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Valvular Heart Disease TABLE 21. Valvular and Other Cardiac Lesions and Their Associated Examination Findings Cardiac Characteristic Location Radiation Associated Findings Severity and Pitfalls Condition Murmur Aortic stenosis Midsystolic; RUSB Right clavicle, Enlarged, nondisplaced Severe aortic stenosis crescendo- carotid; apex apical impulse; Sa; bicuspid findings may include decrescendo valve without calcification decreased 42; high-pitched, will have systolic ejection late-peaking murmur; click followed by murmur diminished and delayed carotid upstroke; radiation o{ murmur to both clavicles and carotids Radiation of murmur down the descending thoracic aorta may mimic mitral regurgitation , Aortic Diastolic; LLSB (valvular) None Enlarged, displaced apical Acute severe regurgitation regurgitation decrescendo or RLSB impulse; 53 or Sa; increased murmur may be masked by (dilated aorta) pulse pressure; bounding tachycardia and short (best heard carotid and peripheral duration of murmur sitting and pu lses Severity in chronic leaning regurgitation is difficult to forward) assess by auscultation Mitral stenosis Diastolic; low- Apex (best None Loud 51; tapping apex beat; lnterval between 52 and pitched, heard in left opening snap after 52 if opening snap is short in decrescendo lateral leaflets mobile; irregular severe m;tral stenosis decubitus pulse i{ atrial fibrillation position) present lntensity of murmur correlates with transvalvular gradient P2 may be loud if pulmonary hypertension present Mitral Systolic; holo-, Apex Axilla or back; Systolic click in mitral valve Acute severe regurgitation regurgitation mid-, or late occasionally prolapse; 53; apical impulse may have soft or no systolic anteriorly to hyperdynamic and may be holosystolic murmur, mitral precordium displaced if dilated left inflow rumble, or 53 ventricle; in mitral valve prolapse, Valsalva maneuver moves onset of clicks and murmur closer to S,; handgrip maneuver increases murmur intensity Tricuspid Holosystolic LLSB LUSB Merged and prominent c Right ventricular impulse regurgitation and v waves in jugular below sternum venous pulse; murmur Pulsatile, enlarged liver with increases during inspiration possible ascites Murmur may be high- pitched if associated with severe pulmonary hypertension Tricuspid Diastolic; low- LLSB None Elevated central venous Low-pitched frequency may stenosis pitched, pressure with prominent a be difficult to auscultate, decrescendo; wave, signs of venous especially at higher heart increased congestion (hepatomegaly, rate intensity during ascites, edema) inspiration Pulmonary valve Systolic; LUSB Left clavicle Pulmonic ejection click after lncreased intensity of stenosrs crescendo- S1 (diminishes with murmur with late peaking decrescendo inspiration) Pulmonary valve Diastolic; LLSB None Loud P2 if pulmonary Murmur may be minimalor regurgitation decrescendo hypertension present absent if severe due to minimaldifference in pulmonary artery and right ventricular diastolic pressures (Continued on the next page)

TABLE 21. Valvular and Other Cardiac Lesions and Their Associated Examination Findings Cardiac Characteristic Location Radiation Associated Findings Severity and Pitfalls Condition Murmur Aortic stenosis Midsystolic; RUSB Right clavicle, Enlarged, nondisplaced Severe aortic stenosis crescendo- carotid; apex apical impulse; Sa; bicuspid findings may include decrescendo valve without calcification decreased 42; high-pitched, will have systolic ejection late-peaking murmur; click followed by murmur diminished and delayed carotid upstroke; radiation o{ murmur to both clavicles and carotids Radiation of murmur down the descending thoracic aorta may mimic mitral regurgitation , Aortic Diastolic; LLSB (valvular) None Enlarged, displaced apical Acute severe regurgitation regurgitation decrescendo or RLSB impulse; 53 or Sa; increased murmur may be masked by (dilated aorta) pulse pressure; bounding tachycardia and short (best heard carotid and peripheral duration of murmur sitting and pu lses Severity in chronic leaning regurgitation is difficult to forward) assess by auscultation Mitral stenosis Diastolic; low- Apex (best None Loud 51; tapping apex beat; lnterval between 52 and pitched, heard in left opening snap after 52 if opening snap is short in decrescendo lateral leaflets mobile; irregular severe m;tral stenosis decubitus pulse i{ atrial fibrillation position) present lntensity of murmur correlates with transvalvular gradient P2 may be loud if pulmonary hypertension present Mitral Systolic; holo-, Apex Axilla or back; Systolic click in mitral valve Acute severe regurgitation regurgitation mid-, or late occasionally prolapse; 53; apical impulse may have soft or no systolic anteriorly to hyperdynamic and may be holosystolic murmur, mitral precordium displaced if dilated left inflow rumble, or 53 ventricle; in mitral valve prolapse, Valsalva maneuver moves onset of clicks and murmur closer to S,; handgrip maneuver increases murmur intensity Tricuspid Holosystolic LLSB LUSB Merged and prominent c Right ventricular impulse regurgitation and v waves in jugular below sternum venous pulse; murmur Pulsatile, enlarged liver with increases during inspiration possible ascites Murmur may be high- pitched if associated with severe pulmonary hypertension Tricuspid Diastolic; low- LLSB None Elevated central venous Low-pitched frequency may stenosis pitched, pressure with prominent a be difficult to auscultate, decrescendo; wave, signs of venous especially at higher heart increased congestion (hepatomegaly, rate intensity during ascites, edema) inspiration Pulmonary valve Systolic; LUSB Left clavicle Pulmonic ejection click after lncreased intensity of stenosrs crescendo- S1 (diminishes with murmur with late peaking decrescendo inspiration) Pulmonary valve Diastolic; LLSB None Loud P2 if pulmonary Murmur may be minimalor regurgitation decrescendo hypertension present absent if severe due to minimaldifference in pulmonary artery and right ventricular diastolic pressures (Continued on the next page) 62 !

I t I Valvular Heart Disease t i t I TABLE 21. Valvular and Other Cardiac Lesions and TheirAssociated Examination Findings (Continued) i

I t I Valvular Heart Disease t i t I TABLE 21. Valvular and Other Cardiac Lesions and TheirAssociated Examination Findings (Continued) i 1 Cardiac Characteristic Location Radiation Associated Findings Severity and Pitfalls I Condition Murmur t Benign Midsystolic; RUSB None Normal intensity of 42; May be present in conditions t (innocent)flow grade 1/6 or normal splitting of 52; no with increased flow (e.9., i murmur 2/6 in intensity radiation pregnancy, fever, anemia, { hyperthyroidism) Hypertroph ic None Murmur may not be present I obstructive Systolic; crescendo LLSB Enlarged, hyperdynamic apical impulse; bifid carotid impulse in nonobstructive CA rdiomyopathy decrescendo t with delay; increased intensity hypertrophic during Valsalva maneuver or cardiomyopathy L with squatting to standing t Atrial septal Systolic; RUSB None Fixed split 52; right May be associated with ! defect crescendo- ventricular heave; rarely, pulmonary hypertension with decrescendo tricuspid inflow murmur increased intensity of P2, ! pulmonary valve regurgitation Holosystolic LLSB I

1 Cardiac Characteristic Location Radiation Associated Findings Severity and Pitfalls I Condition Murmur t Benign Midsystolic; RUSB None Normal intensity of 42; May be present in conditions t (innocent)flow grade 1/6 or normal splitting of 52; no with increased flow (e.9., i murmur 2/6 in intensity radiation pregnancy, fever, anemia, { hyperthyroidism) Hypertroph ic None Murmur may not be present I obstructive Systolic; crescendo LLSB Enlarged, hyperdynamic apical impulse; bifid carotid impulse in nonobstructive CA rdiomyopathy decrescendo t with delay; increased intensity hypertrophic during Valsalva maneuver or cardiomyopathy L with squatting to standing t Atrial septal Systolic; RUSB None Fixed split 52; right May be associated with ! defect crescendo- ventricular heave; rarely, pulmonary hypertension with decrescendo tricuspid inflow murmur increased intensity of P2, ! pulmonary valve regurgitation Holosystolic LLSB I Ventricular None Palpable thrill; murmur Murmur intensity and t septal defect increases with handgrip duration decrease as maneuver pulmonary hypertension t develops (Eisenmenger I syndrome)

Ventricular None Palpable thrill; murmur Murmur intensity and t septal defect increases with handgrip duration decrease as maneuver pulmonary hypertension t develops (Eisenmenger I syndrome) I Cyanosis if Eisenmenger t syndrome develops ! A? = aortic component of52;LLSB=leftlowersternalborder;LUSB=leftuppersternalborder;P,=pulmoniccomponentofSz;RLSB=rightlowersternalborder;RUSB. right upper sternal border. i L

I Cyanosis if Eisenmenger t syndrome develops ! A? = aortic component of52;LLSB=leftlowersternalborder;LUSB=leftuppersternalborder;P,=pulmoniccomponentofSz;RLSB=rightlowersternalborder;RUSB. right upper sternal border. i L I fEY POltIl (ooilnued) I r Medical therapy, although often effective for symptom i A At risk Patients with risk factors for palliation, has not been shown to prevent disease pro t development of VHD gression or improve long term survival in patients with I B Progressive Patients with progressive VHD valvular heart disease. (mild to moderate severity and o For all patients with valvular heart disease in whom asymptomatic) surgical or interventional therapy is being considered, a C Asymptomatic Asymptomatic patients who have severe the criteria for severe VHD: multidisciplinary approach with a heart team consist- ing of a cardiologist, a surgeon, and an interventional C1 : Asymptomatic patients \ with severe VHD in whom the cardiologist is recommended. left or right ventricle remains ! compensated : C2: Asymptomatic patients with severe VHD, with Aoftic Stenosis t decompensation of the left or Clinical Presentation and Evaluation right ventricle Aortic stenosis may be congenital, as in persons with a bicus I D Symptomatic Patients who have developed pid aortic valve, or acquired. The most common cause is severe symptoms as a result of VHD I degeneration of'the valve that occurs with aging (Figure 3O); VHD = valvular heart disease. severe lesions occur in approximiltely 3')(, of persons aged L Reproduced with permission from Oto CM, Nishimura RA, Bonow RO, et al; Writing 65 years or older. Other acquired cluses include rheunlatic Committee Memb,ers. 2020 ACC/AHA guideline for the management oI patients with valvular heart disease: a report of the American College of Cardiology/ disease and chest irradiation. Although rheumatic discase of' American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 202 1 ;77:e25 e1 97. IPMID: 33342586] doi:1 0.1 01 6ij.jacc.2020.1 1 .0 1 8. the mitral valve frequently occurs in isolation, rheumatic aor 02021 American College of Cardiology Foundation and the American Heart tic valve disease almost never occurs without mitral valve Association, lnc. a involvement. Chest irradiation (e.g., mantle therapy Ibr non Hodgkin lymphonra) commonly results in a combination of f,EY POIXIS stenosis and regurgitation. o Many heart valve lesions progress slowly, causing patients Aortic stenosis causes chronic pressure overload of the I to limit their activity unconsciously in response; therefore, left ventricle (lV), leading to concentric LV hypertrophy and a careful history and detailed physical examination are myocardial interstitial fibrosis. Diastolic dysfunction fbtlows, essential' (continued) with eventual systolic heart failure ancl pulmonary congestion. 'fhe disease typically progresses with a decrease in the aortic

I fEY POltIl (ooilnued) I r Medical therapy, although often effective for symptom i A At risk Patients with risk factors for palliation, has not been shown to prevent disease pro t development of VHD gression or improve long term survival in patients with I B Progressive Patients with progressive VHD valvular heart disease. (mild to moderate severity and o For all patients with valvular heart disease in whom asymptomatic) surgical or interventional therapy is being considered, a C Asymptomatic Asymptomatic patients who have severe the criteria for severe VHD: multidisciplinary approach with a heart team consist- ing of a cardiologist, a surgeon, and an interventional C1 : Asymptomatic patients \ with severe VHD in whom the cardiologist is recommended. left or right ventricle remains ! compensated : C2: Asymptomatic patients with severe VHD, with Aoftic Stenosis t decompensation of the left or Clinical Presentation and Evaluation right ventricle Aortic stenosis may be congenital, as in persons with a bicus I D Symptomatic Patients who have developed pid aortic valve, or acquired. The most common cause is severe symptoms as a result of VHD I degeneration of'the valve that occurs with aging (Figure 3O); VHD = valvular heart disease. severe lesions occur in approximiltely 3')(, of persons aged L Reproduced with permission from Oto CM, Nishimura RA, Bonow RO, et al; Writing 65 years or older. Other acquired cluses include rheunlatic Committee Memb,ers. 2020 ACC/AHA guideline for the management oI patients with valvular heart disease: a report of the American College of Cardiology/ disease and chest irradiation. Although rheumatic discase of' American Heart Association Joint Committee on Clinical Practice Guidelines. J Am Coll Cardiol. 202 1 ;77:e25 e1 97. IPMID: 33342586] doi:1 0.1 01 6ij.jacc.2020.1 1 .0 1 8. the mitral valve frequently occurs in isolation, rheumatic aor 02021 American College of Cardiology Foundation and the American Heart tic valve disease almost never occurs without mitral valve Association, lnc. a involvement. Chest irradiation (e.g., mantle therapy Ibr non Hodgkin lymphonra) commonly results in a combination of f,EY POIXIS stenosis and regurgitation. o Many heart valve lesions progress slowly, causing patients Aortic stenosis causes chronic pressure overload of the I to limit their activity unconsciously in response; therefore, left ventricle (lV), leading to concentric LV hypertrophy and a careful history and detailed physical examination are myocardial interstitial fibrosis. Diastolic dysfunction fbtlows, essential' (continued) with eventual systolic heart failure ancl pulmonary congestion. 'fhe disease typically progresses with a decrease in the aortic 63

Valvular Heart Disease TABLE 23. Serial Evaluation of Asymptomatic Patients with Left-Sided Valvular Conditions Factors Considered Lesion Severity Frequency of Evaluation Aortic Stenosis Stenosis severity; rate of progression; LV At risk (V.", <2 m/s) systolic function; ascending aorta dilation Clinical evaluation yearly; echo every 3-5 y Mild (V.", 2.0-2.9 mls or mean gradient if associated with bicuspid aortic valve <20 mm Hg) Moderate (V-.,3.0-3.9 m/s or mean Clinical evaluation yearly; echo every 1-2 y gradient 20-39 mm Hg) Severe (V-u, >4 m/s or mean gradient Clinical evaluation yearly; echo every >40 mm Hg, AVA typically <1 .0 cm2) 6-12 mo Very severe (V-"* )5 m/s or mean gradient Clinical evaluation yearly; echo every >60 mm Hg) 6-12 mo Aortic Regurgitation Regurgitation severity; rate of progression; Mild (VC <0.3 cm, ERO <0.10 cm2, RV Clinical evaluation yearly; echo every 3-5 y LV ejection fraction; LV chamber size; <30 mUbeat, RF <30%); normal EF ascending aorta dilation if bicuspid aortic Moderate (VC 0.3-0.6 cm, ERO 0.1 0-0.29 cm2, Clinical evaluation yearly; echo every 1-2 y valve RV 30-59 mUbeat, RF 30%-49"/") Severe (VC >0.6 cm, ERO >0.3 cm2, RV >60 mUbeat, RF >50%)

Aortic Regurgitation Regurgitation severity; rate of progression; Mild (VC <0.3 cm, ERO <0.10 cm2, RV Clinical evaluation yearly; echo every 3-5 y LV ejection fraction; LV chamber size; <30 mUbeat, RF <30%); normal EF ascending aorta dilation if bicuspid aortic Moderate (VC 0.3-0.6 cm, ERO 0.1 0-0.29 cm2, Clinical evaluation yearly; echo every 1-2 y valve RV 30-59 mUbeat, RF 30%-49"/") Severe (VC >0.6 cm, ERO >0.3 cm2, RV >60 mUbeat, RF >50%) EF >55%; LVESD <50 mm Clinical evaluation every 6-12 mo; echo every 6-12 mo, more frequently for dilating LV EF <55%; LVESD >50 mm Clinical evaluation every 6-12 mo; echo every 6-12 mo, more frequently for dilating LV

EF <55%; LVESD >50 mm Clinical evaluation every 6-12 mo; echo every 6-12 mo, more frequently for dilating LV Mitral Stenosis Stenosis severity Mild and moderate (MVA>1.5 cm2, Clinical evaluation yearly; echo every 3-5 y diastolic pressure half-time <150 ms) Severe (MVA l1 .5 cm2, diastolic pressure Clinical evaluation yearly; echo every 1-2 y half-time>1 50 msor>220 mswithvery for MVA 1 .0-1 .5 cm2, every year for MVA severe stenosis, PASP >50 mm Hg) <1 .0 cm2 t I Mitral Regurgitation Regurgitation severity; rate of progression; At risk (VC <0.3 cm) Clinicalevaluation yearly; echo only if EF; LV chamber size symptomatic Mild and moderate (VC <0.7 cm, ERO Clinicalevaluation yearly; echo every 3-5 y <0.40 cm2, RV <60 mUbeat, RF <507o) for mild severity, every 1-2y for moderate severity Severe (VC >0.7 cm, ERO >0.4 cm2, Clinical evaluation every 6-12 mo; echo RV>60 mUbeat, RF >50%) every 6-12 mo, more frequently for dilating LV

Mitral Stenosis Stenosis severity Mild and moderate (MVA>1.5 cm2, Clinical evaluation yearly; echo every 3-5 y diastolic pressure half-time <150 ms) Severe (MVA l1 .5 cm2, diastolic pressure Clinical evaluation yearly; echo every 1-2 y half-time>1 50 msor>220 mswithvery for MVA 1 .0-1 .5 cm2, every year for MVA severe stenosis, PASP >50 mm Hg) <1 .0 cm2 t I Mitral Regurgitation Regurgitation severity; rate of progression; At risk (VC <0.3 cm) Clinicalevaluation yearly; echo only if EF; LV chamber size symptomatic Mild and moderate (VC <0.7 cm, ERO Clinicalevaluation yearly; echo every 3-5 y <0.40 cm2, RV <60 mUbeat, RF <507o) for mild severity, every 1-2y for moderate severity Severe (VC >0.7 cm, ERO >0.4 cm2, Clinical evaluation every 6-12 mo; echo RV>60 mUbeat, RF >50%) every 6-12 mo, more frequently for dilating LV Recommendations based on Otto CM, Nishimura RA, Bonow RO, et al; Writing Committee Members.2020 ACC/AHA guideline for the management of patients wrth valvular hean disease: a report of the American College o{ Cardiology/American Heart Association Joint Committee on Cl inical Practice Guidelines. J Am Coll Cardtol.2O21:77:e25 e197 . I PMI D: 33342586] d oi:1 O.1 01 64.jacc.2A20. 1 1 .0 1 8

Recommendations based on Otto CM, Nishimura RA, Bonow RO, et al; Writing Committee Members.2020 ACC/AHA guideline for the management of patients wrth valvular hean disease: a report of the American College o{ Cardiology/American Heart Association Joint Committee on Cl inical Practice Guidelines. J Am Coll Cardtol.2O21:77:e25 e197 . I PMI D: 33342586] d oi:1 O.1 01 64.jacc.2A20. 1 1 .0 1 8 valve area of approximately 0.12 cm2 per year, but the rate The characteristic clinical findings of severe aortic steno depends on patient age, underlying severity of the stenosis, sis include a late peaking systolic murmur, a diminished or and comorbid conditions, such as kidney failure and hyper absent aortic component of the 52. and a delay in the carotid tension. Exertional dyspnea, syncope, and angina are the most upstroke (pulsus tardus) that may be accompanied by a common symptoms; however, symptoms may not appear until decreased pulse amplitude due to low cardiac output (pulsus stenosis is severe. Among asymptomatic patients with severe parvus). Clinical findings suggestive of severe aortic stenosis aortic stenosis, 75'7, will die or develop symptoms within should be promptly evaluated (see Table 21). 5 years. Once symptoms occur, Iife expectancy is generally The primary imaging modality for evaluation of aorlic only 1 to 2 years. Thus, serial evaluation every 6 to 12 months stenosis is TTE (Figure 31). Echocardiography can determine is recommended for patients with severe disease (see Table 23). the cause of stenosis, stenosis severity (with gradient and valve 64

Valvular Heart Disease 'i' ! N tIGURE 30.Aorticstenosis.Grossspecimensshowingpathologyof degenerativeao(irstenosis(topleltpanel),bicuspidaorti(stenosis(toprightpanel),andrheumatic overloadfromsevereaorticstenosislarrowheadinbottomrightpanel).Ao=ascendingaorta; lX=leftatrium; N=nonroronarycusp. lmages c0ua(esy of Dr William Edwards, l\layo Cl nic area assessment), LV function and wall thickness, right ven- Severe aortic stenosis is typically defined by a small valve tricular (RV) function, pulmonary artery pressure, and the area (<1.0 cm2), high peak velocity (>4 m/s), and/or high mean presence or absence of other valve pathologr. In some patients, gradient (>40 mm Hg). There are tvvo patient subsets in which echocardiography may underestimate the severity ol aortic severe aortic stenosis may be present with a snrall valve area stenosis. Further evaluation with cardiac catheterization. dur and either low velocity or low gradient (1) patients with ing which cardiac output and the aortic pressure gradient can severe LV dysfunction and low cardiac output (low flow low be measured, is required when there are discrepancies between gradient aortic stenosis) and (2) patients with preserved LV the clinical and echocardiographic findings in symptomatic lunction and paradoxical low floW low gradient aortic steno patients being considered for intervention. Exercise stress test sis. In the former group, dobutamine echocardiography or ing is useful in asymptomatic patients with severe aortic steno dobutamine cardiac catheterization is needed to distinguish sis to confirm asymptomatic status, but it should be performed true aortic stenosis fiom pseudostenosis. In pseudostenosis, under cardiologist supervision. Exercise stress testing is con dobutamine increases cardiac output and the opening lorces traindicated in symptomatic severe aortic stenosis. on the aortic valve, causing the valve area to increase out of the 65

Valvular Heart Disease small LV size and high aortic impedance to flow (e.g., hyper tension) or other causes of low cardiac output (e.g., atrial fibrillation, pulmonary hl,pertension). Determination of lesion severity in paradoxical aortic stenosis requires consideration of the hemodynamics, valve morpholory (e.g., degree of calcifica tion), presence of LV hypertrophy, and clinical presentation of the patient. In patients with suspected low flow low gradient severe aortic stenosis with normal or reduced left ventricular ejection fraction, measurement of aortic valve calcium score by CT is reasonable to further define severity. In patients with either low-flow low gradient severe aortic stenosis with reduced LV function or paradoxical low-floW low-gradient severe aortic stenosis, observational studies have shown improved survival with aortic valve replacement compared with medical therapy.

small LV size and high aortic impedance to flow (e.g., hyper tension) or other causes of low cardiac output (e.g., atrial fibrillation, pulmonary hl,pertension). Determination of lesion severity in paradoxical aortic stenosis requires consideration of the hemodynamics, valve morpholory (e.g., degree of calcifica tion), presence of LV hypertrophy, and clinical presentation of the patient. In patients with suspected low flow low gradient severe aortic stenosis with normal or reduced left ventricular ejection fraction, measurement of aortic valve calcium score by CT is reasonable to further define severity. In patients with either low-flow low gradient severe aortic stenosis with reduced LV function or paradoxical low-floW low-gradient severe aortic stenosis, observational studies have shown improved survival with aortic valve replacement compared with medical therapy. Management Aortic valve replacement is a life-prolonging procedure in patients with severe aortic stenosis. The indications for aortic valve replacement in severe aortic stenosis are the presence of symptoms (e.g., dyspnea, angina, presyncope, syncope, or heart failure), LV systolic dysfunction (ejection fraction <50'/.) in an asymptomatic patient, or a concomitant cardiac surgical procedure for other indications (e.g., coronary artery bypass grafting or ascending aorta surgery). Aortic valve replacement is reasonable in asymptomatic patients with very severe aortic stenosis and low surgical risk and asymptomatic patients with abnormal results on supervised exercise testing, such as poor exercise tolerance, abnormal ECG changes, or hypotension during testing. Aortic valve replacement can be performed with open car- diac surgery (surgical aortic valve replacement [SAVR]) or via transcatheter approach (transcatheter aortic valve implantation [TAVI]) (Figure 32). SAVR and TAVI have similar procedural and long term survival rates, with expected operative mortality rates of1% to 3%. The choice between surgical and transcatheter interventions is based on the presence of symptoms and the patient's surgical risk, as determined through comprehensive multidisciplinary assessment. TAVI is recommended orer SAVR for symptomatic patients with severe aortic stenosis who are older than B0 years or for younger patients with a life expec- tancy less than 10 years. TAVI is also recommended over SAVR for symptomatic patients of any age with severe aortic stenosis and a high or prohibitive surgical risk if predicted postproce t lG U R E 3 1 . Echocardiographic findings in aortic sten0sis. Calcific aortic dure survival is more than 12 months with an acceptable quality stenosis (arowhead) is present in parasternal long-axis (top panel) and short,axis of life. For symptomatic patients who are aged 65 to 80 years. (middle panellviews. Leftventricular(LV) hypertrophy is also present. Doppler either SAVR or TAVI is appropriate following shared decision echocardiogram shows a mean aortic gradient of 56 mm Hg, consistent with severe aortic stenosis (bottom panel\. Ao = ascending aorta; l-A = left atrium. making. Neither SAVR nor TAVI is indicated in patients with limited expectation of survival due to comorbid conditions. severe range. With low-flow, low gradient aortic stenosis, the Although the pathophysiologr of aortic stenosis is known calculated valve area remains in the severe range with dobu to be inflammatory randomized trials have shown medical tamine administration, and the aortic valve gradient and therapy, specifically statins, to be ineffective in slowing disease velocity increase with increased stroke volume. Patients with progression. For patients with coexistent hypertension or heart paradoxical low flow low-gradient aortic stenosis have low failure, guideline directed medical therapy is recommended. stroke volume (<35 ml/m,) resulting from a combination of Vasodilators should be used with caution in patients with

Management Aortic valve replacement is a life-prolonging procedure in patients with severe aortic stenosis. The indications for aortic valve replacement in severe aortic stenosis are the presence of symptoms (e.g., dyspnea, angina, presyncope, syncope, or heart failure), LV systolic dysfunction (ejection fraction <50'/.) in an asymptomatic patient, or a concomitant cardiac surgical procedure for other indications (e.g., coronary artery bypass grafting or ascending aorta surgery). Aortic valve replacement is reasonable in asymptomatic patients with very severe aortic stenosis and low surgical risk and asymptomatic patients with abnormal results on supervised exercise testing, such as poor exercise tolerance, abnormal ECG changes, or hypotension during testing. Aortic valve replacement can be performed with open car- diac surgery (surgical aortic valve replacement [SAVR]) or via transcatheter approach (transcatheter aortic valve implantation [TAVI]) (Figure 32). SAVR and TAVI have similar procedural and long term survival rates, with expected operative mortality rates of1% to 3%. The choice between surgical and transcatheter interventions is based on the presence of symptoms and the patient's surgical risk, as determined through comprehensive multidisciplinary assessment. TAVI is recommended orer SAVR for symptomatic patients with severe aortic stenosis who are older than B0 years or for younger patients with a life expec- tancy less than 10 years. TAVI is also recommended over SAVR for symptomatic patients of any age with severe aortic stenosis and a high or prohibitive surgical risk if predicted postproce t lG U R E 3 1 . Echocardiographic findings in aortic sten0sis. Calcific aortic dure survival is more than 12 months with an acceptable quality stenosis (arowhead) is present in parasternal long-axis (top panel) and short,axis of life. For symptomatic patients who are aged 65 to 80 years. (middle panellviews. Leftventricular(LV) hypertrophy is also present. Doppler either SAVR or TAVI is appropriate following shared decision echocardiogram shows a mean aortic gradient of 56 mm Hg, consistent with severe aortic stenosis (bottom panel\. Ao = ascending aorta; l-A = left atrium. making. Neither SAVR nor TAVI is indicated in patients with limited expectation of survival due to comorbid conditions. severe range. With low-flow, low gradient aortic stenosis, the Although the pathophysiologr of aortic stenosis is known calculated valve area remains in the severe range with dobu to be inflammatory randomized trials have shown medical tamine administration, and the aortic valve gradient and therapy, specifically statins, to be ineffective in slowing disease velocity increase with increased stroke volume. Patients with progression. For patients with coexistent hypertension or heart paradoxical low flow low-gradient aortic stenosis have low failure, guideline directed medical therapy is recommended. stroke volume (<35 ml/m,) resulting from a combination of Vasodilators should be used with caution in patients with 66

Valvular Heaft Disease Aortic Regurgitation Clinical Presentation and Evaluation Aortic regurgitation, manifesting acutely or chronically, arises lrom aortic root pathologr or intrinsic valve disease. Causes of chronic aortic regurgitation include ascending aortic dilata tion and valve abnormalities due to bicuspid disease, calcific degeneration, rheumatic involvement, or chest irradiation. Acute aortic regurgitation may be caused by endocarditis, blunt chest trauma, iatrogenic damage (e.g., complications of balloon aortic valvuloplasty), or aortic dissection. In chronic aortic regurgitation, volume overload causes progressive LV dilatation and eccentric hypertrophy. Chronic aortic regurgitation may be tolerated for many years but may eventually lead to symptoms, including shortness of breath, fatigue, or angina. Clinical findings result from the large stroke volume and LV dilatation and include bounding peripheral pulses, displacement of the LV apex, and a diastolic decrescendo murmur heard either along the right sternal border (suggesting root pathologr) or left sternal border (sug gesting valve patholory) (see Table 21). The large forward F I G UR E 3 2. Transcatheter aortic valve implanlation.lop left panel: Balloon aortic valvuloplasty (anowhead) is fi rst perform ed.Top right panel: Using a transfemoral stroke volume also can cause an early peaking systolic ejec- approach (anows), a transcatheter aortic value(arrowhead)is positioned at the aortic tion murmur. annu lus usi ng aortog raphy. Bottom left panel:Ihe prosthesis (arowhead) is then In acute regurgitation, the abrupt onset of volume over- slowly inflated during rapid pacing from a temporary pacemaker (IP), which creates load may cause acute heart failure or even cardiogenic shock. ventricula r standstill. Bottom right panel: Ihe prosthesis is ful ly deployed. Bounding pulses may not be present because stroke volume has not markedly increased, and murmurs may be softer or aortic stenosis and heart failure symptoms. ln select cases, bal- shorter in duration because of the rapid equalization of pres- loon valvuloplasty may be used to bridge unstable patients to sures between the aorta and LV therapywith TAVI or SAVR. TTE is indicated for evaluation ofaortic regurgitation and LV function. In patients with moderate or severe aortic regur gitation and suboptimal TTE images or a discrepancy between o The characteristic clinical findings of severe aortic ste clinical and TTE fi ndings, transesophageal echocardiography nosis include a late-peaking systolic murmur, a dimin- (TEE), cardiac magnetic resonance (CMR) imaging, or cardiac ished or absent aortic component of the 52, and a weak catheterization is indicated. When endocarditis is suspected, and delayed carotid upstroke. TTE is the initial imaging test in most patients. TEE following o Echocardiography is accurate for defining the severity TTE is recommended for patients with suboptimal TTE results of aortic stenosis in most patients; when there is a dis- or with high initial risk. CT angiography is indicated in patients crepancy between the clinical and echocardiographic with acute AR and aortic dissection because it is highly accu- findings, cardiac catheterization should be considered rate and usually rapidly available. in patients who are candidates for intervention. Criteria for severe aortic regurgitation include a jet width . Patients with low-flow low gradient aortic stenosis have a that occupies 657, or more of the LV outflow tract, vena con- small valve area but low velocity and/or low gradient due tracta (the width of the regurgitant jet at its most narrow por to low stroke volume; dobutamine echocardiography or tion) greater than 0.6 cm, holodiastolic flow in the descending cardiac catheterization can be used to distinguish pseudo- aorta, regurgitation volume of 60 mL or more, and effective severe aortic stenosis from true severe aortic stenosis. regurgitant orifice area of 0.3 cm2 or greater. The LV gpically is dilated in chronic aortic regurgitation. In patients suspected o Aortic valve replacement prolongs life in patients with of having an aortic root abnormality, evaluation with CMR symptomatic severe aortic stenosis; the patient's surgi- imaging, CT, or TEE is recommended. Surveillance is based on cal risk determines whether valve replacement is per- severity ofregurgitation and other factors (see Table 23). formed using open surgery or a transcatheter approach. . Transcatheter aortic valve implantation is indicated for Management symptomatic patients with trileaflet aortic stenosis at In chronic aortic regurgitation, surgery with traditional open any level of surgical risk who do not have concomitant aortic valve replacement is advised for patients with symptoms severe aortic regurgitation. (typically, dyspnea or angina) or LV dysfunction (ejection

Aortic Regurgitation Clinical Presentation and Evaluation Aortic regurgitation, manifesting acutely or chronically, arises lrom aortic root pathologr or intrinsic valve disease. Causes of chronic aortic regurgitation include ascending aortic dilata tion and valve abnormalities due to bicuspid disease, calcific degeneration, rheumatic involvement, or chest irradiation. Acute aortic regurgitation may be caused by endocarditis, blunt chest trauma, iatrogenic damage (e.g., complications of balloon aortic valvuloplasty), or aortic dissection. In chronic aortic regurgitation, volume overload causes progressive LV dilatation and eccentric hypertrophy. Chronic aortic regurgitation may be tolerated for many years but may eventually lead to symptoms, including shortness of breath, fatigue, or angina. Clinical findings result from the large stroke volume and LV dilatation and include bounding peripheral pulses, displacement of the LV apex, and a diastolic decrescendo murmur heard either along the right sternal border (suggesting root pathologr) or left sternal border (sug gesting valve patholory) (see Table 21). The large forward F I G UR E 3 2. Transcatheter aortic valve implanlation.lop left panel: Balloon aortic valvuloplasty (anowhead) is fi rst perform ed.Top right panel: Using a transfemoral stroke volume also can cause an early peaking systolic ejec- approach (anows), a transcatheter aortic value(arrowhead)is positioned at the aortic tion murmur. annu lus usi ng aortog raphy. Bottom left panel:Ihe prosthesis (arowhead) is then In acute regurgitation, the abrupt onset of volume over- slowly inflated during rapid pacing from a temporary pacemaker (IP), which creates load may cause acute heart failure or even cardiogenic shock. ventricula r standstill. Bottom right panel: Ihe prosthesis is ful ly deployed. Bounding pulses may not be present because stroke volume has not markedly increased, and murmurs may be softer or aortic stenosis and heart failure symptoms. ln select cases, bal- shorter in duration because of the rapid equalization of pres- loon valvuloplasty may be used to bridge unstable patients to sures between the aorta and LV therapywith TAVI or SAVR. TTE is indicated for evaluation ofaortic regurgitation and LV function. In patients with moderate or severe aortic regur gitation and suboptimal TTE images or a discrepancy between o The characteristic clinical findings of severe aortic ste clinical and TTE fi ndings, transesophageal echocardiography nosis include a late-peaking systolic murmur, a dimin- (TEE), cardiac magnetic resonance (CMR) imaging, or cardiac ished or absent aortic component of the 52, and a weak catheterization is indicated. When endocarditis is suspected, and delayed carotid upstroke. TTE is the initial imaging test in most patients. TEE following o Echocardiography is accurate for defining the severity TTE is recommended for patients with suboptimal TTE results of aortic stenosis in most patients; when there is a dis- or with high initial risk. CT angiography is indicated in patients crepancy between the clinical and echocardiographic with acute AR and aortic dissection because it is highly accu- findings, cardiac catheterization should be considered rate and usually rapidly available. in patients who are candidates for intervention. Criteria for severe aortic regurgitation include a jet width . Patients with low-flow low gradient aortic stenosis have a that occupies 657, or more of the LV outflow tract, vena con- small valve area but low velocity and/or low gradient due tracta (the width of the regurgitant jet at its most narrow por to low stroke volume; dobutamine echocardiography or tion) greater than 0.6 cm, holodiastolic flow in the descending cardiac catheterization can be used to distinguish pseudo- aorta, regurgitation volume of 60 mL or more, and effective severe aortic stenosis from true severe aortic stenosis. regurgitant orifice area of 0.3 cm2 or greater. The LV gpically is dilated in chronic aortic regurgitation. In patients suspected o Aortic valve replacement prolongs life in patients with of having an aortic root abnormality, evaluation with CMR symptomatic severe aortic stenosis; the patient's surgi- imaging, CT, or TEE is recommended. Surveillance is based on cal risk determines whether valve replacement is per- severity ofregurgitation and other factors (see Table 23). formed using open surgery or a transcatheter approach. . Transcatheter aortic valve implantation is indicated for Management symptomatic patients with trileaflet aortic stenosis at In chronic aortic regurgitation, surgery with traditional open any level of surgical risk who do not have concomitant aortic valve replacement is advised for patients with symptoms severe aortic regurgitation. (typically, dyspnea or angina) or LV dysfunction (ejection 67

Valvular Heart Disease fraction <55'/.) thought to be due to aortic regurgitation or for of life. More than one third of those older than 70 years with patients with severe aortic regurgitation who are undergoing severe aortic stenosis have an underlying bicuspid valve. ! other cardiac surgery. Surgical treatment of aortic regurgita- Bicuspid valvulopathy is often accompanied by aortic tion is reasonable in asymptomatic patients with severe AR, abnormalities, independent of the severity of aortic stenosis or normal left ventricular function, and significant LV dilatation regurgitation, and may be associated with aneurysm, dissec (end systolic dimension >50 mm or indexed end-systolic tion, or coarctation. Therefore, in patients '*,ith a bicuspid dimension >25 mm/m2). In patients with isolated severe aortic aortic valve, the ascending aorta and aortic arch should be regurgitation who have indications for SAVR and are candi- examined for aortopathy with TTE. CMR angiography or CT dates for surgery TAVI should not be performed. angiography is indicated when echocardiographic assessment Medical therapy, preferably with dihydropyridine calcium is suboptimal. Lifelong serial imaging is indicated if abnor- channel blockers, ACE inhibitors, or angiotensin receptor malities are detected. The imaging modality and frequency blockers (ARBs), is indicated in patients with chronic aortic depend on several factors, including the nature (stenosis. regurgitation and concomitant hlpertension. Therapy with regurgitation, or aneurysm) and severity of the abnormality. ACE inhibitors or ARBs and p blockers is reasonable in severe age of the patient, family history and candidacy for surgery. aortic regurgitation with symptoms or LV dysfunction when Importantly, bicuspid aortic valve is a heritable abnormaliry surgery is not an option. and first degree relatives of patients with a bicuspid aortic Significant acute aortic regurgitation due to aortic dissec- valve and aortopathy may be considered for screening with tion is a surgical emergency, requiring aortic dissection repair echocardiography. and aortic valve replacement or repair. For other acute causes, Management of bicuspid aortic valve disease is deter- the indications for surgery depend on the regurgitation sever mined by the predominant lesion Upe (stenosis or regurgita iry presence of symptoms, and hemodynamic stability of the tion) and its severity. In patients with a bicuspid valve patient. undergoing surgery for severe aortic stenosis or regurgitation, surgical repair ofthe ascending aorta is reasonable'nthen the t(EY POlltTS aortic dimension is 4.5 cm or greater. In the absence of surgical o Characteristic clinical findings of chronic aortic regurgi- indications for a stenotic or regurgitant aortic valve, surgical tation include bounding peripheral pulses, displace- repair of the ascending aorta or aortic sinuses is recommended ment of the left ventricular apex, and a diastolic decre- when the aortic dimension is greater than 5.5 cm and may be scendo murrnur heard along the right or left sternal reasonable when the dimension is greater than 5.0 cm with an border. additional risk factor for dissection (e.g., family history rate of o In chronic aortic regurgitation, surgery with traditional progression >0.5 cm/year). open aortic valve replacement is advised for patients with No medical therapies slow aortic dilatation in patients symptoms or left ventricular dysfunction, or who have with aortopathy and a bicuspid aortic valve. Blood pres severe asymptomatic aortic regurgitation and are under- sure should be controlled in patients with concomitant going other cardiac surgery; in slmptomatic patients hypertension. who are not surgical candidates, medical therapy is TEY POITIIS appropriate. r Medical therapy with dihydropyridine calcium channel r Bicuspid morphologr predisposes to early degeneration of the aortic valve, resulting in stenosis in most patients blockers, ACE inhibitors, or angiotensin receptor block- and pure regurgitation in few patients. ers is recommended for patients with chronic aortic regurgitation and concomitant hlpertension. . Patients with a bicuspid aortic valve typically present . Emergent surgery is indicated for patients with acute with an incidental systolic ejection murrnur in adoles cence or young adulthood and gradually progress to aortic regurgitation due to aortic dissection. severe disease in the fifth or sixth decade of life. . Management of bicuspid aortic valve disease follows the recommendations for the predominant valve lesion tlpe Bicuspid Aortic Valve Disease (aortic stenosis or regurgitation) and its severity. Bicuspid aortic valve disease affects approximately l% to 2'/. of the general population. Bicuspid morphologr leads to abnor- mal shear forces and predisposes to early degeneration ofthe valve, resulting in stenosis in most patients (up to 75%) (see Mitral Stenosis Figure 30) and pure regurgitation in a small minority of Clinical Presentation and Evaluation patients (2%-7O'X,). Patients with a bicuspid aortic valve typi The leading cause of mitral stenosis is rheumatic heart disease, cally present with an asymptomatic finding of a systolic which is more common in women than in men (4:1 ratio). ejection murmur in adolescence or young adulthood and Although relatively rare in the United States, rheumatic heart gradually progress to severe disease in the fifth or sixth decade disease is frequent in populations with limited access to

fraction <55'/.) thought to be due to aortic regurgitation or for of life. More than one third of those older than 70 years with patients with severe aortic regurgitation who are undergoing severe aortic stenosis have an underlying bicuspid valve. ! other cardiac surgery. Surgical treatment of aortic regurgita- Bicuspid valvulopathy is often accompanied by aortic tion is reasonable in asymptomatic patients with severe AR, abnormalities, independent of the severity of aortic stenosis or normal left ventricular function, and significant LV dilatation regurgitation, and may be associated with aneurysm, dissec (end systolic dimension >50 mm or indexed end-systolic tion, or coarctation. Therefore, in patients '*,ith a bicuspid dimension >25 mm/m2). In patients with isolated severe aortic aortic valve, the ascending aorta and aortic arch should be regurgitation who have indications for SAVR and are candi- examined for aortopathy with TTE. CMR angiography or CT dates for surgery TAVI should not be performed. angiography is indicated when echocardiographic assessment Medical therapy, preferably with dihydropyridine calcium is suboptimal. Lifelong serial imaging is indicated if abnor- channel blockers, ACE inhibitors, or angiotensin receptor malities are detected. The imaging modality and frequency blockers (ARBs), is indicated in patients with chronic aortic depend on several factors, including the nature (stenosis. regurgitation and concomitant hlpertension. Therapy with regurgitation, or aneurysm) and severity of the abnormality. ACE inhibitors or ARBs and p blockers is reasonable in severe age of the patient, family history and candidacy for surgery. aortic regurgitation with symptoms or LV dysfunction when Importantly, bicuspid aortic valve is a heritable abnormaliry surgery is not an option. and first degree relatives of patients with a bicuspid aortic Significant acute aortic regurgitation due to aortic dissec- valve and aortopathy may be considered for screening with tion is a surgical emergency, requiring aortic dissection repair echocardiography. and aortic valve replacement or repair. For other acute causes, Management of bicuspid aortic valve disease is deter- the indications for surgery depend on the regurgitation sever mined by the predominant lesion Upe (stenosis or regurgita iry presence of symptoms, and hemodynamic stability of the tion) and its severity. In patients with a bicuspid valve patient. undergoing surgery for severe aortic stenosis or regurgitation, surgical repair ofthe ascending aorta is reasonable'nthen the t(EY POlltTS aortic dimension is 4.5 cm or greater. In the absence of surgical o Characteristic clinical findings of chronic aortic regurgi- indications for a stenotic or regurgitant aortic valve, surgical tation include bounding peripheral pulses, displace- repair of the ascending aorta or aortic sinuses is recommended ment of the left ventricular apex, and a diastolic decre- when the aortic dimension is greater than 5.5 cm and may be scendo murrnur heard along the right or left sternal reasonable when the dimension is greater than 5.0 cm with an border. additional risk factor for dissection (e.g., family history rate of o In chronic aortic regurgitation, surgery with traditional progression >0.5 cm/year). open aortic valve replacement is advised for patients with No medical therapies slow aortic dilatation in patients symptoms or left ventricular dysfunction, or who have with aortopathy and a bicuspid aortic valve. Blood pres severe asymptomatic aortic regurgitation and are under- sure should be controlled in patients with concomitant going other cardiac surgery; in slmptomatic patients hypertension. who are not surgical candidates, medical therapy is TEY POITIIS appropriate. r Medical therapy with dihydropyridine calcium channel r Bicuspid morphologr predisposes to early degeneration of the aortic valve, resulting in stenosis in most patients blockers, ACE inhibitors, or angiotensin receptor block- and pure regurgitation in few patients. ers is recommended for patients with chronic aortic regurgitation and concomitant hlpertension. . Patients with a bicuspid aortic valve typically present . Emergent surgery is indicated for patients with acute with an incidental systolic ejection murrnur in adoles cence or young adulthood and gradually progress to aortic regurgitation due to aortic dissection. severe disease in the fifth or sixth decade of life. . Management of bicuspid aortic valve disease follows the recommendations for the predominant valve lesion tlpe Bicuspid Aortic Valve Disease (aortic stenosis or regurgitation) and its severity. Bicuspid aortic valve disease affects approximately l% to 2'/. of the general population. Bicuspid morphologr leads to abnor- mal shear forces and predisposes to early degeneration ofthe valve, resulting in stenosis in most patients (up to 75%) (see Mitral Stenosis Figure 30) and pure regurgitation in a small minority of Clinical Presentation and Evaluation patients (2%-7O'X,). Patients with a bicuspid aortic valve typi The leading cause of mitral stenosis is rheumatic heart disease, cally present with an asymptomatic finding of a systolic which is more common in women than in men (4:1 ratio). ejection murmur in adolescence or young adulthood and Although relatively rare in the United States, rheumatic heart gradually progress to severe disease in the fifth or sixth decade disease is frequent in populations with limited access to 68

I t Valvular Heart Disease trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be

trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be v

trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be v I

trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be v I FIGURE 33.Echocardiogramsshowinganormal mitralvalve(MV) (toprightpanel)andrheurnatrcmrtral stenosiswith(ommissura lusian(arrowheads,topleftpanel).ln

trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be v I FIGURE 33.Echocardiogramsshowinganormal mitralvalve(MV) (toprightpanel)andrheurnatrcmrtral stenosiswith(ommissura lusian(arrowheads,topleftpanel).ln mitral gradient of 1 3 mm Hg, r0nsistent with severe stenosis. Ao - ascendinq aorta; LA= left atrium; LV= left ventricle.

trerturcnt fbr streptococcll pharyngitis. llheur.natic heirrt clis s),stcnric embolizirtion. atrial fibrillrtion. or. in sevcre cases. eirsc results in fusion ol thc rlitral cor.r.rr.r.rissLlres and. in rlrore hen'rolltvsis. He:irt tirilure is the clusc of death it-t .tpproxi ad'tirnced firn-r-rs. calciflcrrtion olthe Virl'u'c irncl abnorrnllities in nratelt'60'li, of prticnts r,t'ith n-ritrirl stenosis. u ith thromboen-t the subvirlvular appirrirtus (Figure 33). Othe.r causes ol r.nitral bolisnr causing nlost others. stenosis arc parachutc rlitral valve, chest irradiation. irr-rd (llir.rical finclings r,r'hen the vrtlr,c is pliable include r tap severe nritrll annular calcitlcation. Nlitral itr-rr-rular calci licirtion ping Ili impulse. a loucl S,. an increrrsecl lrulmonic conrponent is rttore conrmon ir-r thc clderll'and is ilssociiited u,itl-r inf'lrrnt ot'S,. ii cliastolic opcning snap. irr.rcl l diastolic rumble or lou, mirtoll clisorders. peripheral artery ctiseasc. and chronic kicl pitche d llrurnrur.lt tl.re apex (see'lirblc 2l). Signs o{ pulmonary ney discase. or systemic congestior-r n-ray be present depending rin lesion 'l'he natural history of mitral stenosis is chirracterizcd lty sevcrity ancl the puticnt's volume stirlus. slou' progression over clecades. r,r,ith grrrclual left rtrirl (l.A) I'l'lr is highll'ac(urilte fbr irssessing disease severit],,, pul enllrgenrent and lrresen'ation of L\,'tirnction. Sl,mptonrs llav nronrrl pressures. rrnd RV fur-rction ls u'ell as for iclcntiff ing ari se lionr lor'r' carcl iac or-r tput fatiguc). pu l ntonan, con gest ior-r ( cor.rconritilr-rt v:rh,'ular lcsions (scc l'irblc 2tl). Additionirl imag (dyspnea). and pulnronary hypertension r,r,ith right sicled ir-rg stuclies or cirrclilc catheteriz-ation is rarely recluired for heart trrilure (lower extremity edema). I)regnancy, lvith the diirgnosis. Severe mitrirl stenosis is clellned by a nritral valve resultirrg increased bkrocl volume irncl t'lrclilrc output, r'nirv alscr iirer o1 1.5 cmr or lcss. u'hich usturlll,corresponcls to a ntean precipitltc S)'mptoms. 51'n-rptoms rrrc ty'pical11, exerti0nal n-ritrrrl gradierlt of'nrore than 5 to l0 nrnr Ilg at a nurmll heart becrrnse erercise shortens diirstolic lilling tin-re ancl increirses rilte. In patients 'nvith ii discrep:rncl betu,een the clinical the transval.n,ular 1lolr, and cliastolic ntitral gradient, leirding to lncl ccl.rocardi ogrlp h ic li ndings. exercise ecl.rocardiography u,orsening of LA hypertension. P:rtients irlso can present with or exercise testir.rg cluring cardiac cirtheterization should be v I FIGURE 33.Echocardiogramsshowinganormal mitralvalve(MV) (toprightpanel)andrheurnatrcmrtral stenosiswith(ommissura lusian(arrowheads,topleftpanel).ln mitral gradient of 1 3 mm Hg, r0nsistent with severe stenosis. Ao - ascendinq aorta; LA= left atrium; LV= left ventricle. 69

Valvular Heart Disease pursued to assess the response of the mitral gradient and pul papillary muscles, or LV free walls) and may present acutell'or monary pressures. chronically. Causes of acute mitral regurgitation include infective endocarditis, papillary muscle ischemia or rupture. Management trauma (e. g., injury from PB MC or blunt force). or degenerative Percutaneous balloon mitral commissurotomy (PBMC) is the disease with chordal rupture and f'lail leaflet. Chronic mitral procedure of choice for patients with significant rheumatic regurgitation is classified as primary. involving any portion of mitral stenosis when the valve is pliable and not severely calci the mitral annulus. or secondary. invoh,ing causes other than fied. PBMC is indicated for symptomatic patients with severe the annulus (e.g., ventricular dysflunction). Common causes of rheumatic mitral stenosis and favorable valve morphologr and primary mitral regurgitation are mitral valve prolapse (also is reasonable in asymptomatic patients with severe rheumatic know,n as myxomatous or degenerative mitral valve disease), mitral stenosis and a pulmonary artery systolic pressure radiation therap)'. rheumatic disease. and cleft mitral valve. greater than 50 mm Hg. PBMC should not be performed in Mitral regurgitation results in volume overload with LV patients with LA thrombus or moderate or severe mitral regur dilatation and LA hypertension. which may progress and gitation, both of which are optimally evaluated with TEE. In cause pulmonary hypertension and RV failure. In acute mitral appropriately selected patients, the success rate with PBMC is regurgitation, hearl failure symptoms often occur abruptly 95'/,, and complications occur in fewer than 5'1, of patients. because of insufficient time fbr adaptive chamber dilatation Mitral valve surgery is indicated in patients with severe mitral and, in some patients. can result in cardiogenic shock. The stenosis, New York Heart Association functional class III or IV systolic murrnur in acute mitral regurgitation may be brief symptoms, and a nonpliable valve and in asymptomatic because of the rapid equalization of l.A and LV pressures. and patients with severe mitral stenosis undergoing concomitant echocardiography with color flow imaging can underestimate cardiac surgery for another indication. the severity of the regurgitation. Thus, when acute mitral Nearly 50'2, of patients with mitral stenosis have atrial regurgitation is suspected, comprehensive assessment to iden fibrillation, and without anticoagulation, these patients have a tify the potential causes should be pursued. and additional risk for thromboembolism of 20'7, to 25')(,. Patients with mod imaging with TEE should be considered. erate to severe mitral stenosis and concomitant atrial fibrilla Chronic primary mitral regurgitation is predominantly tion should receive a vitamin K antagonist such as warfarin, caused by mitral valve prolapse. which affects approxi with a goal INR of 2.0 to 3.0. Anticoagulation is also indicated mately 27, of the general population in the United States. in patients with a history of LA thrombus or systemic emboli Echocardiography in patients with chronic primary mitral zation. Notably, clinical trials of direct acting oral anticoagu regurgitation may show a range of abnormalities, including lants in atrial fibrillation excluded patients with moderate to prolapse (Figure 34), gross degeneration ofone or both leaf: severe mitral stenosis; therefore. the efficacy and salety of Iets (Barlow syndrome). or chordal rupture' ,ith flail leaflet. these agents have not been demonstrated in this population. Barlow syndrome is more common in young adult patients. In x Because the mitral gradient is heavily dependent on patients who are relatively older. fibroelastic deficiency pre transvalvular flow medical therapy with negative chrono It dominates and frequently results in chordal rupture. In tropic agents, diuretics, and long-acting nitrates can be effec patients with chronic secondary mitral regurgitation, ven- tive for symptom palliation in patients who are not candidates tricular dysfunction causes mitral regurgitation through pap for interventional or surgical therapy. illary muscle displacement and tethering of the mitral leaflets. X EY PO I TITS which impairs coaptation. The mitral valve apparatus is nor mal in patients with chronic secondary mitral regurgitation . In patients with mitral stenosis, transthoracic echocar (Figure 35). diography is highly accurate for assessing disease sever The physical examination in patients with chronic mitral ity, pulmonary pressures, and right ventricular function regurgitation is notable for a blowing holosystolic murmur at as well as identiSring concomitant valvular lesions. the apex. In patients with mitral valve prolapse. one or more . Percutaneous balloon mitral commissurotomy is indi- systolic clicks may precede the murmur. and variation in cated for patients with symptomatic severe mitral ste severity, preload, and afterload can lead to differences in nosis and favorable valve morphologr. murmur onset (holosystolic, midsystolic. or late systolic). o Anticoagulation is recommended for patients with In patients with LV dilatation, the apical impulse may be mitral stenosis and a history of atrial fibrillation, left displaced laterally, and an S,, may be audible. especially in atrial thrombus, or systemic embolization. patients with secondarl, n.tt,tr, regurgitation due to LV dysfunction. TTE readily and accurately assesses the selerity of pri- Mitral Regurgitation mary mitral regurgitation. Severe primary mitral regurgitation Clinical Presentation and Evaluation is defined by several parameters, the most common of which Mitral regurgitation may arise from dysfunction of any portion are an effective regurgitant orifice area of 0.4 cm2 or larger, of the complex valve apparatus (leaflets, annulus, chordae. regurgitant volume of 60 mL or more, and vena contracta of

pursued to assess the response of the mitral gradient and pul papillary muscles, or LV free walls) and may present acutell'or monary pressures. chronically. Causes of acute mitral regurgitation include infective endocarditis, papillary muscle ischemia or rupture. Management trauma (e. g., injury from PB MC or blunt force). or degenerative Percutaneous balloon mitral commissurotomy (PBMC) is the disease with chordal rupture and f'lail leaflet. Chronic mitral procedure of choice for patients with significant rheumatic regurgitation is classified as primary. involving any portion of mitral stenosis when the valve is pliable and not severely calci the mitral annulus. or secondary. invoh,ing causes other than fied. PBMC is indicated for symptomatic patients with severe the annulus (e.g., ventricular dysflunction). Common causes of rheumatic mitral stenosis and favorable valve morphologr and primary mitral regurgitation are mitral valve prolapse (also is reasonable in asymptomatic patients with severe rheumatic know,n as myxomatous or degenerative mitral valve disease), mitral stenosis and a pulmonary artery systolic pressure radiation therap)'. rheumatic disease. and cleft mitral valve. greater than 50 mm Hg. PBMC should not be performed in Mitral regurgitation results in volume overload with LV patients with LA thrombus or moderate or severe mitral regur dilatation and LA hypertension. which may progress and gitation, both of which are optimally evaluated with TEE. In cause pulmonary hypertension and RV failure. In acute mitral appropriately selected patients, the success rate with PBMC is regurgitation, hearl failure symptoms often occur abruptly 95'/,, and complications occur in fewer than 5'1, of patients. because of insufficient time fbr adaptive chamber dilatation Mitral valve surgery is indicated in patients with severe mitral and, in some patients. can result in cardiogenic shock. The stenosis, New York Heart Association functional class III or IV systolic murrnur in acute mitral regurgitation may be brief symptoms, and a nonpliable valve and in asymptomatic because of the rapid equalization of l.A and LV pressures. and patients with severe mitral stenosis undergoing concomitant echocardiography with color flow imaging can underestimate cardiac surgery for another indication. the severity of the regurgitation. Thus, when acute mitral Nearly 50'2, of patients with mitral stenosis have atrial regurgitation is suspected, comprehensive assessment to iden fibrillation, and without anticoagulation, these patients have a tify the potential causes should be pursued. and additional risk for thromboembolism of 20'7, to 25')(,. Patients with mod imaging with TEE should be considered. erate to severe mitral stenosis and concomitant atrial fibrilla Chronic primary mitral regurgitation is predominantly tion should receive a vitamin K antagonist such as warfarin, caused by mitral valve prolapse. which affects approxi with a goal INR of 2.0 to 3.0. Anticoagulation is also indicated mately 27, of the general population in the United States. in patients with a history of LA thrombus or systemic emboli Echocardiography in patients with chronic primary mitral zation. Notably, clinical trials of direct acting oral anticoagu regurgitation may show a range of abnormalities, including lants in atrial fibrillation excluded patients with moderate to prolapse (Figure 34), gross degeneration ofone or both leaf: severe mitral stenosis; therefore. the efficacy and salety of Iets (Barlow syndrome). or chordal rupture' ,ith flail leaflet. these agents have not been demonstrated in this population. Barlow syndrome is more common in young adult patients. In x Because the mitral gradient is heavily dependent on patients who are relatively older. fibroelastic deficiency pre transvalvular flow medical therapy with negative chrono It dominates and frequently results in chordal rupture. In tropic agents, diuretics, and long-acting nitrates can be effec patients with chronic secondary mitral regurgitation, ven- tive for symptom palliation in patients who are not candidates tricular dysfunction causes mitral regurgitation through pap for interventional or surgical therapy. illary muscle displacement and tethering of the mitral leaflets. X EY PO I TITS which impairs coaptation. The mitral valve apparatus is nor mal in patients with chronic secondary mitral regurgitation . In patients with mitral stenosis, transthoracic echocar (Figure 35). diography is highly accurate for assessing disease sever The physical examination in patients with chronic mitral ity, pulmonary pressures, and right ventricular function regurgitation is notable for a blowing holosystolic murmur at as well as identiSring concomitant valvular lesions. the apex. In patients with mitral valve prolapse. one or more . Percutaneous balloon mitral commissurotomy is indi- systolic clicks may precede the murmur. and variation in cated for patients with symptomatic severe mitral ste severity, preload, and afterload can lead to differences in nosis and favorable valve morphologr. murmur onset (holosystolic, midsystolic. or late systolic). o Anticoagulation is recommended for patients with In patients with LV dilatation, the apical impulse may be mitral stenosis and a history of atrial fibrillation, left displaced laterally, and an S,, may be audible. especially in atrial thrombus, or systemic embolization. patients with secondarl, n.tt,tr, regurgitation due to LV dysfunction. TTE readily and accurately assesses the selerity of pri- Mitral Regurgitation mary mitral regurgitation. Severe primary mitral regurgitation Clinical Presentation and Evaluation is defined by several parameters, the most common of which Mitral regurgitation may arise from dysfunction of any portion are an effective regurgitant orifice area of 0.4 cm2 or larger, of the complex valve apparatus (leaflets, annulus, chordae. regurgitant volume of 60 mL or more, and vena contracta of 70

Valvular Heart Disease 4r./1c-: qlfiz rp;-.ril- 'l40mm ADULT ECHO NTHI Generoi .;; Pwr:0dB l"!l:1 9 c,lds s1/ ltt/ 4 Ao fialn: 1dE a:3 LV TE?,l,fiIiE I HR: f,4bpm \LA i!,1 FR 14Hz -47 i 20 r 135,'; ';I 1i" i9r 52"; 1.1'! rz T t ",Ao re. ,,

t ",Ao re. ,, LV -* e - - 10 .,9, :ir - g; 5i 97 bpm "ai: I seen\arrowheads), leading to severe regurgitati0n seen on color flow imaging (arrow, bolton right panel) Ao = ascending aorta; LA- left atrium; LV = left ventricle.

LV -* e - - 10 .,9, :ir - g; 5i 97 bpm "ai: I seen\arrowheads), leading to severe regurgitati0n seen on color flow imaging (arrow, bolton right panel) Ao = ascending aorta; LA- left atrium; LV = left ventricle. 0.7 cm or lar[e r. WI]en T1'I pnrvides insr-rtlicient or rliscordant iire sigt-tiliclnt synlptonrs. llail lerrllct. or LV clilatation. lrr onc infirrr.nation,'l'lrl.l or CMIt inrrging is inclicated. study ol .15t3 patier-rts r,r,ith iisynrptonrxtic sevcrr primary nritral r\pproprirrtc fbllou, u1l of asyn'rlttontiltic piltirltts u,ith regulgitiltion. the 5 y'crrr sun,ivirl rrte \\'as on11, 58')i,. Surgical nlitrirl regurgitiltion is outIinerl ir-r'liible 2:1. rep:rir of thc r-r-ritral 'urrlve is inclicrrtccl fbr chronic severr pri mary mi trll regllrgitrltiolr in syurptonrirtic prrt ients (regr rcl less Management ol LV systolic function) and :rsytuptotnatic prltients with lV l\4edicll therrrpl, rlnd surgical illten'elttion can be lile saving dysfttnction (ejection liitction ((;0'1,, and or IV end s1'stolic in l)rltients u ith ircute se\e rc lritrul regurgitirtion. \'rrsoclilator clir-nension > 10 mnr). Surgical re prrir is relsonirble in ttsl r-npto thcrapy lr,ith I titratiible clrug, such xs nitnrprusside. decreases matic paticllts witl.r prcservecl IV function r,r,lren the expectccl iiortic it-npedlncc and mitral regurgitation, therebf irlproving repair succcss ratc is grclrter tl-ran 95',/, and thc operative risk is ftrnrirrcl carcliirc olltput. An intra aortic birlkror-r punrp can be less tl'rirn l't,. Surgicrrl rcpair is prrlerred ovcr repltrcerlenl in ttsecl to decreirse irlterloacl irnrl irugr-nent slster-r-ric irr.rrl crrror-rar1 irll patie nts. ilnd piiticnts sl-roukl lre re ferrecl to ir surgicril ce ntcr pcrlt-tsion pressLlres. Pronrllt srtrgical correction sl'uukl be con lvitl-r expcrtise to irrlrrove thc chances o{ re1;lir. Meilicrrl sirle rerl Ibr iill prrticnts with acutc sevcrc nritral regurgitltion. therapy with vasoclilators is not bcnelicial in patients r,r,,ith [)irtients '"vith chronic scr,e re prinrrrrl' nritral regurgitrition primary nritrirl regurgitrrtion in thc irl-rsence ol slnrptolt-ts or I11 gencrirlll do poorlv uitholrt surger)l prrrticularll'u'hor there tlvstunction.

0.7 cm or lar[e r. WI]en T1'I pnrvides insr-rtlicient or rliscordant iire sigt-tiliclnt synlptonrs. llail lerrllct. or LV clilatation. lrr onc infirrr.nation,'l'lrl.l or CMIt inrrging is inclicated. study ol .15t3 patier-rts r,r,ith iisynrptonrxtic sevcrr primary nritral r\pproprirrtc fbllou, u1l of asyn'rlttontiltic piltirltts u,ith regulgitiltion. the 5 y'crrr sun,ivirl rrte \\'as on11, 58')i,. Surgical nlitrirl regurgitiltion is outIinerl ir-r'liible 2:1. rep:rir of thc r-r-ritral 'urrlve is inclicrrtccl fbr chronic severr pri mary mi trll regllrgitrltiolr in syurptonrirtic prrt ients (regr rcl less Management ol LV systolic function) and :rsytuptotnatic prltients with lV l\4edicll therrrpl, rlnd surgical illten'elttion can be lile saving dysfttnction (ejection liitction ((;0'1,, and or IV end s1'stolic in l)rltients u ith ircute se\e rc lritrul regurgitirtion. \'rrsoclilator clir-nension > 10 mnr). Surgical re prrir is relsonirble in ttsl r-npto thcrapy lr,ith I titratiible clrug, such xs nitnrprusside. decreases matic paticllts witl.r prcservecl IV function r,r,lren the expectccl iiortic it-npedlncc and mitral regurgitation, therebf irlproving repair succcss ratc is grclrter tl-ran 95',/, and thc operative risk is ftrnrirrcl carcliirc olltput. An intra aortic birlkror-r punrp can be less tl'rirn l't,. Surgicrrl rcpair is prrlerred ovcr repltrcerlenl in ttsecl to decreirse irlterloacl irnrl irugr-nent slster-r-ric irr.rrl crrror-rar1 irll patie nts. ilnd piiticnts sl-roukl lre re ferrecl to ir surgicril ce ntcr pcrlt-tsion pressLlres. Pronrllt srtrgical correction sl'uukl be con lvitl-r expcrtise to irrlrrove thc chances o{ re1;lir. Meilicrrl sirle rerl Ibr iill prrticnts with acutc sevcrc nritral regurgitltion. therapy with vasoclilators is not bcnelicial in patients r,r,,ith [)irtients '"vith chronic scr,e re prinrrrrl' nritral regurgitrition primary nritrirl regurgitrrtion in thc irl-rsence ol slnrptolt-ts or I11 gencrirlll do poorlv uitholrt surger)l prrrticularll'u'hor there tlvstunction. 71

Valvular Heart Disease FIGURE 35. Echocardiogramsdemonstratingsecondarymitral regurgitationinapatientwithpreviousinferiormyocardial infarction.Tetheringof theposteriorleaflet aorta; LA = left atrium; LV = left ventricle. For patients who are not surgical candidates, transcatheter l( EY PO I t{TS (cotttinued) mitral valve repair with a clip device (transcatheter edge-to- . Surgery for chronic severe primary mitral regurgitation edge repair [TEER]) improves coaptation of the mitral valve is indicated in the presence of symptoms. left ventricular leaflets, leading to increased valve closure and a reduction in dilatation. or decreasing ejection fraction. regurgitation (Figure 36). In selected patients with primary mitral regurgitation, success rates with TEER are approximately . Surgical repair is preferred over replacement in patients 90%, with a procedural mortality rate of approximately 2'2,. with chronic primary mitral regurgitation; patients should In patients with chronic secondary mitral regurgitation, be referred to a surgical center with expertise to improve

For patients who are not surgical candidates, transcatheter l( EY PO I t{TS (cotttinued) mitral valve repair with a clip device (transcatheter edge-to- . Surgery for chronic severe primary mitral regurgitation edge repair [TEER]) improves coaptation of the mitral valve is indicated in the presence of symptoms. left ventricular leaflets, leading to increased valve closure and a reduction in dilatation. or decreasing ejection fraction. regurgitation (Figure 36). In selected patients with primary mitral regurgitation, success rates with TEER are approximately . Surgical repair is preferred over replacement in patients 90%, with a procedural mortality rate of approximately 2'2,. with chronic primary mitral regurgitation; patients should In patients with chronic secondary mitral regurgitation, be referred to a surgical center with expertise to improve the primary goal of therapy is to address the underlying ven the chances of repair. tricular dysfunction with guideline directed medical therapy o Transcatheter mitral valve repair with implantation of a and, ifindicated, cardiac resynchronization therapy (see Heart clip device may be considered for patients with chronic Failure). Guideline directed medical therapy for ventricular severe primary mitral regurgitation who are at high sur- dysfunction includes an ACE inhibitor, ARB, or angiotensin gical risk. receptor neprilysin inhibitor; a p blocker; a diuretic: andior o Patients with chronic secondary mitral regurgitation an aldosterone antagonist. Benefits ofvalve repair or replace should be treated with guideline directed medical ther ment in patients with secondary mitral regurgitation are less apy for ventricular dysfunction: surgical intervention certain, although studies have demonstrated favorable LV may be considered for those undergoing concomitant remodeling after surgery. Surgery fbr secondary severe mitral cardiac surgery. regurgitation is reasonable fbr those undergoing coronary artery bypass grafting, but mitral regurgitation may recur after repair because of primary LV dysfunction. TEER has been Tricuspid Valve Disease approved by the FDA for patients with chronic secondary Tricuspid regurgitation. the most common form of tricuspid mitral regurgitation and refractory symptoms despite optimal valve disease, is frequently functional (or secondary) and clini medical therapy for heart failure; however, the effect of TEER cally asymptomatic. Causes of tricuspid regurgitation include on mortality and heart failure hospitalization in this popula cor pulmonale (or pulmonary hypertension) rvith RV failure. tion has varied in studies. pacemaker or defibrillator lead placement. trauma. congenital I(EY POITIS abnormalities. and infective endocarditis. \[r]ren st'mptomatic. . Patients with acute mitral regurgitation may present patients may present with fatigue from [on'cardiac output as well as signs and symptoms of right sided failure. such as with acute heart failure; these patients may be difficult elevated jugular venous pulse (a large c u wave), a palpable RV to diagnose clinically or with echocardiography. lift, hepatic congestion with pulsatile liver, and peripheral (Continued) edema. The murmur ol tricuspid regurgitation is typically a

the primary goal of therapy is to address the underlying ven the chances of repair. tricular dysfunction with guideline directed medical therapy o Transcatheter mitral valve repair with implantation of a and, ifindicated, cardiac resynchronization therapy (see Heart clip device may be considered for patients with chronic Failure). Guideline directed medical therapy for ventricular severe primary mitral regurgitation who are at high sur- dysfunction includes an ACE inhibitor, ARB, or angiotensin gical risk. receptor neprilysin inhibitor; a p blocker; a diuretic: andior o Patients with chronic secondary mitral regurgitation an aldosterone antagonist. Benefits ofvalve repair or replace should be treated with guideline directed medical ther ment in patients with secondary mitral regurgitation are less apy for ventricular dysfunction: surgical intervention certain, although studies have demonstrated favorable LV may be considered for those undergoing concomitant remodeling after surgery. Surgery fbr secondary severe mitral cardiac surgery. regurgitation is reasonable fbr those undergoing coronary artery bypass grafting, but mitral regurgitation may recur after repair because of primary LV dysfunction. TEER has been Tricuspid Valve Disease approved by the FDA for patients with chronic secondary Tricuspid regurgitation. the most common form of tricuspid mitral regurgitation and refractory symptoms despite optimal valve disease, is frequently functional (or secondary) and clini medical therapy for heart failure; however, the effect of TEER cally asymptomatic. Causes of tricuspid regurgitation include on mortality and heart failure hospitalization in this popula cor pulmonale (or pulmonary hypertension) rvith RV failure. tion has varied in studies. pacemaker or defibrillator lead placement. trauma. congenital I(EY POITIS abnormalities. and infective endocarditis. \[r]ren st'mptomatic. . Patients with acute mitral regurgitation may present patients may present with fatigue from [on'cardiac output as well as signs and symptoms of right sided failure. such as with acute heart failure; these patients may be difficult elevated jugular venous pulse (a large c u wave), a palpable RV to diagnose clinically or with echocardiography. lift, hepatic congestion with pulsatile liver, and peripheral (Continued) edema. The murmur ol tricuspid regurgitation is typically a 72

Valvular Heart Disease Medical therapy with loop diuretics and aldosterone antagonists alleviates symptoms of right-sided congestion; risks include low-flow state with impaired cardiac output. Surgery is recommended for patients with severe tricuspid regurgitation who are undergoing left-sided valve surgery. Surgery may be considered in patients with severe sympto matic tricuspid regurgitation refractory to medical therapy. Tricuspid stenosis is almost always caused by rheumatic disease. Other causes include radiation therapy, carcinoid syndrome, and medications (e.g., the ergot agent cabergo- line). Symptoms of tricuspid stenosis (fatigue, cold skin) are typically overshadowed by those caused by the left sided abnormalities of coexistent rheumatic mitral disease. Clinical AoV , C findings include signs of right-sided congestion (elevated

Medical therapy with loop diuretics and aldosterone antagonists alleviates symptoms of right-sided congestion; risks include low-flow state with impaired cardiac output. Surgery is recommended for patients with severe tricuspid regurgitation who are undergoing left-sided valve surgery. Surgery may be considered in patients with severe sympto matic tricuspid regurgitation refractory to medical therapy. Tricuspid stenosis is almost always caused by rheumatic disease. Other causes include radiation therapy, carcinoid syndrome, and medications (e.g., the ergot agent cabergo- line). Symptoms of tricuspid stenosis (fatigue, cold skin) are typically overshadowed by those caused by the left sided abnormalities of coexistent rheumatic mitral disease. Clinical AoV , C findings include signs of right-sided congestion (elevated I I r' <-- * r-l jugular venous pulse, hepatic congestion, peripheral edema) and a diastolic rumble. Surgery for tricuspid stenosis is typically performed in concert with therapy for rheumatic \ mitral disease. TTY POIXIS o Loop diuretics and aldosterone antagonists alleviate symptoms of right-sided congestion in patients with LA { +-- -*r \: significant tricuspid regurgitation. r Tricuspid valve surgery is recommended for patients with severe tricuspid regurgitation undergoing left-sided lalve LV t. ,1 $ugery and may be considered in patients with severe symptomatic tricuspid regurgitation refractory to medical therapy.

I I r' <-- * r-l jugular venous pulse, hepatic congestion, peripheral edema) and a diastolic rumble. Surgery for tricuspid stenosis is typically performed in concert with therapy for rheumatic \ mitral disease. TTY POIXIS o Loop diuretics and aldosterone antagonists alleviate symptoms of right-sided congestion in patients with LA { +-- -*r \: significant tricuspid regurgitation. r Tricuspid valve surgery is recommended for patients with severe tricuspid regurgitation undergoing left-sided lalve LV t. ,1 $ugery and may be considered in patients with severe symptomatic tricuspid regurgitation refractory to medical therapy. Prosthetic Valves The choice ofprosthesis in a patient undergoing surgical valve replacement is multifactorial, considering the patient's age, the expected durability ofthe prosthesis, the surgical risk for reoperation in the event of degeneration, and the ability and willingness of the patient to take warfarin for anticoagulation. For patients of any age, a bioprosthetic valve is recommended for patients who cannot or do not desire to take anticoagula FIGURE 36.Iranscathetermitralvalveclippingforanteriormitral valveflail.,4, tion therapy. According to the American College of Cardiologz/ Atrial view of anterior mitral valve flail segment (arow). B, Ventricular view of American Heart Association. an aortic mechanical valve is anterior mitral valve f lail segment (arow). ( Fusion imaging (three-dimensional reasonable in patients younger than 50 years, a bioprosthesis transesophageal echocardiography, two-dimensional transesophageal echocardiography, and fluoroscopy) of transcatheter mitral clip (arow) deployment is reasonable in patients older than 65 years, and either a bio- t0 anterior mitral valve flail segment. D, Postprocedure atrial view after placement prosthesis or mechanical aortic valve prosthesis is reasonable of three transcatheter clip devices (white arrows) with residual double orifice (red in those aged 50 to 65 years. In patients older than age arows) mitral valve. t, Postprocedure ventricular view after placement of three 65 years, a mitral valve bioprosthesis is reasonable, as is tra nscatheter cl ip deuices (white arrows) with resid ual dou ble orifice (red arows) mechanical valve prosthesis in patients younger than 65 years. mitral valve. AoV = aortic valve; LA = left atrium; LV = left ventricle. However, the final decision on valve type should be reached through a shared decision-making process between the physi- holosystolic murmur heard along the left sternal border that cian and patient. increases during inspiration due to increased venous return. The expected durability ofbioprostheses is 15 years, and Tricuspid regurgitation should be evaluated with TTE, in persons younger than 60 years, approximately 40'X, of valves which also allows assessment of RV function and estimation of have evidence of clinically severe deterioration by 15 years. pulmonary pressures. In patients with tricuspid regurgitation Data on long-term durability ofTAVI prostheses are currently due to pacemaker or defibrillator lead placement, TEE may be limited to a follow-up of 8 to 10 years; however, thus flar, valve required to determine the etiologr more clearly. durability is similar to surgical prostheses.

Prosthetic Valves The choice ofprosthesis in a patient undergoing surgical valve replacement is multifactorial, considering the patient's age, the expected durability ofthe prosthesis, the surgical risk for reoperation in the event of degeneration, and the ability and willingness of the patient to take warfarin for anticoagulation. For patients of any age, a bioprosthetic valve is recommended for patients who cannot or do not desire to take anticoagula FIGURE 36.Iranscathetermitralvalveclippingforanteriormitral valveflail.,4, tion therapy. According to the American College of Cardiologz/ Atrial view of anterior mitral valve flail segment (arow). B, Ventricular view of American Heart Association. an aortic mechanical valve is anterior mitral valve f lail segment (arow). ( Fusion imaging (three-dimensional reasonable in patients younger than 50 years, a bioprosthesis transesophageal echocardiography, two-dimensional transesophageal echocardiography, and fluoroscopy) of transcatheter mitral clip (arow) deployment is reasonable in patients older than 65 years, and either a bio- t0 anterior mitral valve flail segment. D, Postprocedure atrial view after placement prosthesis or mechanical aortic valve prosthesis is reasonable of three transcatheter clip devices (white arrows) with residual double orifice (red in those aged 50 to 65 years. In patients older than age arows) mitral valve. t, Postprocedure ventricular view after placement of three 65 years, a mitral valve bioprosthesis is reasonable, as is tra nscatheter cl ip deuices (white arrows) with resid ual dou ble orifice (red arows) mechanical valve prosthesis in patients younger than 65 years. mitral valve. AoV = aortic valve; LA = left atrium; LV = left ventricle. However, the final decision on valve type should be reached through a shared decision-making process between the physi- holosystolic murmur heard along the left sternal border that cian and patient. increases during inspiration due to increased venous return. The expected durability ofbioprostheses is 15 years, and Tricuspid regurgitation should be evaluated with TTE, in persons younger than 60 years, approximately 40'X, of valves which also allows assessment of RV function and estimation of have evidence of clinically severe deterioration by 15 years. pulmonary pressures. In patients with tricuspid regurgitation Data on long-term durability ofTAVI prostheses are currently due to pacemaker or defibrillator lead placement, TEE may be limited to a follow-up of 8 to 10 years; however, thus flar, valve required to determine the etiologr more clearly. durability is similar to surgical prostheses. 73

Valvular Heart Disease Echocardiography should be performed after implanta I nfective Endocarditis tion to document the baseline hemodynamic performance of the valve, and repeat evaluations should be performed for Diagnosis and Management signs or symptoms of prosthetic dysfunction (new dyspnea or Infective endocarditis (lE) is a life threatening bacterial or fun heart failure. louder murmur or new murmur. thromboembo gal inf'ection ol the endocardium involving native valvular lism, hemolytic anemia). ln patients with a surgical biopros structures or implanted cardiac devices. Such devices include thetic valve, TTE at 5 years and 10 years and then annually cardiac valve prostheses, perrnanent pacemakers. implanted after implantation is reasonable, even in the absence of a cardioverter defibrillators. and occluders for repair ofcongeni change in clinical status. For patients with a transcatheter tal lesions (e.g.. atrial or ventricular septal defect occluders). bioprosthetic valve, annual TTE is reasonable, even in the Risk factors for IE include advanced age, diabetes mellitus, absence of a change in clinical status. immunosuppression. injection drug use. indwelling intrave- Lif'elong warfarin anticoagulation is indicated fbr all nous catheters. congenital heart disease. cardiac transplanta- patients with a mechanical valve. Therapy should target a spe' tion with valvulopathy, and an implanted cardiac device. lE cific INR value rather than a range. In patients with a mechan- carries significant risk fbr morbidity and mortality. with high ical aortic valve prosthesis (bileaflet or current generation rates of in hospital mortality (zo7.), t year mortality (40'x'). single-tilting disc) with no additional risk lactors fbr thrombo peripheral embolization (23'7,), stroke (17'x,). and need for car embolism (history of embolization. hypercoagulable disorder. diac surgery (a8%). Early diagnosis. targeted antimicrobial LV dysfunction, atrial fibrillation), the goal INR for warfarin therapy. and recognition ofthe need for early surgical interven anticoagulation is 2.5. ln patients with a mechanical aortic tion enhance patient survival. valve prosthesis with heightened risk factors for thromboem Diagnosis of definite IE requires pathologic confirmation of bolism, an older generation aortic valve prosthesis (ball in microorganisms demonstrated by culture or histologic examina cage), or any mitral prosthesis, the target tNR is 3.0. An INR tion of a vegetation, a vegetation that has embolized. or an intra- range of 2.5 to 3.5 is acceptable to balance the risks of under cardiac abscess specimen. Much more commonly. the diagnosis and over anticoagulation in a patient with a mechanical mitral is made using the modified Duke criteria (Table 24). The diag- valve. Because of the risk fbr valve thrombosis, direct acting nostic criteria for definite lE include either two major criteria, oral anticoagulants should not be used for anticoagulation in one major criterion plus three minor criteria, or five minor patients with a mechanical valve. Oral anticoagulation with criteria. Possible IE requires one major criterion and one minor warfarin should be considered for 3 to 6 months after implan criterion. or three minor criteria. IE is excluded when there is a tation ola mitral or aortic bioprosthesis. An INR of 2.5 should firm alternative diagnosis, resolution of IE syndrome with anti- be targeted in these patients. biotic therapy for 4 days or less, or no pathologic evidence of Adding aspirin (75 100 mg/d) to warfarin in patients IE at surgery or at autopsy with antibiotic therapy for 4 days with a mechanical prosthesis decreases the risk for stroke or less. and death and may be considered in patients who have an Blood cultures are positive in 90'7, of IE cases. Most cases indication for antiplatelet therapy when the bleeding risk is of culture negative endocarditis occur in patients who have low. Low dose aspirin is reasonable for all patients with a received recent pre culture antibiotics. Serologic testing may bioprosthesis be required to identi[z fastidious organisms that are dilficult to culture. ln patients with a prosthetic valve, IE syndrome is rtY POIXTT classified according to the time from surgery as early (within o A shared decision-making process should guide the 60 days), intermediate (60 365 days), and late (>365 days). choice of prosthetic aortic valve type, especially in Early prosthetic valve endocarditis (PVE) is characterized patients aged 50 to 65 years, in whom either a mechani by infection with hospital acquired microbes. such as cal valve or bioprosthesis is a reasonable choice. Stophyloc'occus oureus. Coagulase negative staphylococci are o Echocardiography is recommended fbr all patients the most common microbes in intermediate PVE. S. oureus and with a surgical bioprosthesis at baseline, 5 and 10 years coagulase negative staphylococci are important causes of late after surgery and then annually thereafter and with PVE; however, the microbes in late PVE more typically resem any change in clinical status suggesting prosthetic ble those of'native valve endocarditis. dysfunction. TTE is indicated to identify vegetations and associated . Echocardiography is recommended for all patients with hemodynamic derangements. TEE is indicated with interme a transcatheter bioprosthesis at baseline and annually diate or high suspicion lbr lE when TTE is not diagnostic. thereafter and with any change in clinical status sug- especially in the setting of a prosthetic valve, intracardiac gesting prosthetic valve dysfunction. device, or myocardial abscess, which in turn is suggested by new conduction abnormalities on ECG or persistent bactere . Lifelong warfarin anticoagulation is recommended in all mia despite antibiotic therapy. In patients with IE who have a patients with a mechanical valve prosthesis; low dose change in clinical signs or symptoms, TTE and/or TEE are aspirin is reasonable for all patients with a bioprosthesis. recommcnded fbr reevaluation.

Echocardiography should be performed after implanta I nfective Endocarditis tion to document the baseline hemodynamic performance of the valve, and repeat evaluations should be performed for Diagnosis and Management signs or symptoms of prosthetic dysfunction (new dyspnea or Infective endocarditis (lE) is a life threatening bacterial or fun heart failure. louder murmur or new murmur. thromboembo gal inf'ection ol the endocardium involving native valvular lism, hemolytic anemia). ln patients with a surgical biopros structures or implanted cardiac devices. Such devices include thetic valve, TTE at 5 years and 10 years and then annually cardiac valve prostheses, perrnanent pacemakers. implanted after implantation is reasonable, even in the absence of a cardioverter defibrillators. and occluders for repair ofcongeni change in clinical status. For patients with a transcatheter tal lesions (e.g.. atrial or ventricular septal defect occluders). bioprosthetic valve, annual TTE is reasonable, even in the Risk factors for IE include advanced age, diabetes mellitus, absence of a change in clinical status. immunosuppression. injection drug use. indwelling intrave- Lif'elong warfarin anticoagulation is indicated fbr all nous catheters. congenital heart disease. cardiac transplanta- patients with a mechanical valve. Therapy should target a spe' tion with valvulopathy, and an implanted cardiac device. lE cific INR value rather than a range. In patients with a mechan- carries significant risk fbr morbidity and mortality. with high ical aortic valve prosthesis (bileaflet or current generation rates of in hospital mortality (zo7.), t year mortality (40'x'). single-tilting disc) with no additional risk lactors fbr thrombo peripheral embolization (23'7,), stroke (17'x,). and need for car embolism (history of embolization. hypercoagulable disorder. diac surgery (a8%). Early diagnosis. targeted antimicrobial LV dysfunction, atrial fibrillation), the goal INR for warfarin therapy. and recognition ofthe need for early surgical interven anticoagulation is 2.5. ln patients with a mechanical aortic tion enhance patient survival. valve prosthesis with heightened risk factors for thromboem Diagnosis of definite IE requires pathologic confirmation of bolism, an older generation aortic valve prosthesis (ball in microorganisms demonstrated by culture or histologic examina cage), or any mitral prosthesis, the target tNR is 3.0. An INR tion of a vegetation, a vegetation that has embolized. or an intra- range of 2.5 to 3.5 is acceptable to balance the risks of under cardiac abscess specimen. Much more commonly. the diagnosis and over anticoagulation in a patient with a mechanical mitral is made using the modified Duke criteria (Table 24). The diag- valve. Because of the risk fbr valve thrombosis, direct acting nostic criteria for definite lE include either two major criteria, oral anticoagulants should not be used for anticoagulation in one major criterion plus three minor criteria, or five minor patients with a mechanical valve. Oral anticoagulation with criteria. Possible IE requires one major criterion and one minor warfarin should be considered for 3 to 6 months after implan criterion. or three minor criteria. IE is excluded when there is a tation ola mitral or aortic bioprosthesis. An INR of 2.5 should firm alternative diagnosis, resolution of IE syndrome with anti- be targeted in these patients. biotic therapy for 4 days or less, or no pathologic evidence of Adding aspirin (75 100 mg/d) to warfarin in patients IE at surgery or at autopsy with antibiotic therapy for 4 days with a mechanical prosthesis decreases the risk for stroke or less. and death and may be considered in patients who have an Blood cultures are positive in 90'7, of IE cases. Most cases indication for antiplatelet therapy when the bleeding risk is of culture negative endocarditis occur in patients who have low. Low dose aspirin is reasonable for all patients with a received recent pre culture antibiotics. Serologic testing may bioprosthesis be required to identi[z fastidious organisms that are dilficult to culture. ln patients with a prosthetic valve, IE syndrome is rtY POIXTT classified according to the time from surgery as early (within o A shared decision-making process should guide the 60 days), intermediate (60 365 days), and late (>365 days). choice of prosthetic aortic valve type, especially in Early prosthetic valve endocarditis (PVE) is characterized patients aged 50 to 65 years, in whom either a mechani by infection with hospital acquired microbes. such as cal valve or bioprosthesis is a reasonable choice. Stophyloc'occus oureus. Coagulase negative staphylococci are o Echocardiography is recommended fbr all patients the most common microbes in intermediate PVE. S. oureus and with a surgical bioprosthesis at baseline, 5 and 10 years coagulase negative staphylococci are important causes of late after surgery and then annually thereafter and with PVE; however, the microbes in late PVE more typically resem any change in clinical status suggesting prosthetic ble those of'native valve endocarditis. dysfunction. TTE is indicated to identify vegetations and associated . Echocardiography is recommended for all patients with hemodynamic derangements. TEE is indicated with interme a transcatheter bioprosthesis at baseline and annually diate or high suspicion lbr lE when TTE is not diagnostic. thereafter and with any change in clinical status sug- especially in the setting of a prosthetic valve, intracardiac gesting prosthetic valve dysfunction. device, or myocardial abscess, which in turn is suggested by new conduction abnormalities on ECG or persistent bactere . Lifelong warfarin anticoagulation is recommended in all mia despite antibiotic therapy. In patients with IE who have a patients with a mechanical valve prosthesis; low dose change in clinical signs or symptoms, TTE and/or TEE are aspirin is reasonable for all patients with a bioprosthesis. recommcnded fbr reevaluation. 74

Valvular Heart Disease TABLE 24. Major and Minor Criteria Used in the Modified Duke Criteria Major Criteria Blood culture positive for infeaive endocarditis Typical microorganisms consistent with infective endocarditis from two separate blood cultures: Viridans streptococci, Streptococcus bovis, HACEK gToup (Haemophllus spp., Actin obacillus actinomycetemcomitans, Cardiobacterium hominis, Eikenella spp., and Kngella kingae), Staphylococcus aureus; or community-acquired enterococci, in the absence o{ a primary focus; or Microorganisms consistent with infective endocarditis from persistently positive blood cultures, defined as follows: At least two positive cultures of blood samples drawn 1 2 h apart; or All ofthreeoramajorityof atleastfourseparateculturesof blood(withfirstandlastsamplesdrawnatleastl hapart) Single blood culture positive flor Coxiella burnetii or antiphase I lgG antibody titer >1 :800 Evidence of endocardial involvement Echocardiogram positive for infective endocarditis (TEE recommended in patients with prosthetic valves, rated at least "possible infective endocarditis" by clinical criteria, or complicated infective endocarditis Iparavalvular abscess]; TTE as firsttest in other patients), defined as follows: Oscillating intracardiac mass on valve or supporting structures, in the path of regurgitant jets, or on implanted material in the absence of an alternative anatomic explanation; or Abscess; or New partial dehiscence of prosthetic valve New valvular regurgitation (worsening or changing of pre-existing murmur not sufficient) Minor Criteria Predisposition, predisposing heart condition, or injection drug use Fever (temperature >38'C [1 00.4'F]) Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions lmmunologic phenomena: glomerulonephritis, Osler nodes, Roth spots, and rheumatoid factor Microbiologic evidence: positive blood culture but does not meet a major criterion as noted above" or serologic evidence of active infection with organism consistent with infective endocarditis TEE = transesophageal echocardiography; TTE = transthoracic echocardiography.

Vascular phenomena: major arterial emboli, septic pulmonary infarcts, mycotic aneurysm, intracranial hemorrhage, conjunctival hemorrhages, and Janeway lesions lmmunologic phenomena: glomerulonephritis, Osler nodes, Roth spots, and rheumatoid factor Microbiologic evidence: positive blood culture but does not meet a major criterion as noted above" or serologic evidence of active infection with organism consistent with infective endocarditis TEE = transesophageal echocardiography; TTE = transthoracic echocardiography. uExcludes single culture positive {or coagulase negative staphylococci and organisms that do not cause endocarditis. Reproduced with permission from Li JS, Sexton DJ, Mick N, et al. Proposed modifications to the Duke criteria for the diagnosis of infective endocarditis. Clin lnfect Dis. 2000;30:6338.lPMlD:1O770721ldoi:10.1086/313753@2000OxlordUniversityPress. Appropriate antimicrobial therapy should be promptly o Severe valvular dysfunction resulting in symptomatic initiated in high risk patients, such as those with severe sep- heart failure sis, once cultures have been obtained. Empiric treatment o Leflt-sided IE caused by S. oureus, fungal infections, or should consider patient risk factors, such as intravenous drug other highly resistant organisms use or the presence of an indwelling intravenous catheter, and o Associated complications, such as annular or aortic ab- focus on the most common causes of acute endocarditis scess, destructive penetrating lesions, or heart block Stophylococcus (methicillin susceptible and methicillin resistant), Streptococcus, and Enterococcus species. Regimens o Persistent bacteremia or fevers lasting longer than 5 to for empiric antibiotic therapy in critically ill patients with 7 days after onset of appropriate antimicrobial therapy

Appropriate antimicrobial therapy should be promptly o Severe valvular dysfunction resulting in symptomatic initiated in high risk patients, such as those with severe sep- heart failure sis, once cultures have been obtained. Empiric treatment o Leflt-sided IE caused by S. oureus, fungal infections, or should consider patient risk factors, such as intravenous drug other highly resistant organisms use or the presence of an indwelling intravenous catheter, and o Associated complications, such as annular or aortic ab- focus on the most common causes of acute endocarditis scess, destructive penetrating lesions, or heart block Stophylococcus (methicillin susceptible and methicillin resistant), Streptococcus, and Enterococcus species. Regimens o Persistent bacteremia or fevers lasting longer than 5 to for empiric antibiotic therapy in critically ill patients with 7 days after onset of appropriate antimicrobial therapy acute endocarditis are provided in Table 25. Antimicrobials Early surgery is reasonable in patients with recurrent emboli should not be administered to patients with VHD and unex and persistent valve vegetations despite 5 to 7 days of antibiotic plained fever before several blood cultures are drawn. Empiric therapy, and early surgery may be considered in the presence ofa antibiotic combinations can be more focused when microbio large (>10-mm) left-sided vegetation to prevent embolic events. logic results are available. Infectious disease consultants When IE is associated with a pacemaker or deflbrillator, the should advise on the duration oftherapy. entire system (generator and leads) must be removed. The decision to pursue surgery for treatment of IE and the timing of such surgery is complex and requires a multidiscipli Prophylaxis nary approach. Early surgery (during hospitalization and Endocarditis prophylaxis is recommended for a specific group before completion of an antimicrobial course) is recom of patients before dental procedures that involve manipulation mended for patients with any of the following: of gingival tissue or the periapical region of the teeth or

acute endocarditis are provided in Table 25. Antimicrobials Early surgery is reasonable in patients with recurrent emboli should not be administered to patients with VHD and unex and persistent valve vegetations despite 5 to 7 days of antibiotic plained fever before several blood cultures are drawn. Empiric therapy, and early surgery may be considered in the presence ofa antibiotic combinations can be more focused when microbio large (>10-mm) left-sided vegetation to prevent embolic events. logic results are available. Infectious disease consultants When IE is associated with a pacemaker or deflbrillator, the should advise on the duration oftherapy. entire system (generator and leads) must be removed. The decision to pursue surgery for treatment of IE and the timing of such surgery is complex and requires a multidiscipli Prophylaxis nary approach. Early surgery (during hospitalization and Endocarditis prophylaxis is recommended for a specific group before completion of an antimicrobial course) is recom of patients before dental procedures that involve manipulation mended for patients with any of the following: of gingival tissue or the periapical region of the teeth or 75

Valvular Heart Disease TABLE 25. EmpiricAntibioticTherapyforCritically lll TABLE 26. Prophylactic lnfective Endocarditis Regimens Patients with Acute Endocarditis for Adults at Highest Risk of an Adverse Outcome Before a Condition European Society American Dental Procedure of Cardiology Heart Situation Agent Dosage" ) (201s) Association Oral Amoxicillin 2g (2OOs) 2glMorlV V"n.ornvon -l Unable to take oral Ampicillin Community- Ampicillin medication acquired native i or with with valve endocarditis or late prosthetic Cefazolin or lglMorlV (Flu)cloxacillin or Cefepime or ceftriaxone valve endocarditis oxacillin ceftriaxone (for (>12 months after prosthetic valve Allergic to Cephalexinb,' 29 surgery) with penicillin or endocarditis) ampicillin - oral or Gentamicin Clindamycin 600 mg Community- Vancomycin No specific acquired native regimen or with valve endocarditis provided Azithromycin or 500 mg or late prosthetic Gentamicin clarithromycin valve endocarditis (>12 rnonths after Allergic to Cefazolin or lglMorlV surgery), penicillin penicillin or ceftriaxone allergic patient ampicillin and or unable to take oral Early prosthetic Vancomycin Vancomycin medication Clindamycin 600 mg lM or lV valve endocarditis (<12 months after with with lM = intramusrular: lV intravenous. surgery) or health Gentamicin Gentamicin care-associated 'Regimen consists of a single dose 30 to 60 mrnutes before the dental procedure, with with or, if inadvertently not administered, drug may be given up to 2 hours after the endocarditis procedure. Rifampinu Rifampinu t'Or other first- or second generation oral cephalosporin in equivalent adult d osag e. with Cefepime 'Cephalosporins should not be used in an individual with a history of anaphylaxrs, angioedema, or urticaria with penicillins or ampicillin. asome experts recommend delaying the start of rifampin for 3 5 days in an lnformation from Wilson W Taubert KA, Gewiu N4, et ai; American Heart attempt to prevent the emergence of resistance to rifampin. Associat on Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee. Prevention of infective endocarditis: guidelines from the American Heart lnformation from Habib G, Lancellotti P, Antunes MJ, et al; ESC Scientific Association: a guideline from the American Heart Association Rheumatic Fever, Document Group. 201 5 ESC Auidelines for the management of infective Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular endocarditis: The Task Force for the Management of lnfective Endocarditis of the Disease in the Young, and the Council on Clinical Cardiology, Council on European Society of Cardiology (ESC). Endorsed by European Association for Cardiovascular Surgery and Anesthesia, and the Oualrty of Care and Outcomes Cardio Thoracic Surgery (EACTS), the European Association of Nuclear Medicine Research lnterdisciplinary Working Group. Circulation.2007;116.1136 54. IPMID: (EANI'/). Eur HeartJ.2015;36:3075 3128. IPMID: 26320109]doi:10.1093/ 17 4464421 do :1 0.1 1 6 1 i Cl RCULATIONAHA. l 06.1 83095 eurheartj/ehv319 and Baddour LM, Wilson WR, Bayer AS, et al; Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. lnfective endocarditis: diagnosis, antimicrobial therapy, and management of complications: a statement for healthcare professionals from the Committee on Rheumatic Fever, Endocardrtis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the o A defect that has been repaired with prosthetic mate- Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Association: endorsed by the lnfectious Diseases Society of America. Circulation. 2005;1 1 1 :e394-434. [PMID: 1 59561 45] doi:1 0.1 1 61 iCIRCULATIONAHA. rial (surgical or catheter-based) within the previous 1 05.1 65564 6 months

TABLE 25. EmpiricAntibioticTherapyforCritically lll TABLE 26. Prophylactic lnfective Endocarditis Regimens Patients with Acute Endocarditis for Adults at Highest Risk of an Adverse Outcome Before a Condition European Society American Dental Procedure of Cardiology Heart Situation Agent Dosage" ) (201s) Association Oral Amoxicillin 2g (2OOs) 2glMorlV V"n.ornvon -l Unable to take oral Ampicillin Community- Ampicillin medication acquired native i or with with valve endocarditis or late prosthetic Cefazolin or lglMorlV (Flu)cloxacillin or Cefepime or ceftriaxone valve endocarditis oxacillin ceftriaxone (for (>12 months after prosthetic valve Allergic to Cephalexinb,' 29 surgery) with penicillin or endocarditis) ampicillin - oral or Gentamicin Clindamycin 600 mg Community- Vancomycin No specific acquired native regimen or with valve endocarditis provided Azithromycin or 500 mg or late prosthetic Gentamicin clarithromycin valve endocarditis (>12 rnonths after Allergic to Cefazolin or lglMorlV surgery), penicillin penicillin or ceftriaxone allergic patient ampicillin and or unable to take oral Early prosthetic Vancomycin Vancomycin medication Clindamycin 600 mg lM or lV valve endocarditis (<12 months after with with lM = intramusrular: lV intravenous. surgery) or health Gentamicin Gentamicin care-associated 'Regimen consists of a single dose 30 to 60 mrnutes before the dental procedure, with with or, if inadvertently not administered, drug may be given up to 2 hours after the endocarditis procedure. Rifampinu Rifampinu t'Or other first- or second generation oral cephalosporin in equivalent adult d osag e. with Cefepime 'Cephalosporins should not be used in an individual with a history of anaphylaxrs, angioedema, or urticaria with penicillins or ampicillin. asome experts recommend delaying the start of rifampin for 3 5 days in an lnformation from Wilson W Taubert KA, Gewiu N4, et ai; American Heart attempt to prevent the emergence of resistance to rifampin. Associat on Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee. Prevention of infective endocarditis: guidelines from the American Heart lnformation from Habib G, Lancellotti P, Antunes MJ, et al; ESC Scientific Association: a guideline from the American Heart Association Rheumatic Fever, Document Group. 201 5 ESC Auidelines for the management of infective Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular endocarditis: The Task Force for the Management of lnfective Endocarditis of the Disease in the Young, and the Council on Clinical Cardiology, Council on European Society of Cardiology (ESC). Endorsed by European Association for Cardiovascular Surgery and Anesthesia, and the Oualrty of Care and Outcomes Cardio Thoracic Surgery (EACTS), the European Association of Nuclear Medicine Research lnterdisciplinary Working Group. Circulation.2007;116.1136 54. IPMID: (EANI'/). Eur HeartJ.2015;36:3075 3128. IPMID: 26320109]doi:10.1093/ 17 4464421 do :1 0.1 1 6 1 i Cl RCULATIONAHA. l 06.1 83095 eurheartj/ehv319 and Baddour LM, Wilson WR, Bayer AS, et al; Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. lnfective endocarditis: diagnosis, antimicrobial therapy, and management of complications: a statement for healthcare professionals from the Committee on Rheumatic Fever, Endocardrtis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the o A defect that has been repaired with prosthetic mate- Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Association: endorsed by the lnfectious Diseases Society of America. Circulation. 2005;1 1 1 :e394-434. [PMID: 1 59561 45] doi:1 0.1 1 61 iCIRCULATIONAHA. rial (surgical or catheter-based) within the previous 1 05.1 65564 6 months t(EY P0lltT5

TABLE 25. EmpiricAntibioticTherapyforCritically lll TABLE 26. Prophylactic lnfective Endocarditis Regimens Patients with Acute Endocarditis for Adults at Highest Risk of an Adverse Outcome Before a Condition European Society American Dental Procedure of Cardiology Heart Situation Agent Dosage" ) (201s) Association Oral Amoxicillin 2g (2OOs) 2glMorlV V"n.ornvon -l Unable to take oral Ampicillin Community- Ampicillin medication acquired native i or with with valve endocarditis or late prosthetic Cefazolin or lglMorlV (Flu)cloxacillin or Cefepime or ceftriaxone valve endocarditis oxacillin ceftriaxone (for (>12 months after prosthetic valve Allergic to Cephalexinb,' 29 surgery) with penicillin or endocarditis) ampicillin - oral or Gentamicin Clindamycin 600 mg Community- Vancomycin No specific acquired native regimen or with valve endocarditis provided Azithromycin or 500 mg or late prosthetic Gentamicin clarithromycin valve endocarditis (>12 rnonths after Allergic to Cefazolin or lglMorlV surgery), penicillin penicillin or ceftriaxone allergic patient ampicillin and or unable to take oral Early prosthetic Vancomycin Vancomycin medication Clindamycin 600 mg lM or lV valve endocarditis (<12 months after with with lM = intramusrular: lV intravenous. surgery) or health Gentamicin Gentamicin care-associated 'Regimen consists of a single dose 30 to 60 mrnutes before the dental procedure, with with or, if inadvertently not administered, drug may be given up to 2 hours after the endocarditis procedure. Rifampinu Rifampinu t'Or other first- or second generation oral cephalosporin in equivalent adult d osag e. with Cefepime 'Cephalosporins should not be used in an individual with a history of anaphylaxrs, angioedema, or urticaria with penicillins or ampicillin. asome experts recommend delaying the start of rifampin for 3 5 days in an lnformation from Wilson W Taubert KA, Gewiu N4, et ai; American Heart attempt to prevent the emergence of resistance to rifampin. Associat on Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee. Prevention of infective endocarditis: guidelines from the American Heart lnformation from Habib G, Lancellotti P, Antunes MJ, et al; ESC Scientific Association: a guideline from the American Heart Association Rheumatic Fever, Document Group. 201 5 ESC Auidelines for the management of infective Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular endocarditis: The Task Force for the Management of lnfective Endocarditis of the Disease in the Young, and the Council on Clinical Cardiology, Council on European Society of Cardiology (ESC). Endorsed by European Association for Cardiovascular Surgery and Anesthesia, and the Oualrty of Care and Outcomes Cardio Thoracic Surgery (EACTS), the European Association of Nuclear Medicine Research lnterdisciplinary Working Group. Circulation.2007;116.1136 54. IPMID: (EANI'/). Eur HeartJ.2015;36:3075 3128. IPMID: 26320109]doi:10.1093/ 17 4464421 do :1 0.1 1 6 1 i Cl RCULATIONAHA. l 06.1 83095 eurheartj/ehv319 and Baddour LM, Wilson WR, Bayer AS, et al; Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease. lnfective endocarditis: diagnosis, antimicrobial therapy, and management of complications: a statement for healthcare professionals from the Committee on Rheumatic Fever, Endocardrtis, and Kawasaki Disease, Council on Cardiovascular Disease in the Young, and the o A defect that has been repaired with prosthetic mate- Councils on Clinical Cardiology, Stroke, and Cardiovascular Surgery and Anesthesia, American Heart Association: endorsed by the lnfectious Diseases Society of America. Circulation. 2005;1 1 1 :e394-434. [PMID: 1 59561 45] doi:1 0.1 1 61 iCIRCULATIONAHA. rial (surgical or catheter-based) within the previous 1 05.1 65564 6 months t(EY P0lltT5 perforation of the oral mucosa (Table 26). Although endocar o Initial diagnostic tests for suspected infective endocar ditis prophylaxis was previously advised for a broad popula- ditis (lE) include blood cultures and transthoracic echo- tion, current guidelines recommend its use only for the cardiography (TTE); TTE is followed by transesophageal following: echocardiography in patients with suboptimal TTE results or with high initial risk for IE. . o HistoU olendocarditis o Cardiac transplantation with valve regurgitation due to a . Early surgery is indicated for patients with acute infec tive endocarditis (lE) presenting with acute and wors- structurally abnormal valve ening valve regurgitation resulting in heart failure; . Prosthetic valve left-sided IE caused by Stophylococcus oureus, fungal, . Prosthetic material used for cardiac valve repair, including or other highly resistant organisms; IE complicated by annuloplasty rings and chords heart block, annular or aortic abscess, or destructive . Unrepaired congenital heart disease penetrating lesion; and IE with persistent bacteremia . Repaired congenital heart disease with residual defects at or fevers lasting longer than 5 to 7 days after starting antibiotic therapy. the site or adjacent to the site ofa prosthetic patch or device

perforation of the oral mucosa (Table 26). Although endocar o Initial diagnostic tests for suspected infective endocar ditis prophylaxis was previously advised for a broad popula- ditis (lE) include blood cultures and transthoracic echo- tion, current guidelines recommend its use only for the cardiography (TTE); TTE is followed by transesophageal following: echocardiography in patients with suboptimal TTE results or with high initial risk for IE. . o HistoU olendocarditis o Cardiac transplantation with valve regurgitation due to a . Early surgery is indicated for patients with acute infec tive endocarditis (lE) presenting with acute and wors- structurally abnormal valve ening valve regurgitation resulting in heart failure; . Prosthetic valve left-sided IE caused by Stophylococcus oureus, fungal, . Prosthetic material used for cardiac valve repair, including or other highly resistant organisms; IE complicated by annuloplasty rings and chords heart block, annular or aortic abscess, or destructive . Unrepaired congenital heart disease penetrating lesion; and IE with persistent bacteremia . Repaired congenital heart disease with residual defects at or fevers lasting longer than 5 to 7 days after starting antibiotic therapy. the site or adjacent to the site ofa prosthetic patch or device 76

Myocardial Disease triad). Patients with dynamic obstruction may develop dysp- Myocardial Disease nea, presyncope, or syncope during periods ofincreased ven tricular contractility (exercise) or with decreases in ventricular Hypertrophic Ca rdiomyopathy preload or afterload, all of which may worsen the degree of Clinical Presentation obstruction. Hypertrophic cardiomyopathy (HCpf) is an autosomal domi- Atrial fibrillation is common in patients with HCM, and nant heritable disorder related to mutations in the genes that risk increases with age. During atrial fibrillation with rapid primarily encode sarcomeric proteins. It is characterized by ventricular response, diastolic filling periods shorten, worsen increased left ventricular (LV) wall thickness in the absence of ing diastolic dysfunction; reduced LV filling may exacerbate loading conditions (e.g., hypertension) or other underlying the LVOT gradient. causes. HCM affects approximately I in 500 persons and may be identified in all age groups, although in the United States, Evaluation index cases typically present within the third to fourth dec Physical examination findings may be normal in patients ades of life. without IVOT obstruction. The most common feature related Most patients with HCM are asymptomatic and have nor- to LVOT obstruction is a cardiac murmur, and dynamic mal life expectancy, with diagnosis often resulting lrom evalu maneuvers during examination are helpful in differentiating ation of a heart murmur or abnormal ECG. However. certain HCM from fixed valvular obstruction (Table 27). subsets of patients are more likely to develop symptomatic Twelve-lead ECG reveals abnormal findings, such as disease and are at risk for sudden cardiac death (SCD). increased QRS voltage, evidence of left atrial enlargement, LV Symptomatic individuals usually present with signs and conduction abnormalities, pathologic Q waves, and significant symptoms of heart failure (dyspnea, fatigue) or arrhythmias repolarization abnormalities, in 75"1, to 95% of persons with (palpitations, syncope). SCD may be the initial manifestation HCM (Figure 37); however, there is substantial interpersonal in some patients. variability in the degree of abnormalities. Heart failure symptoms are attributable to abnormal LV fill The diagnosis of HCM is most commonly established ing (diastolic dysfunction) and dynamic left ventricular outflow by transthoracic echocardiography (TTE) (Figure 38). Echocar- tract (LVOT) obstruction. Diastolic dysfunction is multifactorial, diography demonstrates the magnitude and distribution ol involving increased chamber stiffness, progressive fibrosis, and hypertrophy (>15 mm in any LV region or 13 14 mm in a per myocardial ischemia due to mismatch of coronary flow and LV son with a first-degree relative with HCM), reveals the pres mass. Dynamic LVOT obstruction, characterized by asymmet- ence and degree of dynamic LVOT obstruction and mitral ric LV hypertrophy with prominent interventricular septal regurgitation, and characterizes diastolic LV filling. Doppler thickening, is the classic form of HCM. Outflow tract obstruc echocardiography is the modality of choice for quantiffing the tion in HCM is found on echocardiography in 30'X, of patients LVOT gradient to difl'erentiate obstructive from nonobstructive at rest and in an additional 407, of patients with provocation; HCM, an important distinction in symptom management. In 30'1, of patients have no obstruction. During ventricular systole, patients with HCM and resting LVOT gradient less than 50 mm anterior motion of the mitral valve results in early to midsys Hg, echocardiography with provocative maneuvers (Valsalva, tolic obstruction of the LVOT and subsequent mitral regurgita- squatting) is recommended to establish the diagnosis. ll tion related to leaflet malcoaptation ("eject obstruct leak" provocative maneuvers fail to elicit an outflow gradient in

triad). Patients with dynamic obstruction may develop dysp- Myocardial Disease nea, presyncope, or syncope during periods ofincreased ven tricular contractility (exercise) or with decreases in ventricular Hypertrophic Ca rdiomyopathy preload or afterload, all of which may worsen the degree of Clinical Presentation obstruction. Hypertrophic cardiomyopathy (HCpf) is an autosomal domi- Atrial fibrillation is common in patients with HCM, and nant heritable disorder related to mutations in the genes that risk increases with age. During atrial fibrillation with rapid primarily encode sarcomeric proteins. It is characterized by ventricular response, diastolic filling periods shorten, worsen increased left ventricular (LV) wall thickness in the absence of ing diastolic dysfunction; reduced LV filling may exacerbate loading conditions (e.g., hypertension) or other underlying the LVOT gradient. causes. HCM affects approximately I in 500 persons and may be identified in all age groups, although in the United States, Evaluation index cases typically present within the third to fourth dec Physical examination findings may be normal in patients ades of life. without IVOT obstruction. The most common feature related Most patients with HCM are asymptomatic and have nor- to LVOT obstruction is a cardiac murmur, and dynamic mal life expectancy, with diagnosis often resulting lrom evalu maneuvers during examination are helpful in differentiating ation of a heart murmur or abnormal ECG. However. certain HCM from fixed valvular obstruction (Table 27). subsets of patients are more likely to develop symptomatic Twelve-lead ECG reveals abnormal findings, such as disease and are at risk for sudden cardiac death (SCD). increased QRS voltage, evidence of left atrial enlargement, LV Symptomatic individuals usually present with signs and conduction abnormalities, pathologic Q waves, and significant symptoms of heart failure (dyspnea, fatigue) or arrhythmias repolarization abnormalities, in 75"1, to 95% of persons with (palpitations, syncope). SCD may be the initial manifestation HCM (Figure 37); however, there is substantial interpersonal in some patients. variability in the degree of abnormalities. Heart failure symptoms are attributable to abnormal LV fill The diagnosis of HCM is most commonly established ing (diastolic dysfunction) and dynamic left ventricular outflow by transthoracic echocardiography (TTE) (Figure 38). Echocar- tract (LVOT) obstruction. Diastolic dysfunction is multifactorial, diography demonstrates the magnitude and distribution ol involving increased chamber stiffness, progressive fibrosis, and hypertrophy (>15 mm in any LV region or 13 14 mm in a per myocardial ischemia due to mismatch of coronary flow and LV son with a first-degree relative with HCM), reveals the pres mass. Dynamic LVOT obstruction, characterized by asymmet- ence and degree of dynamic LVOT obstruction and mitral ric LV hypertrophy with prominent interventricular septal regurgitation, and characterizes diastolic LV filling. Doppler thickening, is the classic form of HCM. Outflow tract obstruc echocardiography is the modality of choice for quantiffing the tion in HCM is found on echocardiography in 30'X, of patients LVOT gradient to difl'erentiate obstructive from nonobstructive at rest and in an additional 407, of patients with provocation; HCM, an important distinction in symptom management. In 30'1, of patients have no obstruction. During ventricular systole, patients with HCM and resting LVOT gradient less than 50 mm anterior motion of the mitral valve results in early to midsys Hg, echocardiography with provocative maneuvers (Valsalva, tolic obstruction of the LVOT and subsequent mitral regurgita- squatting) is recommended to establish the diagnosis. ll tion related to leaflet malcoaptation ("eject obstruct leak" provocative maneuvers fail to elicit an outflow gradient in TAB LE 2 7.Physical Examination Findings of Dynamic Left Ventricular Outflow Tract Obstruction Versus Fixed Valvular Aortic Stenosis Finding or Maneuver Hypertrophic Cardiomyopathy with Valvular Aortic Stenosis Dynamic LVOT Obstruction Characteristic of murmur Ejection-quality murmur usually best heard at Ejection-quality murmur usually best heard at left lower sternal border; generally does not right second intercostal space with radiation to radiate to the carotid arteries the carotid arteries Carotid impulse Brisk upstroke; may have two impulses for each Upstroke is often diminished and delayed ejection (bifid) (parvus ettardus)

TAB LE 2 7.Physical Examination Findings of Dynamic Left Ventricular Outflow Tract Obstruction Versus Fixed Valvular Aortic Stenosis Finding or Maneuver Hypertrophic Cardiomyopathy with Valvular Aortic Stenosis Dynamic LVOT Obstruction Characteristic of murmur Ejection-quality murmur usually best heard at Ejection-quality murmur usually best heard at left lower sternal border; generally does not right second intercostal space with radiation to radiate to the carotid arteries the carotid arteries Carotid impulse Brisk upstroke; may have two impulses for each Upstroke is often diminished and delayed ejection (bifid) (parvus ettardus) Valsalva maneuver lncrease in intensity of murmur during strain No change or diminished murmur intensity phase(LR+=14) during strain phase Position lncrease in intensity of murmur with standing No significant change in intensity of murmur from squat or seated position (LR+ = 6.0); with position decrease in murmur with elevation of legs when supine (LR+ = 7.6) Peripheral pulse after PVC No change or decrease in intensity of pulse lncrease in intensity of pulse LR+ = positive likelihood ratio; LVOT = left ventricular outflow tract; PVC = premature ventricular contraction- 77