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I Pleural Disease TAELE 25. Criteria for Exudative Pleural Effusion Test Sensitivity (%) Specificity (%) Combined Light's criteria (1 or more of the following 3): 97 85 Ratio o{ pleural fluid protein to serum protein level >0.5 90 90 Ratio of pleural fluid LDH level to serum LDH level >0.6 88 91 Pleural-fluid LDH level >2/3the upper limit of normal {or serum LDH 88-89 93-1 00 Pleuralfluid cholesterol level >55 mg/dL 85-94 9s-99 Ratio of pleural-fluid cholesterolto serum cholesterol >0.3 93 94 LDH = lactate dehydrogenase. Data from Wilcox ME, Chong CA, Stanbrook MB, et al. Does this patient have an exudative pleural effusion? The Rational Clinical Examination systematic review. JAMA. 201 4;3 1 1 2422-2431 . IPMID:249385651 doi:1 0.1 00 1./jama.201 4.5552

Pleuralfluid cholesterol level >55 mg/dL 85-94 9s-99 Ratio of pleural-fluid cholesterolto serum cholesterol >0.3 93 94 LDH = lactate dehydrogenase. Data from Wilcox ME, Chong CA, Stanbrook MB, et al. Does this patient have an exudative pleural effusion? The Rational Clinical Examination systematic review. JAMA. 201 4;3 1 1 2422-2431 . IPMID:249385651 doi:1 0.1 00 1./jama.201 4.5552 TABLE 26. Causes of Transudates and Exudates Neutrophil predominant effusions are secondary to an acute process such as pneumonia (parapneumonic effusion) or pul Transudates Exudates monary embolus. Lymphocyte predominance (more than 50%) Very Common is common in chronic effusions. The most common causes Heart failure Parapneumonic worldwide of lymphocyte predominant effusions are tubercu Cirrhosis Malignancy losis and cancer. An effusion with greater than l0'2, eosinophils Less Common is most commonly caused by current or recent air or blood in the pleural space and is a nonspecific finding (Table 27). Nephrotic syndrome Pulmonary embolism Hypoalbuminemia Tuberculosis Chemical Anolysis Unexpandable (trapped) lung Autoimmune diseases (RA, SLE) Pleural fluid acidosis (pH less than 7.3) is nonspecific and occurs Peritoneal dialysis Benign asbestos effusion in malignant effusions, complicated parapneumonic effusions, Atelectasis Coronary artery bypass esophageal rupture, and inflammatory conditions such as rheu surgery matoid and lupus pleuritis. A pH less than7.2 in a parapneu- Urinothorax Pancreatitis monic effusion indicates that a complicated pleural effusion is Constrictive pericarditis Myocardial infarction present and tube thoracostomy drainage is needed. Glucose Meigs syndrome (ovarian fibroma with ascites) Yellow nail syndrome normally difftrses freely across the pleural membrane. A pleural (lymphatic disorders) glucose concentration of less than 60 mg/dl (3.33 mmol/L) nar Myxedema Drugs (e.g., nitrofurantoin, rows the differential significantly and suggests that the effu Pulmonary arterial hypertension dantrolene, methysergide, sion is secondary to malignancy, empyema or complicated dasatinib, amiodarone, parapneumonic effusion, tuberculosis, esophageal rupture, or methotrexate, phenytoin, interleukin-2) rheumatoid or lupus pleuritis. Pleural fluid amylase concentration should be checked if Sarcoidosis there is concern that the effusion may be a result of pancreatitis Abdominal disease (e.9., pancreatitis, esophageal or esophageal mpture. Pleural fluid amylase greater than the perforation) upper limit of normal for serum amylase or a pleural fluid to RA = rheumatoid anhritis; SLE = systemic lupus ery/thematosus. serum amylase ratio greater than 1.0 are indicative of pancrea titis or esophageal rupture.

TABLE 26. Causes of Transudates and Exudates Neutrophil predominant effusions are secondary to an acute process such as pneumonia (parapneumonic effusion) or pul Transudates Exudates monary embolus. Lymphocyte predominance (more than 50%) Very Common is common in chronic effusions. The most common causes Heart failure Parapneumonic worldwide of lymphocyte predominant effusions are tubercu Cirrhosis Malignancy losis and cancer. An effusion with greater than l0'2, eosinophils Less Common is most commonly caused by current or recent air or blood in the pleural space and is a nonspecific finding (Table 27). Nephrotic syndrome Pulmonary embolism Hypoalbuminemia Tuberculosis Chemical Anolysis Unexpandable (trapped) lung Autoimmune diseases (RA, SLE) Pleural fluid acidosis (pH less than 7.3) is nonspecific and occurs Peritoneal dialysis Benign asbestos effusion in malignant effusions, complicated parapneumonic effusions, Atelectasis Coronary artery bypass esophageal rupture, and inflammatory conditions such as rheu surgery matoid and lupus pleuritis. A pH less than7.2 in a parapneu- Urinothorax Pancreatitis monic effusion indicates that a complicated pleural effusion is Constrictive pericarditis Myocardial infarction present and tube thoracostomy drainage is needed. Glucose Meigs syndrome (ovarian fibroma with ascites) Yellow nail syndrome normally difftrses freely across the pleural membrane. A pleural (lymphatic disorders) glucose concentration of less than 60 mg/dl (3.33 mmol/L) nar Myxedema Drugs (e.g., nitrofurantoin, rows the differential significantly and suggests that the effu Pulmonary arterial hypertension dantrolene, methysergide, sion is secondary to malignancy, empyema or complicated dasatinib, amiodarone, parapneumonic effusion, tuberculosis, esophageal rupture, or methotrexate, phenytoin, interleukin-2) rheumatoid or lupus pleuritis. Pleural fluid amylase concentration should be checked if Sarcoidosis there is concern that the effusion may be a result of pancreatitis Abdominal disease (e.9., pancreatitis, esophageal or esophageal mpture. Pleural fluid amylase greater than the perforation) upper limit of normal for serum amylase or a pleural fluid to RA = rheumatoid anhritis; SLE = systemic lupus ery/thematosus. serum amylase ratio greater than 1.0 are indicative of pancrea titis or esophageal rupture. TABLE 2?. Pleural Cell Counts and Clinical Conditions Cell Type Cell Count Clinical Conditions Erythrocyte 5000-1 0,000/pL(5-1 0 x 1 Qell) Hemothorax if pleural fluid hematocrit >50% peripheral hematocrit Nucleated cells >50,000/pL(50 x 1 oell) Complicated parapneumonic effusions and empyema >1 0,000/1rL(1 0 x 1 oelL) Simple parapneumonic effusion, acute pancreatitis, and lupus pleuritis <5000/pL(5x1Oe/L) Chronic exudates (TB pleuritis and malignancy) Lymphocytes >80% TB, lymphoma, malignancy, RA pleuritis, sarcoidosis, late post-CABG effusions Eosinophils >10o/" Air or blood in the pleural space; also parapneumonic effusions, drug-induced pleuritis, eosinophilic granulomatosis with polyangiitis, benign asbestos effusions, malignancy (lymphoma), pulmonary infarction, parasitic disease CABG = coronary artery bypass graft; RA = rheumatoid anhritis; TB = tuberculosas.

TABLE 2?. Pleural Cell Counts and Clinical Conditions Cell Type Cell Count Clinical Conditions Erythrocyte 5000-1 0,000/pL(5-1 0 x 1 Qell) Hemothorax if pleural fluid hematocrit >50% peripheral hematocrit Nucleated cells >50,000/pL(50 x 1 oell) Complicated parapneumonic effusions and empyema >1 0,000/1rL(1 0 x 1 oelL) Simple parapneumonic effusion, acute pancreatitis, and lupus pleuritis <5000/pL(5x1Oe/L) Chronic exudates (TB pleuritis and malignancy) Lymphocytes >80% TB, lymphoma, malignancy, RA pleuritis, sarcoidosis, late post-CABG effusions Eosinophils >10o/" Air or blood in the pleural space; also parapneumonic effusions, drug-induced pleuritis, eosinophilic granulomatosis with polyangiitis, benign asbestos effusions, malignancy (lymphoma), pulmonary infarction, parasitic disease CABG = coronary artery bypass graft; RA = rheumatoid anhritis; TB = tuberculosas. 38

! i Pleural Disease I a b Pleural fluid triglycerides elevated above 110 mg/dl bacteriologr of pleural space infection differs depending on i (t.Z+ mmollL) support the diagnosis of chylothorax. If the whether it is communit5z-acquired or hospital-acquired. In the I : triglyceride level is between 50 and 110 mg/dl (0.56 and communigz, Streptococcus pneumoniae, Streptococcus pAo- I.24 mmollL), the presence of chylomicrons should be genes, Staphylococcus qureus, and Streptococcus onginosus checked. A true chylothorax results from a disruption of the group are the organisms typically associated with pleural infec- ! : thoracic duct and is usually the result of thoracic surgery tion. Methicillin-resistant S. oureus and Enterobacteriqceoe or trauma. Other causes include malignancy (lymphoma), are more prevalent in nosocomial empyema. Anaerobic bacte tuberculosis, and lymphatic malformations. ria are cultured in greater th an 20% of pleural space infections l and may be a result of the common association with aspiration Tests for Tuberculous Effusions orthe anaerobic environment ofthe pleural space. Polymicrobial The diagnosis oftuberculosis should be considered in a patient infections are common, and empiric antibiotic regimens with a lymphocl.te-predominant exudative effusion of unclear before obtaining culture results should include coverage for cause-however, confirming the diagnosis may be challeng anaerobes. ing. An acid-fast smear of pleural fluid has a sensitivity of less Complicated parapneumonic effusions and empyema than 5%, and mycobacterial culture has a sensitivity of only require drainage. Options for drainage include early surgical lO%to 20% because of the low mycobacterial load. Adenosine intervention via video assisted thoracoscopic surgery and deaminase is an enzyme present in lymphocytes that is ele- chest tube drainage with or without the combined use of vated in most tuberculous pleural effusions (sensitivity, 90%). intrapleural fibrinolytics (streptokinase, urokinase, tissue In countries with a low incidence of tuberculosis, testing for plasminogen activator) and a mucolytic agent (deoxyribonu adenosine deaminase can be useful because a negative test clease, or DNase). There is controversy regarding which helps to exclude tuberculosis. Pleural biopsy is useful for his- approach is best, and this question is under investigation. A tologz and is also the most likely source to yield a positive multidisciplinary discussion should take place to help inform mycobacterial culture (greater than 70%). this decision.

b Pleural fluid triglycerides elevated above 110 mg/dl bacteriologr of pleural space infection differs depending on i (t.Z+ mmollL) support the diagnosis of chylothorax. If the whether it is communit5z-acquired or hospital-acquired. In the I : triglyceride level is between 50 and 110 mg/dl (0.56 and communigz, Streptococcus pneumoniae, Streptococcus pAo- I.24 mmollL), the presence of chylomicrons should be genes, Staphylococcus qureus, and Streptococcus onginosus checked. A true chylothorax results from a disruption of the group are the organisms typically associated with pleural infec- ! : thoracic duct and is usually the result of thoracic surgery tion. Methicillin-resistant S. oureus and Enterobacteriqceoe or trauma. Other causes include malignancy (lymphoma), are more prevalent in nosocomial empyema. Anaerobic bacte tuberculosis, and lymphatic malformations. ria are cultured in greater th an 20% of pleural space infections l and may be a result of the common association with aspiration Tests for Tuberculous Effusions orthe anaerobic environment ofthe pleural space. Polymicrobial The diagnosis oftuberculosis should be considered in a patient infections are common, and empiric antibiotic regimens with a lymphocl.te-predominant exudative effusion of unclear before obtaining culture results should include coverage for cause-however, confirming the diagnosis may be challeng anaerobes. ing. An acid-fast smear of pleural fluid has a sensitivity of less Complicated parapneumonic effusions and empyema than 5%, and mycobacterial culture has a sensitivity of only require drainage. Options for drainage include early surgical lO%to 20% because of the low mycobacterial load. Adenosine intervention via video assisted thoracoscopic surgery and deaminase is an enzyme present in lymphocytes that is ele- chest tube drainage with or without the combined use of vated in most tuberculous pleural effusions (sensitivity, 90%). intrapleural fibrinolytics (streptokinase, urokinase, tissue In countries with a low incidence of tuberculosis, testing for plasminogen activator) and a mucolytic agent (deoxyribonu adenosine deaminase can be useful because a negative test clease, or DNase). There is controversy regarding which helps to exclude tuberculosis. Pleural biopsy is useful for his- approach is best, and this question is under investigation. A tologz and is also the most likely source to yield a positive multidisciplinary discussion should take place to help inform mycobacterial culture (greater than 70%). this decision. Te sts for Pleural MalignancA Malignant Pleural Effusion Cytologic examination of pleural fluid has an average sensitiv The diagnosis of a malignant pleural effusion signifies ity of 60%. Sensitivity is slightly higher in adenocarcinoma advanced disease and overall poor prognosis (usually (79%) and significantly lower in mesothelioma (6%), squa- 4 7 months from the time of diagnosis). In these cases, the mous cell carcinoma (r+%), and lymphoma (+o%). There is goal of management is the relief of symptoms. Several thera- minimal benefit to obtaining and sending more than two fluid peutic options exist, and treatment decisions should be based samples. If cy.tolory is negative and malignancy is still sus on symptoms, prognosis, degree of anticipated lung reexpan- pected, pleural biopsy under direct visualization known as sion, and patient performance status. Repeat therapeutic thoracoscopy-may be the next step in evaluation. This proce thoracentesis is appropriate for patients with poor prognosis dure has a diagnostic sensitivity for malignant disease of (less than 3 months) and slow reaccumulation of fluid. Patients

Te sts for Pleural MalignancA Malignant Pleural Effusion Cytologic examination of pleural fluid has an average sensitiv The diagnosis of a malignant pleural effusion signifies ity of 60%. Sensitivity is slightly higher in adenocarcinoma advanced disease and overall poor prognosis (usually (79%) and significantly lower in mesothelioma (6%), squa- 4 7 months from the time of diagnosis). In these cases, the mous cell carcinoma (r+%), and lymphoma (+o%). There is goal of management is the relief of symptoms. Several thera- minimal benefit to obtaining and sending more than two fluid peutic options exist, and treatment decisions should be based samples. If cy.tolory is negative and malignancy is still sus on symptoms, prognosis, degree of anticipated lung reexpan- pected, pleural biopsy under direct visualization known as sion, and patient performance status. Repeat therapeutic thoracoscopy-may be the next step in evaluation. This proce thoracentesis is appropriate for patients with poor prognosis dure has a diagnostic sensitivity for malignant disease of (less than 3 months) and slow reaccumulation of fluid. Patients greater than 90%. Closed pleural biopsy provides only a ran- with rapid reaccumulation of fluid and dyspnea should be dom sample of pleural tissue without visualization of pleural offered more definitive management. Indwelling pleural cath abnormalities. It is less sensitive than fluid cytologz and has eters with intermittent outpatient drainage provide significant been replaced by thoracoscopy in the diagnostic evaluation of symptom relief, and approximately 50% of patients achieve pleural malignancy. spontaneous pleurodesis after 6 to B weeks. Chemical pleu rodesis refers to obliteration of the pleural space with a scle Management rosing agent (typicatty talc). Talc can be introduced through a Parapneumonic Efftrsions and Empyema thoracostomy tube (talc slurry) or during a thoracoscopy A pleural effusion associated with a bacterial pneumonia is (talc poudrage). Talc pleurodesis is very effective, with a suc- called a parapneumonic effusion. It can be uncomplicated cess rate of 60'/" to 90%, depending on the degree of lung (sterile and free flowing) or complicated (either infected or reexpansion. loculated). Uncomplicated effusions are typically small and resolve on their own with treatment of the pneumonia. Nonmalignant Pleural Eftrsion I Complicated parapneumonic effusions occur with significant Recurrent benign pleural effusions portend a poor prognosis, t inflammation or when bacteria invade the pleural space. An as they are a marker of worsening of the underlying disease. empyema is defined as a bacterial infection of the pleural space, Mortality rates of 50% in one year have been associated with t which results in purulent fluid or a positive Gram stain. Pleural effusions secondary to heart failure, 46% in those with kidney i

greater than 90%. Closed pleural biopsy provides only a ran- with rapid reaccumulation of fluid and dyspnea should be dom sample of pleural tissue without visualization of pleural offered more definitive management. Indwelling pleural cath abnormalities. It is less sensitive than fluid cytologz and has eters with intermittent outpatient drainage provide significant been replaced by thoracoscopy in the diagnostic evaluation of symptom relief, and approximately 50% of patients achieve pleural malignancy. spontaneous pleurodesis after 6 to B weeks. Chemical pleu rodesis refers to obliteration of the pleural space with a scle Management rosing agent (typicatty talc). Talc can be introduced through a Parapneumonic Efftrsions and Empyema thoracostomy tube (talc slurry) or during a thoracoscopy A pleural effusion associated with a bacterial pneumonia is (talc poudrage). Talc pleurodesis is very effective, with a suc- called a parapneumonic effusion. It can be uncomplicated cess rate of 60'/" to 90%, depending on the degree of lung (sterile and free flowing) or complicated (either infected or reexpansion. loculated). Uncomplicated effusions are typically small and resolve on their own with treatment of the pneumonia. Nonmalignant Pleural Eftrsion I Complicated parapneumonic effusions occur with significant Recurrent benign pleural effusions portend a poor prognosis, t inflammation or when bacteria invade the pleural space. An as they are a marker of worsening of the underlying disease. empyema is defined as a bacterial infection of the pleural space, Mortality rates of 50% in one year have been associated with t which results in purulent fluid or a positive Gram stain. Pleural effusions secondary to heart failure, 46% in those with kidney i r fluid cultures identiflz pathogens in only 60% of cases. If infec- failure, and 25% in those with liver failure. As such, refractory I tion is suspected, culture bottles should be inoculated at the benign effusions also deserve prompt attention. If diagnostic a bedside to increase yield. Drainage is required ifthere is a posi- uncertainty persists, additional evaluation with imaging tive Gram stain or culture or when the pH is less than 7 .2. The (chest CT with contrast) and possible thoracoscopy should be I

r fluid cultures identiflz pathogens in only 60% of cases. If infec- failure, and 25% in those with liver failure. As such, refractory I tion is suspected, culture bottles should be inoculated at the benign effusions also deserve prompt attention. If diagnostic a bedside to increase yield. Drainage is required ifthere is a posi- uncertainty persists, additional evaluation with imaging tive Gram stain or culture or when the pH is less than 7 .2. The (chest CT with contrast) and possible thoracoscopy should be I t 39 I

Pleural Disease TABLE 2 8. Risk Factors for Pneumothorax Risk Factors for PSP Smoking Family history Thoracic endometriosis Tall stature Risk Factors for SSP COPD lnterstitial lung disease Tuberculosis Cystic fibrosis Malignancy Necrotizing pneumonia Marfan syndrome PSP = primary spontaneous pneumothorax; SSP = secondary spontaneous pneumothorax.

TABLE 2 8. Risk Factors for Pneumothorax Risk Factors for PSP Smoking Family history Thoracic endometriosis Tall stature Risk Factors for SSP COPD lnterstitial lung disease Tuberculosis Cystic fibrosis Malignancy Necrotizing pneumonia Marfan syndrome PSP = primary spontaneous pneumothorax; SSP = secondary spontaneous pneumothorax. considered. Options for management include tunneled pleural FIGUnE I 5. Large left*ided pneumothorax(>2 cm atthe hilum). catheters, pleurodesis, or both after careful discussion with a multidisciplinary team. location and the presence of a lung point confirming the edge of a pneumothorax. Pneumothorax Management Evaluation Management of pneumothorax is driven by clinical symptoms. Pneumothorax is defined as air in the pleural space, which can A tension pneumothorax (large and hemodynamically signifi occur spontaneously or as a result of trauma or a procedural cant) should be managed by emergent needle thoracostomy complication. A primary spontaneous pneumothorax (PSP) followed by thoracostomy tube placement and hospitalization. occurs in someone without known underlying lung disease. A Observation alone has been shown to be safe for small pneu- secondary spontaneous pneumothorax (SSP) occurs in someone mothoraces in patients with minimal symptoms (Table 29). with a known underlying lung disease such as COPD. Risk for recurence for PSP is 23% to 50'/u ov€r th€ course of 1 to 5 years and greater than 50% over the course of1 to 3 years in those with TABLE 29. Management of Pneumothorax SSP Risk factors for pneumothorax are listed in Table 28. Size" and Clinical Symptoms Management Symptoms include sudden onset of dyspnea and sharp <2 cm on chest radiograph, Needle aspiration or admit pleuritic chest pain. The symptoms are typically more severe minimal symptoms to hospital for observation with SSP because patients have less respiratory reserve. and supplemental oxygen (PSP may be managed as an Physical examination findings can be subtle but usually reveal outpatient if good access to reduced lung expansion, hyperresonance to percussion, and medical care) diminished breath sounds on the side of the pneumothorax. >2 cm on chest radiograph, lnsertion of a small-bore Tension pneumothorax should be suspected in patients pre- breathlessness, and chest pain (<1 4 Fr) thoracostomy tube

considered. Options for management include tunneled pleural FIGUnE I 5. Large left*ided pneumothorax(>2 cm atthe hilum). catheters, pleurodesis, or both after careful discussion with a multidisciplinary team. location and the presence of a lung point confirming the edge of a pneumothorax. Pneumothorax Management Evaluation Management of pneumothorax is driven by clinical symptoms. Pneumothorax is defined as air in the pleural space, which can A tension pneumothorax (large and hemodynamically signifi occur spontaneously or as a result of trauma or a procedural cant) should be managed by emergent needle thoracostomy complication. A primary spontaneous pneumothorax (PSP) followed by thoracostomy tube placement and hospitalization. occurs in someone without known underlying lung disease. A Observation alone has been shown to be safe for small pneu- secondary spontaneous pneumothorax (SSP) occurs in someone mothoraces in patients with minimal symptoms (Table 29). with a known underlying lung disease such as COPD. Risk for recurence for PSP is 23% to 50'/u ov€r th€ course of 1 to 5 years and greater than 50% over the course of1 to 3 years in those with TABLE 29. Management of Pneumothorax SSP Risk factors for pneumothorax are listed in Table 28. Size" and Clinical Symptoms Management Symptoms include sudden onset of dyspnea and sharp <2 cm on chest radiograph, Needle aspiration or admit pleuritic chest pain. The symptoms are typically more severe minimal symptoms to hospital for observation with SSP because patients have less respiratory reserve. and supplemental oxygen (PSP may be managed as an Physical examination findings can be subtle but usually reveal outpatient if good access to reduced lung expansion, hyperresonance to percussion, and medical care) diminished breath sounds on the side of the pneumothorax. >2 cm on chest radiograph, lnsertion of a small-bore Tension pneumothorax should be suspected in patients pre- breathlessness, and chest pain (<1 4 Fr) thoracostomy tube senting with signifi cant cardiorespiratory distress (worsening with connection to a high- volume low-pressure suction dyspnea, hypotension, absent breath sounds on one side, tra- system cheal deviation, and distended neck veins). Cardiovascular compromise Emergent needle A chest radiograph is an appropriate initial test and can (hypotension, increasing decompression followed by confirm the diagnosis and determine the size of the pneumo- breath lessness) regard less thoracostomy tu be insertion thorax. If the lung margin is greater than 2 cm away from the of size Note: lf persistent air leak chest wall at the level of the hilum, it is considered a large (>48 hours), refer to an pneumothorax (Figure 15). Cl is the most sensitive imaging interventional pulmonologist or thoracic modality for small pneumothoraces and is particularly useful surgeon in patients with bullous emphysema. Ultrasound can also be PSP = primary spontaneous pneumothorax. helpful in the diagnosis of pneumothorax, with the presence uMeasured between lung and chest wall at the level of the hilum. of Iung sliding indicating no pneumothorax at that specific

senting with signifi cant cardiorespiratory distress (worsening with connection to a high- volume low-pressure suction dyspnea, hypotension, absent breath sounds on one side, tra- system cheal deviation, and distended neck veins). Cardiovascular compromise Emergent needle A chest radiograph is an appropriate initial test and can (hypotension, increasing decompression followed by confirm the diagnosis and determine the size of the pneumo- breath lessness) regard less thoracostomy tu be insertion thorax. If the lung margin is greater than 2 cm away from the of size Note: lf persistent air leak chest wall at the level of the hilum, it is considered a large (>48 hours), refer to an pneumothorax (Figure 15). Cl is the most sensitive imaging interventional pulmonologist or thoracic modality for small pneumothoraces and is particularly useful surgeon in patients with bullous emphysema. Ultrasound can also be PSP = primary spontaneous pneumothorax. helpful in the diagnosis of pneumothorax, with the presence uMeasured between lung and chest wall at the level of the hilum. of Iung sliding indicating no pneumothorax at that specific 40

Pulmonary Vascular Disease Recurrence prevention is recommended after the second edema, chest pain, palpitations, and in some cases syncope. As episode of pneumothorax on the ipsilateral side in PSP pulmonary vascular remodeling in PH progresses, pulmonary and after the first occurrence in SSP. Patients should be artery (PA) pressures rise. Right ventricular (RV) remodeling encouraged to stop smoking; the lifetime incidence rates for and compensation initially occur to maintain normal cardiac PSP are much higher in men who are lifelong heavy smokers output. In the face of disease progression, the compensatory than in menwho have neversmoked (12'l" vs 0.1%). Intervention mechanisms of the RV eventually fail. At this point, patients to prevent recurrence includes both chemical and mechanical begin to experience the classic symptoms of RV failure associ- pleurodesis. Air travel should be avoided until complete reso ated with PH. lution of the pneumothorax, and scuba diving is not recom Findings on physical examination frequently include an mended unless definitive therapy such as surgical pleurectomy accentuated or persistently split second heart sound. A tri- has been applied. cuspid regurgitant murmur may be detected, and jugular venous distention, an RV heave, hepatomegaly, ascites, and peripheral edema occur in the face of progressive RV failure. Pulmonary findings reflect underlying lung disease when Pulmonary Vascular present. Disease If PH is suspected, transthoracic echocardiography should be pursued (Figure 16). Echocardiography provides an estima- Pulmonary Hypertension tion ofPA systolic pressure and assessment ofboth right and Pulmonary hypertension (PH) has been defined as a resting left heart size and function (Figure 17). Ifthe echocardiogram mean pulmonary artery pressure (mPAP) of 25 mm Hg or suggests PH, the patient should be referred for further evalua greater, measured by right heart catheterization. A normal tion, including right heart catheterization. Echocardiography resting mPAP is around 14 mm Hg and is rarely above 20 mm should not be used to confirm a PH diagnosis because the PA Hg. In 2018, based on growing evidence that patients with pressure measurement is often inaccurate when measured mPAP above 20 mm Hg are at increased risk for early mortal- using echocardiography. ity, the Sixth World Symposium on Pulmonary Hypertension Right heart catheterization is essential to conflrm a diag revised the definition of PH to inciude all patients with a rest nosis of PH, as it provides accurate hemodynamic data and ing mPAP of 20 mm Hg or greater. helps distinguish the primary cause of elevated PA pressure. PH can occur for a varie$/ ofreasons. The current classi An evaluation for suspected PH should also include pul- fication system separates PH into five groups on the basis of monary function tests, high resolution CT (if interstitial lung similarities in mechanisms, hemodynamics, clinical presenta disease is a consideration), and tests for sleep related breath- tion, and approach to treatment (Table 30). ing disorders; to detect the etiologr of some forms of PH, serologic tests for underlying connective tissue disease, hepa Evaluation of Suspected titis, and HIV are advised. All patients with suspected PH Pulmonary Hypertension should receive a ventilation/perfusion (V/Q) scan to evaluate Symptoms of PH are nonspecific; thus, the average time from for chronic thromboembolic PH (CTEPH). V/Q offers improved

Recurrence prevention is recommended after the second edema, chest pain, palpitations, and in some cases syncope. As episode of pneumothorax on the ipsilateral side in PSP pulmonary vascular remodeling in PH progresses, pulmonary and after the first occurrence in SSP. Patients should be artery (PA) pressures rise. Right ventricular (RV) remodeling encouraged to stop smoking; the lifetime incidence rates for and compensation initially occur to maintain normal cardiac PSP are much higher in men who are lifelong heavy smokers output. In the face of disease progression, the compensatory than in menwho have neversmoked (12'l" vs 0.1%). Intervention mechanisms of the RV eventually fail. At this point, patients to prevent recurrence includes both chemical and mechanical begin to experience the classic symptoms of RV failure associ- pleurodesis. Air travel should be avoided until complete reso ated with PH. lution of the pneumothorax, and scuba diving is not recom Findings on physical examination frequently include an mended unless definitive therapy such as surgical pleurectomy accentuated or persistently split second heart sound. A tri- has been applied. cuspid regurgitant murmur may be detected, and jugular venous distention, an RV heave, hepatomegaly, ascites, and peripheral edema occur in the face of progressive RV failure. Pulmonary findings reflect underlying lung disease when Pulmonary Vascular present. Disease If PH is suspected, transthoracic echocardiography should be pursued (Figure 16). Echocardiography provides an estima- Pulmonary Hypertension tion ofPA systolic pressure and assessment ofboth right and Pulmonary hypertension (PH) has been defined as a resting left heart size and function (Figure 17). Ifthe echocardiogram mean pulmonary artery pressure (mPAP) of 25 mm Hg or suggests PH, the patient should be referred for further evalua greater, measured by right heart catheterization. A normal tion, including right heart catheterization. Echocardiography resting mPAP is around 14 mm Hg and is rarely above 20 mm should not be used to confirm a PH diagnosis because the PA Hg. In 2018, based on growing evidence that patients with pressure measurement is often inaccurate when measured mPAP above 20 mm Hg are at increased risk for early mortal- using echocardiography. ity, the Sixth World Symposium on Pulmonary Hypertension Right heart catheterization is essential to conflrm a diag revised the definition of PH to inciude all patients with a rest nosis of PH, as it provides accurate hemodynamic data and ing mPAP of 20 mm Hg or greater. helps distinguish the primary cause of elevated PA pressure. PH can occur for a varie$/ ofreasons. The current classi An evaluation for suspected PH should also include pul- fication system separates PH into five groups on the basis of monary function tests, high resolution CT (if interstitial lung similarities in mechanisms, hemodynamics, clinical presenta disease is a consideration), and tests for sleep related breath- tion, and approach to treatment (Table 30). ing disorders; to detect the etiologr of some forms of PH, serologic tests for underlying connective tissue disease, hepa Evaluation of Suspected titis, and HIV are advised. All patients with suspected PH Pulmonary Hypertension should receive a ventilation/perfusion (V/Q) scan to evaluate Symptoms of PH are nonspecific; thus, the average time from for chronic thromboembolic PH (CTEPH). V/Q offers improved symptom onset to diagnosis can exceed 2 years. Symptoms sensitivity over CT angiography (CTA) when evaluating for include exertional dyspnea or lightheadedness, lower extremity CTEPH. Assessment of functional status with the 6 minute walk test provides prognostic information and a baseline for TABTE 30. Classification of Pulmonary Hypertension assessment of therapeutic response. 1 Pulmonary arterial hypertension (includes idiopathic XEY POII{IS and heritable disease, disease associated with use of drugs or toxins, connective tissue diseases, HIV . If pulmonary hypertension is suspected, transthoracic infection, congenital heart disease, schistosomiasis, and echocardiography should be performed to estimate pul- portal hypertension; also includes vasoresponsive PAH and PAH attributable to pulmonary venoocclusive monary artery systolic pressure and assessment of right disease or pulmonary capillary hemangiomatosis) and left heart size and function. 2 Pulmonary hypertension due to left-sided heart disease . Right heart catheterization is essential to confirm a HVC

symptom onset to diagnosis can exceed 2 years. Symptoms sensitivity over CT angiography (CTA) when evaluating for include exertional dyspnea or lightheadedness, lower extremity CTEPH. Assessment of functional status with the 6 minute walk test provides prognostic information and a baseline for TABTE 30. Classification of Pulmonary Hypertension assessment of therapeutic response. 1 Pulmonary arterial hypertension (includes idiopathic XEY POII{IS and heritable disease, disease associated with use of drugs or toxins, connective tissue diseases, HIV . If pulmonary hypertension is suspected, transthoracic infection, congenital heart disease, schistosomiasis, and echocardiography should be performed to estimate pul- portal hypertension; also includes vasoresponsive PAH and PAH attributable to pulmonary venoocclusive monary artery systolic pressure and assessment of right disease or pulmonary capillary hemangiomatosis) and left heart size and function. 2 Pulmonary hypertension due to left-sided heart disease . Right heart catheterization is essential to confirm a HVC 3 Pulmonary hypertension due to lung diseases or hypoxia diagnosis of pulmonary hypertension, as it provides 4 Chronic thromboembolic pulmonary hypertension and accurate hemodynamic data and helps distinguish the other pulmonary artery obstructions primary cause of elevated pulmonary artery pressure. tr Pulmonary hypertension with unclear or multifactorial causes Pulmonary Arterial PAH = pulmonary arterial hypertension. Hypertension (Group 1) lnformation from Simonneau G, Ny'ontani D, Celermajer DS, et aL. Haemodynamic definitions and updated clinical classification of pulmonary hypeftension. Eur Pulmonary arterial hypertension (PAH) is defined by ele Respir J.2019;53. IPMID: 30545968] doi:T 0.1 1 83/1 3993003.01 91 3'20T 8 vated pulmonary vascular resistance in the small pulmonary

3 Pulmonary hypertension due to lung diseases or hypoxia diagnosis of pulmonary hypertension, as it provides 4 Chronic thromboembolic pulmonary hypertension and accurate hemodynamic data and helps distinguish the other pulmonary artery obstructions primary cause of elevated pulmonary artery pressure. tr Pulmonary hypertension with unclear or multifactorial causes Pulmonary Arterial PAH = pulmonary arterial hypertension. Hypertension (Group 1) lnformation from Simonneau G, Ny'ontani D, Celermajer DS, et aL. Haemodynamic definitions and updated clinical classification of pulmonary hypeftension. Eur Pulmonary arterial hypertension (PAH) is defined by ele Respir J.2019;53. IPMID: 30545968] doi:T 0.1 1 83/1 3993003.01 91 3'20T 8 vated pulmonary vascular resistance in the small pulmonary 41