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Cardiovascular Medicrne Restrictive Ca rd iomyopathy Diagnosis Presenting symptoms include dyspnea, peripheral edema, and exercise intolerance. Hepatomegaly and ascites may be late find ings. Jugular veins may engorge with inspiration (Kussmaul sign). Primary restrictive cardiomyopathy must be differentiated from other conditions with restrictive physiolog,: Restriction and normal wall size: . radiation induced fibrosis . eosinophilic diseases o hemochromatosis Restriction and increased wall thickness: o amyloidosis Testing Echocardiogram shows biatrial enlargement and severe diastolic dysfunction in the setting of normal ventricular size, wall thickness, and systolic function. PH is commonly present, as are tricuspid and mitral valve regurgitation. 5?UDY TABLE: Clues to Underlying Systemic Diseases Causing Restrictive Physiology Disease Clues Amyloidosis Neuropathy, proteinuria, hepatomegaly, periorbital ecchymosis, bruising, and low-voltage ECG despite increased LV hypertrophy. CMR imaging will support the diagnosis but not differentiate AL amyloidosis from hereditary transthyretin amyloidosis. A positive technetium pyrophosphate scan is diagnostic of hereditary transthyretin amyloidosis. ln patients with AL amyloidosis, diagnosis can often be confirmed with abdominalfat pad aspiration. Sarcoidosis Bilateral hilar lymphadenopathy; possible pulmonary reticular opacities; and skin, joint, or eye lesions. Cardiac involvement is suggested by the presence of arrhythmias, conduction blocks, or HF. Diagnosis is supported by CMR imaging with gadolinium. Hemochromatosis Abnormal aminotransferase levels, OA, diabetes, erectile dys{unction, and HF; elevated serum ferritin and transferrin saturation level. Restrictive cardiomyopathy must be differentiated from constrictive pericarditis (see Cardiac Effusion and Tamponade and Constrictive Pericarditis sections).

5?UDY TABLE: Clues to Underlying Systemic Diseases Causing Restrictive Physiology Disease Clues Amyloidosis Neuropathy, proteinuria, hepatomegaly, periorbital ecchymosis, bruising, and low-voltage ECG despite increased LV hypertrophy. CMR imaging will support the diagnosis but not differentiate AL amyloidosis from hereditary transthyretin amyloidosis. A positive technetium pyrophosphate scan is diagnostic of hereditary transthyretin amyloidosis. ln patients with AL amyloidosis, diagnosis can often be confirmed with abdominalfat pad aspiration. Sarcoidosis Bilateral hilar lymphadenopathy; possible pulmonary reticular opacities; and skin, joint, or eye lesions. Cardiac involvement is suggested by the presence of arrhythmias, conduction blocks, or HF. Diagnosis is supported by CMR imaging with gadolinium. Hemochromatosis Abnormal aminotransferase levels, OA, diabetes, erectile dys{unction, and HF; elevated serum ferritin and transferrin saturation level. Restrictive cardiomyopathy must be differentiated from constrictive pericarditis (see Cardiac Effusion and Tamponade and Constrictive Pericarditis sections). Treatment Treat the underlying disorder when present and any underlying disease or condition that affects diastolic function (AF, hyper- tension, diabetes, ischemic heart disease). Loop diuretics are used to treat dyspnea and peripheral edema. B-Blockers or nondi- hydropyridine calcium channel antagonists may enhance diastolic function. TE TYOURSELF A 63-year-old man develops dyspnea and fatigue. Physical examination shows IVD, a prominent jugular o wave, a prominent S.,, and a grade 2/6 holosystolic murmur at the left sternal border. The lungs are clear. Other findings include an enlarged, tender liver; l petechiae over the feet; and periorbital ecchymoses. ANSWER: The diagnosis is AL amyloid cardiomyopathy, indicated by the noncardiac symptoms and signs. 1

TE TYOURSELF A 63-year-old man develops dyspnea and fatigue. Physical examination shows IVD, a prominent jugular o wave, a prominent S.,, and a grade 2/6 holosystolic murmur at the left sternal border. The lungs are clear. Other findings include an enlarged, tender liver; l petechiae over the feet; and periorbital ecchymoses. ANSWER: The diagnosis is AL amyloid cardiomyopathy, indicated by the noncardiac symptoms and signs. 1 1 12 I

Cardiovascular Medicine ul UI s: l .i vt Cadiac Amyloidosis: The ECG shows low voltage, the most common ECG abnormality associated with cardiac amyloidosis. Palpitations and Syncope Testing In a patient with palpitations and syncope, the key diagnostic test is an ECG recorded during the clinical event. Obtain an echocardiogram in patients with suspected structural heart disease. See General Internal Medicine chapter for major causes of syncope.

Cadiac Amyloidosis: The ECG shows low voltage, the most common ECG abnormality associated with cardiac amyloidosis. Palpitations and Syncope Testing In a patient with palpitations and syncope, the key diagnostic test is an ECG recorded during the clinical event. Obtain an echocardiogram in patients with suspected structural heart disease. See General Internal Medicine chapter for major causes of syncope. 5?llllY 'i"*.*ii.: Diagnostic Studies for Suspected Arrhythmias Diagnostic Test Utility Advantages Limitations Resting ECG lnitial diagnostic test in all Diagnostic if recorded during Most arrhythmias are intermittent patients the arrhythmia and not recorded on a resting ECG ! Ambulatory (24-hour) lndicated for frequent (at least Records every heartbeat Not helpful if arrhythmia is I ECG daily) arrhythmias during a 24-hour period infrequent Exercise ECG lndicated for arrhythmias Allows diagnosis o{ exercise- Physician supervision required provoked by exercise related arrhythmias Event monitor lndicated for infrequent Small recorder is held to the Limited to symptomatic arrhythmias arrhythmias >1-2 min in duration chest when symptoms are that persist long enough for patient present to activate the device; not a viable ! choice for patients with syncope

5?llllY 'i"*.*ii.: Diagnostic Studies for Suspected Arrhythmias Diagnostic Test Utility Advantages Limitations Resting ECG lnitial diagnostic test in all Diagnostic if recorded during Most arrhythmias are intermittent patients the arrhythmia and not recorded on a resting ECG ! Ambulatory (24-hour) lndicated for frequent (at least Records every heartbeat Not helpful if arrhythmia is I ECG daily) arrhythmias during a 24-hour period infrequent Exercise ECG lndicated for arrhythmias Allows diagnosis o{ exercise- Physician supervision required provoked by exercise related arrhythmias Event monitor lndicated for infrequent Small recorder is held to the Limited to symptomatic arrhythmias arrhythmias >1-2 min in duration chest when symptoms are that persist long enough for patient present to activate the device; not a viable ! choice for patients with syncope I Loop recorder lndicated for infrequent Saves previous 30 s to 2 min ECG leads limit patient activities symptomatic brief arrhythmias ECG signal when patient I activates the recorder; can be i activated following syncopal t I event to capture arrhythmia I lmplanted recorder lndicated for very infrequent Long-term continuous ECG lnvasive procedure with some risk I arrhythmias monitoring I

I Loop recorder lndicated for infrequent Saves previous 30 s to 2 min ECG leads limit patient activities symptomatic brief arrhythmias ECG signal when patient I activates the recorder; can be i activated following syncopal t I event to capture arrhythmia I lmplanted recorder lndicated for very infrequent Long-term continuous ECG lnvasive procedure with some risk I arrhythmias monitoring I t Electrophysiology study Can be used for inducing, The origin and mechanism of lnvasive procedure with some risk identifying, and clarifying an arrhythmia can be precisely I mechanism of arrhythmia as well defined l> as fortreatment (e.9., catheter I L ablation); studies are not used i for initial diagnosis L 13 r