Dilated cardiomyopathy with conduction disease and arrhythmia

NK Lakdawala, MM Givertz - Circulation, 2010 - Am Heart Assoc
NK Lakdawala, MM Givertz
Circulation, 2010Am Heart Assoc
527 cardiac sarcoidosis (CS). 3 Cardiac disease is one of the more common causes of
death in sarcoidosis and may be preventable with the use of appropriate therapies.
Approximately 5% of patients will have cardiac-predominant disease and present without
characteristic pulmonary, dermatologic, or ocular features. CS most commonly affects the
myocardium but may also affect the pericardium and endocardium. Myocardial infiltration
may be associated with VT, aneurysm formation, or global systolic dysfunction, but the most …
527 cardiac sarcoidosis (CS). 3 Cardiac disease is one of the more common causes of death in sarcoidosis and may be preventable with the use of appropriate therapies. Approximately 5% of patients will have cardiac-predominant disease and present without characteristic pulmonary, dermatologic, or ocular features. CS most commonly affects the myocardium but may also affect the pericardium and endocardium. Myocardial infiltration may be associated with VT, aneurysm formation, or global systolic dysfunction, but the most common clinical feature is conduction disease. 3 The noncaseating granuloma is the characteristic pathological lesion of sarcoidosis (Figure, A); however, granulomas are not specific to sarcoidosis and can be the result of various infectious and noninfectious causes. Diagnostic criteria for CS have been proposed4 that rely on pathological demonstration of cardiac granulomas or noninvasive evidence of cardiac involvement in a patient with pathologically proven extracardiac sarcoidosis (online-only Data Supplement Table I). Owing to patchy involvement, often of the LV, a right ventricular (RV) endomyocardial biopsy provides diagnostic evidence of CS in only 25% to 50% of autopsy-confirmed cases. 3 Contemporary imaging with fluorodeoxyglucose (18F-FDG) positron emission tomography or cardiac magnetic resonance can identify inflammation (Figure, B and C), has better diagnostic accuracy than older techniques5 (thallium 201 or gallium 67 scintigraphy), provides complementary information, 6 and can predict adverse events. 7
Am Heart Assoc