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. Myocardial infiltration may be associated with VT, aneurysm formation, or global systolic dysfunction, but the most common clinical feature is conduction disease. CS most commonly affects the myocardium but may also affect the pericardium and endocardium. Approximately 5% of patients will have cardiac-predominant disease and present without characteristic pulmonary, dermatologic, or ocular features. 3 Cardiac disease is one of the more common causes of death in sarcoidosis and may be preventable with the use of appropriate therapies.
SVT WITH IVCD SERIES
Autopsy series suggest that up to 50% of patients with sarcoidosis have some degree of cardiac involvement, but only a fraction of these had previously recognized cardiac sarcoidosis (CS). 2 After an early stage of granulomatous inflammation, sarcoidosis may resolve or progress to end-organ fibrosis. Sarcoidosis is an inflammatory disease of unknown origin characterized by noncaseating granuloma formation in multiple organ systems.
Emphasis is placed on diagnoses that are relatively common or for which the clinical management would be impacted significantly by recognition of the underlying cause. In this Clinician Update, we focus on the diagnostic approach to patients with DCM+E. Clinical features that are suggestive of DCM+E include supraventricular arrhythmias or conduction disease that precedes cardiomyopathy, multiple VT morphologies, and features suggestive of ischemic heart disease (Q waves, regional wall-motion abnormalities, perfusion defects, ventricular aneurysm) in the absence of epicardial stenoses. Cardiac presentation associated with these conditions is distinct from more common causes of DCM such as ischemic heart disease, viral myocarditis, valvular dysfunction, pregnancy, or substance abuse. Diverse causes can result in DCM+E and fall into general categories of inflammatory, infectious, hereditary, and infiltrative processes. This patient presents with dilated cardiomyopathy (DCM) with electric instability (DCM+E), which we define as conduction disease and arrhythmia out of proportion to the severity of LV systolic dysfunction. What are the diagnostic considerations for this patient, and what further evaluations are indicated? Multiple episodes of nonsustained ventricular tachycardia (VT) are documented on continuous ECG monitoring. Nuclear imaging is notable for heterogeneous myocardial uptake of technetium Tc99m sestamibi, and coronary angiography reveals widely patent epicardial vessels. Now, she has echocardiographic evidence of moderate to severe left ventricular (LV) systolic dysfunction with regional wall-motion abnormalities.
One year earlier, a permanent pacemaker was placed after she complained of fatigue and was found to have high-grade atrioventricular block. Case presentation: A 48-year-old woman presents with exertional dyspnea and recurrent syncope.
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