Cardiomyopathy (CMP) from sarcoidosis occurs in 2-7% of patients suffering from sarcoidosis, they have a five-year mortality of 50-60%, and usually is from congestive heart failure (CHF) and sudden death. We present a case of sarcoid CMP. A trial of corticosteroids (CS) dramatically improved symptoms and cardiac function.CASE REPORT: 55 y/o black male with steroid related hyperglycemia and hypertension presented with a 3-year history of chronic cough and a 2-week history of worsening dyspnea. Dyspnea was initially with exertion, later at rest and during sleep (PND). He was diagnosed with CHF and bifascicular block on admission (see figure 1
ECG). Recent evaluation for chronic cough revealed mild restrictive lung disease (TLC of 62% predicted & DLCO of 38%). Thoracic CT scan (see figure 2
CT on admission) showed ground glass opacities in the upper lobes with bronchovascular beading. Nonischemic CMP was confirmed by cardiac catheterization with a left ventricular ejection fraction of 25%. Significant laboratory findings included an elevated ACE of 57 U/L (7-46) and CRP of 2.59 mg/dl (0.02-0.8).An endomyocardial biopsy performed was non-diagnostic (see figure 3
Biopsy of the heart). Bronchoscopic transbronchial biopsies revealed non-necrotizing granulomatous inflammation raising the possibility of cardiac sarcoidosis (se figure 4
Biopsy of the lung). The patient was started on prednisone, an ACE inhibitor and a beta-blocker. There was a dramatic improvement in LV function and in symptoms. Repeat CT scan revealed resolution of the opacities of the upper lobes and the interstitial infiltrates (see figure 5
CT of follow-up 5 months later). Prednisone was discontinued after three months. Five months later, he had a normal left ventricular function of 50-60% on echocardiogram and a normal spirometry with a DLCO that improved to 68%.DISCUSSION: Cardiomyopathy from sarcoidosis is rare. Our patient presented with CHF with an EF of 25%, bifascicular block, with non-obstructive coronaries on angiogram. Because of preceding history of chronic cough, it prompted further diagnostic evaluation. Corticosteroid is an effective treatment for sarcoid cardiomyopathy. Among steroid treated patients the 10-year survival rate is 90% if the EF is >50% versus 30% if the EF is < 50%.
We present a case of cardiomyopathy with a preceding history of chronic cough. A clinical diagnosis of sarcoid cardiomyopathy was made based on transbronchial lung biopsy. The patient responded very well to a short course of steroid.
I.R. de Chazal, None.