Described herein are clinical and morphologic findings in 2 patients who underwent heart transplantation because of severe heart failure resulting from cardiac sarcoidosis. Although the explanted hearts in each patient had characteristic gross changes of cardiac sarcoidosis, one patient who had been treated with prednisone, had no residual sarcoid granulomas in the myocardium, whereas the other patient, in whom diagnosis was not made until heart transplantation, had innumerable sarcoid granulomas in her heart. This report suggests that prednisone can eliminate sarcoid granulomas in the heart but that their replacement is by dense fibrous tissue, something also likely the result of the granulomas themselves, creating a situation where the treated (prednisone) and the non-treated sarcoid heart may appear similar by gross examination.
Although it is a systemic disease, sarcoidosis uncommonly affects the heart and when it does the non-cardiac organs are usually minimally affected and they function normally. On occasion, cardiac sarcoidosis may lead to such severe heart failure that heart transplantation (HT) is the only reasonable therapy. In such situations, diagnosis of cardiac sarcoidosis is usually not made until HT, the usual pre-HT diagnosis being “non-ischemic cardiomyopathy”. Recently, 2 patients underwent HT at Baylor University Medical Center (BUMC) in the same month and examination of the explanted heart in one disclosed innumerable myocardial sarcoid granulomas (not diagnosed clinically) and, in the other patient, despite some similar gross findings in the heart, no myocardial granulomas were seen (diagnosed clinically by an earlier biopsy). This report describes findings in these 2 patients to point out the huge spectrum of myocardial changes in patients with cardiac sarcoidosis, and the potential effect of long term prednisone therapy in this condition.
Patients Studied
Pertinent findings in each of the 2 patients are detailed in Table 1 . Both patients were in their 50s; both were white; both had severe (4+/4+) heart failure; both had evidence of cardiac dysfunction for approximately 4 years; both had periodic runs of non-sustained ventricular tachycardia; both had some type of heart block (complete in case #1, and right bundle branch block in case #2); neither had dysfunction of a non-cardiac body organ, and neither patient had narrowing of the epicardial coronary arteries. Diagnosis of sarcoidosis before HT was made in patient #1 by biopsy of a mediastinal lymph node 21 months before HT although 2 years earlier a cardiac biopsy had shown “granulomas”. Patient #1, who had no granulomas in his explanted heart, was placed on prednisone after the granulomas were seen in the lymph node and he received this drug for 21 months, at very high doses for 10 months before dose-tapering began. Patient #2, whose sarcoidosis was not diagnosed until HT, never received corticosteroid therapy before HT and her explanted heart contained innumerable granulomas typical of sarcoidosis. Photographs of the heart in each patient are shown in Figures 1 to 4 .
| Variable | Case | |
|---|---|---|
| #1 | #2 | |
| Gender | Man | Woman |
| Age (years) | ||
| At heart transplantation | 53 | 57 |
| Onset of symptoms | 49 | 53 |
| Electrophysiology | ||
| Heart block | Complete | RBBB |
| Ventricular tachycardia | + | + |
| Pacemaker | + | 0 |
| Intracardiac defibrillator | + | + |
| Systemic hypertension | + | 0 |
| Body mass index (Kg/m 2 ) | 31.8 | 29.9 |
| Hemodynamic data | ||
| Lowest left ventricular EF (%) | 20 | 15 |
| Cardiac index (L/min/m 2 ) | 2.01 | 1.36 |
| Pressures (mm Hg) | ||
| Blood pressure, indirect (s/d) | 100/85 | 85/40 |
| Right atrial mean | 6 | 2 |
| Right ventricle (s/d) | 26/10 | 48/6 |
| Pulmonary artery (s/d) | 26/14 | 44/11 |
| Pulmonary artery wedge mean | 11 | 14 |
| Prednisone therapy (months) | + (21) | 0 |
| Heart weight (g) | 535 | 380 |
| Cardiac granuloma (0-4+) | 0 | 4+ |
| Left ventricular cavity (cm) | 7.5 | 5.5 |
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