Comparison of necropsy findings in patients with sarcoidosis dying suddenly from cardiac sarcoidosis versus dying suddenly from other causes

Abstract

The clinical diagnosis of cardiac sarcoidosis can be difficult and is largely dependent on newer imaging modalities. A retrospective search of sudden cardiac deaths was performed from a reference laboratory and statewide medical examiner system for a 12-year period. Planimetry was performed on gross photographs of transverse short-axis sections, and the phase of the lesion and the portion of myocardium extent was estimated histologically. Lesions were classified histologically as early (primarily lymphocytic), intermediate (primarily granulomatous), and late (primarily scar). A total of 41 cases were found, including 25 in which the death was ascribed to sarcoidosis of the heart (group 1) and 16 in which sudden death was due to other findings (group 2). No significant differences were found in age or activity at death, although gross scars and epicardial nodules were more frequent in group 1 (p <0.0001). In the hearts with gross scars, the ventricular septum had the largest percentage of involvement (32%) followed by the posterior wall (25%). Histologically, the intermediate phase predominated in group 1, and the late phase predominated in group 2. Approximately 50% of the cases in group 1 had involvement in the right ventricular apex and septum, suggesting a positive yield by biopsy. In conclusion, cardiac sarcoidosis causing sudden death is characterized by extensive active granulomas with a predilection for the subepicardium and ventricular septum.