Cardiomyopathy
Comparison of Necropsy Findings in Patients With Sarcoidosis Dying Suddenly from Cardiac Sarcoidosis Versus Dying Suddenly from Other Causes

https://doi.org/10.1016/j.amjcard.2009.03.068Get rights and content

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.

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Methods

A retrospective search was performed from 1995 to 2008 for hearts seen in consultation at the Armed Forces Institute Pathology, Office of the Armed Forces Medical Examiner, and Office of the Chief Medical Examiner, evaluated in a uniform fashion by 1 of the coauthors (A.B.). All cases with the diagnosis of “sarcoid” in the history or diagnosis, with gross and microscopic materials available, were retained for the present study. Only cases of sudden death were included.

The gross extent of

Results

A total of 46 cases of sudden death were found with “sarcoid” in either the clinical history or cardiac findings. Of these 46 patients, 16 had a history of sarcoid, and, of these, 5 had no cardiac involvement, 6 died from cardiac sarcoid, and 5 had incidental cardiac sarcoidosis at autopsy. Of the 41 patients with cardiac sarcoidosis at autopsy, the cause of sudden death was attributed to sarcoid in 25 (group 1, Table 1) and other factors in 16 (group 2, Table 2). No differences were found in

Discussion

The present autopsy study sampled 1 subset of symptomatic cardiac sarcoidosis, namely that resulting in sudden death. Because of autopsy selection bias, especially in cases referred to the Medical Examiner's Office, only a small proportion of patients (1 in the entire study) had a history of cardiac sarcoidosis, because patients with a history of heart disease are often not subject to forensic evaluation. Therefore, the study findings are based on a selected group of patients with cardiac

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