Exercise-related sudden cardiac death of an American football player with arrhythmogenic right ventricular dysplasia/cardiomyopathy AND sarcoidosis

Abstract

This case emphasizes the value of cardiac MRI and genetic testing in the early phase of ARVD/C. It also emphasizes the increased risk of SCD for patients with ARVD/C participating in competitive sports, even with immediate cardiopulmonary resuscitation.

Keywords: ARVC; ARVD/C; American football; arrhythmogenic right ventricular dysplasia/cardiomyopathy; athlete; exercise; magnetic resonance imaging; sarcoidosis; sudden cardiac death; syncope.

Figure 1

Illustrate parts of the diagnostic puzzle that finally led to the diagnosis ARVD/C and extracardiac sarcoidosis in a 28‐year‐old American football player with two exercise‐related syncopes. A, Unremarkable ECG (paper speed 50 mm/s): typical ARVD/C features as epsilon wave, T‐wave inversion or delayed terminal activation duration cannot be observed. B, Unremarkable signal‐averaged electrocardiogram (SAECG). C and D, Cardiac magnetic resonance imaging: cine images in 4‐chamber (C) and short‐axis views (D) in end‐systolic phase demonstrating a severely dilated right ventricle, ratio of RV end‐diastolic volume to BSA = 130 mL/m² with bulging (arrows), regional dyssynchrony and moderately decreased right ventricular systolic function, RV ejection fraction = 42%. Cardiac MRI fulfilled one major criterion for ARVD/C

Figure 2

Illustrate parts of the diagnostic puzzle that finally led to the diagnosis ARVD/C and extracardiac sarcoidosis in a 28‐year‐old American football player with two exercise‐related syncopes. A, Macroscopic cross‐section of the right ventricle shows fat deposits (arrows) indicative for ARVD/C. B, Microscopic pattern of the right ventricle (stained with hematoxylin‐eosin) with typical fibrofatty replacement (arrows) of the myocardium and estimated <50% residual myocytes. C, Microscopic pattern of the lung (stained with hematoxylin‐eosin) with non‐necrotizing (non‐caseating) granulomas (arrow), typical for sarcoidosis. D, Microscopic pattern of the liver (stained with hematoxylin‐eosin) with non‐necrotizing (non‐caseating) granulomas (arrow), typical for sarcoidosis