PATHOLOGY IMAGE OF THE MONTH: Sudden Cardiac Death Caused by an Uncommon Disease

A 57-year-old female, found dead lying supine in bed, was transferred to the autopsy service for an unrestricted autopsy to be performed under the authorization by the coroner. Medical history was unknown. At the time of autopsy, an implantable cardioverter-defibrillator (ICD) was identified in the subcutaneous tissues of the left subclavicular chest, with distal leads terminating in a small amount of fibrous tissue within the right auricular appendage and along the medial wall of the right ventricle. The heart was enlarged at 430gm (312 ±78) and cross sections were notable for left ventricular hypertrophy at 1.9cm (1.0-1.5cm) and for dilatation of the right ventricular chamber on initial apical cross section. All cross sections, from cardiac apex to subvalvular base, showed broad patches of white-yellow myocardial discoloration, without obvious hemorrhage, along the free wall of the left ventricle, the free wall of the right ventricle, and within the anterior interventricular septum (Figure 1). Additional notable findings at autopsy included a vena caval filter devoid of thromboembolic material, a patent foramen ovale (0.7cm) and microscopic plexogenic arteriopathy, low grade, consistent with pulmonary hypertension within the intrapulmonary vasculature. Histology from the discolored patches of myocardium is seen in Figure 2. Special stains for microorgansims (periodic acid-Schiff, Gomori methanamine silver, and Fite) were all negative.