Case 1/2016 – 56-Year-Old Male with Atrial Septal Defect, Pulmonary Arterial Hypertension, Hospitalized Due to Eisenmenger Syndrome

Postmortem examination

The heart was enlarged and globoid, weighing 832 g. On gross examination, the RV was prominent. The opening of the cavities evidenced eccentric hypertrophy of the four chambers, mainly the right ones, which showed marked dilation. A large ASD was identified in the fossa ovalis (ostium secundum type), measuring 3.5 x 3.0 cm (). The ventricular septum was intact, no other heart malformation being identified. The microscopic examination revealed hypertrophy of the cardiomyocytes and myocardial interstitial fibrosis in both ventricles, with no evidence of ischemic lesions (infarction). The pulmonary trunk was dilated (diameter of 4.0 cm) and showed complicated atherosclerosis, with calcified areas. The right pulmonary artery showed calcified atherosclerosis, organized partial thrombosis, and markedly dilation of its parenchymatous branches. The process was similar in the left lung, but the thrombosis was more extensive, affecting numerous arterial branches, in an occlusive way, including the pulmonary hilum (). The microscopic examination of the lungs confirmed the extensive organizing arterial thrombosis detected on gross examination, in addition to revealing: organized and rechanneled arteriolar thrombosis (); recent focal thrombosis of a small artery and small hemorrhagic infarction in the lower lobe of the right lung (); areas of alveolar hemorrhage; anthracosis; microscopic evidence of chronic bronchitis; and diffuse pulmonary emphysema. In addition, a small in situ adenocarcinoma was found in the right lung, measuring 9 mm. Moderate aorta atherosclerosis was identified, with calcified plaques. Neither pneumothorax nor bronchopneumonia were evidenced.

[…]