However, from the pathology standpoint, 3 distinct pathologic patterns might be found in those patients: plexogenic
pulmonary arteriopathy, recurrent pulmonary thromboembolic disease, and pulmonary veno-occlusive disease (PVOD).
Additional findings at autopsy included cardiomegaly at 720 gm (234-390 gm), bilateral pulmonary edema containing hemosiderin laden macrophages, congestive hepatosplenomegaly and plexogenic arteriopathy on pulmonary microscopic examination.
(11) With no evidence of thromboemboli or endocarditis in the current case along with the autopsy findings of congestive splenomegaly and plexogenic arteriopathy in the pulmonary vascular system coupled with antemortem historical evidence of recent shortness of breath, the decedent's proximate cause of death was presumably heart failure due to chronic rheumatic mitral valvulopathy.
D'Armini et al., "Plaque composition in plexogenic
and thromboembolic pulmonary hypertension: the critical role of thrombotic material in pultaceous core formation," Heart, vol.
Thrombotic lesions in primary plexogenic
(56) The hallmark histopathologic lesion is plexogenic
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.
arteriopathy associated with pulmonary vasculitis in systemic lupus erythematosus.
The microscopic features ascribed to PH can broadly be called plexogenic arteriopathy (PA).
The autopsy case depicted here of a young adult with SCD demonstrates the hallmark plexiform lesion (Grade IV) seen in plexogenic arteriopathy (PA) of PH.
In a series of 80 cases, 28% had plexogenic
arteriopathy, but the authors did not describe cholesterol granulomas.