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A7000 - Cardiac Tamponade: As a Manifestation of Pulmonary Veno-Occlusive Disease (PVOD)
Author Block: M. Rives-Sanchez, N. AlQsous, A. Quintos, A. Niroula, A. Salehbeiki, S. Sharma; Pulmonary Critical Care, Einstein Healthcare Network, Philadelphia, PA, United States.
Pulmonary veno-occlusive disease (PVOD) is one of the rare causes of pulmonary hypertension. The estimated prevalence of PVOD among patients clinically diagnosed with idiopathic PAH varies from 5-20%. Based on the incidence of primary pulmonary hypertension in the general population, annual incidence of PVOD is 0.1-0.2 case per million persons . Misdiagnosis and delayed treatment of PVOD is very common because of similarity to Idiopathic Pulmonary Hypertension (IPH). Case reports describing PVOD are found in literature, however report of cardiac tamponade as a manifestation of PVOD has not existed so far. We present a case of a 44-year-old AA female, smoker with past medical history of COPD on 4L NC, HTN, CHF, ESRD on HD, pulmonary hypertension, that presented with complains of worsening dyspnea. Initial examination remarkable for respiratory distress, tachycardia, wheezing on auscultation and LE edema. Admission workup revealed CXR with mild pulmonary edema, cardiomegaly and right lower lobe opacity; on echocardiography large pericardial effusion seen circumferentially with findings consistent with tamponade physiology. Patient underwent pericardiocentesis and 2L of bloody fluid were removed after RHC also showing equalization of pressures in cardiac chambers. Pericardial drain left in place with evidence of fluid re-accumulation. Patient with previous admissions and concern for sarcoidosis given extensive lymphadenopathy, and bilateral ground glass opacities. To further workup chronic findings, VATS was performed. Pathology examination revealed pulmonary veno occlusive disease. After establishing the diagnosis of PVOD and due to her background of ESRD patient was transferred for lung and kidney dual transplant. PVOD is occlusion or narrowing of the pulmonary veins and venules. Etiology of pulmonary veno-occlusive disease is not clear completely. However, genetic, immune mediated, infection and radiation have been proposed as trigger of this disease. Most patients experience progressive worsening and death without appropriate treatment within two years of diagnosis. Symptoms, testing and physical examination are not specific and are very similar to pulmonary hypertension and for this many patient with PVOD are misdiagnosed. Although lung biopsy is the gold standard for definitive diagnosis of PVOD, It is rarely done since PVOD is not usually thought of on initial differential diagnosis and unfortunately is usually a post mortem diagnosis. There is not any confirmed treatment for PVOD except lung transplant. We report a case with cardiac tamponade secondary to PVOD. In our experience, pericardial effusion or cardiac tamponade could be first reported manifestation of PVOD.