SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters II
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM
INTRODUCTION: PVOD is a poorly understood syndrome characterized by progressive dyspnea and hypoxemia from PH due to extensive occlusion of the pulmonary venous circulation by fibrous tissue and thrombi.
CASE PRESENTATION: A 58 year-old female presents for evaluation of worsening dyspnea and hypoxemia. Symptoms began 3 years ago after a presumed respiratory infection. Over the next 2 years she had multiple evaluations for dyspnea—all felt secondary to pneumonia and pleurisy. Last year she was diagnosed with a right upper lobe pulmonary embolism and completed 6 months of rivaroxaban. Past history revealed incidental identification of an enlarged axillary lymph node (LN) on mammogram at age 48. LN excision showed benign lymphatic tissue with non-caseating granulomas. Present evaluation revealed the following: normal spirometry with a significantly decreased DLCO (52%); six minute walk test of 700 feet with nadir oximetry of 79%; echocardiogram with mildly dilated right ventricle with reduced function, normal left ventricular function, and estimated pulmonary systolic pressure of 69mmHg; no evidence of mismatches on V/Q study; and computed tomography revealed diffuse mosaic attenuation pattern consistent with ground glass opacities with interlobular septal thickening and pulmonary venous obstruction with thrombi. Right heart catheterization revealed normal wedge pressure and pulmonary hypertension (PH) (55/20; mean 35mmHg). In addition to oxygen, warfarin and prednisone were initiated. The latter treatment was trialed given her remote history of non-caseating granulomas on a LN biopsy—raising the question of sarcoidosis as a unifying diagnosis where granulomata are responsible for venous obstruction. We referred our patient for lung transplantation as a therapeutic option.
DISCUSSION: The diagnosis of PVOD is strongly suggested by PH with normal wedge pressures and radiographic findings of pulmonary edema. Estimated annual incidence is 1-2 cases per 10 million persons. The etiology is incompletely understood but may include hypercoagulability, genetic predisposition, prior respiratory infection, antineoplastic agents, and autoimmune disorders. Oxygen therapy is indicated in all hypoxemic PVOD patients and anticoagulation is recommended based on data from Group 1 PH patients. There is no convincing role for vasodilator therapy and a theoretical risk of precipitating life-threatening pulmonary edema exists. Immunosuppressives have been used anecdotally in patients with features of autoimmune diseases.
CONCLUSIONS: Lung transplantation is the only treatment option that may confer survival advantage to date.
Reference #1: Montani D et al. Pulmonary veno-occlusive disease. Medicine 2008;87:220
Reference #2: Montani D et al. Pulmonary veno-occlusive disease. Respir Med 2010; 104 Suppl1:S23
DISCLOSURE: The following authors have nothing to disclose: Ana Bonetti, Chirag Shah, Nataly Vasquez Encalada
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