A Rare and Unexpected Cause of Pulmonary Hypertension: Pulmonary Capillary Hemangiomatosis

Abstract

Pulmonary capillary hemangiomatosis (PCH) is an idiopathic disease characterized with pulmonary hypertension (PH) caused by the proliferation of numerous capillaries within alveolar walls of the lung. Administration of vasodilator therapy, in contrast to primary PH, is risky due to possible fatal pulmonary edema; therefore, differentiation of PCH and primary PH is of significant importance. Since the clinical features of PCH are vague and histopathologic examination may not be usually feasible due to unstable conditions of the patients, radiological findings may help establish the diagnosis. A 17-year-old girl presented with exertional dyspnea and fatigue. PH was observed with both two-dimensional Doppler echocardiography and right heart catheterization. The diffuse ground glass opacifications of both lungs and the signs of PH on computed tomography (CT) raised the suspicion of PCH. The diagnosis was then confirmed with histopathologic examination. We herein report this rare pediatric case of PCH with emphasis on CT imaging findings.