Pulmonary capillary hemangiomatosis: An unusual cause of primary pulmonary hypertension in a child with characteristic computed tomography imaging features.

Abstract

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of primary pulmonary hypertension (PPH) diagnosed in children and young adults with a nonspecific clinical presentation of dyspnea, cough, chest pain, and fatigue. It is characterized by extensive proliferation of pulmonary capillaries within alveolar septa. The imaging features include diffuse centrilobular ground-glass opacities with features of pulmonary hypertension. We present a case of PCH in an 11-year-old boy who was diagnosed with PPH in echocardiography and referred for diagnostic imaging.