An Adolescent with Pulmonary Hypertension Secondary to Idiopathic Fibrosing Mediastinitis: Case Report

Pulmonary hypertension is an important determinant of morbidity and mortality in many diseases, including congenital heart disease and respiratory disease. Fibrosing mediastinitis is a rare disorder caused by proliferation of acellular collagen. In fibrosing mediastinitis, clinical presentation is determined by compression to the vital mediastinal organs. Fibrosing mediastinitis may rarely lead to pulmonary veno-occlusive disease. In this case report, a 16 year old boy with pulmonary arterial hypertension was presented. His right pulmonary artery was completely occluded secondary to idiopathic fibrosing mediastinitis. The patient died after mediastinal biopsy. Herein, we discussed pulmonary arterial hypertension secondary to idiopathic fibrosing mediastinitis in children.