Surgery for fibrosing mediastinitis with severe pulmonary hypertension due to pulmonary venous stenosis

Abstract

A 21-year-old woman with severe pulmonary hypertension and circulatory collapse was referred to our hospital for possible lung transplantation with extracorporeal membrane oxygenation support. Computed tomography revealed severe stenosis of all 4 pulmonary veins, and fibrosing mediastinitis was suspected. Surgical reconstruction of the pulmonary veins was performed, and extracorporeal membrane oxygenation support was weaned off. After surgery, pulmonary vascular resistance normalized. This successful case demonstrates that surgical pulmonary venous reconstruction is an important treatment for fibrosing mediastinitis induced by pulmonary venous stenosis and pulmonary hypertension.

Keywords: Pulmonary venous stenosis; bovine patch repair; extracorporeal membrane oxygenation; fibrosing mediastinitis; pulmonary hypertension.