A pressing matter—mediastinal fibrosis with near obliteration of the pulmonary arteries

Abstract

Idiopathic mediastinal fibrosis is a rare, histologically benign condition which often presents with symptoms attributable to compression of vital mediastinal structures. Diagnosis can be difficult and individualized treatments are required for patients, with possible intervention including pharmacotherapy, surgery, and percutaneous stenting. We present a case of idiopathic mediastinal fibrosis present in a 50-year-old woman as compression and near obliteration of the pulmonary arteries. A percutaneous approach was utilized with bilateral balloon expandable kissing stents simultaneous deployed from the main pulmonary artery to the right and left pulmonary arteries. There was instantaneous improvement in the pulmonary and systemic hemodynamics. Her immediate postprocedure course was complicated by reperfusion injury to the right lung, requiring intubation and ventilation. The patient made a full recovery and remains well at 6 months. Our case highlights the procedural and postprocedural difficulties that exist in such cases, and reinforces the value of endovascular stenting strategies in the management of patients with this rare condition.