Pulmonary artery dissection with patent ductus arteriosus presenting with differential cyanosis: a case report and review of literature

Abstract

Spontaneous pulmonary artery (PA) dissection is an extremely rare and life-threatening complication of chronic pulmonary hypertension, most commonly associated with congenital heart disease. We report a 54-year-old woman who presented with sharp chest pain, progressive dyspnea, and differential cyanosis between the upper and lower extremities. Laboratory evaluation revealed secondary erythrocytosis and elevated NT-proBNP. Transthoracic echocardiography demonstrated a markedly dilated pulmonary trunk, severe pulmonary hypertension, and a large patent ductus arteriosus with bidirectional shunting. Contrast-enhanced computed tomography confirmed spontaneous PA dissection involving the main pulmonary trunk and both pulmonary arteries. Because of established Eisenmenger physiology, surgical repair was not feasible; the patient declined further intervention and was managed conservatively with diuretics, pulmonary vasodilators, and carefully monitored anticoagulation. She was subsequently discharged against medical advice and lost to follow-up. This case highlights differential cyanosis as a bedside marker of right-to-left ductal shunting, summarizes previously reported cases and their outcomes, and discusses the management challenges of this rare condition.