Pulmonary Artery Dissection and Rupture in a Patient With Eisenmenger Syndrome

Abstract

INTRODUCTION: Eisenmenger syndrome (ES) is frequently associated with hemoptysis and sudden death. We describe a patient with ES presenting with chest pain and hemoptysis due to pulmonary artery (PA) dissection with subsequent death from rupture. CASE PRESENTATION: A 44-year-old man with ES was transferred to our facility for further evaluation of hemoptysis and chest pain. Past history included a large muscular ventricular septal defect with ineffective PA banding at ten months of age and subsequent irreversible pulmonary vascular disease and cyanosis. He had a known large PA aneurysm and one prior episode of massive hemoptysis requiring coil embolization of pulmonary collaterals. He had been treated with bosentan but had been noncompliant at times and was lost to follow-up for six years prior to presentation. He presented to an outside hospital with left-sided pleuritic chest pain and fevers. Despite antibiotics, he had persistent chest pain and had four episodes of hemoptysis. At the time of transfer, oxygen saturation was 71%, heart rate 118 bpm, and blood pressure 113/62. Chest x-ray revealed a large left pleural effusion and dilatation of the pulmonary arteries. Thoracentesis yielded one liter of bloody fluid. Transthoracic echo showed severe right ventricular enlargement and an estimated right ventricular systolic pressure of 120 mmHg. Computed tomography (CT) showed massive dilatation of the main PA and a dissection flap extending from the intimal disruption caused by the pulmonary band. The patient experienced pulseless electrical activity arrest and resuscitation was unsuccessful. Autopsy revealed a ruptured main PA which was massively dilated with chronic dissection present. The rupture occurred in the outer wall of the dissection channel and migration of the PA band through the vessel wall was also noted. DISCUSSION: PA dissection is a rare complication of pulmonary hypertension and is usually fatal. Dissection and rupture are more common with PA aneurysms, which occur more often in patients with ES due to increased pulmonary blood flow. In contrast to aortic dissection which develops a re-entry tear, in PA dissections the false lumen is more likely to rupture (1). In this case, the patient was likely more prone to dissection due to the PA band that had migrated through the wall of the vessel. Surgical repair of PA dissection has been reported but the overall prognosis remains poor (2). CONCLUSIONS: PA dissection or rupture is a rare complication in patients with ES, is often fatal, and should be suspected in patients presenting with hemoptysis.