Cardiovascular Disease |

Pulmonary Artery Dissection and Rupture in a Patient With Idiopathic Pulmonary Artery Hypertension FREE TO VIEW

Neal Holland, DO; Ruxana Sadikot, MD; Hiren Mehta, MD; Vipul Patel, MD
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University of Florida, Gainesville, FL

Chest. 2013;144(4_MeetingAbstracts):156A. doi:10.1378/chest.1703198
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SESSION TITLE: Cardiovascular Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary arterial hypertension (PAH) is characterized by progressive increase in pulmonary vascular resistance eventually causing right ventricular failure. PAH rarely causes dissection of the pulmonary artery which may lead to rupture of the vessel, a consequence manifested as cardiogenic shock or sudden death. This is diagnosed postmortem(1).

CASE PRESENTATION: A 78 year-old woman was diagnosed with idiopathic PAH. She was treated with incremental doses of bosentan, sildenafil and trepostinil for eight years. She was admitted with atrial fibrillation with rapid ventricular rate. CT pulmonary angiography (CTPA) showed an enlarged pulmonary artery. The pulmonary artery diameter had significantly increased over the past 6 months compared to prior imaging. During hospitalization, she developed asystolic cardiopulmonary arrest. Cardio-pulmonary resuscitation was unsuccessful. Her autopsy showed dissection and rupture of the main pulmonary artery resulting in hemo-pericardium. The pericardial sac contained 700 cc of blood with a clot weighing 230 g. The pulmonary artery was grossly enlarged with 4.0 cm separation of the intimal surface and a perforation of 0.5 cm located 2.0 cm superior to the pulmonic valve. Gross and histological examination showed prior dissections with evidence of healing. Histology showed hypertrophy of the media of the pulmonary arteries.

DISCUSSION: Spontaneous rupture of the pulmonary artery is a rare event and has only been reported anecdotally. An increase in pulmonary artery diameter has not been associated with prognosis. In this case we have reported there was rapid increase in pulmonary artery diameter noted on CTPA over a six month period. Whether this was a harbinger of impending rupture and dissection is matter of speculation. Although PAH is routinely not followed by serial CT scan, we suggest that rapid change in diameter should warrant further evaluation by imaging with MRA to exclude such a catastrophic event. Our case suggests that monitoring pulmonary artery diameter by some form of imaging may provide prognostic clues. To date there are no definitive reports that suggests a role for surgical intervention for management of a rapidly expanding pulmonary artery to prevent dissection.

CONCLUSIONS: The cause of sudden death in patients with PAH is often attributed to a massive PE or RV failure. This case suggests that a rapid increase in pulmonary artery diameter should be considered as an impending dissection or rupture and should be considered in patients with PAH who develop sudden cardiovascular collapse.

Reference #1: Mohammad K, et al. Idiopathic Pulmonary Artery Dissection: A Case Report. Journal of Medical Case Reports. 2009, 3:7426.

DISCLOSURE: The following authors have nothing to disclose: Neal Holland, Ruxana Sadikot, Hiren Mehta, Vipul Patel

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