Pulmonary Vascular Disease |

A Rare Case of Chest Pain: Extensive Pulmonary Artery Dissection Presenting With Cardiac Tamponade in a Patient With Idiopathic Pulmonary Hypertension FREE TO VIEW

Chintan Patel, MD; Inas Abuali, MD; Mustafa Al-Shammari, MD; Maria Carrillo, MD; Pablo Garcia, MD
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Internal Medicine, Saint Agnes Hospital, Baltimore, MD

Chest. 2014;146(4_MeetingAbstracts):897A. doi:10.1378/chest.1994539
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SESSION TITLE: Pulmonary Vascular Disease Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Pulmonary artery dissection is a very rare phenomenon known to occur in patients with pulmonary hypertension. Typically cases are diagnosed postmortem with only a few cases discovered antemortem. We present a case of a patient with known primary pulmonary hypertenion who presented to the ER with chest pain.

CASE PRESENTATION: A 62 year old female was brought to the emergency room after having acute onset of severe, non-radiating chest pain associated with light-headedness.. The patient has a history of severe primary pulmonary hypertension that was diagnosed in 1988. One year prior, she was found to have enlarged pulmonary artery measuring 6.1 cm in diameter with right & left pulmonary arteries measuring 4.0cm & 2.4 cm, respectively with no evidence of aneurysm. An EKG was concerning for ST elevations in anterior & lateral leads so the patient was emergently taken for left heart catheterization. There was no significant coronary artery occlusion identified, but during the procedure the patient became hypotensive & hypoxic requiring vasopressor support & additional oxygen. ICU was consulted for concern for possible pulmonary embolism. A CT pulmonary angiogram identified a pulmonary artery dissection extending from the pulmonary root through the right & left pulmonary arteries & extending to the first order of the pulmonary arteries along with a large pericardial effusion. A bedside echocardiogram was performed indicating cardiac tamponade. The patient was emergently transferred to a tertiary care center for veno-arterial extracoporeal membrane oxygenation. Shortly after the transfer, the patient's suffered from complete right heart failure and expired.

DISCUSSION: Published case reports of pulmonary artery dissection are very rare with even fewer seen in patients that were alive at the time of diagnosis. Chronic pulmonary hypertension has been known to cause pulmonary artery dilation through medial degeneration with fragmentation of elastic fibers thereby causing a weakened arterial wall leading to shear stress & intimal tear. The main pulmonary trunk is often the site of dissection however usually does not involve the branches. In our case the dissection extended beyond the initial bifurcation & involved the formation of a pericardial effusion.

CONCLUSIONS: Pulmonary artery dissection should be considered as a differential for chest pain in a patient with a history of significant pulmonary hypertension and established diagnosis of enlarged pulmonary arteries. Time is of the essence in these patients and immediate identification and evaluation for surgical options can be life-saving.

Reference #1: Khattar,R.S.,Fox, D.J.,Alty,J.E.,& Arora, A.(2005). Pulmonary artery dissection:an emerging cardiovascular complication in surviving patients with chronic pulmonary hypertension. Heart, 91(2),142-145.

Reference #2: Steurer J, Jenni R, Medici TC, et al. Dissecting aneurysm of the pulmonary artery with pulmonary hypertension. Am Rev Respir Dis 1990;142:1219-21.

DISCLOSURE: The following authors have nothing to disclose: Chintan Patel, Inas Abuali, Mustafa Al-Shammari, Maria Carrillo, Pablo Garcia

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