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Cardiovascular Disease |

Sudden Death in a 43-Year-Old Female With Pulmonary Arterial Hypertension

Sandeep Sahay*, MD; Adriano Tonelli, MD
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Akron General Medical Center, Cuyahoga Falls, OH


Chest. 2012;142(4_MeetingAbstracts):125A. doi:10.1378/chest.1388836
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Abstract

SESSION TYPE: Cardiovascular Case Report Posters

PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM

INTRODUCTION: Sudden death is most commonly a manifestation of underlying ischemic heart disease. Sudden death is observed in pulmonary arterial hypertension (PAH); however very rarely this is due to ischemic ventricular fibrillation (VF) due to the extramural compression of the left main coronary artery by an enlarged pulmonary artery, a likely underrecognized association.

CASE PRESENTATION: A 43-year old female with ventricular septa defect (VSD) repaired during childhood, complicated by pulmonary hypertension presented with VF arrest. She was successfully defibrillated. Electrocardiogram on arrival showed T-wave inversions in leads V1-6. Echocardiography showed severely dilated and dysfunctional right ventricle with estimated right ventricular systolic pressure of 88 mmHg. A large right-to-left interatrial shunt and a relatively small intraventricular left-to-right shunt at the site of the previous repair of the VSD were seen. Right heart catheterization revealed pulmonary artery pressures of 74/34 mmHg. A coronary CT angiogram revealed a compressed left main coronary artery by a severely dilated main pulmonary artery with slit like ostium measuring 6.2 X 2.6 mm (Figure 1). A bare metal stent was placed in the left main coronary artery at the site of compression (Figure 2). The patient also received an intra-cardiac defibrillator and was discharged in the stable state.

DISCUSSION: Extrinsic compression of the left main coronary artery by an enlarged pulmonary artery is an increasingly recognized and potentially reversible cause of angina, left ventricular dysfunction and rarely sudden death in PAH. Percutaneous coronary intervention appears to be a feasible, safe, and effective treatment option for such patients.

CONCLUSIONS: The diagnosis of extrinsic left main coronary artery compression requires a high index of suspicion and should be considered in patients with severe pulmonary hypertension.

1) Lee MS, Oyama J, Bhatia R, Kim YH, Park SJ. Left main coronary artery compression from pulmonary artery enlargement due to pulmonary hypertension: a contemporary review and argument for percutaneous revascularization. Catheter Cardiovasc Interv. 2010 ;76:543-50.

DISCLOSURE: The following authors have nothing to disclose: Sandeep Sahay, Adriano Tonelli

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Akron General Medical Center, Cuyahoga Falls, OH

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