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Cardiothoracic Surgery |

Alcapa Syndrome: A Rare Congenital Cardiac Anomaly Diagnosed Postpregnancy in an Adult

Hiren Patel, MD; Brian Swirsky, MD
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Baton Rouge General Medical Center, Baton Rouge, LA


Chest. 2015;148(4_MeetingAbstracts):37A. doi:10.1378/chest.2251480
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Abstract

SESSION TITLE: Cardiothoracic Surgery Student/Resident Case Report Posters

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) accounts for less than 0.5% of all congenital cardiac anomalies and is associated with a 90% mortality rate in the first year of life. ALCAPA causes myocardial ischemia due to a coronary steal phenomenon leading to congestive heart failure and even sudden cardiac death. Diagnosis of this anomaly in adulthood is extremely rare. Herein, we present a case of ALCAPA diagnosed in an adult female eleven years after a successful pregnancy.

CASE PRESENTATION: A 33-year-old female presented with worsening angina since her successful pregnancy at age 22. Physical exam and vitals were within normal limits. EKG had non-specific ST changes. She underwent a coronary angiography after having a positive stress test. It showed retrograde filling of the left coronary artery system via collaterals from the dilated right coronary artery and eventually draining into the pulmonary artery. The diagnosis of ALCAPA was confirmed with a computerized tomography angiogram. Transthoracic echocardiography showed moderate mitral regurgitation and moderate to severe global hypokinesis. The patient eventually underwent reimplantation of the left coronary system to the aortic root without any complications. Over the next 4 years she continues to have normal routine follow up ever since the reimplantation.

DISCUSSION: ALCAPA is a rare life-threatening anomaly. Without surgery, the mortality rate is 90% in the first year of life. In extremely rare cases, patients make it to adulthood, even through pregnancy as illustrated in this case, due to adequate collateral blood supply from the right coronary artery to the left coronary system. The diagnosis is often made due to the development of persistent angina caused by myocardial ischemia. This ischemia is due to a coronary steal phenomenon from a left to right shunt via the collateral vessels resulting in decreased perfusion pressure as seen on our patient’s coronary angiogram. Overtime, myocardial ischemia leads to the development of congestive heart failure, mitral insufficiency, malignant arrhythmias, and even sudden cardiac death, therefore surgical correction to reimplant the coronary artery to the aorta is crucial.

CONCLUSIONS: The presentation of ALCAPA in adulthood even after pregnancy, though extremely rare, is possible with persistent symptoms of angina usually leading to the diagnosis; this in turn allows early surgical correction to provide excellent long-term prognosis as seen in our presented patient.

Reference #1: Dodge-Khatami A, et al. Anomalous origin of the left coronary artery from the pulmonary artery: collective review of surgical therapy. Ann Thorac Surg. 2002;74:946-955.

Reference #2: Wollenek G D, et al. Anomalous origin of the left coronary artery: a review of surgical management in 13 patients. J Cardiovasc Surg 1993;34:399-405.

Reference #3: Edwards JE. Anomalous coronary arteries with special reference to arteriovenous-like communications. Circulation.1958;17:1001-1006.

DISCLOSURE: The following authors have nothing to disclose: Hiren Patel, Brian Swirsky

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