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CLINICAL RESEARCH: CONGENITAL HEART DISEASE

Anomalous Aortic Origin of a Coronary Artery With an Interarterial Course

Should Family Screening Be Routine?

Julie A. Brothers, MD^_*,_*, Paul Stephens, MD^_*, J. William Gaynor, MD, Richard Lorber, MD, Luca A. Vricella, MD and Stephen M. Paridon, MD, FACC^_*

^_* Division of Cardiology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Division of Cardiothoracic Surgery, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
Department of Pediatric Cardiology, Cleveland Clinic, Cleveland, Ohio
Division of Cardiac Surgery, Johns Hopkins Hospital, Baltimore, Maryland.

Manuscript received November 20, 2007; revised manuscript received December 19, 2007, accepted January 7, 2008.

^_* Reprint requests and correspondence: Dr. Julie A. Brothers, The Children's Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Division of Cardiology, Main Building, Philadelphia, Pennsylvania 19104. (Email: brothersj{at}email.chop.edu).

Objectives: We sought to present cases of familial occurrence of anomalous aortic origin of a coronary artery with an interarterial course (AAOCA) to determine if it would alter our current screening and management recommendations.

Background: Anomalous aortic origin of a coronary artery with an interarterial course is a rare congenital anomaly that carries an increased risk of sudden death in children and young adults. There are no reports in the literature of familial AAOCA in the pediatric population.

Methods: In preparation for a multi-institutional prospective study evaluating patient management and surgical outcomes in children and young adults with AAOCA, a questionnaire was sent to multiple pediatric institutions in North and South America. Several respondents indicated caring for families with more than 1 member with AAOCA. These patients were identified and charts were retrospectively reviewed.

Results: We identified 5 families in which a child was diagnosed with AAOCA and another family member was subsequently identified through screening with echocardiography. The odds of this occurring are significantly greater than what would be expected by chance. All identified by screening were asymptomatic and had anomalous right coronary artery despite 2 of the 5 index cases having anomalous left coronary artery.

Conclusions: It is possible that there is a genetic link for AAOCA. Future research into this is warranted. Due to the potential risk of myocardial ischemia and sudden death associated with AAOCA, screening first-degree relatives for AAOCA using transthoracic echocardiography would be the prudent approach to potentially prevent a sudden catastrophic event.

Abbreviations and Acronyms   AAOCA = anomalous aortic origin of a coronary artery with an interarterial course   ALCA = anomalous origin of left main coronary artery   ARCA = anomalous origin of right coronary artery   CHSS = Congenital Heart Surgeon Society   TTE = transthoracic echocardiography

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