Anomalous Aortic Origin of a Coronary Artery with an Interarterial Course: Understanding Current Management Strategies in Children and Young Adults

The Children's Hospital of Philadelphia, Cardiology, Philadelphia, PA, USA.
Pediatric Cardiology (Impact Factor: 1.31). 07/2009; 30(7):911-21. DOI: 10.1007/s00246-009-9461-y
Source: PubMed


Anomalous aortic origin of a coronary artery from the opposite sinus of Valsalva with an interarterial course (AAOCA) is a rare congenital anomaly that is the second leading cardiovascular cause of sudden death in children and adolescents. Management practices with respect to this lesion vary among clinicians. We surveyed practitioners' opinions on treatment and management strategies for patients with AAOCA. A 17-question "Patient Management Questionnaire" was sent to all Congenital Heart Surgeons' Society (CHSS) members (n = 90), who were asked to respond and to distribute the questionnaire to pediatric cardiologists and nurse practitioners at their institutions. Questions addressed patient presentation, diagnostic modalities, therapeutic intervention, complications to therapy, follow-up visits, and type and frequency of follow-up testing. Descriptive statistics were used for analyses. We received 113 responses. Almost all respondents agree that ischemia at presentation or with provocative testing warrants surgical repair, whereas the majority believes that any patient with symptoms but no evidence of ischemia should have surgery. Approximately one-half and one-third of the respondents manage patients who are awaiting surgery or not having surgery by completely restricting exercise or not allowing competitive athletics, respectively. In asymptomatic patients, patient age determines timing of surgical repair but almost one-quarter did not believe that an asymptomatic child should undergo surgery. Among patients managed medically, there were six sudden deaths; there were two deaths in those who had surgical repair. There is marked heterogeneity in physician opinions regarding AAOCA treatment and management. A multicenter registry would facilitate the development of evidence-based treatment and management guidelines for children and young adults with AAOCA.

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    • "Ce type de traitement peut exposer au risque d'involution précoce des conduits artériels du fait de l'absence de réduction importante du flux artériel à l'état basal dans l'artère ectopique. Ces considérations expliquent que les registres montrent généralement des pratiques peu en adéquation avec les recommandations, en particulier pour une population d'adultes jeunes où l'ANOCOR peut être découverte fortuitement [19] "
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    ABSTRACT: Among the wide spectrum of congenital abnormalities of coronary arteries, a single coronary artery is often confused with an ectopic coronary artery connected with the contralateral coronary artery. Both abnormalities are characterized by a single coronary ostium, but they differ by the lack or not of an initial ectopic course. The prognosis of anomalous connections of coronary arteries depends mainly on the type of the initial course in relation to other cardiac structures. Therefore, the distinction between a single coronary artery and an ectopic coronary artery connected with the contralateral artery is of importance.
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    ABSTRACT: Anomalous origin of the coronary artery from the contralateral sinus of Valsalva, coursing between the aorta and pulmonary artery, has garnered much attention because of its association with sudden death. Current medical opinion is heavily skewed toward "successful" intervention. However, two key issues have not been critically analyzed: what is the true risk of sudden death from an anomalous coronary artery, and how does this risk balance against the risk of surgical intervention? Common misconceptions about risk derive from citations of autopsy data. In fact, the scant available data suggest a far lower absolute risk than commonly cited. The risks of surgical intervention, while likely quite low, remain real, and include aortic valve damage and neurological sequelae. The lack of long-term outcomes data precludes any definite recommendations in most patients. The decision to intervene is thus not straightforward, and should be approached cautiously and only after appropriate counseling of the patient.
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