Abstract

Anomalous aortic origin of a coronary artery (AAOCA) from the inappropriate sinus of Valsalva with an interarterial, intraconal, or intramural course is a rare heart defect associated with an increased risk of sudden death in children.

CHSS AAOCA study

In 2009, The Congenital Heart Surgeons' Society (CHSS) established a North American Registry in order to study a large multi-institutional cohort of patients with AAOCA. This initiative is intended to generate new knowledge concerning the natural history of AAOCA, to describe the outcomes of surgical intervention versus observation in children and young adults with AAOCA, and to generate evidence to support risk stratification among patients with AAOCA and eventually suggest evidence-based guidelines for management.

CHSS AAOCA study | Rationale and objectives

The AAOCA is a rare birth defect in the heart that occurs when a coronary artery arises from the wrong location on the main blood vessel, the aorta.

Children and young adults with these defects can die suddenly, especially during or just after exercise. In fact, AAOCA is the second leading cause of sudden cardiac death in children and adolescents in the United States behind hypertrophic cardiomyopathy. The prevalence is estimated at 0.1% to 0.3% of the general population. Neither the true risk of sudden death nor the best way to treat these patients is known with certainty. Because of the risk of sudden death, doctors face the pressure to “do something” but in the absence of long-term follow-up data, the risks and benefits of different management options are unconfirmed. This study will create a pool of information that may guide future choice of treatment options for these children and young adults.

This study will be ongoing for 15 years. It is expected that approximately 1000 patients will be enrolled.

This funding to start the registry was provided by The Children's Heart Foundation, The Cardiac Center at The Children's Hospital of Philadelphia and from CHSS member institutions.

CHSS AAOCA study | Enrollment criteria

Patients who are diagnosed with AAOCA at or before age 30 years are eligible for this study. They should have otherwise normal heart or only minor defects such as Atrial septal defect, Ventricular septal defect, Patent ductus arteriosus, bicuspid aortic valve, mild pulmonary stenosis etc.

Patients who have other major heart problems that require operations are currently not included in this Cohort study. Any other problems with coronary arteries are also not included.

CHSS AAOCA study | Current status

The Registry has been enrolling new patients from participating institutions that are member of the Congenital Heart Surgeons' Society. Hospitals from across North America continue to join the study group and enroll patients. Over 140 patients with AAOCA have been enrolled by June 2011, making it the largest cohort ever assembled of this anomaly.

CHSS AAOCA study | ARCAPA

Anomalous origin of the right coronary artery originating from the pulmonary trunk (ARCAPA) is a rare but potentially fatal anomaly. The goal of surgical therapy is establishment of a physiologic bi-coronary circulation.