Anomalous Aortic Origin of a Coronary Artery With an Interarterial Course Should Family Screening Be Routine?

ObjectivesWe 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.BackgroundAnomalous 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.MethodsIn 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.ResultsWe 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.ConclusionsIt 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

Technical modifications that might improve long-term outcome of the ross procedure in children

BACKGROUND: Failure of the pulmonary autograft (PVA) following the Ross procedure (RP) has discouraged its widespread use and led to modifications or alternatives to the procedure. We sought to analyze whether certain technical modifications could improve results of the RP in children. METHODS: Sixty-nine patients (median age 12 years, range 0.25 to 17.9) underwent the RP between 01/1996 and 12/2018. Concomitant Konno procedure was performed on 20/69 (29%). Prior interventions included balloon valvuloplasty in 30/69 (44%), and/or surgical valvuloplasty in 39/69 (57%). Technical modifications included utilizing the native aortic root for external annuloplasty, implanting the autograft using uniplanar horizontal sutures through the aortic wall, normalizing the sinotubular junction and wrapping the native root remnant around the PVA. RESULTS: Operative mortality was 1/69 (1.5%), with no late death. No patient had neoaortic valvar stenosis and 7/68 (10%) had mild regurgitation on discharge echocardiogram. At latest follow-up (median 9.4 years, range 0.4 - 21.3) there was no significant change in the latest follow up Z scores of annulus, sinus or sinotubular junction diameters when compared to those at discharge. Three patients (4.4%) required late autograft replacement, two PVA repair, and two resection of pseudoaneurysm. Actuarial freedom from PVA replacement was 87% at 20 yrs. Freedom from right ventricular outflow tract catheter reintervention or reoperation was 83% and 80% respectively. CONCLUSIONS: Technical modifications of the RP used in this cohort might successfully prolong the life of the PVA without compromising its growth, an important advantage in pediatric patients