Coronary Artery from the Wrong Sinus of Valsalva: A Physiologic Repair Strategy

Abstract

The most commonly reported coronary artery malformation leading to sudden death in children and young athletes is an anomalous coronary origin from the wrong aortic sinus, with or without a proximal course of the coronary artery between the aorta and the pulmonary artery. Although the incidence is difficult to assess, there is a 0.17% incidence in autopsy series (all variants included) and a 0.1 to 0.3% in catheter-echo diagnostic series. There may be a regional variation. In a prospective study of 1950 consecutive patients undergoing coronary angiography, anomalous origin of the right coronary artery from the left aortic sinus was found in 0.92% of cases, whereas the incidence of left coronary artery arising from the right aortic sinus was 0.15%. 1 In a study of 2388 patients who had transthoracic echocardiographic examination of their proximal coronary anatomy as part of innocent murmur investigation and evaluation of performance status, four patients (0.2%) were found to have an anomalous origin of the coronary artery from the wrong sinus. 2 Whatever the true prevalence, this lesion predisposes to fatal episodes of myocardial ischemia that may occur during or shortly after strenuous exercise. 3-5 Timely diagnosis requires a high index of suspicion but is frequently impossible, as the majority of patients with the anomaly are asymptomatic and thus do not seek medical attention. Therefore, sudden death is frequently the first manifestation, especially in young athletes, with only 30% of patients reporting prodromal symptoms. 5,6 Screening young adults before participation in competitive sports has been unsuccessful. Stress testing is unreliable because of high false-positive and falsenegative results; echocardiogram, echocardiography, and stress testing all lack predictive value. 4,5 Finally, although the major association of sudden death is with young athletes, cases have also been reported in children under 1 year of age.