Coronary Anomalies and Sudden Death in Athletes
July 1, 2000 4 minutes read
Coronary Anomalies and Sudden Death in Athletes
abstract & commentary
Synopsis: Routine rest and exercise ECG screening of athletes are not reliable to exclude coronary artery anomalies, but premonitory symptoms are helpful in selecting patients for further study.
Source: Basso C, et al. J Am Coll Cardiol 2000;35: 1493-1501.
Sudden death in athletes is a rare but catastrophic event, the etiology of which includes congenital coronary artery anomalies. These defects are readily correctable by surgery if detected premortem. However, little information exists about their clinical presentation and the feasibility of early recognition. Thus, Basso and associates reviewed two large registries of sudden death in young competitive athletes who experienced sudden death in the United States and Italy. The entry criteria for patient selection were: 1) origin of a coronary artery from the wrong sinus of Valsalva without other cardiac defects; 2) a competitive athlete; 3) age younger than 35 years; and 4) no evidence of substance abuse. Clinical information in the U.S. cases was obtained for the evaluation of symptoms, whereas in Italy there is a systematic screening program for all competitive athletes that includes a 12-lead electrocardiogram (ECG) and a limited step stress test. The study population consisted of 27 cases, of which 23 had left main origin from the right coronary sinus and four had right coronary origins from the left sinus. There were 22 men and five women aged 9-32 years (mean 16), most of whom were schoolchildren. There were only a few collegiate or professional athletes. White races predominated (n = 16), followed by African Americans (9) and Asians (2). Many sporting activities were represented, but 15 of the cases involved basketball or soccer, popular sports in the two countries. Sudden death occurred during (n = 25) or shortly after intense exercise either during training (n = 16) or during the competition (n = 11). In 44%, the intense activity lasted for more than 30 minutes and in 70% it occurred between 3 and 9 pm, the usual time of after-school activities. In 15 cases, there was no clinical information from life. The 12 in whom clinical information was available did not seem to differ in any meaningful way from the others. Premonitory symptoms were present in 10:4 syncope, two recurrent; five chest pain, three with exertion; and three palpitations, one with exertion. All the symptoms had been present for less than 24 months. None of the cases had a family history of sudden death at a young age. Pathology showed and angled take off of the anomalous artery with a slit-like ostial opening, tunneling in the media of the aorta, and a course between the aorta and the pulmonary artery. No atherosclerotic coronary artery lesions were seen, but three had evidence of old myocardial damage and six had histologic evidence of recent ischemia. Interestingly, only those with origin of the left main from the right sinus had symptoms: mainly chest pain and syncope. Basso et al conclude that routine rest and exercise ECG screening of athletes is not reliable to exclude coronary artery anomalies, but premonitory symptoms are helpful in selecting patients for further study.
Comment by Michael H. Crawford, MD
Sudden death in young athletes is rare, occurring in 0.5 to 1.6/100,000/yr. In the United States, the most common cause is hypertrophic cardiomyopathy because we have no systematic screening program for school-age athletes; whereas in Italy, where they do have such programs, the most common cause is arrhythmogic right ventricular dysplasia. Coronary anomalies make up about 20% of the cases in both countries, but origin from the wrong sinus seems to carry the highest risk of sudden death, which is why it was the subject of this report. The exact pathophysiology of sudden death in these anomalies is unknown, but several factors may be important: 1) intermittent closure of the slit-like opening; 2) compression of the anomalous artery between the aorta and pulmonary artery, especially during exercise when the great arteries expand due to increasing pressure and flow; 3) coronary spasm; and 4) endothelial damage. This study supports the notion that ischemia is sporadic in these patients and may not always be precipitated by exercise.
The major findings from this study are that premonitory symptoms are the best clue to this diagnosis and that ECG stress testing is not sensitive enough to rely upon on for screening. Especially important are the symptoms of syncope and chest pain that occurred most frequently with anomalous origin of the left main. Unfortunately, anomalous origin of the right coronary was silent in this study, but not in others. Thus, Basso et al recommend that athletes, or, for that matter, any children who complain of chest pain or syncope, have an echocardiogram to look for absence of coronary artery origins in the two cusps. In older children, transesophageal echo may be necessary or perhaps magnetic resonance imaging or computerized tomography scanning. When suspicion of coronary anomalies is present, cardiac catheterization should be considered with an eye toward surgical correction. The standard surgical operation is coronary bypass, but reimplantation of the anomalous coronary is sometimes possible or there is a new procedure that involves unroofing the intra-aortic part of the artery. The latter operation would only be of benefit if the between-the-great-arteries course was not considered to be important in the production of ischemia. Regardless of the therapy chosen, such children should be prohibited from competitive sports.
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