This is a novel re-appraisal of an understudied and misunderstood group of important coronary anomalies. The general name of the group is "anomalous origin of the left coronary artery," but several additional details should be included in this group of anomalies and the explanation of their pathophysiology. The most lethal form in young athletes or military recruits features intramural aortic proximal course. This comprehensive review is based on a large experience at a dedicated center for coronary artery anomalies, using evolving knowledge (over 20 years) while employing prospective and disciplined programs of evaluation and treatment, according to the nature and severity of each anomaly. The most common pathogenic mechanism of coronary dysfunction relates to intramural coronary course, with or without ectopic origin, leading to variable lateral compression and stenosis inside the aortic tunica media; this compression is present at rest and worsens with exertion. We propose that such variable and dynamic stenoses can be best studied by examining their specific anatomy and clinical presentation, stress testing, and, most importantly, in vivo evaluation by intravascular ultrasonography. Such methods should be used to support individual risk evaluation and selection among treatment options.
Improving preparticipation screening of candidates for sports necessitates establishing the prevalence of high-risk cardiovascular conditions (hr-CVC) that predispose young people to sudden cardiac death (SCD). Our accurate, novel protocol chiefly involved the use of cardiac magnetic resonance (CMR) to estimate this prevalence.
Middle and high school students from a general United States population were screened by means of questionnaires, resting electrocardiograms, and CMR to determine the prevalence of 3 types of hr-CVC: electrocardiographic abnormalities, cardiomyopathies, and anomalous coronary artery origin from the opposite sinus with intramural coronary course (ACAOS-IM). We examined the range of normal left ventricular size and function in the main study cohort (schoolchildren 11–14 yr old). We defined diagnostic criteria for hr-CVC and compared the cardiac measurements of these younger participants with those of older children whom we examined (age, 15–18 yr).
From 5,169 completed diagnostic studies (mean participant age, 13.06 ± 1.78 yr), CMR results revealed 76 previously undiagnosed cases of hr-CVC (1.47% of the total cohort): 11 of dilated cardiomyopathy (14.5%), 3 of nonobstructive hypertrophic cardiomyopathy (3.9%), 23 ACAOS-IM cases (30.3%; 6 left-ACAOS and 17 right-ACAOS), 4 Wolff-Parkinson-White patterns (5.3%), 34 prolonged QT intervals (44.7%), and 1 Brugada pattern (1.3%). Cardiomyopathies were significantly more prevalent in the older children. Of note, we identified 959 cases (18.5%) of left ventricular noncompaction.
If our estimate is accurate, only 1.47% of school-age sports participants will need focused secondary evaluations; the rest can probably be reassured about their cardiac health after one 30-minute screening study.
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