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June 16, 2026Journal of the American College of Cardiology1,236 citations

Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes

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CBCristina BassoBMBarry J. MaronDCDomenico Corrado

Key Result

Among 27 young athletes who died suddenly from anomalous coronary artery origin, standard cardiovascular testing was normal in all tested, but premonitory symptoms occurred in 10 of 12 with data.

Key Points

  • The study aims to characterize congenital coronary artery anomalies and identify clinical markers for detection in young athletes.
  • Reviewed registries of young competitive athletes who experienced sudden death in the U.S. and Italy.
  • Categorized causes of death related to coronary artery anomalies identified at autopsy.
  • Analyzed available clinical data for athletes who showed premonitory symptoms.
  • Identified 27 sudden deaths due to coronary anomalies, occurring mainly during intense exertion.
  • 55% of athletes had no prior cardiovascular symptoms; 45% presented with symptoms like syncope and chest pain.
  • Standard ECG testing failed to reliably detect myocardial ischemia in athletes with these anomalies.

Study Design

Type

Observational (n=27)

Multicenter

Yes

Structured PICO

P
Population
27 young competitive athletes who died suddenly due to anomalous coronary artery origin from the wrong aortic sinus, identified from U.S. and Italian registries.
O
Outcome
Clinical profile, premonitory symptoms, and cardiovascular testing results prior to sudden death

Standard resting and exercise ECGs are unreliable for screening anomalous coronary artery origins in athletes, but a history of exertional syncope or chest pain should prompt targeted evaluation to prevent sudden death.

Abstract

OBJECTIVES: The purpose of this study is to characterize the clinical profile and identify clinical markers that would enable the detection during life of anomalous coronary artery origin from the wrong aortic sinus (with course between the aorta and pulmonary trunk) in young competitive athletes. BACKGROUND: Congenital coronary artery anomalies are not uncommonly associated with sudden death in young athletes, the catastrophic event probably provoked by myocardial ischemia. Such coronary anomalies are rarely identified during life, often because of insufficient clinical suspicion. However, since anomalous coronary artery origin is amenable to surgical treatment, timely clinical identification is crucial. METHODS: Because of the paucity of available data characterizing the clinical profile of wrong sinus coronary artery malformations, we reviewed two large registries comprised of young competitive athletes who died suddenly, assembled consecutively in the U.S. and Italy. RESULTS: We reported 27 sudden deaths in young athletes, identified solely at autopsy and due to either left main coronary artery from the right aortic sinus (n = 23) or right coronary artery from the left sinus (n = 4). Each athlete died either during (n = 25) or immediately after (n = 2) intense exertion on the athletic field. Fifteen athletes (55%) had no clinical cardiovascular manifestations or testing during life. However, in the remaining 12 athletes (45%) aged 16 +/- 7, certain clinical data were available. Premonitory symptoms had occurred in 10, including syncope in four (exertional in three and recurrent in two, 3 to 24 months before death) and chest pain in five (exertional in three, all single episodes, < or =24 months before death). All cardiovascular tests were within normal limits, including 12-lead electrocardiogram (ECG) pattern (in 9/9), stress ECG with maximal exercise (in 6/6) and left ventricular wall motion and cardiac dimensions by two-dimensional echocardiography (in 2/2). CONCLUSIONS: With regard to congenital coronary artery anomalies of wrong aortic sinus origin in young competitive athletes, 1) standard testing with ECG under resting or exercise conditions is unlikely to provide clinical evidence of myocardial ischemia and would not be reliable as screening tests in large athletic populations, 2) premonitory cardiac symptoms not uncommonly occurred shortly before sudden death (typically associated with anomalous left main coronary artery), suggesting that a history of exertional syncope or chest pain requires exclusion of this anomaly. These observations have important implications for the preparticipation screening of competitive athletes.

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Cite This Study

Basso et al. (2000) conducted an observational in Congenital coronary artery anomalies with origin from the wrong aortic sinus (n=27). Anomalous coronary artery origin from the wrong aortic sinus was evaluated on Clinical profile, premonitory symptoms, and cardiovascular test results prior to sudden death. Among 27 young athletes who died suddenly from anomalous coronary artery origin, standard cardiovascular testing was normal in all tested, but premonitory symptoms occurred in 10 of 12 with data.

synapsesocial.com/papers/6a311504dc2b2c6f755102f7https://doi.org/10.1016/s0735-1097(00)00566-0
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