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

Cristina Basso, MD, PhD^*, Barry J. Maron, MD, FACC, Domenico Corrado, MD and Gaetano Thiene, MD^*

^* Department of Pathology, University of Padua Medical School, Padua, Italy
Department of Cardiology, University of Padua Medical School, Padua, Italy
Minneapolis Heart Institute Foundation, Minneapolis, Minnesota, USA

View larger version (27K): [in a new window]   Figure 1 Flow-chart showing clinical data available and findings in the present study group of athletes who died of wrong aortic sinus coronary artery anomalies.

View larger version (80K): [in a new window]   Figure 2 A 15-year-old male Italian soccer player with a history of exertional syncope one year before death who died suddenly while running during the second half of a game. (A) A 12-lead ECG performed 10 months before death, as part of routine preparticipation screening, is within normal limits. (B) View of the aortic root; RCA arises normally from the right aortic sinus (arrow), and the LMCA arises anomalously from the right sinus with an acute angle take-off producing a slit-like lumen (arrowhead).

View larger version (78K): [in a new window]   Figure 3 From the same Italian soccer player shown in Figure 2, with anomalous left main coronary artery (LMCA) and intramural course. (A) Transverse section of the aortic root at the commissural level. The anomalous LMCA shows an intramural aortic course just behind the commissure between the two coronary cusps. (B) Panoramic histology showing that the LMCA, which arises from the right sinus (RS) instead of from the left sinus (LS), and the aorta share the same media without an interpositioned adventitia, accounting for a collapsed coronary lumen (Weigert van Gieson stain, x3) (C = commissure). (C) Histologic section of the region of left ventricular myocardium supplied by the anomalous LMCA showing diffuse myocyte necrosis and neutrophilic infiltrates (Haematoxylin-Eosin stain, x240). (D) Multiple patchy areas of replacement-type fibrosis that stain blue are diffusely distributed throughout the myocardium (Heidenhain trichrome stain, x30).