Survived Sudden Cardiac Death in a Young Marathon Runner
Cardiac Ischemic Event Attributed to an Interarterial Course of the Right Coronary Artery
A healthy man, aged 20 years, experienced sudden cardiac death caused by ventricular fibrillation while running a half marathon. Cardiopulmonary resuscitation was initiated within 2 minutes of latency and was continued for 9 minutes with return of spontaneous circulation after defibrillation. The ECG showed ST-elevations in leads II, III, and aVF (Figure 1).
A coronary artery disease could be excluded by coronary angiography. The left coronary artery had its orthotopic source on the left coronary cusp and arose anatomically regular with identification of a myocardial bridge in segment 7 (Figure 2 and Movies I and II in the online-only Data Supplement). The right coronary artery (RCA) arose atypically from the left coronary cusp. The proximal segment of the RCA was slightly narrowed with a length of 11 mm (Figure 3). The intubation of the RCA proved to be atypical. However, all of the coronary arteries showed thrombolysis in myocardial infarction 3 flow. No atherosclerotic lesions were detected (Figure 4 and Movies III through VII in the online-only Data Supplement).
Acute aortic dissection could be ruled out by transesophageal echocardiography. Furthermore, the left coronary artery was free-visible up to the bifurcation. A second small vessel was identified next to the left coronary artery in the left coronary cusp (Figure 5). Postresuscitation care phase and weaning proceeded rapidly with no neurologic dysfunctions.
Because of the treatment history of a common cold, the absence of coronary artery disease, and suggestive laboratory results (creatine kinase max, 35 µkat/L [normal value, <2.85 µkat/L], creatine kinase MB, 2.18 µkat/L [normal value, <0.42 µkat/L], high-sensitivity troponin T, 2068 ng/L [normal value, <14 ng/L], and d-dimer, 0.85 mg FEU/L [normal value, <0.5 FEU/L]), initially a myocarditis was considered as a relevant cause for the ventricular fibrillation. To test for myocarditis lesions, a cardiac magnetic resonance imaging (MRI) scan was performed. Surprisingly, a pattern of posterior wall infarction was shown by the MRI, yet there was no evidence of acute myocarditis or hemodynamic relevant muscle bridge of the left anterior descending artery with stress MRI. The perfusion sequences showed an inferior perfusion failure. Accordant to this, a late gadolinium enhancement was seen inferior in the late gadolinium enhancement sequences (Figure 6). The subsequent coronary MRI showed an interarterial course of the RCA between the pulmonary trunk and the ascending aorta. The proximal part of the RCA appeared to be (intussusceptive) intramural within the aortic root wall (Figure 7).
Surgical correction was performed with a reimplantation of the atypical originating RCA to the right side of the aortic root. The muscle bridge on the left anterior descending artery was not unroofed because of the missing perfusion failure in the left anterior descending artery territory and the clear target of fibrosis in the RCA territory. The operative correction proceeded without any difficulties.
The present case of a young athlete experiencing sudden cardiac death illustrates the importance of suspecting the presence of coronary anomalies, especially in younger patients. In this case, the RCA arose abnormally from the left coronary cusp coursing partially intramural in the aortic root and interarterial between the aorta and the pulmonary trunk. The intramural course leads to partially hypoplastic portions, which, especially during exertion, causes a further compression of these parts that lead to deterioration of the hemodynamic relevance of these arteries.1–3
Medical treatment, coronary angioplasty with stent deployment, and surgical repair (unroofing, debranching, or bypassing) are possible treatment options.1 In this case, the decision was made for surgical treatment. An off-label stent deployment would not have been feasible because of the difficult intubatable RCA, as well as the increased risk of restenosis and stent thrombosis with involvement of a large territory at risk for myocardial infarction.
The prevalence of coronary anomalies is reported with ≈5% of coronary anomalies.1 The subgroup of anomalous origination of a coronary artery from the opposite sinus is a serious anomaly with an incidence of ≈1% of all coronary anomalies. Coronary angiography and cardiac MRI should be performed in ambiguous situations. The relevance of myocardial bridges is controversially discussed. They were observed in >1% of coronary anomalies in an unselected population, which is considered to be a normal variant.4 The intramural-interarterial course of a coronary artery is an especially severe and rare disorder. The cause and the best medical treatment for this disorder remain unclear.
The online-only Data Supplement is available with this article at http://circulation.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.113.001418/-/DC1.
- © 2013 American Heart Association, Inc.
- Angelini P
- Angelini P,
- Velasco JA,
- Flamm S
- Cox ID,
- Bunce N,
- Fluck DS