Case 2 -

Anomalous Origin of Circumflex Coronary Artery from Right Sinus of Valsalva Stephen Cohle, Spectrum-Health Blodgett Campus, Grand Rapids, MI

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Clinical History This 6-year-old boy, wearing a life jacket, fell backward into a swimming pool. Seconds later he was removed from the pool. He was pronounced dead shortly thereafter in an emergency room. He had no family history of sudden death and no history of seizures, syncope, or chest pain. A physical exam 4 months before was unremarkable. Pertinent Laboratory Data: A drug screen was negative. Shown is a photograph of the right sinus of Valsalva. To the right of the round right coronary ostium is the slit like ostium of the anomalous origin of the circumflex coronary artery (see arrow in enlargement). Case 2 - Figure 1 Coronary ostia showing anomalous origin of circumflex coronary artery from right sinus of Valsalva Pathological/Microscopic Findings and any Immunohistochemical or Other Studies: The circumflex artery arose anomalously from the right sinus of Valsalva. There was no myocardial infarct. Differential Diagnoses: Normal variation vs cause of death Final Diagnosis: Anomalous Origin of Circumflex Coronary Artery from Right Sinus of Valsalva Case Discussion: Anomalous origin of the coronary arteries as a cause of death have an acute takeoff from the aorta, pass through the aorta wall, and usually pass between the aorta and pulmonary artery. Victims often have physical or emotional stress at the time of collapse. Review of the Literature/Treatment Options (if applicable): The most common vessel to have anomalous origin is the left coronary artery arising from the right sinus of Valsalva. Treatment, which is mandatory in this variation (and recommended in patients with other variations who are symptomatic) includes bypass grafting, re-implantation, and unroofing. The mechanism of death is ischemia, usually but not always including infarct. Conclusion(s): The location, shape, and course of anomalous coronary arteries needs to be documented, as well as the activity at the time of collapse. References: Cohle SD, Graham MA, Pounder DJ Nonatherosclerotic sudden coronary artery death. Pathology Annual 1986; 21 (part 2): 217-249. Basso C, Maron BJ, Corrado D, et al. Clinical profile of congenital coronary artery anomalies with origin from the wrong aortic sinus leading to sudden death in young competitive athletes. JACC 2000; 35: 1493-1501 Basso C, Corrado D, Thiene G. Congenital coronary anomalies as an important cause of sudden death in the young. Cardiol in Review 2000; 9: 312-317 Brothers J, Stephens D, Gaynor W et al. Anomalous aortic origin of a coronary artery with an interarterial course. JACC 2008; 51: 2062-2064 Cohle S and Sampson B. The negative autopsy: Sudden cardiac death or other? Cardiovasc Pathol; 10: 219-222 Catanzaro J, Makaryus A, Catanese C. Sudden cardiac death associated with an extremely rare coronary anomaly of the left and right coronary arteries arising exclusively from the posterior (non-coronary) sinus of Valsalva. Clin Cardiol 2005; 28: 542-544 Davies J, Burkhart H, Degrani J, et al. Surgical management of anomalous aortic origin of a coronary artery. Ann Thor Surg 2009; 88: 844-848 Gersony W. Management of anomalous coronary artery from the contralateral coronary sinus. JACC 2007; 50: 2083-2084 Bernanke, D and Velkey, J. Development of coronary artery blood supply, changing concepts and current ideas. The Anatomical Record (2) Anat. 2002, 269: 198-208. Tomanek, R. Formation of coronary vasculature during development. Angiogenesis 2005; 8: 273-84.