An exceedingly rare coronary anomaly - right coronary artery take-off from the left ventricular outflow tract

• Published in: The Thoracic and Cardiovascular Surgeon
• Main Authors: Biermann, D., Subbotina, I., Stumm, M., Arndt, F., Schemm, A., Arunagirinathan, U., Reichenspurner, H., Riso, A., Sachweh, J.S.
• Format: Conference Proceeding
• Language: English
• Published: 10.02.2014
• ISSN: 0171-6425; 1439-1902
• DOI: 10.1055/s-0034-1367352

Introduction: The incidence of congenital coronary anomalies in the general population is 0.2-1.2% and in most cases of no hemodynamic relevance. However, if coronary perfusion is altered, e.g. in large coronary fistulas, myocardial malfunction will develop over time. Thus, surgical or interventional therapy is generally warranted. We present an exceedingly rare case with right coronary artery (RCA) take-off from the left ventricular outflow tract (LVOT) and absent proximal ostium of the RCA. Background: A 15 month-old otherwise healthy boy was presented to rule out an atrial septal defect. Initial echocardiography revealed a large diastolic flow from the RCA to the LVOT as the only pathological finding. Electrocardiogram (ECG) was unremarkable. Subsequent angiography confirmed retrograde perfusion of the heavily dilated RCA via multiple collaterals from the left coronary artery and diastolic drainage into the LVOT. An RCA ostium in the aortic root was not present. Although, at this time there were no criteria for myocardial malfunction, long-term sequaele of fistula related coronary flow are well known. Thus, surgical therapy was recommended. Surgery was done on mild hypothermic cardiopulmonary bypass with cardioplegic arrest and consisted of closing the RCA-LVOT connection at ventricular level and creating a communication between the aortic root and the proximal RCA. The operative and postoperative course was uneventful and the patient was discharged home on postoperative day 8. At follow-up 3 months after surgery the patient was well, ECG was unremarkable and echocardiography revealed a closed RCA-LVOT connection and a typical diastolic perfusion of RCA via the aortic root. Discussion: The spectrum of coronary anomalies is wide and is demanding in terms of therapeutically decision making. This is particularly the case in patients with no objective data for myocardial malfunction. However, if relevant pathophysiology (e.g. large fistula with massive coronary artery dilatation) is present, restoration of normal anatomy and physiology early in life may prevent from myocardial deterioration over time.