Anomalous origin of the circumflex coronary artery from the pulmonary artery: a case report

Abstract Case description: A 4-year old boy without significant prior history, presented with pronounced dyspnea. During clinical examination only mild hepatomegaly was present. Thoracic radiography showed significant cardiomegaly. Echocardiography revealed left ventricular dilatation and poor systolic function. Both Troponin and NT-pro-BNP level were elevated. Treatment with diuretics and inotropics was started with clinical improvement. The diagnosis of an anomalous left circumflex artery out of the right pulmonary artery was suspected by coronarography and confirmed by coronary CT. Coronary reimplantation was performed without complications. Subsequently, left ventricular dimensions normalized progressively in the next months. Discussion: Without congenital heart disease, an anomalous left circumflex artery arising from the pulmonary artery is rarely present. Symptoms and prognosis are determined by the amount of collateral circulation to provide adequate myocardial perfusion. Diagnosis of an anomalous left circumflex artery (LCx) is mostly made at adult age. However, our patient presented at young age. Even though the origin of both coronary arteries was normal, a coronary anomaly lies at the origin of his cardiac dysfunction. Treatment consisting of surgical reimplantation or coronary bypass is the primary choice. Conclusion: We present this case to highlight the importance of having a high index of suspicion of coronary artery abnormalities in children with dilated cardiomyopathy, as the prognosis can be very favorable after surgical repair. Transthoracic echocardiography is not always conclusive in showing a normal origin of both coronary arteries and certainly not for visualizing the LCx reliably. Therefore, further imaging should be considered in children with a new diagnosis of unexplained DCM.