Congenital Left Main Coronary Artery to Main Pulmonary Artery Fistula with Bicuspid Aortic Valve: A Case Report and Review of Literature

Abstract

Congenital coronary artery fistulas (CAFs) are uncommon abnormalities. A connection between the left main stem and main pulmonary artery is extremely rare. Congenital CAFs are frequently associated with another congenital heart disease. Associated anomalies include atrial septal defect, tetralogy of Fallot, patent ductus arteriosus, ventricular septal defect, and pulmonary atresia. Association of bicuspid aortic valve with CAF has not been reported in literature to date. We report on a 68-year-old man with congenital left main to pulmonary artery fistula associated with bicuspid aortic valve and moderate aortic stenoses, who underwent successful aortic valve replacement with ligation of CAF and also review the natural history, pathophysiology, and management of CAF.

Coronary arteriovenous fistula with coexisting atrial septal defect

“…In 20–45% cases, other associated congenital cardiac anomalies are evident 4. Drainage is primarily into the right heart system (90%) with 15–17% PA drainage 2 4.…”

“…In 20–45% cases, other associated congenital cardiac anomalies are evident 4. Drainage is primarily into the right heart system (90%) with 15–17% PA drainage 2 4. Complications include angina (coronary steal), heart failure, myocardial infarction and death 1 4.…”

“…Drainage is primarily into the right heart system (90%) with 15–17% PA drainage 2 4. Complications include angina (coronary steal), heart failure, myocardial infarction and death 1 4. Concurrent ASD is reported to have a significantly higher Qp/Qs ratio 2.…”

“…Concurrent ASD is reported to have a significantly higher Qp/Qs ratio 2. Despite coronary angiogram being the gold standard of diagnosis, alternate modalities such as TEE, CT angiogram and MRI are increasingly being advocated 3 4…”

Detection of patent ductus arteriosus with intraoperative transesophageal echocardiography in a patient undergoing closure of coronary artery to pulmonary artery fistula

“…Coronary artery to pulmonary artery fistula is an unusual vascular anomaly, and the shunt ratio of this fistula is usually small [ 1 , 2 ]. This anomaly is also known to be associated with various congenital heart diseases, such as tetralogy of Fallot and patent ductus arteriosus (PDA) [ 3 , 4 ]. In this case, we calculated the left-to-right shunt ratio after placement of a pulmonary artery catheter in a patient undergoing closure of coronary artery to pulmonary artery fistula, and the ratio was unexpectedly large.…”