Unusual case of anomalous origin of the left coronary artery from the distal right pulmonary artery.

A full-term born, female baby with prenatal diagnosis of aortic coarctation underwent successful coarctectomy at 8 days of life. However, despite full anti-congestive therapy, she showed failure to thrive (body weight, 3.5 kg; 25th percentile) and congestive heart failure. At 2 months of age, two-dimensional echocardiography revealed a progression of mitral regurgitation from mild to moderate, dilated left ventricle, and decreased left ventricular ejection fraction of 40%. Cardiac angiography revealed an anomalous origin of the left main coronary artery from the distal right pulmonary artery, since only the right coronary artery was visualized (Fig 1), and the patient was scheduled for surgery. Through a midline sternotomy, the aorta was cannulated close to the origin of the innominate artery, and a single venous cannula was inserted in the right atrium. On moderately hypothermic (32°C) cardiopulmonary bypass, we identified the left coronary artery, originating together with the seno-atrial nodal artery, from the right pulmonary artery, next to the hilar portion (Fig 2;DtAo = distal aorta; LCa = left coronary artery; Pa = pulmonary artery; PrAo = proximal aorta; RA = right atrium; RV = right ventricle; S-ANa = senoatrial nodal artery; SVC = superior vena cava). In Figure 2, it is valuable to note the anomalous origin of the left coronary artery and of the senoatrial node artery from the distal right pulmonary artery. After delicate dissection of the right pulmonary artery, the aorta was cross clamped close to the aortic cannula, and a cold cardioplegic cardiac arrest was obtained. After the aorta was completely transected, the left coronary ostium was identified through the lumen of the right pulmonary artery and was then harvested with a large button of the right pulmonary arterial wall (Fig 3;DtAO = distal aorta; LCa = left coronary artery; Pa = pulmonary artery; PrAO = proximal aorta; RA = right atrium; RV = right ventricle; S-ANa = seno-atrial nodal artery; SVC = superior vena cava). Note the coronary button of the anomalous left coronary artery detached from the distal right pulmonary artery. The coronary button was then re-implanted directly, with a trap door technique, using a continuous 8-0 polypropylene suture anastomosis to the left lateral wall of the ascending aorta, avoiding compression or kinking from the right pulmonary branch (Fig 4;Ao = aorta; LCa = left coronary artery; Pa = pulmonary artery; RA = right atrium; RPa = right pulmonary artery; RV = right ventricle; SVC = superior vena cava). Note the reimplanted left coronary artery on the neo-ascending aorta. Eventually, the aortic ends were directly anastomosed with a continuous 7.0 polypropylene suture. After the aortic cross clamp was released on the beating heart, the right pulmonary wall was reconstructed with autologous pericardium. Despite limited cross-clamp time (61 minutes), the patient could not be weaned off cardiopulmonary bypass and required left ventricular assist device (Bio-Medicus; Medtronic Inc, Eden Prairie, MN) for 48 hours postoperatively. After the chest was closed, the remaining postoperative stay was uneventful, and the patient was discharged home on postoperative day 8 on oral digoxin and furosemide. At her 6-month follow-up, an echocardiogram showed a normalized left ventricular function ejection fraction of 70%, with trivial mitral regurgitation.