Minimally Invasive Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) and Mitral Valve Repair

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This video presents the case of a minimally invasive mitral valve (MV) and anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) repair in a twenty-one-month-old female patient.

The Patient

An echocardiography showed poor left ventricle contractility with 25 percent LVEF and a small apical aneurysm, severe MV insufficiency that was due to anomalous posterior leaflet and annular dilatation, and the left coronary artery arising from anterior sinus of the pulmonary artery. Computed tomography confirmed an ALCAPA diagnosis and showed dilatation of left chambers with no additional abnormalities.

The operative plan consisted of a right axillary minithoracotomy in the fourth intercostal space, central cardiopulmonary bypass (CPB) cannulation, aortic cross-clamp and cardioplegia administration, MV repair through the left atrium, and left coronary artery translocation.

The Surgery

To gain right axillary access, the patient was rotated to be in the left lateral decubitus position. A horizontal skin incision was made over the fourth intercostal space, between the anterior and posterior axillary lines. The thymus was then excised. After pericardiotomy, cannulation for cardiopulmonary bypass was performed. Suture loops were used to snare the superior vena cava and inferior vena cava. Before the aortic cross-clamp, the main pulmonary artery (PA) was also snared.

After aortic cross-clamp was applied, antegrade cold blood cardioplegia was administered, and a left atrial incision was performed. Mitral valve revision showed marked annulus dilation and A2 segment prolapse because of lateral papillary muscle fibrosis and an anomalous posterior leaflet. The surgical team decided to perform Alfieri edge-to-edge repair between A2 and P2 segments and suture an MV annuloplasty in the posterior leaflet region close to lateral commissure. Bent instruments and bayonet forceps were used throughout the repair. A water probe showed minimal regurgitation and the left atrium was closed. 

Next, the pulmonary artery was transected 1 cm above the pulmonary valve. The left coronary artery ostium was resected with the wide, 10 x 20 mm area of the pulmonary artery wall, which was used to elongate the coronary vessel. Surgeons then performed tubular elongation of the left coronary artery at the expense of the pulmonary artery wall. To improve the exposure of PA, the pericardial stay sutures at the right side were left loose and pulled tighter at the left side. This maneuver rotated the heart and positioned the left coronary artery in the middle of operation field. After completing the elongation of the left coronary artery, the wall defect in the PA was reconstructed using a pericardial patch.

The ascending aorta was then opened anteriorly to the left coronary sinus. A left coronary artery to aorta anastomosis was performed and the rest of aortotomy was closed. The inferior part of the distal PA anastomosis was controlled using a side-biting clamp that was positioned during the aortic cross-clamping. Then the aorta was unclamped and deaeration of the left chambers was performed. Pulmonary artery anastomoses between the distal PA and proximal PA that was reconstructed with the autopericardium was done again with the beating heart and sinus rhythm was restored. CPB was then disconnected and drainage and wound closure were completed.

The duration of the operation time was 450 min and CPB time was 275 min. The aorta was cross-clamped for 159 min. The patient’s postoperative course was unremarkable. She stayed in the intensive care unit for two days and was discharged from the hospital on the fifth postoperative day. A postoperative coronary angiograph was performed two weeks after operation and showed good results.

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