Long term results of surgical management of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) and mitral incompetence

Abstract

Establishment of two coronary systems is not the end point of challenge in management of anomalous origin of the left coronary artery from pulmonary artery (ALCAPA). To touch or not touch the mitral valve if there is severe mitral regurgitation is still a point of big challenge, another point is the timing and criteria for mechanical support (ECMO). Forty-seven cases with a median age of 1.29 years (28 days to 8 years) underwent ALCAPA repair from January 1985 to July 2012. Seven cases (14.89%) had associated lesions. Ligation of anomalous left coronary artery was performed in four cases (8.51%), Takeuchi’s repair was done for three cases (6.38%) and 40 cases (85.1%) underwent direct aortic implantation of left coronary artery. Preoperative echo showed moderate to severe left ventricular dysfunction with severe mitral regurgitation in 15 cases (31.91%). Mortality rate was 17.02% (eight cases). Four cases (8.51%) needed ECMO support in operating room and two patients needed ECMO support in intensive care unit. Mitral valve repair was adopted in three cases (6.38) and mitral valve replacement was performed in one case (2.12%). Early postoperative echo showed moderate to severe mitral regurge with severe LV dysfunction in eight cases (17.02%) which improved in the first year of follow up. One case (2.12%) returned back with left coronary artery origin stenosis after Takeuchi’s repair. Early diagnosis and repair of ALCAPA improve outcome of surgery. Aortic implantation of left coronary artery is the best technique of ALCAPA repair and can be done easily. In severe mitral regurgitation, it is better not to touch the mitral valve as repair is very technically demanding especially in small babies without expected good results and consumes a lot of cross clamp time with subsequent bad impact on surgical outcome. Mechanical support should be decided according to the degree of LV dysfunction and severity of mitral valve regurgitation.