BACKGROUND: The study evaluates the long-term results of surgery for anomalous left coronary artery from the pulmonary artery (ALCAPA) with special attention on the left ventricular (LV) function and mitral regurgitation. METHODS: Twenty-one children underwent surgery for ALCAPA over 23 years (1987-2010). All patients underwent establishment of a two-coronary system, by direct reimplantation (n = 13) or by intrapulmonary tunnel technique (n = 8), with concomitant mitral valve repair in one. The follow-up echocardiograms were evaluated to assess LV function and mitral regurgitation. RESULTS: Five patients died. The age of the nonsurvivors was lower, 4.2 ± 1.3 versus 22.7 ± 29.4 months, P = .04. All nonsurvivors had moderate or severe mitral regurgitation preoperatively and higher LV diameter z score than the survivors: 11.8 (9-14.6) versus 4.6 (1.9-13.1), P = .01. At last follow-up, all survivors were asymptomatic; the diastolic LV diameter was normal, with z scores: 0.3 (0.1-1.9) versus 7 (1.9-14.6) preoperatively, P = .001, as was the LV ejection fraction: 66% (61%-78%) versus 38% (16%-70%) preoperatively, P = .001. Fifteen patients had moderate or severe mitral regurgitation at initial presentation and it eventually regressed to insignificant in all survivors (P = .001). No subsequent interventions on the coronary arteries or the mitral valve were needed. Four patients with intrapulmonary tunnel had mild suprapulmonary obstruction with Doppler peak gradients between 20 and 30 mm Hg. CONCLUSIONS: In our experience, establishment of a two-coronary circulation without mitral valve repair leads to normalization of LV dimension and systolic function and to improvement of mitral regurgitation in the surviving patients. Mortality is related to low age and to the associated higher degree of LV dysfunction.
BACKGROUND: The study evaluates the long-term results of surgery for anomalous left coronary artery from the pulmonary artery (ALCAPA) with special attention on the left ventricular (LV) function and mitral regurgitation. METHODS: Twenty-one children underwent surgery for ALCAPA over 23 years (1987-2010). All patients underwent establishment of a two-coronary system, by direct reimplantation (n = 13) or by intrapulmonary tunnel technique (n = 8), with concomitant mitral valve repair in one. The follow-up echocardiograms were evaluated to assess LV function and mitral regurgitation. RESULTS: Five patients died. The age of the nonsurvivors was lower, 4.2 ± 1.3 versus 22.7 ± 29.4 months, P = .04. All nonsurvivors had moderate or severe mitral regurgitation preoperatively and higher LV diameter z score than the survivors: 11.8 (9-14.6) versus 4.6 (1.9-13.1), P = .01. At last follow-up, all survivors were asymptomatic; the diastolic LV diameter was normal, with z scores: 0.3 (0.1-1.9) versus 7 (1.9-14.6) preoperatively, P = .001, as was the LV ejection fraction: 66% (61%-78%) versus 38% (16%-70%) preoperatively, P = .001. Fifteen patients had moderate or severe mitral regurgitation at initial presentation and it eventually regressed to insignificant in all survivors (P = .001). No subsequent interventions on the coronary arteries or the mitral valve were needed. Four patients with intrapulmonary tunnel had mild suprapulmonary obstruction with Doppler peak gradients between 20 and 30 mm Hg. CONCLUSIONS: In our experience, establishment of a two-coronary circulation without mitral valve repair leads to normalization of LV dimension and systolic function and to improvement of mitral regurgitation in the surviving patients. Mortality is related to low age and to the associated higher degree of LV dysfunction.