The outcome after surgical repair of anomalous origin of the left coronary artery from the pulmonary trunk (ALCAPA) remains incompletely defined, and transplantation has been advocated for patients with severe left ventricular dysfunction. We reviewed our institutional experience with ALCAPA since 1977 in 38 patients whose repair established a dual coronary circulation (7 early cases with LCA ligation were excluded) to assess whether cardiac function predicts outcome, the use of left ventricular assist device (LVAD), and postoperative complications of the current operative techniques. Surgical techniques included direct coronary reimplantation to the aorta in 11 pts, intrapulmonary baffling (Takeuchi) when the LCA could not reach the aorta directly in 25 pts, and modified Takeuchi in which an extrapulmonary baffle was fashioned from anterior and posterior walls of the pulmonary artery in 2 pts. The operative survival was 91%, 88%, and 100%, respectively (89% overall). All pts who died were < lyr old (3/19 Takeuchi, 1/10 reimplantation). Since its introduction at our institution in 1990, LVAD has been used in 4/16 pts, 1 Takeuchi pt who died and 3 reimplantation pts who all survived. Survival in pts < 1 yr old was not different in those with severe dysfunction (shortening fraction (SF) < 15%,18/20) compared with moderate dysfunction (SF 16–25%, 4/5). Function returned to normal in all pts for whom followup is known past one year, regardless of the severity of preoperative dysfunction. Postoperative complications for Takeuchi pts included baffle leaks in 6/21 pts who underwent postoperative echocardiogram, supravalvar pulmonary stenosis in 13/20 pts for whom followup is known (< 25 mmHg in 9 pts, 26-50 mmHg in 2 pts, > 50 mmHg in 2 pts requiring reoperation), and progressive aortic regurgitation requiring aortic valve replacement in 1 pt. There were no complications in the two modified Takeuchi pts (6 and 12 mos followup) or in reimplantation pts. (1) The degree of LV dysfunction at presentation does not predict survival in pts < 1yr old, (2) current operative survival for ALCAPA does not justify consideration of transplantation, (3) LVAD may improve survival for some patients and should be available for postoperative management, and (4) supravalvar pulmonary stenosis occurs in a majority of Takeuchi pts, some of whom require reoperation; a modified technique may improve this postoperative complication rate.
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