alexa Anomalous origin of the left coronary artery from the pulmonary artery associated with severe left ventricular dysfunction: results in normothermia.
Genetics & Molecular Biology

Genetics & Molecular Biology

Journal of Genetic Syndromes & Gene Therapy

Author(s): Belli E, Roussin R, Ly M, Roubertie F, Le Bret E,

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Abstract BACKGROUND: Repair of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) carries a high risk of operation, particularly in those with poor left ventricular function. In this study, we assessed the outcomes of patients who presented with severe preoperative left ventricular dysfunction (shortening fraction <15\%) who underwent the repair under normothermic bypass. METHODS: Since September 2002, 21 infants with severe left ventricular dysfunction underwent surgical repair using a direct coronary reimplantation technique. Mean age of patients was 5.3 +/- 3.8 months. Moderate or severe mitral regurgitation present in 5 patients was not addressed at the operation. Repair was performed under normothermic cardiopulmonary bypass and myocardial protection was achieved by intermittent antegrade normothermic blood cardioplegia. RESULTS: Mean cardiopulmonary bypass and cross-clamp times were 153.5 +/- 51 and 50 +/- 14.5 minutes, respectively. There was one (4.7\%) early death because of sudden cardiac arrest 48 hours after successful weaning from mechanical ventilation. One patient required urgent left coronary artery stenting on the 34th postoperative day because of early reimplantation failure. There was no late death. Echocardiographic evaluation revealed a significant improvement in terms of shortening fraction (preoperative, 10.3 +/- 3.2\% vs follow-up, 33 +/- 7.7\%, p < 0.0001). CONCLUSIONS: Our results suggest that the repair of anomalous origin of the left coronary artery from the pulmonary artery can be accomplished with acceptable mortality and morbidity rates even in patients having severely depressed left ventricular function. Normothermic cardiopulmonary bypass is safe and effective in children who have a limited tolerance to further ischemic insult. 2010 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved. This article was published in Ann Thorac Surg and referenced in Journal of Genetic Syndromes & Gene Therapy

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