The surgical outcome of anomalous origin of the left coronary artery from the pulmonary artery.

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly which represents one of the most common causes of myocardial ischemia and infarction in children. This anomaly, if left untreated, results in a very high mortality rate within the first year of life. Yet, immediate surgical correction can lead to excellent results.

Objectives: The present study aimed to determine the surgical outcome of ALCAPA.

Methods: This study was conducted on 53 patients with ALCAPA operated from January 2005 to December 2012. Surgical repair was carried out as soon as the diagnosis was made. Surgery was thus undertaken on an urgent basis (within 48 hours) in the patients with congestive heart failure or critical clinical status and on a semi- elective basis (within a few days) in the remaining children. Operations for all the patients were performed through a median sternotomy using established standard cardiopulmonary bypass technique. Grouped variables were compared using chi-square test with Yates' correction. Besides, McNemar's test was used to assess the relationship between preoperative ejection fraction and mitral incompetence. All the analyses were performed using the SPSS statistical software, version 11.5 (SPSS Inc., Chicago, IL).

Results: The patients' median age at presentation was 4 months. The mean preoperative ejection fraction was 36.5%. The results showed a significant relationship between age at presentation and impairment of ejection fraction (P < 0.001). At first, 23% of our patients presented with ejection fraction < 35%. However, 6 months after the operation, the ejection fraction improved to a mean of 53.07% (SD = 8.5) ranging from 38 - 66%. There were 5 postoperative hospital deaths with an overall mortality rate of 9.6%.

Conclusions: Excellent results with desirable long-term outcomes can be achieved in the infants with ALCAPA using coronary artery implantation techniques. The best potential for recovery of the left ventricular function is in younger symptomatic infants despite the worst initial presentation. Normalization of cardiac function is expected within the first year in all operative survivors with a patent dual coronary system.