Early and midterm results after surgical repair of anomalous origin of the left coronary artery from the pulmonary artery

Abstract

Introduction/Objective. The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is rare congenital disease, which causes myocardial ischemia and subsequent heart failure in infants. The aim is early and mid-term follow up evaluation of the heart function after surgical repair of ALCAPA. Methods. Investigation was retrospective and included medical records of the ALCAPA patients treated surgically, between 2009 and 2017, at the tertiary referent heart center. Results. Five patients (four girls) with coronary anomaly were included in the study. All patients had significantly increased left ventricular end diastolic diameter (z-score 6.6 ± 2.43) and left atria size (z-score 3.09 ± 0.37), along with decreased systolic function (ejection fraction 34.8 ± 7.4% and fractional shortening 15.5 ± 3.4%). The surgery was performed on average at the age of 8.2 ± 7.8 months. Operative treatment was associated with early improvement in echocardiographic parameters (except the size of the left atria). Patients were followed for 4.5 ± 2.6 years. Improvement in echocardiographic parameters was age-related. Patients under four months had recovery early after surgery, those treated at 5.5–6 months of age had normalization after 12 months, and patient who was recognized in the second year of life had late recovery (after ≥ 24 months). Conclusion. Operative treatment in the first 3–4 months of life is related with the most favorable prognosis and rapid normalization of the echocardiographic parameters.

Keywords

• alcapa
• cardiomyopathy
• echocardiography