A Single-Center Retrospective Study of Patients with ALCAPA

Abstract

Introduction: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare but fatal disease. Untreated cases have a first-year mortality rate of 90%. We aimed to evaluate the early outcomes of patients who were operated on for ALCAPA in our clinic. Patients and Methods: We retrospectively reviewed the cases of seven patients who were operated on in our clinic for ALCAPA between 2013 and 2019. Preoperative, early postoperative, and first year echocardiography results were compared. Results: Five patients (71.4%) underwent reimplantation and two patients (28.6%) underwent Takeuchi repair. The median age was 25 months and 71.4% (n= 5) of the patients were aged ≤1 year. In preoperative echocardiograms, mean ejection fraction (EF) was 32.1 ± 4.9% and mean preoperative MR grade was 2.1 ± 0.7. Two patients with severe MR (grade 3) underwent simultaneous mitral annuloplasty. The mean EF % significantly increased (p= 0.023) and the mean MR grade significantly decreased (p= 0.039, p< 0.05) in the early postoperative period. This finding was not statistically associated with surgical technique or age. The mortality rate was 14.3% (n= 1). The patient who died had severe preoperative LV dysfunction and MR, which did not improve after surgical intervention. Conclusion: Early improvement in LV function and MR grade after ALCAPA repair is crucial for survival regardless of age. Moderate or mild functional MR may spontaneously improve after the surgical correction of LV dysfunction. Simultaneous mitral valve intervention is needed in cases of severe MR and patients aged ≥1 year.