Berra Zümrüt Tan Recep1, Aybala Tongut2, Ali Can Hatemi1, Nihat Cine1, Eylem Tunçer1, Hakan Ceyran1

1Clinic of Pediatric Cardiovascular Surgery, Koşuyolu High Specialization Training and Research Hospital, İstanbul, Türkiye
2Clinic of Pediatric Cardiovascular Surgery, Children’s National Hospital, Washington, United States

Keywords: Left coronary artery; pulmonary artery; coronary artery reimplantation


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.

Cite this article as: Tan Recep BZ, Tongut A, Hatemi AC, Cine N, Tunçer E, Ceyran H. A single-center retrospective study of patients with alcapa. Koşuyolu Heart J 2022;25(2):200-207.

Ethics Committee Approval

The study protocol was approved by the Kartal Koşuyolu High Specialization Training and Research Hospital Eth- ics Committee. The study was conducted in accordance with the principles of the Declaration of Helsinki (Decision no: 2021/1/423 Date: 12.01.2021).

Peer Review

Externally peer-reviewed.

Author Contributions

Concept/Design - BZTR; Analysis/Interpreta- tion - BZTR, NC; Data Collection - NC; Writing - BZTR, ACH; Critical Revision - ACH; Final Approval - ACH, HC; Statistical Analysis - AT, ET; Overall Responsibility - ACH, HC.

Conflict of Interest

The authors declared that there was no conflict of interest during the preparation and publication of this article.

Financial Disclosure

The authors declared that this study has received no financial support.