Effectiveness and Safety of Mitral Valve Plasty in Patients with an Anomalous Origin of the Coronary Artery from the Pulmonary Artery

Abstract

The study aimed to determine the effectiveness and safety of anomalous coronary artery from pulmonary artery (ACAPA) patients with moderate or severe mitral valve regurgitation (MVR) receiving mitral valve plasty (MVP) concurrently. Consecutive ACAPA patients undergoing surgery between 2015 and 2021 were retrospectively included. Patients were divided into three groups: moderate MVR without MVP (non-MVP (moderate) N = 14), moderate MVR with MVP (MVP (moderate) N = 13), and severe MVR with MVP (MVP (severe) N = 13). The primary safety endpoint was in-hospital surgery-related complications. The primary effectiveness outcome was left ventricular ejection function (LVEF) and left ventricular end-diastolic diameter (LVEDD) z-score at 2- and 24-month follow-ups. Multivariable linear regression models were used to obtain the β coefficient. The median age of the included patients was 7.5 years (IQR 1.4-26.5). The in-hospital surgery-related complication rates were 7.1%, 15.4%, and 7.7% in non-MVP (moderate), MVP (moderate), and MVP (severe) groups, separately. At the 2-month follow-up, the non-MVP (moderate) group had a better LVEF and LVEDD z-score compared with the MVP (moderate) group (LVEF β = 9.22, 95%CI 1.09 to 17.35; LVEDD z-score β = -2.49, 95%CI -4.53 to -0.45). At the 24-month follow-up, the LVEF of all patients and the LVEDD z-score of 90% of patients in the three groups returned to normal. For ACAPA patients with moderate MVR, MVP was not necessary, especially for pediatric patients (age < 3 years) and patients with secondary MVR. Further studies for ACAPA patients with severe MVR are still needed.

Keywords: anomalous coronary artery from the pulmonary artery; anomalous origin of the left coronary artery from the pulmonary artery; mitral valve regurgitation; simultaneous mitral valve plasty; total coronary artery from the pulmonary artery.

Figure 1

Flow chart of patient selection and follow-up. Abbreviation: ALCAPA, anomalous origin of the left coronary artery from the pulmonary artery; ARCAPA, anomalous origin of the right coronary artery from the pulmonary artery; MVR, mitral valve regurgitation; MVP, mitral valve plasty; TCAPA, total anomalous origin of the coronary arteries from the pulmonary artery.

Figure 2

Longitudinal assessment for the primary outcome of ACAPA patients with and without mitral valve plasty. (A) LVEF% at pre-operation, post-operation, and follow-up. (B) LVEED z-score at pre-operation, post-operation, and follow-up. (C) Longitudinal changes in the degree of mitral valve incompetence with the corresponding number of patients at risk. Abbreviation: LVEED, left ventricular end-diastolic diameter; LVEF, left ventricular ejection fraction; MVR, mitral valve regurgitation; MVP, mitral valve plasty; Postop, post-operation; Preop, pre-operation; Pts, patients.

Figure 3

Longitudinal assessment for the primary outcome of each TCAPA patient with and without mitral valve plasty. (A) LVEF% of each TCAPA patient at pre-operation, post-operation, and follow-ups. (B) LVEED z-score of each TCAPA patient at pre-operation, post-operation, and follow-ups. (C) Longitudinal changes in the degree of mitral valve incompetence with the corresponding number of patients at risk. A (blue) indicated non-MVP (moderate) group, B (orange) indicated MVP (moderate) group, and C (purple) indicated MVP (severe) group. Abbreviation: LVEED, left ventricular end-diastolic diameter; LVEF, left ventricular ejection fraction; MVR, mitral valve regurgitation; Postop, post-operation; Preop, pre-operation; Pts, patients; TCAPA, total anomalous origin of the coronary arteries from the pulmonary artery.