Long-Term Systolic and Diastolic Function in Patients with Repaired Anomalous Left Coronary Artery from the Pulmonary Artery.

Abstract

Long-term data on systolic and diastolic function following anomalous left coronary artery from the pulmonary artery (ALCAPA) repair are limited. We evaluated trends in these parameters and clinical outcomes over long-term follow-up. We reviewed pediatric patients who underwent ALCAPA repair from 1983-2021with ≥ 6 months of echocardiographic follow-up, assessing diastolic function indices including indexed left atrial (LA) volume, mitral inflow (E/A, E-wave deceleration time [DT]), tissue Doppler E', and E/E'. A diastolic function score (DFS; range 0-4) was calculated from these indices, with persistent diastolic dysfunction (DD) defined as DFS ≥ 2 at ≥ 6 months post-repair. Kaplan-Meier analysis assessed time-to normalization for left ventricular (LV) ejection fraction (EF) and volumes. Regression splines analyzed trends, and Fisher's exact test evaluated associations with a composite clinical outcome (heart failure or ventricular tachyarrhythmia). Among 37 patients, median preoperative LV EF was 26.5% [IQR 20.8, 45]). Median time-to EF normalization was 11.3 months [IQR 3.1, 72], with 34 patients (92%) normalizing. LV volumes down-trended, but 27% remained dilated. Persistent LV DD was observed in 21 patients (57%), only three of which normalized. Higher LA volume at discharge was associated with persistent LV DD (p = 0.047). E' Z-scores remained depressed, and E/E' Z-scores elevated (p > 0.05). Persistent LV DD correlated with the composite (p = 0.05), whereas abnormal LV EF did not (p = 1.00). LV systolic function normalized in most patients post-ALCAPA repair, but 27% exhibited persistent LV dilation. The majority of patients had persistent DD, and it tended to remain abnormal long-term. Persistent DD was associated with worse clinical outcomes.