McGeoghegan Patrick B, Borgelt Luke, Lipsitz Stuart, Sleeper Lynn A, Kobayashi Ryan, Feins Eric, Baird Chris, Thatte Nikhil, Friedman Kevin
Department of Cardiology, Boston Children's Hospital, Boston, MA, USA.
, 1 Baylor Plz, Houston, TX, 77030, USA.
Pediatr Cardiol. 2025 Jul 18. doi: 10.1007/s00246-025-03957-8.
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.
关于肺动脉起源异常左冠状动脉（ALCAPA）修复术后收缩和舒张功能的长期数据有限。我们评估了这些参数的长期随访趋势及临床结局。我们回顾了1983年至2021年接受ALCAPA修复且超声心动图随访≥6个月的儿科患者，评估舒张功能指标，包括左心房（LA）容积指数、二尖瓣血流（E/A、E波减速时间[DT]）、组织多普勒E'和E/E'。根据这些指标计算舒张功能评分（DFS；范围0 - 4），持续性舒张功能障碍（DD）定义为修复后≥6个月时DFS≥2。Kaplan-Meier分析评估左心室（LV）射血分数（EF）和容积恢复正常的时间。回归样条分析趋势，Fisher精确检验评估与复合临床结局（心力衰竭或室性快速心律失常）的相关性。37例患者中，术前LV EF中位数为26.5%[四分位间距20.8, 45]）。EF恢复正常的中位时间为11.3个月[四分位间距3.1, 72]，34例患者（92%）恢复正常。LV容积呈下降趋势，但27%仍有扩大。21例患者（57%）观察到持续性LV DD，其中仅3例恢复正常。出院时较高的LA容积与持续性LV DD相关（p = 0.047）。E' Z评分仍较低，E/E' Z评分升高（p>0.05）。持续性LV DD与复合结局相关（p = 0.05），而LV EF异常则不然（p = 1.00）。大多数患者在ALCAPA修复术后LV收缩功能恢复正常，但27%表现为持续性LV扩大。大多数患者存在持续性DD，且长期倾向于保持异常。持续性DD与更差的临床结局相关。
