Baidoun Mohammad, Galas James, Farooqi Ahmad, Singh Gautam, Eddine Ahmad Charaf
Division of Pediatric Cardiology, Department of Pediatrics, Detroit, MI, USA.
Division of Pediatric Cardiology, Department of Pediatrics, Children's Hospital of Michigan/Central Michigan University, Michigan, USA.
Pediatr Cardiol. 2025 Jun 17. doi: 10.1007/s00246-025-03915-4.
Tetralogy of Fallot (TOF) is the most common form of cyanotic congenital heart disease. Patients who undergo surgical repair for TOF or TOF with pulmonary atresia (PA) may develop chronic severe pulmonary regurgitation (PR), which leads to right ventricle (RV) dilation and dysfunction. Pulmonary valve replacement is performed to restore pulmonary valve function and reverse the RV remodeling. Traditionally, pulmonary valve replacement has been performed surgically, but over the past two decades, transcatheter pulmonary valve replacement (TPVR) has emerged as an alternative approach. However, the optimal timing for TPVR remains a clinical challenge and a highly debated topic. Our study's primary aim is to evaluate the mid-term effect of TPVR on biventricular systolic function in patients with long-standing PR post-repair of TOF or TOF with PA. This retrospective single-center study included 30 patients with repaired TOF or TOF/PA who underwent first-time TPVR between 2012 and 2022. Echocardiographic assessments of right ventricular (RV) and left ventricular (LV) function were conducted at baseline and up to 3-5 years post-TPVR. Key parameters included RV fractional area change (FAC), RV strain, RV dP/dt, LV ejection fraction (EF), and LV longitudinal strain (LVLS). Statistical comparisons were made between pre- and post-TPVR time points. At baseline, most patients showed reduced RV and LV systolic function. Following TPVR, RV function did not show significant improvement on follow-up. However, LV longitudinal strain gradually improved over time and reached statistical significance at long-term follow-up (p = 0.004). LV EF showed an upward trend but did not reach significance. biventricular systolic function is decreased in many patients with repaired TOF or TOF/PA and long-standing severe PR. Transcatheter pulmonary valve replacement in this cohort resulted in improved LV strain but no improvement in RV function on mid-term follow up. These findings suggest the need to reconsider the timing of TPVR. Future studies should investigate whether earlier valve replacement could lead to better outcome of ventricular function in this patient population.
法洛四联症(TOF)是最常见的青紫型先天性心脏病。接受TOF或合并肺动脉闭锁(PA)的TOF手术修复的患者可能会出现慢性重度肺动脉反流(PR),这会导致右心室(RV)扩张和功能障碍。进行肺动脉瓣置换以恢复肺动脉瓣功能并逆转右心室重塑。传统上,肺动脉瓣置换是通过手术进行的,但在过去二十年中,经导管肺动脉瓣置换术(TPVR)已成为一种替代方法。然而,TPVR的最佳时机仍然是一个临床挑战和备受争议的话题。我们研究的主要目的是评估TPVR对TOF或合并PA的TOF修复术后长期PR患者双心室收缩功能的中期影响。这项回顾性单中心研究纳入了30例在2012年至2022年间首次接受TPVR的TOF或TOF/PA修复患者。在基线时以及TPVR后3至5年进行右心室(RV)和左心室(LV)功能的超声心动图评估。关键参数包括右心室面积变化分数(FAC)、右心室应变、右心室dp/dt、左心室射血分数(EF)和左心室纵向应变(LVLS)。在TPVR前后的时间点之间进行统计学比较。在基线时,大多数患者的右心室和左心室收缩功能降低。TPVR后,随访时右心室功能没有显著改善。然而,左心室纵向应变随时间逐渐改善,并在长期随访时达到统计学意义(p = 0.004)。左心室EF呈上升趋势,但未达到显著水平。许多接受TOF或TOF/PA修复且长期存在严重PR的患者双心室收缩功能降低。该队列中的经导管肺动脉瓣置换术在中期随访时导致左心室应变改善,但右心室功能没有改善。这些发现表明需要重新考虑TPVR的时机。未来的研究应调查早期瓣膜置换是否能使该患者群体的心室功能获得更好的结果。