Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
Department of Pediatric Cardiology, Cleveland Clinic Children's Hospital, Cleveland, Ohio.
Ann Thorac Surg. 2021 Jul;112(1):188-196. doi: 10.1016/j.athoracsur.2020.05.170. Epub 2020 Aug 5.
Although outcomes for pediatric cardiomyopathy (CMP) patients have improved, an understanding of outcomes by CMP phenotype is essential. This study assessed changes in waitlist and post-transplant survival in nondilated cardiomyopathy (DCM) patients over 2 decades, explored ventricular assist device (VAD) utilization in this cohort, and identified risk factors for waitlist and posttransplant mortality in the current era.
Pediatric patients with a diagnosis of CMP listed for heart transplantation during three eras: Era 1: March 5, 1999 to December 31, 2004; Era 2: January 1, 2005 to December 15, 2011; and Era 3 (current era): December 16, 2011 to February 28, 2018 were included. Multivariable Cox proportional hazards regression was performed to assess waitlist and posttransplant survival.
Compared with patients with DCM, those with hypertrophic and restrictive cardiomyopathy in the current era are less likely to be on VAD (23.4% vs 2.7% vs 4.5%); listed United Network for Organ Sharing Status 1A (75.6% vs 39.8% vs 34.8%), and more likely to have longer waitlist times (P < .01 for all). Only 3.3% hypertrophic and 2.4% restrictive cardiomyopathy patients had VAD implantation, although VAD use did not adversely impact waitlist survival in weighted non-DCM patients. Significant improvements have occurred in waitlist survival of hypertrophic and posttransplant survival of both types of non-DCM patients.
Currently, waitlist and posttransplant survival is similar for all CMP phenotypes. VAD use is low in patients with non-DCM, although this did not increase waitlist mortality in adjusted analysis. Further studies in patients with non-DCM are needed to determine optimal timing and anatomic characteristics most likely to benefit from VAD implantation during the waitlist period.
尽管儿科心肌病（CMP）患者的预后有所改善，但了解 CMP 表型的预后至关重要。本研究评估了 20 多年来非扩张型心肌病（DCM）患者在等待移植名单和移植后生存率的变化，探讨了该队列中心室辅助装置（VAD）的应用，并确定了当前时代等待移植名单和移植后死亡的危险因素。
研究纳入了在三个时代被诊断为 CMP 并接受心脏移植的儿科患者：时代 1：1999 年 3 月 5 日至 2004 年 12 月 31 日；时代 2：2005 年 1 月 1 日至 2011 年 12 月 15 日；以及时代 3（当前时代）：2011 年 12 月 16 日至 2018 年 2 月 28 日。采用多变量 Cox 比例风险回归评估等待移植名单和移植后的生存率。
与 DCM 患者相比，当前时代肥厚型和限制型心肌病患者更不可能使用 VAD（23.4% vs. 2.7% vs. 4.5%）；列入联合器官共享网络（UNOS）1A 状态的比例（75.6% vs. 39.8% vs. 34.8%），等待名单时间也更长（所有 P<0.01）。只有 3.3%的肥厚型和 2.4%的限制型心肌病患者接受了 VAD 植入，尽管在加权非 DCM 患者中，VAD 的使用并未对等待名单的生存率产生不利影响。肥厚型患者的等待名单生存率和非 DCM 患者的移植后生存率均有显著改善。
目前，所有 CMP 表型的等待名单和移植后生存率相似。非 DCM 患者的 VAD 使用率较低，尽管在调整分析中，这并未增加等待名单的死亡率。需要对非 DCM 患者进行进一步研究，以确定在等待名单期间最有可能从 VAD 植入中受益的最佳时机和解剖特征。
