Hospital for Sick Children, Ontario, Canada.
J Heart Lung Transplant. 2009 Dec;28(12):1312-21. doi: 10.1016/j.healun.2009.05.019. Epub 2009 Sep 26.
Dilated (DCM), restrictive (RCM), and hypertrophic (HCM) cardiomyopathies (CM) in children have varying clinical courses and therapeutic options. Heart transplantation (HTx) offers a chance for long-term survival; but outcomes after listing have not been well defined.
A multi-institutional registry of 3,147 patients listed for HTx (January 1993-December 2006) was used to compare outcomes of 1,320 children with CM (42%) and 1,827 with non-CM (58%) etiologies. Comparisons were made between sub-groups: 1,098 DCM (83%), 145 RCM (11%), and 77 HCM (6%).
CM patients had a waitlist mortality of 17% vs 32% for non-CM patients (p < 0.0001), with no difference between the CM sub-groups. Risk factors were younger age, black race (relative risk [RR], 1.65; p = 0.009), mechanical ventilation (RR, 3.17; p < 0.001), and extracorporeal membrane oxygenation (RR, 2.16; p < 0.001). Ten-year survival after listing was 66% for CM vs 53% for non-CM (p < 0.0001). HCM and RCM patients aged < 1 year at the time of listing had the highest waitlist mortality and the lowest overall survival. CM patients had a better 10-year survival after HTx (68% vs 61%, p < 0.0001). Risk factors for death early after HTx included mechanical ventilation at HTx (RR, 3.07; p < 0.001), longer ischemic time (RR, 1.27; p = 0.01), and earlier era (RR, 1.77; p = 0.002). Late risk factors included black race (RR, 3.01; p < 0.001), HCM or RCM (RR, 1.93; p = 0.007), and older age (RR, 1.9; p < 0.001).
Children with CM have a lower waitlist mortality and better survival post-HTx than children with a non-CM diagnosis. DCM patients have the best and HCM or RCM patients aged younger than 1 year have the worst overall outcomes.
儿童扩张型心肌病（DCM）、限制型心肌病（RCM）和肥厚型心肌病（HCM）的临床表现和治疗选择各异。心脏移植（HTx）可提供长期生存的机会，但等待移植名单上的患者结局尚未明确。
使用一个多机构的 HTx 登记处（1993 年 1 月至 2006 年 12 月）的 3147 名患者数据，比较了 1320 例心肌病（42%）和 1827 例非心肌病（58%）病因的患者的结局。在亚组之间进行了比较：1098 例 DCM（83%）、145 例 RCM（11%）和 77 例 HCM（6%）。
CM 患者的等待移植死亡率为 17%，而非 CM 患者为 32%（p < 0.0001），CM 亚组之间无差异。危险因素包括年龄较小、黑人种族（相对风险 [RR]，1.65；p = 0.009）、机械通气（RR，3.17；p < 0.001）和体外膜肺氧合（RR，2.16；p < 0.001）。CM 患者的 10 年生存率为 66%，而非 CM 患者为 53%（p < 0.0001）。HCM 和 RCM 患者在登记时年龄 < 1 岁，等待移植死亡率最高，整体生存率最低。CM 患者在 HTx 后 10 年生存率更高（68% vs 61%，p < 0.0001）。HTx 后早期死亡的危险因素包括机械通气（RR，3.07；p < 0.001）、缺血时间较长（RR，1.27；p = 0.01）和时代较早（RR，1.77；p = 0.002）。晚期危险因素包括黑人种族（RR，3.01；p < 0.001）、HCM 或 RCM（RR，1.93；p = 0.007）和年龄较大（RR，1.9；p < 0.001）。
与非 CM 诊断的儿童相比，CM 患儿的等待移植死亡率较低，HTx 后生存率较高。DCM 患者的结局最好，年龄 < 1 岁的 HCM 或 RCM 患者的整体结局最差。
