Department of Pediatric Cardiology, Cohen Children's Medical Center/Northwell Health, New Hyde Park, New York.
Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, New York.
Ann Thorac Surg. 2023 Sep;116(3):588-597. doi: 10.1016/j.athoracsur.2022.05.037. Epub 2022 Jun 9.
Heart transplantation (HT) is standard therapy for end-stage hypertrophic cardiomyopathy (HCM); however, few studies have described outcomes of older children and young adults with HCM listed for HT. Our objective was to compare waitlist and post-HT outcomes among pediatric and young adult patients with HCM and dilated cardiomyopathy (DCM).
The Scientific Registry of Transplant Recipients was queried for patients with HCM and DCM listed at ≤25 years of age. Patient characteristics, waitlist and post-HT survival were compared between younger (≤5 years of age) and older (>5 to ≤25 years of age) HCM patients and between HCM and DCM patients.
Among 6252 patients listed for HT at ≤25 years of age with DCM and HCM, 3926 and 250 were in the older cohort and 1944 and 132 were in the younger cohort, respectively. Older HCM patients were less likely to be critically ill at listing compared with younger HCM patients (P = .0001). Waitlist mortality was similar between HCM and DCM patients in both age cohorts. Post-HT survival in HCM patients was similar between the age cohorts. In the younger cohort, early post-HT survival was worse in HCM compared with DCM (P = .009), with no difference in long-term survival. Survival was similar between the older cohorts.
Older children and young adults with HCM are less critically ill than the younger cohort and show waitlist and post-HT survival similar to DCM patients. The young children with HCM had worse early posttransplantation survival, though long-term survival was same as DCM.
心脏移植(HT)是治疗终末期肥厚型心肌病(HCM)的标准疗法;然而,很少有研究描述过接受 HT 治疗的大龄儿童和年轻成人 HCM 患者的结局。我们的目的是比较 HCM 和扩张型心肌病(DCM)患者在等待名单和 HT 后的结果。
科学移植受者登记处被查询了在 ≤25 岁时被列入 HCM 和 DCM 名单的患者。在 HCM 患者中,年轻(≤5 岁)和年长(>5 至 ≤25 岁)患者之间,以及 HCM 和 DCM 患者之间,比较了患者特征、等待名单和 HT 后生存情况。
在 ≤25 岁的 6252 名患有 DCM 和 HCM 的 HT 患者中,3926 名和 250 名在年长队列中,1944 名和 132 名在年轻队列中。与年轻 HCM 患者相比,年长 HCM 患者在列入名单时病情不太危急(P =.0001)。在两个年龄组中,HCM 和 DCM 患者的等待名单死亡率相似。HCM 患者的 HT 后生存率在两个年龄组中相似。在年轻队列中,HCM 患者的早期 HT 后生存率较 DCM 患者差(P =.009),但长期生存率无差异。在年长队列中,生存率相似。
年长的儿童和年轻的成人 HCM 患者比年轻的队列病情不那么危急,与 DCM 患者的等待名单和 HT 后生存率相似。患有 HCM 的幼儿在移植后早期的生存率较差,但长期生存率与 DCM 相同。