Long-Term Outcomes of Orthotopic Heart Transplantation for Hypertrophic Cardiomyopathy
Long-Term Outcomes of Orthotopic Heart Transplantation for Hypertrophic Cardiomyopathy
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DOI：
10.1016/j.transproceed.2013.12.052
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发表时间：
2014-06-01
影响因子：
0.9
通讯作者：
Kobashigawa, J.
中科院分区：
文献类型：
--
作者：
Lee, M. S.;Zimmer, R.;Kobashigawa, J.
背景资料。肥厚型心肌病(HCM)是一种遗传性心肌疾病，其特征是在没有明显临床病因的情况下表现为不对称或对称性的心肌肥厚。尽管进行了内科治疗和外科间隔肌切除术，但仍有顽固性症状的患者可接受原位心脏移植。然而，关于肥厚性心肌病患者接受OHTs治疗的结果的数据很少。回顾收集了1996年至2004年在加州大学洛杉矶分校医学中心连续接受OHT的462名患者的数据。对11例肥厚型心肌炎患者的临床资料进行分析。本组病例以男性为主(%)。患者平均年龄45+/-8岁，供者平均年龄35+/-18岁。平均缺血时间226±60分钟。有1例院内死亡继发于感染性休克。在中位随访时间为4.5年(平均4.4+/-3.2年)时，增加了3例死亡。与未接受HCM的451名OHT患者相比，在存活率(P=.13)、心脏移植物血管病变(P=.46)或排斥反应(P=.71)方面没有差异。供者心脏活检中没有发现肥厚性心肌病复发的证据。对于标准疗法无效的终末期肥厚型心肌病患者，OHT是一种可行的治疗选择。
Background. Hypertrophic cardiomyopathy (HCM) is a genetic heart muscle disease characterized by asymmetric or symmetric ventricular hypertrophy in the absence of an obvious clinical cause. Orthotopic heart transplantation (OHT) has been performed in patients who have refractory symptoms despite medical therapy and surgical septal myectomy. However, there is a paucity of data on outcomes of HCM patients who undergo OHT.Methods. Data on 462 consecutive patients who underwent OHT at UCLA Medical Center from 1996 to 2004 were retrospectively collected. The clinical data on the 11 patients with HCM were identified.Results. The majority of the HCM patients were male (64%). The mean age of the patient was 45 +/- 8 years, and the mean donor age was 35 +/- 18 years. The mean ischemia time was 226 +/- 60 minutes. There was 1 in-hospital death secondary to septic shock. At a median duration of follow-up of 4.5 years (mean, 4.4 +/- 3.2 years), there were 3 additional deaths. Compared with the 451 OHT patients who did not have HCM, there was no difference in survival (P = .13), development of cardiac allograft vasculopathy (P = .46), or rejection (P = .71). There was no evidence of HCM recurrence in biopsies from the donor heart.Conclusions. OHT is a viable treatment option for patients with end-stage HCM refractory to standard therapies.