Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles.
J Heart Lung Transplant. 2012 Dec;31(12):1269-75. doi: 10.1016/j.healun.2012.09.018. Epub 2012 Oct 15.
Restrictive cardiomyopathy (RCM) represents a spectrum of disorders with a common physiology but divergent etiologies. RCM commonly leads to progressive heart failure and the need for heart transplantation (HTx). Pediatric RCM is a more homogeneous disorder with post-HTx outcomes comparable to those for non-RCM patients. However, post-HTx outcomes in adult RCM patients have not been studied to date.
Demographic, clinical and survival outcomes of 38,190 adult HTx-only recipients from 1987 to 2010 were acquired from the registry of the United Network of Organ Sharing. The study population included 544 RCM patients (1.4%) and 37,646 non-RCM patients (98.6%). RCM diagnoses included idiopathic (n = 227, 42%), amyloid (n = 142, 26%), sarcoid (n = 81, 15%), radiation/chemotherapy (XRT) (n = 35, 6%) and other (n = 59, 11%).
Follow-up began at the time of HTx (74±64 months). During the follow-up period, 224 (41%) patients in the RCM group died, whereas 18,791 (50%) in the non-RCM group died. Crude 1-, 5- and 10-year survival for RCM patients was 84%, 66% and 45%, and for non-RCM patients was 85%, 70% and 50%, respectively. The overall unadjusted hazard ratio of RCM vs non-RCM for all-cause mortality was 1.07 (confidence interval [CI] 0.93 to 1.22). Multivariate Cox proportional hazards regression analysis yielded a hazard ratio of 1.06 (CI 0.91 to 1.25). RCM subgroup analysis showed decreased survival at 1, 5 and 10 years in the XRT (71%, 47% and 32%) and amyloid (79%, 47% and 28%) patient groups. The unadjusted hazard ratio for the XRT and amyloid subgroups vs RCM for all-cause mortality was 1.81 (p = 0.002) and 1.85 (p = 0.0004), respectively.
Outcomes for RCM patients post-HTx are comparable to those of non-RCM patients. However, RCM subgroup analysis suggests increased mortality for XRT and amyloid subgroups. Further analysis is warranted to understand the contributing factors.
限制型心肌病（RCM）代表了一种具有共同生理学但不同病因的疾病谱。RCM 通常导致进行性心力衰竭和需要心脏移植（HTx）。儿科 RCM 是一种更为同质的疾病，其 HTx 后结局与非 RCM 患者相当。然而，迄今为止，尚未对成人 RCM 患者的 HTx 后结局进行研究。
从 1987 年至 2010 年，从器官共享联合网络的登记处获得了 38190 名仅接受 HTx 的成年患者的人口统计学、临床和生存结局数据。研究人群包括 544 名 RCM 患者（1.4%）和 37646 名非 RCM 患者（98.6%）。RCM 诊断包括特发性（n=227，42%）、淀粉样变性（n=142，26%）、结节病（n=81，15%）、放疗/化疗（XRT）（n=35，6%）和其他（n=59，11%）。
随访始于 HTx 时（74±64 个月）。在随访期间，RCM 组有 224 名（41%）患者死亡，而非 RCM 组有 18791 名（50%）患者死亡。RCM 患者的粗 1、5 和 10 年生存率分别为 84%、66%和 45%，而非 RCM 患者分别为 85%、70%和 50%。总体未调整的 RCM 与非 RCM 患者全因死亡率的风险比为 1.07（93%置信区间 [CI] 0.93 至 1.22）。多变量 Cox 比例风险回归分析得出的风险比为 1.06（91%置信区间 [CI] 0.91 至 1.25）。RCM 亚组分析显示，XRT（71%、47%和 32%）和淀粉样变性（79%、47%和 28%）患者组的 1、5 和 10 年生存率降低。XRT 和淀粉样变性亚组与 RCM 患者的全因死亡率的未调整风险比分别为 1.81（p=0.002）和 1.85（p=0.0004）。
RCM 患者 HTx 后的结局与非 RCM 患者相当。然而，RCM 亚组分析表明 XRT 和淀粉样变性亚组的死亡率增加。需要进一步分析以了解相关因素。
