Singh Tajinder P, Sleeper Lynn A, Lipshultz Steven, Cinar Amy, Canter Charles, Webber Steven A, Bernstein Daniel, Pahl Elfriede, Alvarez Jorge A, Wilkinson James D, Towbin Jeffrey A, Colan Steven D
Department of Cardiology, Children's Hospital Boston, Boston, Mass, USA.
Circ Heart Fail. 2009 Nov;2(6):591-8. doi: 10.1161/CIRCHEARTFAILURE.108.839001. Epub 2009 Sep 28.
In patients with dilated cardiomyopathy, the magnitude of cardiac remodeling often correlates with the clinical severity of heart failure. We sought to determine whether measures of left ventricular (LV) dilation and systolic dysfunction in children with dilated cardiomyopathy at the time of listing for cardiac transplantation are associated with survival while waiting for and early after transplant.
We analyzed echocardiographic data obtained within 6 months of listing for heart transplant and clinical data from 261 children with dilated cardiomyopathy who were included in both the Pediatric Cardiomyopathy Registry and the Pediatric Heart Transplant Study. Median time to listing after diagnosis was 1.9 months and to transplant after listing was 0.8 months. There were 42 deaths (29 waiting and 13 within 6 months after transplant). We found a significant age-dependent association of LV end-diastolic dimension z score (n=204, 31 deaths) with death controlling for race, transplant status, and medical insurance. The association was strongest for infants younger than 6 months at diagnosis (hazard ratio 1.47, P=0.008) and was not significant in children older than 5 years at diagnosis. A similar interaction was identified between age and LV end-systolic dimension z score (P=0.04). Neither LV function nor mass was associated with death, overall, or in subgroups.
The severity of LV dilation at listing for heart transplant is associated with outcome in infants and young children with dilated cardiomyopathy, whereas the severity of LV systolic dysfunction is not. These findings should be considered in risk stratification of these children at listing.
在扩张型心肌病患者中，心脏重塑的程度通常与心力衰竭的临床严重程度相关。我们试图确定扩张型心肌病患儿在列入心脏移植名单时左心室（LV）扩张和收缩功能障碍的指标是否与等待移植及移植后早期的生存率相关。
我们分析了261例纳入儿童心肌病登记处和儿童心脏移植研究的扩张型心肌病患儿在列入心脏移植名单后6个月内获得的超声心动图数据以及临床数据。诊断后至列入名单的中位时间为1.9个月，列入名单后至移植的中位时间为0.8个月。有42例死亡（29例在等待期间，13例在移植后6个月内）。我们发现，在控制种族、移植状态和医疗保险的情况下，LV舒张末期内径z评分（n = 204，31例死亡）与死亡存在显著的年龄依赖性关联。这种关联在诊断时年龄小于6个月的婴儿中最强（风险比1.47，P = 0.008），在诊断时年龄大于5岁的儿童中不显著。在年龄与LV收缩末期内径z评分之间也发现了类似的相互作用（P = 0.04）。总体而言，无论是LV功能还是质量，在总体人群或亚组中均与死亡无关。
心脏移植名单上LV扩张的严重程度与扩张型心肌病婴幼儿的预后相关，而LV收缩功能障碍的严重程度则不然。在这些儿童列入名单时进行风险分层时应考虑这些发现。
