Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry
Cardiomyopathy Phenotypes and Outcomes for Children With Left Ventricular Myocardial Noncompaction: Results From the Pediatric Cardiomyopathy Registry
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DOI：
10.1016/j.cardfail.2015.06.381
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发表时间：
2015-11-01
影响因子：
6
通讯作者：
Towbin, Jeffrey A.
中科院分区：
文献类型：
--
作者：
Jefferies, John L.;Wilkinson, James D.;Towbin, Jeffrey A.
背景：左心室不合作（LVNC）是一种不同的心肌病的形式，其特征是左心室的超额列表。 LVNC表型可能分离出现或与其他心肌病表型发生。预后未完全在儿童中表征。方法和结果：根据1990年至2008年的国家心脏，肺和血液研究所资助的“小儿心肌病登记册”的诊断，3,219名儿童中的155名（4.8％）的155例患有LVNC。每位LVNC患者还被归类为具有相关的超声心动图诊断的心肌病表型：扩张（DCM），肥大（HCM），限制性（RCM），孤立或不确定。表型组之间的死亡或移植时间有所不同（p = .035）。列表心脏移植的时间与表型明显不同（p <.001），移植时间也是如此（p = .015）。 HCM的死亡/移植危险比为4.26（95％置信区间[CI] 0.78-23.3），DCM为6.35（95％CI 1.52-26.6），DCM和5.66（95％）（95％）（95％ CI 1.04-30.9）对于不确定的表型。大多数事件发生在诊断后的第一年。结论：至少有5％的心肌病儿童中存在LVNC。特定的LVNC相关心肌病表型预测死亡或移植的风险，并应为临床管理提供信息。
Background: Left ventricular noncompaction (LVNC) is a distinct form of cardiomyopathy characterized by hypertrabeculation of the left ventricle. The LVNC phenotype may occur in isolation or with other cardiomyopathy phenotypes. Prognosis is incompletely characterized in children.Methods and Results: According to diagnoses from the National Heart, Lung, and Blood Institute-funded' Pediatric Cardiomyopathy Registry from 1990 to 2008, 155 of 3,219 children (4.8%) had LVNC. Each LVNC patient was also classified as having an associated echocardiographically diagnosed cardiomyopathy phenotype: dilated (DCM), hypertrophic (HCM), restrictive (RCM), isolated, or indeterminate. The time to death or transplantation differed among the phenotypic groups (P = .035). Time to listing for cardiac transplantation significantly differed by phenotype (P < .001), as did time to transplantation (P = .015). The hazard ratio for death/transplantation (with isolated LVNC as the reference group) was 4.26 (95% confidence interval [CI] 0.78-23.3) for HCM, 6.35 (95% CI 1.52-26.6) for DCM, and 5.66 (95% CI 1.04-30.9) for the indeterminate phenotype. Most events occurred in the 1st year after diagnosis.Conclusions: LVNC is present in at least 5% of children with cardiomyopathy. The specific LVNC-associated cardiomyopathy phenotype predicts the risk of death or transplantation and should inform clinical management.