Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center
Mortality and Sudden Death in Pediatric Left Ventricular Noncompaction in a Tertiary Referral Center
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DOI：
10.1161/circulationaha.113.002511
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发表时间：
2013-06-04
期刊：
影响因子：
37.8
通讯作者：
Kim, Jeffrey J.
中科院分区：
文献类型：
--
作者：
Brescia, Samuel T.;Rossano, Joseph W.;Kim, Jeffrey J.
左心室致密化不全是一种以左心室过度小梁形成、进行性心肌功能障碍和早期死亡为特征的心肌病。左心室致密化不全有一个异质性的临床表现，包括心律失常和心源性猝死。方法和结果，我们回顾性分析了所有儿童诊断为左心室致密化不全在得克萨斯州儿童医院从1990年1月至2009年1月。排除了先天性心脏病变患者。在研究期间，242名儿童被诊断为孤立性左心室致密化不全。31例（12.8%）死亡，13例（5.4%）接受移植。150例（62%）患者出现或发展为心功能不全。心功能不全的存在与死亡率密切相关（危险比，11; P
Background-Left ventricular noncompaction is a cardiomyopathy characterized by excessive trabeculation of the left ventricle, progressive myocardial dysfunction, and early mortality. Left ventricular noncompaction has a heterogeneous clinical presentation that includes arrhythmia and sudden cardiac death.Methods and Results-We retrospectively reviewed all children diagnosed with left ventricular noncompaction at Texas Children's Hospital from January 1990 to January 2009. Patients with congenital cardiac lesions were excluded. Two hundred forty-two children were diagnosed with isolated left ventricular noncompaction over the study period. Thirty-one (12.8%) died, and 13 (5.4%) were received a transplant. One hundred fifty (62%) presented with or developed cardiac dysfunction. The presence of cardiac dysfunction was strongly associated with mortality (hazard ratio, 11; P