儿童左心室非紧缩性心肌病的长期结果:基于全国人口的研究结果
William Y Shi1,2,3,4, Margarita Moreno-Betancur3,4, Alan W Nugent5
1Department of Cardiac Surgery (W.Y.S.).
Circulation
|March 9, 2018
概括
儿童左心室非紧缩 (LVNC) 主要表现为婴儿期扩张的心脏病. 这些儿童的长期结果与扩张性心肌病患者相比较差.
科学领域:
- 儿童心脏病学
- 心血管研究
- 心脏病的遗传学
背景情况:
- 儿童左心室非紧缩 (LVNC) 是一种罕见的先天性心脏缺陷,长期预后不确定.
- 早期诊断和了解LVNC对于有效的治疗和改善患者的结果至关重要.
研究的目的:
- 研究被诊断为左心室非紧缩 (LVNC) 的儿童的长期结果.
- 将扩张表型 (LVNC-D) 的LVNC患者与扩张心肌病患者的结果进行比较.
主要方法:
- 使用了澳大利亚国家儿童心肌病研究 (1987-1996) 的数据.
- 使用倾向性评分分析进行风险调整的LVNC患者的结果比较.
- 使用竞争风险分析和考克斯回归来评估死亡率和移植风险.
主要成果:
- LVNC影响了9. 2%的心肌病例,诊断时的中位数年龄为0. 3岁.
- 在83%的LVNC病例中,充血性心力衰竭是主要症状,其中93%呈现扩张表型 (LVNC- D).
- 在诊断后15年,LVNC的死亡或移植率为45%,而LVNC- D显示死亡或移植的风险显著增加 (HR2. 3, P=0. 0012).
结论:
- 患有LVNC的症状儿童通常在婴儿期出现扩张的表型.
- 儿童LVNC的长期结果,特别是扩张的表型,比匹配的扩张性心肌病更差.
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