儿童,青少年和年轻成年人的缩性心肌病与当代管理策略的低心血管死亡率相关
Barry J Maron1, Ethan J Rowin2, Susan A Casey2
1From Hypertrophic Cardiomyopathy Center, Minneapolis Heart Institute Foundation, MN (B.J.M., S.A.C., J.R.L., R.F.G., D.M.M.); and Hypertrophic Cardiomyopathy Center, Tufts Medical Center, Boston MA (E.J.R., M.S.M.). hcm.maron@mhif.org.
Circulation
|November 1, 2015
概括
当代治疗显著降低了患有缩性心肌病 (HCM) 的年轻患者的死亡率,这是一种与高风险相关的疾病. 在识别高风险个体和使用可植入心脏变压器 (ICD) 方面取得的进展提高了寿命和生活质量.
科学领域:
- 心血管医学
- 儿童心脏病学
- 遗传学
背景情况:
- 青春期是心肌缩症 (HCM) 的关键风险时期,主要是由于心脏突然死亡.
- 近几十年来,有所改善的风险患者的识别和可植入心脏变压器 (ICD) 的使用.
- 这些管理进展对年轻HCM患者的死亡率的影响尚不清楚.
研究的目的:
- 评估年轻患者的长期结果,诊断出患有多变性心肌病.
- 确定当代治疗策略是否改善了这一高危人群的生存率.
主要方法:
- 研究了474名年龄在7~29岁之间连续患有多变性心肌病的患者队列.
- 长期随访时间均为6. 0年 (范围为3. 010. 0).
- 结果包括死亡率,症状严重程度和危及生命的事件干预.
主要成果:
- 在随访期间,95%的患者无症状或轻度存活.
- 与HCM相关的死亡发生在3%的患者中 (0, 54%/年),心律失常的突然死亡是最常见的原因.
- 高风险患者的死亡率是HCM死亡率的3倍.
- 5年和10年生存率高 (97%和94%) 和中年成人HCM患者相比.
结论:
- 在使用现代心血管治疗的年轻心肌缩病患者中,可实现较低的死亡率.
- 可靠的高危人群鉴定和预防性ICD植入是预防突然死亡的关键.
- 这些策略可以为年轻的HCM患者提供延长寿命和良好的生活质量.
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