The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales.
The Improved Prognosis of Hypoplastic Left Heart: A Population-Based Register Study of 343 Cases in England and Wales.
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DOI：
10.3389/fped.2021.635776
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发表时间：
2021
影响因子：
2.6
通讯作者：
Rankin J
中科院分区：
文献类型：
--
作者：
Best KE;Miller N;Draper E;Tucker D;Luyt K;Rankin J
背景：左心发育不全综合征(HLHS)是一种严重的先天性心脏病(CHD)，其特征是左侧心脏发育不全，左心房、二尖瓣、左心室、主动脉瓣和主动脉弓不同程度发育不全。在英国，1991至1993年间出生的病例12岁存活率为21%。与2000年至2015年出生的病例相对应的英国存活率估计提高了56%，但只检查了五岁以下的存活率。当代的长期存活率评估在诊断后对父母进行咨询方面起着至关重要的作用。这项研究的目的是报告1998至2012年间英格兰和威尔士出生时患有HLHS或左心室发育不良并伴有额外CHD的儿童15岁以下的存活估计。方法：纳入1998-2012年期间向英格兰和威尔士4个先天异常登记处报告的HLHS病例，这些病例与英国国家统计局的死亡信息相匹配。报告了Kaplan-Meier估计的到15岁的生存时间。用COX回归模型检验死亡率的危险因素。结果：HLHS患者244例，左心室发育不良合并其他CHD患者99例，均可追踪生存状态。Kaplan-Meier对HLHS的生存估计在1周时为84.4%，1个月时为76.2%，1岁时为63.5%，5岁时为58.6%，10岁时为54.6%，15岁时为32.6%。左心室发育不良合并其他冠心病的Kaplan-Meier生存率估计在出生后1周为90.9%，1个月为84.9%，1岁为73.7%，5岁至5岁为67.7%，10岁至10岁为59.2%，15岁至15岁为40.3%。早产(p=0.007)、低出生体重(p=0.005)和女性(p=0.005)与死亡率相关。结论：我们已经表明，21世纪与HLHS相关的预后超过了许多以前的基于人群的研究，这可能是由于过去几十年重症监护技术的进步和外科技术的进步。
Background: Hypoplastic Left Heart Syndrome (HLHS) is a severe congenital heart defect (CHD) characterised by the underdevelopment of the left side of the heart with varying levels of hypoplasia of the left atrium, mitral valve, left ventricle, aortic valve and aortic arch. In the UK, age 12 survival for cases born between 1991 and 1993 was 21%. UK survival estimates corresponding to cases born between 2000 and 2015 were improved at 56%, but survival was examined up to age five only. Contemporary long-term survival estimates play a crucial role in counselling parents following diagnosis. The aim of this study was to report survival estimates up to age 15 for children born with HLHS or hypoplastic left ventricle with additional CHD in England and Wales between 1998 and 2012. Methods: Cases of HLHS notified to four congenital anomaly registers in England and Wales during 1998–2012, matched to Office for National Statistics mortality information, were included. Kaplan-Meier survival estimates to age 15 were reported. Cox regression models were fitted to examine risk factors for mortality. Results: There were 244 cases of HLHS and 99 cases of hypoplastic left ventricle co-occurring with other CHD, with traced survival status. Kaplan-Meier survival estimates for HLHS were 84.4% at age 1 week, 76.2% at 1 month, 63.5% at age 1 year, 58.6% at age 5 years, 54.6% at age 10 years, and 32.6% to age 15 years. The Kaplan-Meier survival estimates for cases of hypoplastic left ventricle co-occurring with additional CHD were 90.9% at age 1 week, 84.9% at 1 month, 73.7% at age 1 year, 67.7% to age 5 years, 59.2% to age 10 years, and 40.3% to age 15 years. Preterm birth (p = 0.007), low birth weight (p = 0.005), and female sex (p = 0.01) were associated with mortality. Conclusions: We have shown that prognosis associated with HLHS in the twenty first century exceeds that of many previous population-based studies, likely due to improvements in intensive care technologies and advances in surgical techniques over the last few decades.
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