Mat Bah Mohd Nizam, Sapian Mohd Hanafi, Jamil Mohammad Tamim, Alias Amelia, Zahari Norazah
Department of Pediatrics, Hospital Sultanah Aminah Johor Bahru, Persiaran Abu Bakar Sultan, 80100, Johor Bahru, Johor, Malaysia.
Department of Pediatrics, Faculty of Medicine, University of Malaya, Jalan University, 50603, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia.
Pediatr Cardiol. 2018 Oct;39(7):1389-1396. doi: 10.1007/s00246-018-1908-6. Epub 2018 May 14.
Critical congenital heart disease (CCHD) is associated with significant morbidity and mortality. However, data on survival of CCHD and the risk factors associated with its mortality are limited. This study examined CCHD survival and the risk factors for CCHD mortality. Using a retrospective cohort study of infants born with CCHD from 2006 to 2015, survival over 10 years was estimated using Kaplan-Meier analysis, and the risk factors for mortality were analyzed using multivariate Cox proportional hazards regression. A total of 491 CCHD cases were included in the study, with an overall mortality rate of 34.8% (95% confidence interval [CI] 30.6-39.2). The intervention/surgical mortality rate was 9.8% ≤ 30 days and 11.5% > 30 days after surgery, and 17% died before surgery or intervention. The median age at death was 2.7 months [first quartile: 1 month, third quartile: 7.3 months]. The CCHD survival rate was 90.4% (95% CI 89-91.8%) at 1 month, 69.3% (95% CI 67.2-71.4%) at 1 year, 63.4% (95% CI 61.1-65.7%) at 5 years, and 61.4% (95% CI 58.9-63.9%) at 10 years. Weight of < 2 kg at diagnosis, associated syndromes, poor pre-operative condition, and non-duct-dependent CCHD were independent risk factors for poor survival, with hazard ratios of 2.61, 2.10, 2.22, and 1.70, respectively. CCHD is associated with a high mortality rate. Low weight, poor pre-operative condition, associated syndromes, and non-duct-dependent CCHD are significant risk factors affecting the survival of infants with CCHD.
危重先天性心脏病(CCHD)与显著的发病率和死亡率相关。然而,关于CCHD生存率及其死亡相关危险因素的数据有限。本研究调查了CCHD的生存率及CCHD死亡的危险因素。采用回顾性队列研究,纳入2006年至2015年出生的患有CCHD的婴儿,使用Kaplan-Meier分析估计10年生存率,并使用多变量Cox比例风险回归分析死亡危险因素。本研究共纳入491例CCHD病例,总死亡率为34.8%(95%置信区间[CI]30.6 - 39.2)。干预/手术死亡率在术后≤30天为9.8%,>30天为11.5%,17%在手术或干预前死亡。死亡的中位年龄为2.7个月[第一四分位数:1个月,第三四分位数:7.3个月]。CCHD的生存率在1个月时为90.4%(95%CI 89 - 91.8%),1岁时为69.3%(95%CI 67.2 - 71.4%),5岁时为63.4%(95%CI 61.1 - 65.7%),10岁时为61.4%(95%CI 58.9 - 63.9%)。诊断时体重<2kg、合并综合征、术前状况差以及非导管依赖性CCHD是生存不良的独立危险因素,风险比分别为2.61、2.10、2.22和1.70。CCHD与高死亡率相关。低体重、术前状况差、合并综合征以及非导管依赖性CCHD是影响CCHD婴儿生存的重要危险因素。