Child and Adolescent Health Department, Ministry of Health, General Directorate of Public Health, Ankara, Turkey.
Department of Social Pediatrics, Hacettepe University Institute of Child Health, Ankara, Turkey.
BMC Pregnancy Childbirth. 2023 Dec 16;23(1):871. doi: 10.1186/s12884-023-06193-1.
The aim of this study is to examine the features of critical congenital heart disease (CCHD).
The study was planned as a retrospective cohort study. Data for the study were obtained through national data collection systems and 2018-2020 CCHD cohort was established. In this study, we divided the patients into two groups: Group 1 included seven primary target diseases of the newborn CCHD screening program and Group 2 included secondary target diseases.
There were 9884 CCHD cases, with a prevalence of 27.8 per 10,000 live births. Of the cases 44.4% were in Group 1 (12.3 per 10,000) and 54.8% were in Group 2 (15.2 per 10,000). Of all cases 55.5% were male and the female/male ratio was 1/1.2. While 21.8% of the cases were premature, 23.0% were babies with low birth weight (LBW), 4.8% were born from multiple pregnancies. The highest prevalence of CCHD was found in LBW (84.8 per 10,000), premature infants (57.8 per 10,000) (p < 0.001). The fatality rate in the cohort was 16.6% in the neonatal period, 31.6% in the first year of life respectively. The mean estimated survival time in the birth cohort was 40.0 months (95% CI: 39.5-40.6). The mean survival time for Group 1 diseases was 33.4 months (95% CI: 32.5-34.2), while it was 45.4 months (95% CI: 44.7-46.0) for Group 2 diseases (p < 0.001). Preterm birth, LBW, maternal age and region were evaluated as factors associated with mortality risk.
This study showed that CCHDs are common in Turkey and mortality rates are high. There are regional differences in CCHD both prevalence and survival. Improving prenatal diagnosis rates and expanding neonatal CCHD screening are of key importance.
本研究旨在探讨严重先天性心脏病（CCHD）的特征。
本研究计划为回顾性队列研究。研究数据通过国家数据收集系统获得，并建立了 2018-2020 年 CCHD 队列。在本研究中，我们将患者分为两组：第 1 组包括新生儿 CCHD 筛查计划的七个主要目标疾病，第 2 组包括次要目标疾病。
共有 9884 例 CCHD 病例，患病率为每 10000 例活产儿 27.8 例。病例中 44.4%为第 1 组（每 10000 例 12.3 例），54.8%为第 2 组（每 10000 例 15.2 例）。所有病例中，男性占 55.5%，男女比例为 1/1.2。病例中 21.8%为早产儿，23.0%为低出生体重儿（LBW），4.8%为多胎妊娠。CCHD 患病率最高的是 LBW（每 10000 例 84.8 例）和早产儿（每 10000 例 57.8 例）（p<0.001）。该队列的新生儿期死亡率为 16.6%，1 岁时死亡率为 31.6%。出生队列的平均估计生存时间为 40.0 个月（95%CI：39.5-40.6）。第 1 组疾病的平均生存时间为 33.4 个月（95%CI：32.5-34.2），第 2 组疾病的平均生存时间为 45.4 个月（95%CI：44.7-46.0）（p<0.001）。早产、LBW、母亲年龄和地区被评估为与死亡风险相关的因素。
本研究表明，CCHD 在土耳其很常见，死亡率很高。CCHD 的患病率和生存率在不同地区存在差异。提高产前诊断率和扩大新生儿 CCHD 筛查至关重要。
