Congenital heart defects (CHD) are the most frequent group of congenital anomalies representing a significant burden of mortality and morbidity and health service load.

In the Northern Ireland population, served by a single paediatric cardiology centre, we determine the prevalence and trends of CHD among live births.

This is a descriptive cross-sectional population-based study, using the paediatric cardiology database. The study included a total of 245,120 live births representing all children born in Northern Ireland 2005-2014.

A total of 11,410 children (4.65% of live births in Northern Ireland) received an echocardiogram for suspected CHD, and 3,059 children were subsequently diagnosed with a major CHD (prevalence = 12.48 per 1,000 live births (95% CI 12.04–12.93)) of whom 490 (16.02%) had genetic or chromosomal disorders including Down syndrome. The prevalence of non-genetic or chromosomal cases was 10.48 per 1,000 live births (95% CI 10.08–10.89) and did not change significantly over time (p = 0.91). The prevalence of CHD diagnosed in the first year of life was 8.46 per 1,000 live births (95% CI 8.10–8.83), which increased over time (p < 0.01). The prevalence of severe CHD was 2.02 per 1,000 live births (95% CI 1.85–2.21).

Northern Ireland has a high prevalence of CHD among European countries, which may be associated with complete ascertainment of both early and late diagnosed cases recorded in the paediatric cardiology database, as well as being one of the few European countries where terminations of pregnancy for foetal anomaly was illegal during the study period.

先天性心脏缺陷 (CHD) 是最常见的一组先天性异常，代表着死亡率和发病率的重大负担以及卫生服务负荷。

在由单一儿科心脏病学中心提供服务的北爱尔兰人口中，我们确定了活产中冠心病的患病率和趋势。

这是一项基于人口的描述性横断面研究，使用儿科心脏病学数据库。该研究共包括 245,120 例活产，代表 2005-2014 年在北爱尔兰出生的所有儿童。

共有 11,410 名儿童（占北爱尔兰活产的 4.65%）接受了疑似冠心病的超声心动图检查，随后有 3,059 名儿童被诊断出患有严重的冠心病（患病率 = 12.48 / 1,000 名活产 (95% CI 12.04–12.93)）其中 490 人 (16.02%) 患有包括唐氏综合症在内的遗传或染色体疾病。非遗传或染色体病例的患病率为每 1,000 例活产 10.48 例 (95% CI 10.08–10.89)，并且随时间没有显着变化 (p = 0.91)。出生后第一年诊断出冠心病的患病率为每 1,000 例活产 8.46 例 (95% CI 8.10–8.83)，该患病率随时间增加 (p < 0.01)。严重冠心病的患病率为每 1,000 例活产 2.02 例（95% CI 1.85–2.21）。

北爱尔兰在欧洲国家中冠心病的患病率很高，这可能与儿科心脏病学数据库中记录的早期和晚期诊断病例的完全确定有关，也是少数几个因胎儿异常而终止妊娠的欧洲国家之一在学习期间是非法的。