Cavero-Carbonell Clara, García-Villodre Laura, Barrachina-Bonet Laia, Moreno-Marro Sandra, Páramo-Rodríguez Lucía, Guardiola-Vilarroig Sandra, Zurriaga-Llorens Óscar
Unidad Mixta de Investigación en Enfermedades Raras, FISABIO-UVEG. Valencia. España.
Dirección General de Salud Pública y Adicciones. Conselleria Sanitat Comunitat Valenciana. Valencia. España.
Rev Esp Salud Publica. 2021 Jan 29;95:e202101019.
Tetralogy of Fallot is characterized by the presence of four congenital heart defects. Objective: to describe the temporal trend and distribution of Tetralogy of Fallot, in children under one year in the Valencian Region.
Cases with Tetralogy of Fallot (code Q21.3 from the ICD10-British Paediatric Association) were selected from the Congenital Anomalies Population-based Registry between 2007-2017. Prevalence per 10,000 births with 95%CI was calculated, and a descriptive analysis of sociodemographic and clinical variables was made.
165 cases were identified (43.6% male, 30.9% female and 25.5% unknown). The overall prevalence was 3.1/10,000 births (95%CI:2.6-3.6), being 2015 and 2017 the years with the highest (4.3/10,000 births and 4.7/10,000 births respectively) and 2011 with the lowest (1.8/10,000 births). 72.1% were live births, 24.8% Termination of Pregnancy for Fetal Anomaly (TOPFA) and 3.0% stillbirths. The prevalence in live births was 2.2/10,000 births (95%CI:1.8-2.7) and in TOPFA it was 0.8/10,000 births (95%CI:0.5-1.0), identifying an increasing trend along the period in the last one. 10.1% of live births died during the first year of life and 55.8% were diagnosed prenatally. Mothers younger than 20 years had the highest prevalence (4.8/10,000 births).
The prevalence obtained in the Valencian Region was slightly lower than EUROCAT's but coincides with that of the registries that are closer geographically, and in all of them it is noted that their increasing trend specifically affects cases ending in TOPFA.
法洛四联症的特征是存在四种先天性心脏缺陷。目的:描述巴伦西亚地区一岁以下儿童中法洛四联症的时间趋势和分布情况。
从2007年至2017年基于人群的先天性异常登记处选取法洛四联症病例(ICD10 - 英国儿科学会编码Q21.3)。计算每10000例出生中的患病率及95%置信区间,并对社会人口统计学和临床变量进行描述性分析。
共识别出165例病例(男性占43.6%,女性占30.9%,未知性别占25.5%)。总体患病率为3.1/10000例出生(95%置信区间:2.6 - 3.6),2015年和2017年患病率最高(分别为4.3/10000例出生和4.7/10000例出生),2011年患病率最低(1.8/10000例出生)。72.1%为活产,24.8%为因胎儿异常终止妊娠(TOPFA),3.0%为死产。活产中的患病率为2.2/10000例出生(95%置信区间:1.8 - 2.7),TOPFA中的患病率为0.8/10000例出生(95%置信区间:0.5 - 1.0),发现后者在此期间呈上升趋势。10.1%的活产在出生后第一年内死亡,55.8%在产前被诊断出。年龄小于20岁的母亲患病率最高(4.8/10000例出生)。
巴伦西亚地区获得的患病率略低于欧洲先天性异常监测网络(EUROCAT),但与地理位置较近的登记处一致,并且在所有这些登记处中都注意到其上升趋势特别影响以TOPFA告终的病例。