Vigilancia epidemiológica de las anomalías congénitas cardíacas: La Tetralogía de Fallot en la Comunitat Valenciana, 2007-2017

Background: 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. Methods: 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. Results: 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). Conclusions: 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. Fundamentos: La Tetralogía de Fallot está caracterizada por la presencia de cuatro anomalías congénitas cardíacas. El objetivo de este trabajo fue describir la tendencia temporal y distribución de la Tetralogía de Fallot en menores de un año en la Comunitat Valenciana. Métodos: Se seleccionaron los casos con Tetralogía de Fallot (código Q21.3 de la CIE10 de la Asociación Pediátrica Británica) nacidos entre 2007-2017 del Registro Poblacional de anomalías congénitas de la Comunitat Valenciana. Se calculó la prevalencia por 10.000 nacimientos con IC95% y se realizó un análisis descriptivo de las variables sociodemográficas y clínicas. Resultados: Se identificaron 165 casos (43,6% niños, 30,9% niñas y 25,5% de sexo desconocido). La prevalencia global fue 3,1/10.000 nacimientos (IC95%:2,6– 3,6), siendo los años de mayor prevalencia 2015 y 2017 (4,3/10.000 nacimientos y 4,7/10.000 nacimientos respectivamente)