RESUMEN
BACKGROUND: Congenital heart defects (CHDs) are the most common type of congenital anomaly. The precise etiology is unknown and the development of successful primary prevention strategies is challenging. Folic acid may have a protective role; however published results have been inconsistent. This study examines the impact of mandatory folic acid fortification (FAF) on the prevalence of CHDs. METHODS: CHD cases were ascertained using the Alberta Congenital Anomalies Surveillance System, Pediatric Cardiology Clinics, Pathology, and hospital records. The birth prevalence and odds ratios (OR) of isolated CHD cases (i.e., without noncardiac anomalies) were calculated comparing pre-FAF (1995-1997) with post-FAF (1999-2002). RESULTS: The prevalence of isolated CHD cases remained relatively unchanged when pre-FAF (9.34, 95% confidence interval [CI] 8.79-9.92) was compared with post-FAF (9.41, 95% CI, 8.93-9.91). Left ventricular outflow tract obstruction (LVOTO) decreased post-FAF (OR, 0.76; 95% CI, 0.61-0.94). Coarctation of the aorta contributed to this decline (OR, 0.55; 95% CI, 0.32-0.92). Atrial septal defect (ASD) (OR, 1.42; 95% CI, 1.13-1.80) and ASD with ventricular septal defect (OR, 1.52; 95% CI, 1.10-2.10) increased post-FAF. The remaining types of CHDs were unchanged. CONCLUSION: FAF alone does not have an impact on the prevalence of CHDs as a group and the majority of selected types of CHDs in Alberta. The decrease in LVOTO, particularly coarctation of the aorta, may be due to FAF or other environmental factors. The increase in ASD and ASD with ventricular septal defect may reflect an increase in diagnosis and ascertainment.
Asunto(s)
Ácido Fólico/administración & dosificación , Cardiopatías Congénitas/epidemiología , Cardiopatías Congénitas/prevención & control , Exposición Materna/efectos adversos , Alberta/epidemiología , Femenino , Humanos , PrevalenciaRESUMEN
BACKGROUND: Although the majority of congenital heart defects (CHDs) occur in isolation, a significant number occur with noncardiac anomalies. This study determined the proportion of noncardiac anomalies among CHD cases in Alberta. METHODS: Records of infants and children born in Alberta between January 1, 1995, to December 31, 2002, were searched using multiple sources of ascertainment in addition to the Alberta Congenital Anomalies Surveillance System (ACASS) (Alberta Health and Wellness, 2012). Each case was assigned to one CHD category and was further categorized into one of the following groups: isolated CHD, syndromes, chromosomal, associations and sequences, teratogens, Mendelian, neoplasia, heterotaxy, multiple minor anomalies, and multiple major anomalies. RESULTS: Of all 3751 CHD cases (prevalence 12.42/1000 total births: confidence interval, 12.03-12.83), 75% were isolated, 10% had a chromosomal etiology, and 9% had multiple major anomalies. All other categories accounted for <2% each. The most commonly associated major noncardiac anomalies were musculoskeletal (MSK) (24%) followed by anomalies of the urinary tract (14%), gastrointestinal system (GI) (11%), and central nervous system (CNS) (11%). CONCLUSIONS: This is both a population-based and clinical study using a classification scheme that could help to determine possible etiologic factors contributing to CHD. By eliminating known etiologies such as chromosomal and single gene, future studies can focus on the remainder to evaluate possible preventive measures. The most commonly associated major noncardiac anomalies involve the MSK system, followed by the urinary, GI, and CNS systems.
Asunto(s)
Enfermedades del Sistema Nervioso Central/epidemiología , Enfermedades Gastrointestinales/epidemiología , Cardiopatías Congénitas/epidemiología , Anomalías Musculoesqueléticas/epidemiología , Enfermedades Urológicas/epidemiología , Alberta/epidemiología , Enfermedades del Sistema Nervioso Central/congénito , Enfermedades del Sistema Nervioso Central/genética , Niño , Preescolar , Comorbilidad , Femenino , Enfermedades Gastrointestinales/congénito , Enfermedades Gastrointestinales/genética , Cardiopatías Congénitas/genética , Humanos , Lactante , Recién Nacido , Masculino , Anomalías Musculoesqueléticas/genética , Vigilancia de la Población , Prevalencia , Estudios Retrospectivos , Enfermedades Urológicas/congénito , Enfermedades Urológicas/genéticaRESUMEN
BACKGROUND: Congenital heart defects (CHDs) are the most common type of congenital anomaly, with a wide range of reported birth prevalence estimates. This quality assurance study describes CHD case ascertainment by the Alberta Congenital Anomalies Surveillance System (ACASS). METHODS: ACASS data for CHD cases were compared with additional sources including the two Pediatric Cardiology clinics in Alberta, the Alberta Children's Hospital Department of Pathology, and hospital records. Cases included live births, stillbirths, and fetal deaths at less than 20 weeks' gestation born in Alberta, Canada, between 1995 and 2002. The birth prevalence of cases and chi-square linear trend analyses were calculated for specific types of heart defects for the total study period. RESULTS: The ascertainment of CHD cases by ACASS was 45%. The total prevalence of CHD cases was 5.59 per 1000 total births (TBs; 95% confidence interval [CI], 5.32-5.86) when ACASS was the only data source and increased to 12.42 per 1000 TBs (95% CI, 12.03-12.83) when all data sources were used. Although the total prevalence of CHD cases remained stable during 1995 to 2002, the prevalence of atrial septal defect (ASD) and cases with an ASD and ventricular septal defect (VSD) significantly increased. The prevalence of left ventricular outflow tract obstruction cases significantly decreased during the study period. CONCLUSIONS: Pediatric cardiology clinics are worth including as additional ascertainment sources to contribute to more accurate prevalence estimates. The significant increases of ASD and cases with both an ASD and VSD may reflect differences in diagnostic and ascertainment practices.
Asunto(s)
Muerte Fetal/epidemiología , Cardiopatías Congénitas/epidemiología , Nacimiento Vivo/epidemiología , Vigilancia de la Población , Mortinato/epidemiología , Adulto , Alberta/epidemiología , Femenino , Hospitales Pediátricos , Humanos , Recién Nacido , Estudios Longitudinales , Masculino , Embarazo , Prevalencia , Distribuciones EstadísticasRESUMEN
Newly emerging health technologies are being developed to care for children with complex cardiac defects. Neurodevelopmental and childhood school-related outcomes are of great interest to parents of children receiving this care, care providers, and healthcare administrators. Since the 1970s, neonatal follow-up clinics have provided service, audit, and research for preterm infants as care for these at-risk children evolved. We have chosen to present for this issue the mechanism for longitudinal follow-up of survivors that we have developed for western Canada patterned after neonatal follow-up. Our program provides registration for young children receiving complex cardiac surgery, heart transplantation, ventricular assist device support, and extracorporeal life support among others. The program includes multidisciplinary assessments with appropriate neurodevelopmental intervention, active quality improvement evaluations, and outcomes research. Through this mechanism, consistently high (96%) follow-up over two years is maintained.
RESUMEN
OBJECTIVES: To determine 18-month neurodevelopmental outcome of survivors of complex open heart surgery at =6 weeks of age through an interprovincial program and to explore preoperative, operative, and postoperative outcome predictors. Study design Of 85 children from this inception cohort (21% mortality), 67 18-month-old survivors received multidisciplinary assessment including the Bayley Scales of Infant Development-II. Cumulative risk for adverse outcome was determined through univariate and multivariate analyses. RESULTS: Survival of the 85 children included 23 of 23 after arterial switch, 16 of 26 after Norwood, six of six after total anomalous pulmonary venous drainage repair, and 22 of 30 after miscellaneous repair. Outcomes were as follows: in-hospital death, 14 (16%); postdischarge death, four (5%); motor/sensory disability, three (4%); motor/mental delay (<70), 21 (25%); and intact survivors, 43 (50%). Cohort mental (84+/-17) and motor (80+/-22) scores were lower for those with chromosomal abnormalities, 67+/-16 and 61+/-17, respectively. Fifty-five percent of the outcome variance was explained by duration of preoperative ventilation, 18%; genetic anomaly, 5%; intraoperative variables, 18%; and postoperative variables, 14%. CONCLUSIONS: Risk for adverse outcome is cumulative, with preoperative determinants contributing significantly to total variance. Potentially modifiable variables should be sought in an attempt to improve outcome.