Paediatric and congenital heart disease in South America: an overview.

Congenital heart diseases are one of the most common structural defects present at birth, with an approximate incidence of 8 per 1000 live births. As most countries in South America have a high birth rate, they are a significant public health concern. This paper provides a brief overview of the burden of congenital heart disease in South America, focusing on its local prevalence, facilities for treatment and outcomes after medical, surgical or catheter intervention for the most common diseases.

Health-related quality of life and right ventricular function in the midterm follow-up assessment after tetralogy of fallot repair.

BACKGROUND: Few data are available on quality of life after surgical repair of tetralogy of Fallot (ToF), and on its relationship to right ventricle function. METHODS: Patients with at least 1 year of follow-up evaluation after surgery were studied. Right ventricle function was evaluated by the Doppler-derived myocardial performance index (MPI), and health-related quality of life (HRQL) was assessed by the Child Health Questionnaire, Parent Form-50. Findings were compared with those for an age- and sex-matched group of healthy children. RESULTS: The study enrolled 35 successfully repaired ToF patients with 4.9 years (range, 3-7 years) of follow-up evaluation after surgery and 36 healthy children. The MPI demonstrated right ventricle dysfunction in patients compared with controls (0.34; range, 0.26-0.49 vs 0.2; range, 0.13-0.27; p < 0.01), although HRQL for the psychosocial domain was similar between patients and healthy children (summary score, 47.9; range, 45-52 vs 47.5; range, 44.5-50; p = 0.17). A trend for poorer physical area results was observed in patients (summary score, 44; range, 31-52 vs 48.5; range, 45.7-51.7; p = 0.06). Neither physical nor psychosocial summary scores for HRQL had any correlation with right ventricle MPI. CONCLUSIONS: Right ventricle dysfunction is present in postoperative ToF patients. The psychosocial domain of HRQL is preserved after surgery. A trend for worse results was observed in the physical domain. The right ventricle function is not related to quality of life after surgical repair of ToF.

Ebstein's anomaly with imperforate tricuspid valve. Prenatal diagnosis.

Ebstein's anomaly is an uncommon congenital heart defect, with a prevalence of 0.3-0.5%. Its association with an imperforate tricuspid valve is an even more rare situation (less than 10% of cases). Prenatal diagnosis of this association by means of fetal echocardiography has not been reported. We describe here this association diagnosed before birth and confirmed after birth. The diagnostic potential and importance of fetal echocardiography during prenatal evaluation of cardiac malformations allows for adequate perinatal planning and management, with an obvious impact on morbidity and mortality.