RESUMEN
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.
Asunto(s)
Cardiopatías Congénitas/epidemiología , Adulto , Niño , Preescolar , Atención a la Salud/organización & administración , Cardiopatías Congénitas/terapia , Trasplante de Corazón , Humanos , Incidencia , Lactante , Recién Nacido , Prevalencia , América del Sur/epidemiología , Resultado del TratamientoRESUMEN
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.
Asunto(s)
Estado de Salud , Evaluación de Resultado en la Atención de Salud , Calidad de Vida , Tetralogía de Fallot/cirugía , Función Ventricular Derecha/fisiología , Niño , Preescolar , Ecocardiografía Doppler en Color , Electrocardiografía , Femenino , Estudios de Seguimiento , Ventrículos Cardíacos/diagnóstico por imagen , Ventrículos Cardíacos/fisiopatología , Humanos , Tiempo de Internación , Masculino , Periodo Posoperatorio , Estudios Retrospectivos , Tetralogía de Fallot/diagnóstico por imagen , Tetralogía de Fallot/fisiopatología , Factores de TiempoRESUMEN
BACKGROUND: Polyarteritis nodosa is a necrotising vasculitis of the medium sized and small muscular arteries. The inflammatory and subsequent reparative processes may alter the arterial mechanical properties. The effect of vasculitic damage on arterial distensibility has never been explored however. AIM: To determine the normal values and the effect of childhood vasculitis on arterial distensibility in children and teenagers. METHODS: Distensibility of the brachioradial arterial segment was studied using pulse wave velocity (PWV proportional, variant 1/ radical distensibility), in 13 children with polyarteritis nodosa at a median age of 11.8 (range 4.9-16) years. As a control group, 155 healthy schoolchildren (6-18 years, 81 boys) were studied. PWV was assessed using a photoplethysmographic technique; blood pressure was measured by an automatic sphygmomanometer (Dinamap). Data from patients were expressed as z scores adjusted for age and compared to a population mean of 0 by a single sample t test. Determinants of PWV in normal children were assessed by univariate and multivariate linear regression analyses. RESULTS: Age, height, weight, and systolic blood pressure correlated individually with the brachioradial PWV. Multivariate analysis identified age as the only independent determinant. Ten of the patients were in clinical remission, while three had evidence of disease activity at the time of study. The PWV in the patient group as a whole was significantly greater than those in healthy children (mean z score +0.99). Raised C reactive protein concentration (>2 mg/dl) in the three patients with active disease was associated with a higher PWV when compared to those in remission (z score +2.78 v +0.45). The diastolic blood pressure of the patients was higher than those of the controls (z score +1.04) while the systolic pressure was similar (z score -0.36). CONCLUSIONS: PWV in the brachioradial arterial segment increases gradually during childhood independent of body weight, height, mass, and blood pressure. Increased PWV, and hence decreased distensibility, in this peripheral arterial segment occurs in polyarteritis nodosa and is amplified during acute inflammatory exacerbation.
Asunto(s)
Arterias/fisiología , Poliarteritis Nudosa/fisiopatología , Adolescente , Envejecimiento/fisiología , Análisis de Varianza , Arterias/fisiopatología , Velocidad del Flujo Sanguíneo , Niño , Preescolar , Elasticidad , Femenino , Humanos , Modelos Lineales , Masculino , Fotopletismografía/métodos , Flujo Pulsátil , Resistencia VascularRESUMEN
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.