Características y resultados del Registro español de válvula aórtica bicúspide en pediatría (REVAB) / Characteristics and outcomes of the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB)

Introducción y objetivos: La válvula aórtica bicúspide (VAB) es la cardiopatía congénita más frecuente. El objetivo de este estudio es describir las características de los pacientes en edad pediátrica con VAB en un registro poblacional. Métodos: Los datos de los pacientes se obtuvieron del Registro español de válvula aórtica bicúspide (REVAB) en pediatría (menores de 18 años). Para el análisis de datos, se dividió a los pacientes en 2 grupos según sus características: pacientes con VAB solo y pacientes con VAB y cardiopatía congénita concomitantes. Resultados: Se analizó a un total de 1.681 pacientes de 33 hospitales. La mayoría (1.158, 69,6%) eran varones. La morfología de la válvula fue horizontal en el 63,4% (1.012), y puras (tipo 0 Sievers), el 28,4% (469). El 63,7% (1.060) tenían solo VAB y en el 23,4% (390) concurrían lesiones obstructivas del lado izquierdo. Del total, el 8,6% (145) precisó alguna intervención en la válvula aórtica. Conclusiones: Estos datos representan la primera descripción de base poblacional de la presentación clínica y los resultados en los pacientes del REVAB Pediátrico.(AU)

Introduction and objectives: Bicuspid aortic valve (BAV) disorder is the most common congenital heart disease. The aim of this study was to describe the characteristics of 0- to 18-year olds with BAV in a population-based registry. Methods: Data from all pediatric patients were obtained from the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB) (< 18 years). For data analysis, patients with BAV were divided into 2 groups by their features: isolated BAV and BAV with associated congenital heart disease. Results: We included 1681 patients from 33 hospitals. Males accounted for 69.6% (n = 1158). Valve morphology was horizontal in 63.4% (n = 1012) and pure (Sievers type 0) in 28.4% (n=469). Isolated BAV was present in 63.7% (n=1060), and concomitant left-sided obstructive lesions in 23.4% (n=390). Interventions were required in 8.6% (n=145). Conclusion: These data represent the first large, population-based description of the clinical presentations and outcomes of patients enrolled in the Spanish registry for pediatric patients with bicuspid aortic valve.(AU)

Characteristics and outcomes of the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB).

INTRODUCTION AND OBJECTIVES: Bicuspid aortic valve (BAV) disorder is the most common congenital heart disease. The aim of this study was to describe the characteristics of 0- to 18-year olds with BAV in a population-based registry. METHODS: Data from all pediatric patients were obtained from the Spanish registry for pediatric patients with bicuspid aortic valve (REVAB) (< 18 years). For data analysis, patients with BAV were divided into 2 groups by their features: isolated BAV and BAV with associated congenital heart disease. RESULTS: We included 1681 patients from 33 hospitals. Males accounted for 69.6% (n = 1158). Valve morphology was horizontal in 63.4% (n = 1012) and pure (Sievers type 0) in 28.4% (n=469). Isolated BAV was present in 63.7% (n=1060), and concomitant left-sided obstructive lesions in 23.4% (n=390). Interventions were required in 8.6% (n=145). CONCLUSION: These data represent the first large, population-based description of the clinical presentations and outcomes of patients enrolled in the Spanish registry for pediatric patients with bicuspid aortic valve.

Cor triatriatum con comunicación interauricular asociada / Cor triatriatum with associated atrial septal defect

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A Venous Malformation: An Unusual Primary Cardiac Tumor in Children.

This case report describes a primary cardiac tumor, classified as venous malformation, diagnosed in an asymptomatic child. The tumor was located in the left atrium near the mitral valve without affecting the mitral valve's functioning. Complete resection of the lesion was performed because of the risk of systemic embolism. The lesion consisted of fibrous tissue with multiple venous vascular channels. The patient did not have similar lesions in other locations. Vascular primary cardiac tumors are extremely rare. Hemangiomas and lymphangiomas have been described previously, but to our knowledge, this is the first primary cardiac tumor identified as a venous malformation.