Perfil de apresentação clínica dos tumores cardíacos em crianças e adolescentes

Abstract

Background: Entity rare, intracardiac tumors affecting between 1.7 to 28 per 10,000 live births. Most tumors have a benign evolution and presents spontaneous regression still in the first year of life. Despite this favorable outcome in childhood, tumors can present with multiple symptomatology and adverse hemodynamic consequences from mild to fatal. This outcome can be avoided with early diagnosis and institution of appropriate therapy to overcome the clinical implications pending the regression of tumors. Objective: This work aims to study the incidence and patterns of presentation of intracardiac tumors in childhood and its natural history. Method: Research was conducted with retrospective analysis of medical records and imaging studies of patients between 0 and 12 years with a diagnosis of intracardiac tumors between years 1995 and 2010 in a referral hospital. We performed univariate analysis and comparison of groups by the chi-square and Fisher exact for categorical variables, being adopted significance level of p <0.05. In parallel with the data collection in the Sectors of Echocardiography and Neonatology, research was done in the international literature through international databases and references. Results: We found 16 cases of intracardiac tumors, that correspond to incidence of 0.029% (considering children born in the same hospital during period of study). Rhabdomyomas predominated in the first year of life. Half of cases were diagnosed intrautero. Most children with tumors was oligo or asymptomatic. The fatal outcome was more significant in patients with hemodynamic changes on echocardiogram. There was partial or complete regression in 60% of cases with rhabdomyomas characteristics in echocardiography. Conclusions: The incidence of intracardiac tumors found in childhood confers with international literature. In our series, there was an increase in diagnosis with advent of fetal echocardiography.