Hipertensão pulmonar irreversível secundária a cardiopatia congênita de hiperfluxo pulmonar em crianças e adolescentes

Abstract

Introduction: Pulmonary arterial hypertension (PAH) is a progressive pulmonary vascular disease characterized by elevation of mean pulmonary artery pressure, being greater than or equal to 25 mmHg at rest. It can be caused by many diseases and is a common complication of congenital heart diseases with increased pulmonary blood flow, with two developmental stages: reversible and irreversible. The Eisenmenger syndrome is the irreversible stage and resembles the idiopathic pulmonary arterial hypertension (IPAH). Although, the Eisenmenger syndrome´s prognosis is much better when compared to other forms of PAH especially IPAH. Objective: This study aims to conduct a descriptive analysis of the patient´s profile with PAH due to congenital heart disease with increased pulmonary blood flow; determine the prevalence of PAH in children and adolescents with pulmonary hyperflow heart disease, with and without genetic syndrome; identify the factors involved in development as well as prevention measures. Patients and Methods: We conduct a retrospective study of patient charts from the last 10 years including 214 children and adolescents between 0 and 18 years old, diagnosed with congenital heart disease with increased pulmonary blood flow, with and without pulmonary hypertension, referred for surgical correction at the Clinics Hospital of the Federal University of Minas Gerais. Was used for the statistical analysis, the chi-square test with Yates correction and Fisher's exact test to compare the groups. Was set at 0.05 the level of rejection of the null hypothesis. Results: We studied 214 patients with congenital heart disease with shunt and 28% of them had some degree of pulmonary hypertension. Eisenmenger syndrome was identified in eight patients, and most of these had associated Down syndrome. It was found that patients with post-tricuspid shunt disease had higher frequency of PHA than those with pre-tricuspid shunt disease. Conclusion: Although it is a small sample, the findings are the same as described in the literature. Given the high possibility of the development of PAH in children with congenital heart disease with shunt, and especially those associated with genetic syndromes, the best prevention to PAH is the diagnosis and surgical treatment of every patient with congenital heart disease with increased pulmonary blood flow in due time, especially those with post-tricuspid shunt.