Hipertensión arterial pulmonar en niños con síndrome de Down y cardiopatía congénita. Es realmente más severa? / Pulmonary hypertension in children with Down's syndrome and congenital heart disease. Is it really more severe?

ABSTRACT

OBJECTIVE: To compare the hemodynamic state, the severity and reversibility of pulmonary arterial hypertension (PAH) in patients with Down's syndrome and congenital heart disease (CHD) with respect to those without chromosomal pathologies. MATERIAL AND METHODS: 30 patients with congenital heart disease and left to right shunt were studied, corroborated by echocardiography; 16 patients had Down's syndrome and CHD and the control group was constituted by 14 patients without chromosomal abnormalities and with CHD. The age was R = 4.7 +/- 5.8 years for the Down's syndrome group and x = 5.3 +/- 4.5 years for the control group. All patients were subjected to a complete hemodynamic study, as well as to structural analysis by pulmonary wedge angiography (PWA), tested with oxygen administration. RESULTS: The most frequent diagnosis was ventricular septal defect for the control group and common atrioventricular canal for the Down's group. The systolic and mean pulmonary pressure depicted very similar values in both groups, with an average of 84.87 +/- 13.16 mm Hg for the Down's group and 84.21 +/- 22.05 for the control group. After oxygen administration, a tendency of increased Qp/Qs was found with a drop in pulmonary resistance in both groups, but being more important in the control group. During PWA assessment no quantitative differences were observed in PAH between both groups nor after the angiography with oxygen administration. CONCLUSIONS: Although patients with Down's syndrome present CHD with greater predisposition to develop irreversible pulmonary arterial hypertension like common atrioventricular canal, the hemodynamic behavior of pulmonary hypertension and during the challenge with oxygen was similar in both groups.