RESUMO
Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.
Assuntos
Hipertensão Pulmonar , Artéria Pulmonar , Criança , Humanos , Hipertensão Pulmonar Primária Familiar/cirurgia , Artéria Pulmonar/cirurgia , Anastomose Cirúrgica , Hipertensão Pulmonar/tratamento farmacológico , Hipertensão Pulmonar/cirurgia , Aorta Torácica/cirurgiaRESUMO
ABSTRACT Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.