RESUMO
Pulmonary atresia with ventricular septal defect, non-confluent pulmonary arteries, and bilateral arterial duct is a rare and complex CHD. Physiologic ductal closure may lead to life-threatening hypoxia. We present a case of successful bilateral ductal stenting as a bridge to further lower-risk surgical repair.
RESUMO
We report the case of a fetus with anamnios sequence and VACTERL syndrome, having a circumflex right aortic arch. Two arterial ducts join anteriorly to form a common vessel that connects to the pulmonary trunk with confluent pulmonary branches. Embryologically, the dorsal right 6th aortic arch did not disappear and the aortic arch development stopped in a symmetrical state with an exceptional "Y-shaped" merged bilateral arterial duct.
Assuntos
Canal Anal/anormalidades , Síndromes do Arco Aórtico/patologia , Esôfago/anormalidades , Cardiopatias Congênitas/patologia , Rim/anormalidades , Deformidades Congênitas dos Membros/patologia , Artéria Pulmonar/anormalidades , Artéria Pulmonar/patologia , Coluna Vertebral/anormalidades , Traqueia/anormalidades , Aborto Induzido , Adulto , Canal Anal/patologia , Síndromes do Arco Aórtico/congênito , Esôfago/patologia , Feminino , Feto , Humanos , Rim/patologia , Masculino , Coluna Vertebral/patologia , Traqueia/patologiaRESUMO
We describe a rare case of right aortic arch with bilateral arterial duct and disconnected left pulmonary artery. Prenatal diagnosis allowed to plan delivery at the referral center and to maintain perfusion of the disconnected pulmonary artery through prostaglandin E1 infusion until surgical reconnection was performed. Early postnatal treatment avoided functional loss of the left lung that would have happened if malformation had not been identified before birth.