Interruption of the aortic arch associated with total atrioventricular septum defect in a patient with down syndrome

Abstract

Objective: To report a case of complex congenital cardiac association. Case detail: Late preterm newborn, appropriate for gestational age, male, prenatal exams suggested trisomy 21. Fetal echocardiogram with a suggestive diagnosis of aortic coarctation associated with total atrioventricular septal defect (AVSD). Scheduled birth in a high-risk maternity hospital. Post-birth echocardiogram showed interrupted aortic arch (IAA) + total AVSD. As a congenital defect of the dependent ductus arteriosus, a prostaglandin infusion was started and the pediatric surgical and cardiology teams were called in. Computed tomography angiography of the heart and basal vessels was performed for a better anatomical study and planning of the intervention. At 21 days, he underwent stent implantation in the ductus arteriosus via hemodynamics, which was successful. On the 7th day post-procedure, hemodynamically stable, with device well positioned in the channel, good flow and preserved cardiac function. Anticongestive medications have been adjusted, outpatient follow-up with pediatric cardiology, pending full surgical correction of the defect. Final considerations: IAA has morbidity and mortality. Diagnosis and effective management allowed survival in the face of systemic hypoflow heart disease, dependent on the ductus arteriosus. Failure to intervene early is the etiology for severe heart failure, cardiogenic shock and death. Prenatal diagnosis enables the preparation of the care team.