Prenatal diagnosis of hypoplastic aortic arch without intracardiac malformations: The nevada experience.

Objective: We reviewed our center's experience with neonatal and infant hypoplastic aortic arch, unassociated with intracardiac malformations, and investigated changes in prenatal detection rates over time for those requiring therapeutic procedures.

Methods: We identified all prenatal diagnoses of hypoplastic aortic arch with situs solitus, unassociated with intracardiac malformations, made in Nevada between May 2017 and April 2022. In addition, we identified all those 0-180 days old, with prenatal care, that underwent a surgical or interventional cardiac catheterization aortic arch procedure, whether prenatally or postnatally diagnosed. We excluded those with ventricular septal defects, functionally univentricular hearts, interrupted aortic arches, or any associated malformation requiring an additional surgical or interventional procedure ≤6 months old. Additionally, we calculated prenatal detection rates for those undergoing a surgical or interventional catheterization procedure for each of the 5 years.

Results: We identified 107 patients prenatally and postnatally. Of the 107 patients, 56 (34 prenatally diagnosed and 22 postnatally diagnosed) underwent an aortic arch procedure, and 51 additionally prenatally diagnosed, live-born infants did not undergo a procedure. Of the 56 procedures, 2 were by interventional catheterization, and 54 underwent a surgical repair. Prenatal detection for those undergoing a procedure statistically significantly increased over the 5 years from 38% to 82%, rho = 0.95 (p = .04).

Conclusions: Currently in Nevada, our prenatal detection rate is >80% in the general population for those between 0 and 6 months old who require a therapeutic procedure for aortic arch obstruction without intracardiac malformations.