A rare conotruncal anomaly: absent pulmonary valve syndrome, interrupted aortic arch, and severe aortic stenosis

Abstract

The coexistence of absent pulmonary valve syndrome, Type B interrupted aortic arch, and severe aortic stenosis represents an exceptionally rare and complex congenital cardiac phenotype. The diagnosis was established in a newborn using multimodality imaging. Given the profound airway compression secondary to the massive pulmonary artery dilatation and the associated poor prognosis, palliative care was chosen.