In utero valvuloplasty for pulmonary atresia with hypoplastic right ventricle: techniques and outcomes.

Abstract

Prenatal intervention for fetuses with pulmonary atresia with an intact ventricular septum (PA/IVS) has the potential to alter right heart physiologic features in utero, facilitating right heart growth and improving the prospect of a biventricular outcome after birth. Since 2002, we have considered prenatal intervention for fetal PA/IVS in patients with (1) membranous pulmonary atresia, with identifiable pulmonary valve (PV) leaflets or membrane; (2) an intact or highly restrictive ventricular septum; and (3) right heart hypoplasia, with a tricuspid valve annulus z score of -2 or below and an identifiable but small right ventricle. Intervention was performed through direct cardiac puncture under ultrasound guidance, with percutaneous access or access through a limited laparotomy. Ten fetuses underwent attempted balloon dilation of the PV in utero. The first 4 procedures were technically unsuccessful, and the most-recent 6 were technically successful. Compared with control fetuses with PA/IVS who did not undergo prenatal intervention and had univentricular outcomes after birth, the tricuspid valve annulus, right ventricle length, and PV annulus grew significantly more from midgestation to late gestation in the 6 fetuses who underwent successful interventions. In utero perforation and dilation of the PV in midgestation fetuses with PA/IVS is technically feasible and may be associated with improved right heart growth and postnatal outcomes for fetuses with moderate right heart hypoplasia in midgestation. There is an important learning curve for this procedure, and much remains to be learned about the selection of appropriate fetuses for prenatal intervention.