Nonsurgical Palliation for Hypoplastic Left Heart

Background: We report a novel nonsurgical palliation for newborns with hypoplastic left heart syndrome (HLHS), HLHS variants, and hypoplastic left heart complex (HLHC). The aim of this transcatheter approach is replacing Norwood and open-chest hybrid procedures as well as complex biventricular repairs in the neonatal period, postponing them to infancy, therefore reducing the overall risks of these complex cases.

Method: Between 2019 and 2021, we palliate 13 patients with transcatheter endoluminal bilateral pulmonary artery banding and transfemoral ductal stenting. Seven patients had HLHS, two patients had HLHS variants, and four patients had HLHC. Median age was 3 days (2–21) and median weight was 3.3 kg (1.8–3.9). For endoluminal banding, microvascular plug devices (MVP) were used. Initially, the thin polytetrafluoroethylene-covered, nitinol-framed, self-expandable MVPs were manually modified and converted from a plug device to a pulmonary flow restrictor (PFR). Then, through femoral approach PFRs were placed within the branch pulmonary arteries. After bilateral PFR placement, the arterial duct was stented with balloon-expandable stent for treating a narrowed duct and self-expandable stent for stenting a wide open arterial duct. All PFRs were removed by snare or by using dissector during the surgical procedure.

Results: There was no hospital mortality. One patient with HLHS died at home 8 weeks after the procedure. One patient with HLHS and cardiomyopathy was successfully transplanted 2.5 months after the procedure. Eight patients required catheter reinterventions until surgical procedure. Six patients with HLHS have already received comprehensive stage II operation at a median age of 4.5 months. One patient is waiting for the operation. One patient with critical aortic stenosis and severe endocardial fibroelastosis had comprehensive stage II operation but died 6 weeks after the operation due to sepsis. Three patients with HLHC underwent biventricular repair. Three patients needed pulmonary artery catheter reinterventions after surgery. Overall, 11 patients are still alive after the procedure.

Conclusion: This novel transcatheter approach can be successfully used for the first stage palliation of HLHS and HLHC patients as an alternative to surgical procedures. This novel approach has the potential to allow broader treatment of complex congenital defects.

Publication History

Article published online:
03 February 2022