Current State of the Art in Hypoplastic Left Heart Syndrome

Authors

Aditya K Birla
Sunita Brimmer
Walker D Short
Oluyinka O Olutoye
Jason A Shar
Suriya Lalwani
Philippe Sucosky
Anitha Parthiban
Sundeep G Keswani
Christopher A Caldarone
Ravi K Birla

Publication Date

1-1-2022

Journal

Frontiers in Cardiovascular Medicine

DOI

10.3389/fcvm.2022.878266

PMID

36386362

PMCID

PMC9651920

PubMedCentral® Posted Date

10-28-2022

PubMedCentral® Full Text Version

Post-print

Published Open-Access

yes

Keywords

stem cells, congenital heart defect (CHD), genetics, hemodynamic, regenerative medicine

Abstract

Hypoplastic left heart syndrome (HLHS) is a complex congenital heart condition in which a neonate is born with an underdeveloped left ventricle and associated structures. Without palliative interventions, HLHS is fatal. Treatment typically includes medical management at the time of birth to maintain patency of the ductus arteriosus, followed by three palliative procedures: most commonly the Norwood procedure, bidirectional cavopulmonary shunt, and Fontan procedures. With recent advances in surgical management of HLHS patients, high survival rates are now obtained at tertiary treatment centers, though adverse neurodevelopmental outcomes remain a clinical challenge. While surgical management remains the standard of care for HLHS patients, innovative treatment strategies continue to be developing. Important for the development of new strategies for HLHS patients is an understanding of the genetic basis of this condition. Another investigational strategy being developed for HLHS patients is the injection of stem cells within the myocardium of the right ventricle. Recent innovations in tissue engineering and regenerative medicine promise to provide important tools to both understand the underlying basis of HLHS as well as provide new therapeutic strategies. In this review article, we provide an overview of HLHS, starting with a historical description and progressing through a discussion of the genetics, surgical management, post-surgical outcomes, stem cell therapy, hemodynamics and tissue engineering approaches.