Hypoplastic left heart syndrome: From bedside to bench and back

Anita Saraf, Wendy M. Book, Timothy J Nelson, Chunhui Xu

Research output: Contribution to journalReview article

Abstract

Hypoplastic Left Heart Syndrome (HLHS) is a complex Congenital Heart Disease (CHD) that was almost universally fatal until the advent of the Norwood operation in 1981. Children with HLHS who largely succumbed to the disease within the first year of life, are now surviving to adulthood. However, this survival is associated with multiple comorbidities and HLHS infants have a higher mortality rate as compared to other non-HLHS single ventricle patients. In this review we (a) discuss current clinical challenges associated in the care of HLHS patients, (b) explore the use of systems biology in understanding the molecular framework of this disease, (c) evaluate induced pluripotent stem cells as a translational model to understand molecular mechanisms and manipulate them to improve outcomes, and (d) investigate cell therapy, gene therapy, and tissue engineering as a potential tool to regenerate hypoplastic cardiac structures and improve outcomes.

Original languageEnglish (US)
Pages (from-to)109-118
Number of pages10
JournalJournal of Molecular and Cellular Cardiology
Volume135
DOIs
StatePublished - Oct 1 2019

Fingerprint

Hypoplastic Left Heart Syndrome
Norwood Procedures
Induced Pluripotent Stem Cells
Systems Biology
Tissue Engineering
Cell- and Tissue-Based Therapy
Genetic Therapy
Comorbidity
Heart Diseases
Survival
Mortality

Keywords

  • Congenital heart disease
  • Hypoplastic left heart syndrome

ASJC Scopus subject areas

  • Molecular Biology
  • Cardiology and Cardiovascular Medicine

Cite this

Hypoplastic left heart syndrome : From bedside to bench and back. / Saraf, Anita; Book, Wendy M.; Nelson, Timothy J; Xu, Chunhui.

In: Journal of Molecular and Cellular Cardiology, Vol. 135, 01.10.2019, p. 109-118.

Research output: Contribution to journalReview article

@article{5d5a0799cf074556911e78b9ce43d236,
title = "Hypoplastic left heart syndrome: From bedside to bench and back",
abstract = "Hypoplastic Left Heart Syndrome (HLHS) is a complex Congenital Heart Disease (CHD) that was almost universally fatal until the advent of the Norwood operation in 1981. Children with HLHS who largely succumbed to the disease within the first year of life, are now surviving to adulthood. However, this survival is associated with multiple comorbidities and HLHS infants have a higher mortality rate as compared to other non-HLHS single ventricle patients. In this review we (a) discuss current clinical challenges associated in the care of HLHS patients, (b) explore the use of systems biology in understanding the molecular framework of this disease, (c) evaluate induced pluripotent stem cells as a translational model to understand molecular mechanisms and manipulate them to improve outcomes, and (d) investigate cell therapy, gene therapy, and tissue engineering as a potential tool to regenerate hypoplastic cardiac structures and improve outcomes.",
keywords = "Congenital heart disease, Hypoplastic left heart syndrome",
author = "Anita Saraf and Book, {Wendy M.} and Nelson, {Timothy J} and Chunhui Xu",
year = "2019",
month = "10",
day = "1",
doi = "10.1016/j.yjmcc.2019.08.005",
language = "English (US)",
volume = "135",
pages = "109--118",
journal = "Journal of Molecular and Cellular Cardiology",
issn = "0022-2828",
publisher = "Academic Press Inc.",

}

TY - JOUR

T1 - Hypoplastic left heart syndrome

T2 - From bedside to bench and back

AU - Saraf, Anita

AU - Book, Wendy M.

AU - Nelson, Timothy J

AU - Xu, Chunhui

PY - 2019/10/1

Y1 - 2019/10/1

N2 - Hypoplastic Left Heart Syndrome (HLHS) is a complex Congenital Heart Disease (CHD) that was almost universally fatal until the advent of the Norwood operation in 1981. Children with HLHS who largely succumbed to the disease within the first year of life, are now surviving to adulthood. However, this survival is associated with multiple comorbidities and HLHS infants have a higher mortality rate as compared to other non-HLHS single ventricle patients. In this review we (a) discuss current clinical challenges associated in the care of HLHS patients, (b) explore the use of systems biology in understanding the molecular framework of this disease, (c) evaluate induced pluripotent stem cells as a translational model to understand molecular mechanisms and manipulate them to improve outcomes, and (d) investigate cell therapy, gene therapy, and tissue engineering as a potential tool to regenerate hypoplastic cardiac structures and improve outcomes.

AB - Hypoplastic Left Heart Syndrome (HLHS) is a complex Congenital Heart Disease (CHD) that was almost universally fatal until the advent of the Norwood operation in 1981. Children with HLHS who largely succumbed to the disease within the first year of life, are now surviving to adulthood. However, this survival is associated with multiple comorbidities and HLHS infants have a higher mortality rate as compared to other non-HLHS single ventricle patients. In this review we (a) discuss current clinical challenges associated in the care of HLHS patients, (b) explore the use of systems biology in understanding the molecular framework of this disease, (c) evaluate induced pluripotent stem cells as a translational model to understand molecular mechanisms and manipulate them to improve outcomes, and (d) investigate cell therapy, gene therapy, and tissue engineering as a potential tool to regenerate hypoplastic cardiac structures and improve outcomes.

KW - Congenital heart disease

KW - Hypoplastic left heart syndrome

UR - http://www.scopus.com/inward/record.url?scp=85070884876&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85070884876&partnerID=8YFLogxK

U2 - 10.1016/j.yjmcc.2019.08.005

DO - 10.1016/j.yjmcc.2019.08.005

M3 - Review article

C2 - 31419439

AN - SCOPUS:85070884876

VL - 135

SP - 109

EP - 118

JO - Journal of Molecular and Cellular Cardiology

JF - Journal of Molecular and Cellular Cardiology

SN - 0022-2828

ER -