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Author Notes:

Reprint requests and correspondence: Dr. Jeffrey A. Feinstein, Department of Pediatrics (Cardiology)/Stanford University School of Medicine, 750 Welch Road, Suite 305, Palo Alto, California 94304. jeff.feinstein@stanford.edu.

Dr. Feinstein has received research grant support from Pfizer and GlaxoSmithKline.

Dr. Dubin has received fellowship funding from Medtronic.

Dr. Ivy receives salary support for being a consultant for Actelion, Gilead, Pfizer, and United Therapeutics.

All other authors have reported that they have no relationships relevant to the contents of this paper to disclose.

Subjects:

Keywords:

  • Science & Technology
  • Life Sciences & Biomedicine
  • Cardiac & Cardiovascular Systems
  • Cardiovascular System & Cardiology
  • congenital heart defects
  • Fontan procedure
  • Glenn procedure
  • hypoplastic left heart syndrome
  • Norwood procedure
  • PROTEIN-LOSING ENTEROPATHY
  • QUALITY-OF-LIFE
  • PULMONARY-ARTERY CONDUIT
  • BIDIRECTIONAL CAVOPULMONARY ANASTOMOSIS
  • BLALOCK-TAUSSIG SHUNT
  • SINGLE-VENTRICLE PHYSIOLOGY
  • MODIFIED NORWOOD-PROCEDURE
  • VENOUS OXYGEN-SATURATION
  • STAGE-I NORWOOD
  • COMPUTATIONAL FLUID-DYNAMICS

Hypoplastic Left Heart Syndrome Current Considerations and Expectations

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Tools:

Journal Title:

Journal of the American College of Cardiology

Volume:

Volume 59, Number 1

Publisher:

, Pages S1-S42

Type of Work:

Article | Post-print: After Peer Review

Abstract:

In the recent era, no congenital heart defect has undergone a more dramatic change in diagnostic approach, management, and outcomes than hypoplastic left heart syndrome (HLHS). During this time, survival to the age of 5 years (including Fontan) has ranged from 50% to 69%, but current expectations are that 70% of newborns born today with HLHS may reach adulthood. Although the 3-stage treatment approach to HLHS is now well founded, there is significant variation among centers. In this white paper, we present the current state of the art in our understanding and treatment of HLHS during the stages of care: 1) pre-Stage I: fetal and neonatal assessment and management; 2) Stage I: perioperative care, interstage monitoring, and management strategies; 3) Stage II: surgeries; 4) Stage III: Fontan surgery; and 5) long-term follow-up. Issues surrounding the genetics of HLHS, developmental outcomes, and quality of life are addressed in addition to the many other considerations for caring for this group of complex patients.

Copyright information:

© 2012 American College of Cardiology Foundation.

This is an Open Access work distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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