Hypoplastic left heart syndrome: Current considerations and expectations

Jeffrey A. Feinstein*, D. Woodrow Benson, Anne M. Dubin, Meryl S. Cohen, Dawn M. Maxey, William T. Mahle, Elfriede Pahl, Juan Villafae, Ami B. Bhatt, Lynn F. Peng, Beth Ann Johnson, Alison L. Marsden, Curt J. Daniels, Nancy A. Rudd, Christopher A. Caldarone, Kathleen A. Mussatto, David L. Morales, D. Dunbar Ivy, J. William Gaynor, James S. TweddellBarbara J. Deal, Anke K. Furck, Geoffrey L. Rosenthal, Richard G. Ohye, Nancy S. Ghanayem, John P. Cheatham, Wayne Tworetzky, Gerard R. Martin

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

286 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)S1-S42
JournalJournal of the American College of Cardiology
Volume59
Issue number1 SUPPL.
DOIs
StatePublished - Jan 3 2012

Keywords

  • Fontan procedure
  • Glenn procedure
  • Norwood procedure
  • congenital heart defects
  • hypoplastic left heart syndrome

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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