Literature DB >> 22192720

Hypoplastic left heart syndrome: current considerations and expectations.

Jeffrey A Feinstein1, D Woodrow Benson, Anne M Dubin, Meryl S Cohen, Dawn M Maxey, William T Mahle, Elfriede Pahl, Juan Villafañe, 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 Tweddell, Barbara J Deal, Anke K Furck, Geoffrey L Rosenthal, Richard G Ohye, Nancy S Ghanayem, John P Cheatham, Wayne Tworetzky, Gerard R Martin.   

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 © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

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Year:  2012        PMID: 22192720      PMCID: PMC6110391          DOI: 10.1016/j.jacc.2011.09.022

Source DB:  PubMed          Journal:  J Am Coll Cardiol        ISSN: 0735-1097            Impact factor:   24.094


  465 in total

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  113 in total

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