Hypoplastic left heart syndrome: current considerations and expectations.

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.

journal_name

J Am Coll Cardiol

authors

Feinstein JA,Benson DW,Dubin AM,Cohen MS,Maxey DM,Mahle WT,Pahl E,Villafañe J,Bhatt AB,Peng LF,Johnson BA,Marsden AL,Daniels CJ,Rudd NA,Caldarone CA,Mussatto KA,Morales DL,Ivy DD,Gaynor JW,Tweddell JS,Deal BJ,Fu

doi

10.1016/j.jacc.2011.09.022

subject

Has Abstract

pub_date

2012-01-03 00:00:00

pages

S1-42

issue

1 Suppl

eissn

0735-1097

issn

1558-3597

pii

S0735-1097(11)03342-0

journal_volume

59

pub_type

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