Faculty Collaboration Database

Hypoplastic left heart syndrome: current considerations and expectations. J Am Coll Cardiol 2012 Jan 03;59(1 Suppl):S1-42

Date

01/04/2012

Pubmed ID

22192720

Pubmed Central ID

PMC6110391

DOI

10.1016/j.jacc.2011.09.022

Scopus ID

2-s2.0-84055190992 (requires institutional sign-in at Scopus site)   413 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.

Author List

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, Furck AK, Rosenthal GL, Ohye RG, Ghanayem NS, Cheatham JP, Tworetzky W, Martin GR

Authors

Kathleen Mussatto Ph.D. Associate Professor in the School of Nursing department at Milwaukee School of Engineering
Nancy A. Rudd RN APP Inpatient 1 in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Cardiac Surgical Procedures
Child Development
Echocardiography, Doppler
Female
Fontan Procedure
Humans
Hypoplastic Left Heart Syndrome
Infant, Newborn
Male
Monitoring, Physiologic
Perioperative Care
Pregnancy
Prenatal Diagnosis
Prognosis
Risk Assessment
Survival Analysis
Treatment Outcome
Ultrasonography, Doppler