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. Tweddell; Barbara J. Deal; Anke K. Furck; Geoffrey L. Rosenthal; Richard G. Ohye; Nancy S. Ghanayem; John P. Cheatham; Wayne Tworetzky; Gerard R. Martin

doi:10.1016/j.jacc.2011.09.022

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

Pediatrics

Research output: Contribution to journal › Review article › peer-review

436 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 language	English (US)
Pages (from-to)	S1-S42
Journal	Journal of the American College of Cardiology
Volume	59
Issue number	1 SUPPL.
DOIs	https://doi.org/10.1016/j.jacc.2011.09.022
State	Published - 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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10.1016/j.jacc.2011.09.022

Cite this

Feinstein, J. A., Benson, D. W., Dubin, A. M., Cohen, M. S., Maxey, D. M., Mahle, W. T., Pahl, E., Villafae, J., Bhatt, A. B., Peng, L. F., Johnson, B. A., Marsden, A. L., Daniels, C. J., Rudd, N. A., Caldarone, C. A., Mussatto, K. A., Morales, D. L., Ivy, D. D., Gaynor, J. W., ... Martin, G. R. (2012). Hypoplastic left heart syndrome: Current considerations and expectations. Journal of the American College of Cardiology, 59(1 SUPPL.), S1-S42. https://doi.org/10.1016/j.jacc.2011.09.022

@article{fe63b6a3e4224814a839fb3bea77a526,

title = "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.",

keywords = "Fontan procedure, Glenn procedure, Norwood procedure, congenital heart defects, hypoplastic left heart syndrome",

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

note = "Funding Information: 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. ",

year = "2012",

month = jan,

day = "3",

doi = "10.1016/j.jacc.2011.09.022",

language = "English (US)",

volume = "59",

pages = "S1--S42",

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Feinstein, JA, Benson, DW, Dubin, AM, Cohen, MS, Maxey, DM, Mahle, WT, Pahl, E, Villafae, 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 2012, 'Hypoplastic left heart syndrome: Current considerations and expectations', Journal of the American College of Cardiology, vol. 59, no. 1 SUPPL., pp. S1-S42. https://doi.org/10.1016/j.jacc.2011.09.022

TY - JOUR

T1 - Hypoplastic left heart syndrome

T2 - Current considerations and expectations

AU - Feinstein, Jeffrey A.

AU - Benson, D. Woodrow

AU - Dubin, Anne M.

AU - Cohen, Meryl S.

AU - Maxey, Dawn M.

AU - Mahle, William T.

AU - Pahl, Elfriede

AU - Villafae, Juan

AU - Bhatt, Ami B.

AU - Peng, Lynn F.

AU - Johnson, Beth Ann

AU - Marsden, Alison L.

AU - Daniels, Curt J.

AU - Rudd, Nancy A.

AU - Caldarone, Christopher A.

AU - Mussatto, Kathleen A.

AU - Morales, David L.

AU - Ivy, D. Dunbar

AU - Gaynor, J. William

AU - Tweddell, James S.

AU - Deal, Barbara J.

AU - Furck, Anke K.

AU - Rosenthal, Geoffrey L.

AU - Ohye, Richard G.

AU - Ghanayem, Nancy S.

AU - Cheatham, John P.

AU - Tworetzky, Wayne

AU - Martin, Gerard R.

N1 - Funding Information: 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.

PY - 2012/1/3

Y1 - 2012/1/3

N2 - 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.

AB - 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.

KW - Fontan procedure

KW - Glenn procedure

KW - Norwood procedure

KW - congenital heart defects

KW - hypoplastic left heart syndrome

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U2 - 10.1016/j.jacc.2011.09.022

DO - 10.1016/j.jacc.2011.09.022

M3 - Review article

C2 - 22192720

AN - SCOPUS:84055190992

SN - 0735-1097

VL - 59

SP - S1-S42

JO - Journal of the American College of Cardiology

JF - Journal of the American College of Cardiology

IS - 1 SUPPL.

ER -

Hypoplastic left heart syndrome: Current considerations and expectations

Abstract

Keywords

ASJC Scopus subject areas

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