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.
authors.affiliation: Department of Pediatrics, Stanford University School of Medicine, Lucile Salter Packard Children's Hospital, Palo Alto, California 94304, USA. jeff.feinstein@stanford.edu
authors.forename: Jeffrey A
authors.initials: JA
authors.lastname: Feinstein
authors.forename: D Woodrow
authors.initials: DW
authors.lastname: Benson
authors.forename: Anne M
authors.initials: AM
authors.lastname: Dubin
authors.forename: Meryl S
authors.initials: MS
authors.lastname: Cohen
authors.forename: Dawn M
authors.initials: DM
authors.lastname: Maxey
authors.forename: William T
authors.initials: WT
authors.lastname: Mahle
authors.forename: Elfriede
authors.initials: E
authors.lastname: Pahl
authors.forename: Juan
authors.initials: J
authors.lastname: Villafañe
authors.forename: Ami B
authors.initials: AB
authors.lastname: Bhatt
authors.forename: Lynn F
authors.initials: LF
authors.lastname: Peng
authors.forename: Beth Ann
authors.initials: BA
authors.lastname: Johnson
authors.forename: Alison L
authors.initials: AL
authors.lastname: Marsden
authors.forename: Curt J
authors.initials: CJ
authors.lastname: Daniels
authors.forename: Nancy A
authors.initials: NA
authors.lastname: Rudd
authors.forename: Christopher A
authors.initials: CA
authors.lastname: Caldarone
authors.forename: Kathleen A
authors.initials: KA
authors.lastname: Mussatto
authors.forename: David L
authors.initials: DL
authors.lastname: Morales
authors.forename: D Dunbar
authors.initials: DD
authors.lastname: Ivy
authors.forename: J William
authors.initials: JW
authors.lastname: Gaynor
authors.forename: James S
authors.initials: JS
authors.lastname: Tweddell
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Deal
authors.forename: Anke K
authors.initials: AK
authors.lastname: Furck
authors.forename: Geoffrey L
authors.initials: GL
authors.lastname: Rosenthal
authors.forename: Richard G
authors.initials: RG
authors.lastname: Ohye
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Ghanayem
authors.forename: John P
authors.initials: JP
authors.lastname: Cheatham
authors.forename: Wayne
authors.initials: W
authors.lastname: Tworetzky
authors.forename: Gerard R
authors.initials: GR
authors.lastname: Martin
mesh_terms: D006348:Cardiac Surgical Procedures / Q000379:methods / Q000401:mortality; D002657:Child Development / Q000502:physiology; D015150:Echocardiography, Doppler / Q000379:methods; D005260:Female; D018729:Fontan Procedure / Q000379:methods* / Q000401:mortality; D006801:Humans; D018636:Hypoplastic Left Heart Syndrome / Q000175:diagnosis* / Q000401:mortality / Q000601:surgery*; D007231:Infant, Newborn; D008297:Male; D008991:Monitoring, Physiologic / Q000379:methods; D019990:Perioperative Care / Q000379:methods; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*; D011379:Prognosis; D018570:Risk Assessment; D016019:Survival Analysis; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler / Q000379:methods