NCBISkiptomaincontentSkiptonavigationResourcesHowToAboutNCBIAccesskeysMyNCBISignintoNCBISignOutJournalListPaediatrChildHealthv.5(4);May-Jun2000PMC2817797PaediatrChildHealth.2000May-Jun;5(4):219–225.doi: 10.1093/pch/5.4.219PMCID:PMC2817797PMID:20177524Language:English|FrenchHypoplasticleftheartsyndrome:PrognosisandmanagementoptionsDeborahSFruitman,BScAuthorinformationCopyrightandLicenseinformationDisclaimerFacultyofMedicine,DalhousieUniversity,Halifax,NovaScotiaCorrespondenceandreprints:MsDeborahSFruitman,c/oDrErmelindaPelausa,DepartmentofPediatrics,DivisionofNeonatology,IWK-GraceHealthCentre,5850/5980UniversityAvenue,POBox3070,Halifax,NovaScotiaB3J3G9.Telephone902-420-6474,fax902-420-6469,e-mailac.lad.dem.1smcmput@amtiurfdCopyright©2000,PulsusGroupInc.AllrightsreservedThisarticlehasbeencitedbyotherarticlesinPMC.Fewcongenitalheartmalformationshaveraisedasmanysurgical,ethical,socialandeconomicissuesasthetherapyforinfantsdiagnosedwithhypoplasticleftheartsyndrome.Beforethe1980s,thiscomplexmalformationwasassociatedwith95%mortalitywithinthefirstmonthoflife.Inthepasttwodecades,palliativesurgeryandcardiactransplantationhavebecomemanagementoptions,inadditiontocomfortcarefortheinfant.Theseinnovationshaveforcedparentsandphysicianstomakedifficultdecisionsbecausethelongtermresultsoftheadditionaltreatmentoptionsarenotknown.Thisarticledescribesthecurrentriskfactors,diagnosis,treatmentandoutcomeofinfantswithhypoplasticleftheartsyndrome.Prenataldiagnosisprovidesfamilieswithtimeforcounsellingandforbecomingmoreinformedaboutmanagementoptions.Surgicaltherapyprovideshopeforthesurvivaloftheseinfants,buttheirlongtermoutcomesarenotwelldefined.Comfortcareineitherthehomeorhospitalremainsanacceptablemanagementoption.Moreinvestigationstodeterminethelongtermoutcomefollowingpalliativesurgeryandtransplantationareneededbeforetheybecomethestandardsofcare.Keywords:Congenitalheartdisease,Diagnosis,Hypoplasticleftheartsyndrome,Outcomesanalysis,TreatmentPeudemalformationscardiaquescongénitalesontsuscité