What Is Hypoplastic Left Heart Syndrome (HLHS)?. Hypoplastic left heart syndrome (HLHS) is a rare birth defect of a baby's heart. The left side of the heart ...[SkiptoContent]SharetoFacebookSharetoTwitterSharetoPinterestShareviaEmailKidsHealth/forParents/HypoplasticLeftHeartSyndrome(HLHS)HypoplasticLeftHeartSyndrome(HLHS)Reviewedby:MichaelA.Bingler,MDLargertextsizeLargetextsizeRegulartextsizePrintenespañolSíndromedecorazónizquierdohipoplásicoWhatIsHypoplasticLeftHeartSyndrome(HLHS)?Hypoplasticleftheartsyndrome(HLHS)isararebirthdefectofababy'sheart.Theleftsideoftheheartdoesn'tgrowasitshould,makingitsmallerandweakerthannormal.Theleftsideoftheheartissupposedtopumpbloodouttothebody.Butababy'sheartwithHLHScan'tdothejob.Thismakesababyverysick.Withoutmedicinesandaseriesofthreesurgeriestorebuildtheheart,babieswithHLHSwon'tsurvive.Theleftsideoftheheartcan'tbefixed,sothegoalofthesurgeriesistorebuildpartsoftheheartand"redirect"thewaybloodflowstoandfromthebody.Soonafterbirth,ababywithHLHSneedsaround-the-clockcareinahospitalforthefirstweeksormonthsoflife.HowDoestheHeartWork?Theheartisamusclethatpumpsblood.Itactsliketwopumpsinone.Thepumpsarecalledventriclesandeachhasanimportantjob:Therightventriclegetsbloodfromthebodyandpumpsitoutthepulmonaryarterytothelungs.Inthelungs,bloodfillsupwithoxygenandgetsridofcarbondioxide.Theleftventriclegetsthebloodfromthelungsandpumpsitoutthroughtheaortatothebody.Becauseitpumpsbloodtotheentirebody,theleftventricleisastrongerpumpthantherightventricle.WhatHappensinHypoplasticLeftHeartSyndrome?Withhypoplasticleftheartsyndrome:Theleftventricleandaortaaretoosmalltopumpenoughbloodtothebody.Therightventricle,whichisonlysupposedtopumpbloodtothelungs,pumpsbloodtothelungsandthebodythroughanextrapathwaycalledapatentductusarteriosis(PDA).Thatmeanstherightventriclegetsoverworked.BabieswithHLHSarealmostalwaysbornwithanatrialseptaldefect(ASD).Thisisaholeintheupperpartoftheheart.Itletsbloodwithoxygenmixwithbloodlowonoxygen.Thentherightventriclepumpsthebloodtothelung

Hypoplastic left heart syndrome (HLHS) is a group of defects of the heart and large blood vessels. A child is born with this condition (congenital heart defect).nutch_noindexSearchTermMenuButtonDonateContactMyChartLoginFindaDoctorSecondOpinionDonateContactReferaPatientEnEspañolOurLocationsMedicalServicesAboutUsResearch&InnovationPatients&VisitorsForHealthProfessionalsSecondOpinionDonateEnEspañolMyChartLoginRecentlyVisitedOurLocationsMedicalServicesAboutUsResearch&InnovationPatients&VisitorsForHealthProfessionalsSearchTermCANCELViewMoreResultsLoading...Informationaboutthe2019NovelCoronavirus.Readthelatest>Informaciónsobreelcoronavirus2019(COVID-19).Aprendamás>/nutch_noindex HypoplasticLeftHeartSyndrome(HLHS)inChildrenWhatishypoplasticleftheartsyndromeinchildren?Hypoplasticleftheartsyndrome(HLHS)is agroupof defectsoftheheartandlargebloodvessels.Achildisbornwiththiscondition(congenitalheartdefect).Itoccurswhenpartoftheheartdoesn'tdevelopasitshouldduringthefirst 8weeksofpregnancy.InHLHS,mostoftheleftsideoftheheartis small,underdeveloped,orboth.Thefollowingstructuresareusuallyaffected:Mitralvalve. Thisvalvecontrolsbloodflowbetweentheleftatriumandleftventricle.Leftventricle. Thisisthelowerleftchamberoftheheart.Itpumps bloodtothebody.Aorticvalve. Thisvalvecontrolsbloodflowfromtheleftventricleintotheaorta,andthentothebody.Theaortaisthemajorbloodvesselthatdeliversbloodtothebody.Aorta. Thisisthelargearteryleadingfromtheleftventricletothebody.Theleftventricleisnormallyverystrongsoitcanpumpbloodtothebody.Whenitissmallandpoorlydeveloped,itcan'tpumpanyorenoughbloodouttothebody.Forthisreason,ababywithHLHSwillnotlivelongwithoutsurgerytofixthedefect.Whatcauseshypoplasticleftheartsyndromeinachild?Achildisbornwiththiscondition(congenital).Somecongenitalheartdefectsoccurmoreoftenincertainfamilies(geneticdefects).Inmanychildren,HLHSoccursbychance.Thereisnoclearreasonforitsdevelopment.Whatarethesymptomsofhypoplasticleftheartsyndromeinachild?Babieswithhypoplasticleftheartsyndromeusuallyge

Hypoplastic left heart syndrome (HLHS) is one of the most complex cardiac defects seen in the newborn and remains probably the most challenging to manage ...HLHSHomeHealthTopicsHHLHSPrintNavigateThisAreaSingleVentricleInterstageClinicNPCQICollaborativeMeettheTeamHypoplasticLeftHeartSyndrome/NorwoodProcedureHypoplasticleftheartsyndrome(HLHS)isoneofthemostcomplexcardiacdefectsseeninthenewbornandremainsprobablythemostchallengingtomanageofallcongenitalheartdefects.Itisoneofagroupofcardiacanomaliesthatcanbegroupedtogetherunderthedescription singleventricle defects.InachildwithHLHS,allofthestructuresontheleftsideoftheheart(thesidewhichreceivesoxygen-richbloodfromthelungsandpumpsitouttothebody)areseverelyunderdeveloped. Themitralandaorticvalvesareeithercompletely"atretic"(closed),ortheyareverysmall.Theleftventricleitselfistiny,andthefirstpartoftheaortaisverysmall,oftenonlyafewmillimetersindiameter. Viewa3-DgraphicsummaryThisresultsinasituationwheretheleftsideoftheheartiscompletelyunabletosupportthecirculationneededbythebody'sorgans,thoughtherightsideoftheheart(thesidethatdeliversbloodtothelungs)istypicallynormallydeveloped.Bloodreturningfromthelungstotheleftatriummustpassthroughanatrialseptaldefect(ASD)totherightsideoftheheart.Therightventriclemustthendoa"doubleduty"ofpumpingbloodbothtothelungs(viathepulmonaryartery)andouttothebody(viaapatentductusarteriosus).Thepatentductusarteriosus,anormalstructureinthefetus,isoftentheonlypathwaythroughwhichbloodcanreachthebodyfromtheheart.Whentheductusarteriosusbeginstoclose,asittypicallydoesinthefirstdaysoflife,thebloodflowtothebodywillseverelydiminish,resultingindangerouslylowbloodflowtovitalorgansandleadingtoshock.Withouttreatment,hypoplasticleftheartsyndromeisuniformlyfatal,oftenwithinthefirsthoursordaysoflife.SignsandSymptomsInfantswithhypoplasticleftheartsyndromecandeveloplife-threateningshockwhentheductusarteriosusbeginstoclose.Inmostcases,however,theductusarteriosusiswidelyopenatthetimeofbirth,supplyingthebloodflowtothebody,and

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped. It may affect the left ...HypoplasticleftheartsyndromeFromWikipedia,thefreeencyclopediaJumptonavigationJumptosearchMedicalconditionHypoplasticleftheartsyndromeOthernamesCyanoticheartdisease-hypoplasticleftheart[1]IllustrationofheartsufferingfromhypoplasticleftheartsyndromeSpecialtyCardiology Hypoplasticleftheartsyndrome(HLHS)isararecongenitalheartdefectinwhichtheleftsideoftheheartisseverelyunderdeveloped.Itmayaffecttheleftventricle,aorta,aorticvalve,ormitralvalve.[2]Contents1Signsandsymptoms2Causes2.1Environmentalcauses2.2Genetics2.3Riskfactors3Pathophysiology4Diagnosis5Management5.1Medical5.2Surgical5.2.1Norwoodprocedure5.2.2Hybridprocedure5.2.3Glennprocedure5.2.4Fontanprocedure5.2.4.1Fetalsurgery6Prognosis7Epidemiology8References9ExternallinksSignsandsymptoms[edit]InfantwithcyanosisduetohypoplasticleftheartsyndromeClosingoftheductusarteriosusinaheartthatisseverelyunderdevelopedontheleftresultsincyanosisandrespiratorydistresswhichcanprogresstocardiogenicshockanddeath.Thefirstsymptomsarecyanosisthatdoesnotrespondtooxygenadministrationorpoorfeeding.Peripheralpulsesmaybeweakandextremitiescooltothetouch.[3]HLHSoftenco-occurswithlowbirthweightandprematurebirth.[3]Inneonateswithasmallatrialseptaldefect,termed"restrictive",thereisinadequatemixingofoxygenatedanddeoxygenatedblood.Theseneonatesquicklydecompensateanddevelopacidosisandcyanosis.[3]OnEKG,rightaxisdeviationandrightventricularhypertrophyarecommon,butnotindicativeofHLHS.Chestx-raymayshowalargeheart(cardiomegaly)orincreasedpulmonaryvasculature.NeonateswithHLHSdonottypicallyhaveaheartmurmur,butinsomecases,apulmonaryflowmurmurortricuspidregurgitationmurmurmaybeaudible.[3]Co-occurringtricuspidregurgitationorrightventriculardysfunctioncancausehepatomegalytodevelop.[3]Causes[edit]DiagramofahealthyheartandonewithhypoplasticleftheartsyndromeThereisnoknowncauseinthemajorityofHLHScases.[4]S

JumptonavigationTexasChildren'sBlogLatestPostsKaitlyn’sstory:ReachingbigdreamsafterovercomingaseverechildhoodleginjuryCelebratingourcentennialmilestone:DiabetestechnologyacrosstheyearsPlasticSurgery:WhatoursurgeonsdotoenhancethequalityoflifeforourpatientsMovingfromautismawarenesstoacceptance:TipstopromoteacceptanceandinclusionineverydaylifeTipsforparentsoninfantsleeptrainingandsafetyIt’s#ChildrensHospitalWeek,meetourchampionMarch21,2019PHOTO:PaulKuntzEveryyear,eachhospitalaffiliatedwiththeChildren’sMiracleNetworkHospitalsidentifiesa“champion”intheirlocalcommunitytoserveasthefaceforthechildrentheytreat.Theseambassadorsspendtheyearadvocatingforthecharitableneedofchildren’shospitalsacrossthecountry.Here’sthestoryofourchampion:MandiandKyle,expectingtheirsecondchildtogether,arrivedpromptlytotheirappointmentfora20-weekultrasound.Theyhadjustlearnedoftheirbaby’stwo-vesselumbilicalcord–asuspiciousredflagthatcalledfortheadditionalimaging.Thespecialistseemedtofocusintentlyonthebaby’sheartduringtheultrasound.“Ithinksomethingiswrong,”hefinallytoldthem,muchtotheirdismay.TheywerereferredtoTexasChildren’sPavilionforWomenforafetalechocardiogram,whichconfirmedtheirbabyindeedpossessedarare,complexcongenitalheartdefect:hypoplasticleftheartsyndrome(HLHS).[read:]What’safetalechocardiogram?HLHSisamalformationoftheleftsideoftheheart.Theorgan’smitralvalve,leftventricleandaorticvalvearetoosmall,compromisingtheleftside’sabilitytoeffectivelypumpbloodtotherestofthebody.Thebaby’sheartdefectwasn’tsevereenoughtowarrantin-uterotreatment;however,thepregnancywasmonitoredcloselyuntilMandidelivereddaughterLeighAnnatthePavilionforWomenonMarch9,2017.Withindays,LeighAnnquicklyunderwentherfirstopen-heartsurgery–theNorwoodprocedure,thefirstofthreestagedoperationsintendedtocreateanewfunctionalsystemcircuit.[watch:]HypoplasticLeftHeart:TheNorwoodOperationThissurgery,alongsideitssuccessors,isincrediblyrisky.Imagineoperatingonanewbornbaby’sheart–theheartitselfandtheleftventriclearecomparableinsizetoalargegrapea

PediatricHeartCenterHypoplasticLeftHeartSyndrome(HLHS)OverviewSigns&symptomsDiagnosisTreatmentWheretogetcareHelpfulresourcesOverviewInhypoplasticleftheartsyndrome,theleftsideoftheheart—thepartthatpumpsoxygenatedbloodtotherestofthebody—isunderdeveloped.Itstwochambers,calledtheleftatriumandtheleftventricle,andtheirvalvesmaybetiny,blockingtheflowofoxygenatedbloodfromthelungs.Theheartconsistsoffourchambers:thetwoupperchambers,calledatria,wherebloodenterstheheart,andthetwolowerchambers,calledventricles,wherebloodispumpedoutoftheheart.Theflowbetweenthechambersiscontrolledbyasetofvalvesthatactasone-waydoors.Normallybloodispumpedfromtherightsideoftheheartthroughthepulmonaryvalveandthepulmonaryarterytothelungs,wherethebloodisfilledwithoxygen.Fromthelungs,thebloodtravelsbackdowntotheleftatriumandleftventricle.Thenewlyoxygenatedbloodthenispumpedthroughanotherbigbloodvesselcalledtheaortatotherestofthebody.Babieswithhypoplasticleftheartsyndromemayseemhealthyatbirthbecausethepatentductusarteriosus(PDA)isstillopen.ThePDAisabloodvesselthatconnectsthepulmonaryarterytotheaorta,allowingbloodtocontinuecirculatingdirectlyintotheaortaandouttotherestofthebody,bypassingthelungsandthedefectiveleftsideoftheheart.OncethePDAclosesafewdaysafterbirth,bloodflowstothelungsandthentotheleftsideoftheheart,whereitisblockedandcan'tcirculatethroughtherestofthebody.Withouttreatment,babiesafflictedwithhypoplasticleftheartsyndromecandiewithinthefirstdaysorweeksoflife.Treatmentconsistsofahearttransplantoraseriesofoperationstorestorethefunctionoftheleftsideoftheheart.IntravenousmedicationcankeepthePDAopenuntilsurgerycanbeperformed,butisnotapermanenttreatment.Signs&symptomsBabiesbornwithhypoplasticleftheartsyndromemayseemnormalatbirthbutbecomeseverelyillsoonafterbirth.Babiesmayappearashenorgray,haverapidanddifficultbreathingandhavedifficultyfeeding.Thisheartdefectisusuallyfatalwithinthefirstdaysormonthoflifeunlesstreated.Symptomsinclude:Grayish-blueskincolorRapid,difficultbreathingPoorfeedingColdhandsandfeetL

Hypoplastic left heart syndrome (HLHS) is a birth defect that affects normal blood flow through the heart. As the baby develops during pregnancy, the left side of the heart does not form correctly. Hypoplastic left heart syndrome is one type of congenital heart defect. Congenital means present at birth.SkipdirectlytositecontentSkipdirectlytopageoptionsSkipdirectlytoA-ZlinkCongenitalHeartDefects(CHDs)SectionNavigationCDCHomeFacebookTwitterLinkedInSyndicateFactsaboutHypoplasticLeftHeartSyndromeEspañol(Spanish)MinusRelatedPagesClickheretoviewalargerimageClickheretoviewalargerimageHypoplastic(pronouncedhi-puh-PLAS-tik)leftheartsyndromeorHLHSisabirthdefectthataffectsnormalbloodflowthroughtheheart.WhatisHypoplasticLeftHeartSyndrome?Hypoplasticleftheartsyndrome(HLHS)isabirthdefectthataffectsnormalbloodflowthroughtheheart.Asthebabydevelopsduringpregnancy,theleftsideoftheheartdoesnotformcorrectly.Hypoplasticleftheartsyndromeisonetypeofcongenitalheartdefect.Congenitalmeanspresentatbirth.Becauseababywiththisdefectneedssurgeryorotherproceduressoonafterbirth,HLHSisconsideredacriticalcongenitalheartdefect(CCHD).Hypoplasticleftheartsyndromeaffectsanumberofstructuresontheleftsideoftheheartthatdonotfullydevelop,forexample:Theleftventricleisunderdevelopedandtoosmall.Themitralvalvesisnotformedorisverysmall.Theaorticvalveisnotformedorisverysmall.Theascendingportionoftheaortaisunderdevelopedoristoosmall.Often,babieswithhypoplasticleftheartsyndromealsohaveanatrialseptaldefect,whichisaholebetweentheleftandrightupperchambers(atria)oftheheart.Inababywithoutacongenitalheartdefect,therightsideoftheheartpumpsoxygen-poorbloodfromthehearttothelungs.Theleftsideoftheheartpumpsoxygen-richbloodtotherestofthebody.Whenababyisgrowinginamother’swombduringpregnancy,therearetwosmallopeningsbetweentheleftandrightsidesoftheheart:thepatentductusarteriosusandthepatentforamenovale.Normally,theseopeningswillcloseafewdaysafterbirth.Inbabieswithhypoplasticleftheartsyndrome,theleftsideoftheheartcannotpumpoxygen-ri

SkiptomaincontentArticleSurvivalofChildrenWithHypoplasticLeftHeartSyndromeCsabaSiffel,TiffanyRiehle-Colarusso,MatthewE.OsterandAdolfoCorreaPediatricsOctober2015,136(4)e864-e870;DOI:https://doi.org/10.1542/peds.2014-1427CsabaSiffelaNationalCenteronBirthDefectsandDevelopmentalDisabilities,CentersforDiseaseControlandPrevention,Atlanta,Georgia;bCollegeofAlliedHealthSciences,GeorgiaRegentsUniversity,Augusta,Georgia;FindthisauthoronGoogleScholarFindthisauthoronPubMedSearchforthisauthoronthissiteTiffanyRiehle-ColarussoaNationalCenteronBirthDefectsandDevelopmentalDisabilities,CentersforDiseaseControlandPrevention,Atlanta,Georgia;FindthisauthoronGoogleScholarFindthisauthoronPubMedSearchforthisauthoronthissiteMatthewE.OsteraNationalCenteronBirthDefectsandDevelopmentalDisabilities,CentersforDiseaseControlandPrevention,Atlanta,Georgia;cChildren’sHealthcareofAtlanta,EmoryUniversity,Atlanta,Georgia;andFindthisauthoronGoogleScholarFindthisauthoronPubMedSearchforthisauthoronthissiteAdolfoCorreaaNationalCenteronBirthDefectsandDevelopmentalDisabilities,CentersforDiseaseControlandPrevention,Atlanta,Georgia;dDepartmentofMedicine,UniversityofMississippiMedicalCenter,Jackson,MississippiFindthisauthoronGoogleScholarFindthisauthoronPubMedSearchforthisauthoronthissiteArticleFigures&DataInfo&MetricsCommentsThisarticlerequiresasubscriptiontoviewthefulltext.Ifyouhaveasubscriptionyoumayusetheloginformbelowtoviewthearticle.Accesstothisarticlecanalsobepurchased.DownloadPDFAbstractOBJECTIVE:Toexaminethesurvivalofinfantswithhypoplasticleftheartsyndrome(HLHS)andpotentialinfluenceofdemographicandclinicalcharacteristicsonsurvivalusingpopulation-baseddata.METHODS:InfantswithnonsyndromicHLHS(n=212)bornbetween1979and2005wereidentifiedthroughtheMetropolitanAtlantaCongenitalDefectsProgram.Vitalstatuswasascertainedthrough2009basedonlinkagewithvitalrecords.WeestimatedKaplan-Meiersurvivalprobabilitiesstratifiedbyselectdemographicandclinicalcharacteristics.RESULTS:Theoverallsurvivalprobabilityto2009was24%andsi

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

Hypoplastic Left Heart Syndrome HD ... The left side of the heart is very poorly formed and cannot support the main circulation (round the body). The left ventricle ...Stayinformedwiththelatestupdatesoncoronavirus(COVID-19).Findoutmore>>TheRoyalChildren'sHospitalMelbourneHomeAboutNewsCareersShopContactMyRCHPortalAgreatchildren'shospital,leadingthewayHealthProfessionalsPatientsandFamiliesDepartmentsandServicesResearch HealthProfessionals DepartmentsandServices PatientsandFamilies ResearchHomeAboutNewsCareersSupportusContactSearchCardiologyTogglesectionnavigationInthissectionCardiologyProtocols(intranetonly)ParentinformationForhealthprofessionalsHeartdefectsContactusRCH  >  DivisionofSurgery  >  Cardiology  >  HypoplasticLeftHeartSyndromeHDInthissectionCardiologyProtocols(intranetonly)ParentinformationForhealthprofessionalsHeartdefectsContactusHypoplasticLeftHeartSyndromeHDHypoplasticLeftHeartSyndromeHDFactsheet onHypoplasticLeftHeartSyndromeBookletforparentsofRCHpatientsTheleftsideoftheheartisverypoorlyformedandcannotsupportthemaincirculation(roundthebody).Theleftventricleandaortaareabnormallysmall(hypoplastic).Thisisamongstthemostsevereformsofheartdefect.Mostbabiesareveryillintheearlydaysoflifeandneedurgentsurgerytosurvive.Thefirststageofsurgeryiscalleda 'Norwood'operation.GotoNormalHeartNorwoodOperationTheNorwoodoperationinvolvesconnectingtheoriginofthepulmonaryarterytotheaorta,toallowtherightventricletopumpbloodtothemaincirculationanda'Shunt'operation,involvinginsertionofatinypieceofartificialtube(madefromGoretex)betweentherightarmarteryandtherightpulmonaryartery,tomaintainbloodflowtothelungs.LaterinchildhooditmaybepossibletocarryoutamodifiedFontanoperation.Anotheroptionthatissometimesused,involvesaGoretextube(calleda"Conduit")fromtheRVtothepulmonaryarteryinsteadofthe"Shunt".                    GotoNormalHeartAsecondoperationfollowsafteraboutthreemonthsandiscalleda"Cavo-PulmonaryShunt".Atalaterstage(aroundthreetofouryearsold)aFontanOperationmaybeperformed.FontanOp

Overview. Hypoplastic left heart syndrome is a complex and rare heart defect present at birth (congenital). In this condition, the left side of the ...Scheduleyourappointmentnowforsafein-personcare.Learnmore:MayoClinicfactsaboutcoronavirusdisease2019(COVID-19)Our COVID-19patient andvisitorguidelines,plustrustedhealthinformationLateston COVID-19vaccination bysite:ArizonapatientvaccinationupdatesArizona,FloridapatientvaccinationupdatesFlorida,RochesterpatientvaccinationupdatesRochesterandMayoClinicHealthSystempatientvaccinationupdatesMCHSSkiptositenavigationSkiptoContentThiscontentdoesnothaveanEnglishversion.ThiscontentdoesnothaveanArabicversion.SectionsforHypoplasticleftheartsyndromeSymptoms&causesDiagnosis&treatmentDoctors&departmentsCareatMayoClinicPrintWe'rewelcomingpatientsatMayoClinicSeeoursafetyprecautionsinresponsetoCOVID-19.Requestanappointment.OverviewHypoplasticleftheartsyndromeisacomplexandrareheartdefectpresentatbirth(congenital).Inthiscondition,theleftsideoftheheartiscriticallyunderdeveloped.Ifyourbabyisbornwithhypoplasticleftheartsyndrome,theleftsideoftheheartcan'teffectivelypumpbloodtothebody.Instead,therightsideoftheheartmustpumpbloodtothelungsandtotherestofthebody.Medicationtopreventclosureoftheconnection(ductusarteriosus)betweentherightandleftsides,followedbyeithersurgeryorahearttransplant,isnecessarytotreathypoplasticleftheartsyndrome.Advancesincarehaveimprovedtheoutlookforbabiesbornwiththiscondition.Products&ServicesBook:MayoClinicFamilyHealthBook,5thEditionHLHSCausetoCureblogNewsletter:MayoClinicHealthLetter—DigitalEditionToddandKarenWanekFamilyProgramforHypoplasticLeftHeartSyndromeShowmoreproductsfromMayoClinicSymptomsBabiesbornwithhypoplasticleftheartsyndromeusuallyareseriouslyillsoonafterbirth.Signsandsymptomsinclude:Grayish-blueskincolor(cyanosis)Rapid,difficultbreathingPoorfeedingColdhandsandfeetWeakpulseBeingunusuallydrowsyorinactiveIfthenaturalconnectionsbetweentheheart'sleftandrightsides(foramenovaleandductusarteriosus)areallowedtoclosein

Skiptocontent HypoplasticLeftHeartSyndrome(HLHS)OutpatientAppointments215-590-4040SecondOpinions,ReferralsandInformationAboutOurServices267-426-9600ContactUsOnlineWhatishypoplasticleftheartsyndrome(HLHS)?Hypoplasticleftheartsyndrome(HLHS)isaseverecongenitalheartdefectinwhichtheleftsideoftheheartisunderdeveloped.Inanormalheart,theheart'sleftsidehasthejobofpumpingoxygenatedbloodintotheaorta,thelargearterythatcarriesbloodtothebody.InachildwithHLHS:Themitralvalve,whichseparatesthetwoleftchambersoftheheart,istoosmallorcompletelyclosed(atretic).Theleftventricle(thelower,pumpingchamber)isverysmall.Theaorticvalve,whichseparatestheleftventricleandtheaorta,istoosmallorcompletelyclosed(atretic).InadditiontothemostcommonformofHLHSshownintheanimationbelow,thereareanumberofcomplexcardiacconditionswithvariationsinthestructuresasdescribed.Inthesechildren,whereoneventricleisalsosmall(sometimescalled"HLHSvariants")thetreatmentstrategyissimilartothosewiththemoretypicalHLHS,whichisshownintheanimationsbelow.ViewthisvideowithatranscriptLearnmoreaboutHLHSfromtheexpertsatChildren'sHospitalofPhiladelphia'sCardiacCenterinthisvideoseries.SignsandsymptomsofHLHSThefollowingsymptomsofhypoplasticleftheartsyndromemaybepresentatbirthorseveraldayslater:Blueorpurpletinttolips,skinandnails(cyanosis)DifficultybreathingDifficultyfeedingLethargy(sleepyorunresponsive)TestinganddiagnosisofHLHSOften,hypoplasticleftheartsyndromeisdiagnosedbeforebirth,withfetalechocardiogram(ultrasound).OurFetalHeartProgramcanhelpyouprepareaplanfordeliveryandcareimmediatelyafterbirth.SometimesHLHSisdiagnosedhoursordaysafterbirthandthebabywillneedimmediatetherapy.DiagnosisofHLHSmayrequiresomeorallofthesetests:Echocardiogram(alsocalled"echo"orultrasound):soundwavescreateanimageoftheheartElectrocardiogram(ECG):arecordoftheelectricalactivityoftheheartChestX-rayPulseoximetry:anoninvasivewaytomonitortheoxygencontentofthebloodCardiaccatheterization:athintubeisinsertedintotheheartthroughaveinand/orarteryineitherthelegorthroughtheumbi