Hypoplastic Left Heart Syndrome (HLHS) | Hypoplastic left heart syndrome
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
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hypoplastic left heart syndrome中文Hypoplastic left heart syndrome pathophysiologyHypoplastic right heart syndromeHypoplastic left heart syndrome surgeryTruncus arteriosusTetralogy of FallotCongenital heart disease