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


常見健康問答


延伸文章資訊