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