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1、CongenitalHeartDiseases IISamerAbbas,MDCardiologyUIC/Christ02/27/2008ClassificationCyanosiswithlowpulmflow:-TOF-EbstienAnamoly-HypoplasticofRV-Tricuspidatresia-PulmatresiaCyanosiswithhighpulmflow:TGADoubleoutletV.DoubleinletVTAPVD.EisenmengerSyndromeIn1897VictorEisenme
2、ngerpublishedapaperentitled“CongenitalDefectsoftheVentricularSeptum.”In1958,PaulWoodsummarizedEisenmenger’saccounts:“Thepatientwasapowerfullybuiltmanof32whogaveahistoryofcyanosisandmoderatebreathlessnesssinceinfancy.HemanagedwelluntilJanuaryof1894whendyspneaincreasedan
3、dedemasetin.Sevenmonthslaterhewasadmittedtothehospitalinastateofheartfailure……Heimprovedwithrestanddigitalis,butcollapsedanddiedsuddenlyonNovember13followingalargehemoptysis”EisenmengerSyndromeLargelefttorightshunt:severepulmonaryvasculardiseaseleadingtoshuntreversalIn
4、itialreversiblechanges:MedialhypertrophyofthepulmonaryvasculatureIntimalproliferationEisenmengerSyndromeProgressiveirreversiblechanges:plexiformlesionsnecrotizingarteritisAstheincreasedPVRapproachesorexceedstheSVRtheshuntisreversed.AsRtoLshuntingdevelopscyanosisappears
5、.Mostpatientswilldevelopexertionaldyspneaandimpairedexercisetolerance.EisenmengerSyndromePalpitationsoccurin>50%ofpatientsA.fib/flutterin40%VTin10%)Hemoptysisin~20%PE,angina,syncope,endocarditis~10%SignsofPHTNRVheave,palpableP2,andrightsidedS4Pulmonaryejectionclickanda
6、softscratchySEMd/tdilatedpulmonarytrunkHighpitcheddecrescendodiastolicmurmur(Graham-Steele)audibleinmostpatientsUsuallynoperipheraledemauntilRHFensuesEisenmengerSyndromeECGshowsRVH,RAEandRADAtrialarrhythmiasmaybepresentEisenmengerSyndromeCXRrevealsprominentcentralpulmo
7、naryarteriesanddecreasedvascularmarkings(pruning)oftheperipheralvesselsEisenmengerSyndromeLargevariationinlifeexpectancyinadultswithEisenmengersyndromeRateofsurvivalamongpatientswithEisenmengersyndromeis:80%at10years77%at15years42%at25yearsRecentstudyof109adultsreveale
8、dfollowingasindependentpredictorsofmortality:AgeatpresentationSupraventriculararrhythmiasPoorNYHAfunctionalclass(IIIo
