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Introduc)on to Adult Congenital Heart Disease DavidLaughrun,M.D.,F.A.C.C. Disclosure of Relevant Financial Rela)onships Employee—MercyDesMoines Adult Congenital Heart Disease Defini&on Examples Demographics Evolu>onofSpecialtyCare UniqueMedicalandNon-medicalConcerns Adult Congenital Heart Disease Structuralheartabnormalitypresentatbirth. RangefromSimpletoModeratelyComplextoHighly Complex. RepairedorUnrepaired. Cyano>corNon-cyano>c. Down Syndrome Trisomy21. AVCanalDefect,VSD,ASD,TOF,ormul>pledefects. Turner Syndrome AbsentorabnormalXchromosome(~50%are45XO). Coarcta>on,PAPVR. Noonan Syndrome SimilartoTurnerSyndromebutwithnormalcomplementof chromosomes.MutatedPTPN11orKRASgene.Autosomal dominant. PS,PAS,ASD. Holt-Oram (“Heart-Hand”) Syndrome Muta>onofTBX5gene;autosomaldominant. Abnormalradial,wrist,thenarandthumbbones. ASD,VSD(s),AVBlock,atrialfibrilla>on. Adult Congenital Heart Disease: E)ology Gene>c(complexpa^erns;approximately25%). Environmentaltoxins(ETOH,maternalRubella,drugs). Mul>factorial(egDM). MajorityofisolatedcasesofCHDhaveno apparentcause. Adult Congenital Heart Disease Defini>on Examples Demographics Evolu>onofSpecialtyCare UniqueMedicalandNon-medicalConcerns Normal Heart ACHD-Simple: Secundum Atrial Septal Defect Secundum ASD: Percutaneous or Surgical Closure if RA and RV Enlargement Secundum ASD: Percutaneous Closure Repaired Secundum ASD Followup PercutaneousClosure:3monthsto1yearthen“periodically” thereacer.Watchfor:migra>on,erosion,thrombosis. SurgicalClosure:Indefinitefollowupifadultat>meofsurgery, pulmonaryHTN,arrhythmias,RVdysfunc>onorassociatedlesions. ACHD-Moderately Complex: Repaired Tetralogy of Fallot TOF Repair of RVOT and PA Obstruc)on Pulmonary Regurgita)on s/p TOF Repair Repaired TOF Pa)ent: Long Term Followup Re-opera>onforpulmonicregurgita>on,residualRVOTobstruc>on oraor>cregurgita>on? Heritablecause(eg:22q11dele>on)? Arrhythmias? IncreasedriskofSCD. Favorablelongtermprognosis(86%30yearspostop)but: RepairedTOFisnotcuredTOF. ACHD-Highly Complex: VSD with Eisenmenger Physiology VSDcorrectedbeforeEisenmengerphysiologydevelopshasan excellentlongtermoutlook. VSD with Eisenmenger Physiology VSD with Eisenmenger Physiology Ini>alLtoRshuntleadstomedialthickeningofpulmonary vasculature. Resultantincreaseinpulmonaryvascularresistanceul>matelyleads toRtoLshunt. O2-unresponsivehypoxemiaresults. VSD with Eisenmenger Physiology WhenPVRexceeds70%ofSVRduetoirreversiblechangesinthe pulmonaryvasculature,theriskofsurgicalrepairoftheVSDbecomes prohibi>veduetothelikelihoodofpostopera>vedeathfromRV failure. Eisenmenger Syndrome VSD with Eisenmenger Physiology Progressivedyspneaonexer>on. Secondaryerythrocytosisandirondeficiencycanleadto hyperviscosityproblems(cerebrovascular,renal). Rightheartfailure. Paradoxicalembolism. Angina(RVischemiaorcoronaryarterycompressionbydilatedPA). Deathfrom:SCD,hemoptysis,HF,pregnancy,non-cardiacsurgery, brainabscess,infec>ousendocardi>s,stroke. VSD with Eisenmenger Syndrome: Mangement Absoluteavoidanceofpregnancy. Avoid:airbubblesinIV,dehydra>on,moderateorgreaterexercise (especiallyisometric),excessiveheat,highal>tude. Maintainadequateironstores. Uncommonlyusetherapeu>cphlebotomy(Hb>20withsymptoms). MedicaltreatmentofPAH. Considerheart-lungtransplantorVSDrepair-lungtransplant. VSD with Eisenmenger: Transplant Considera)ons 10yearsurvivals/pHLTapproximately20% WithoutHLT: ACHD-Highly Complex: Dextrocardia, DORV, VSD, L-TGA, Pulmonary Atresia Modified Blalock-Taussig Shunt: Subclavian Artery to Pulmonary Artery Glenn Shunt: Superior Vena Cava to Pulmonary Artery ACHD-Highly Complex: Dextrocardia, DORV, VSD, L-TGA, Pulmonary Atresia Infant:LGlennShunt Infant:RBlalock-TaussigShunt 11y.o.:Rsidedunifocaliza>onsurgerywithbovinepericardialgrac. 12y.o.:PatchclosureofmorphologicRAVvalve+excisionof interatrialseptum. 23y.o.:Successfulpregnancy. 27y.o.:Pulmonaryarterystent+coilingofGlenn“pop-off” collaterals. 30y.o.:CoilingofnewGlenncollaterals. AlthoughVivienThomas(MosDef),ablackmaninthe1930s,isoriginallyhiredasajanitor,heproveshimselfadeptatassis>ng the''BlueBabydoctor,''AlfredBlalock(AlanRickman),withhismedicalresearch.WhenBlalockinsiststhatThomasfollowhimto JohnsHopkinsUniversity,theymustfindawaytoskirtaracistsystemtocon>nuetheirstudyofinfantheartdisease.Thomasis indispensabletoBlalock'sprogress,butBlalockistheonlyonewhoisallowedtoreceivetheacclaim. Vivien Thomas InstructorofSurgeryandHonoraryDoctorateJohnsHopkins University1976. Adult Congenital Heart Disease Defini>on Examples Demographics Evolu>onofSpecialtyCare UniqueMedicalandNon-medicalConcerns ACHD-Demographics Approximately1in100birthshavesomeformofheartdefect. In1960,<40%survivedtoadulthood. Today,>90%survivetoadulthood. >1millionadultsinU.S.livingwithCHD. ACHDpopula>ongrowingatanes>mated5%peryear. Improved CHD Survival Improvedimagingandearlydiagnosis. Improvedsurgicalandinterven>onaltechniques. Advancesincri>calcareandEP. ACHD-Demographics MoreadultsthanchildrenarenowlivingwithCHD. Adult Congenital Heart Disease Defini>on Examples Demographics Evolu&onofSpecialtyCare UniqueMedicalandNon-medicalConcerns ACHD-Evolu)on of Specialty Care Es>mated>50%ofCHDpa>entsarelosttofollow-upacer adolescence.Only10%receivesubspecialtycare. AdultCardiologyFellowshipsrequireonly6hoursoflecturetraining inCHD. In2012,76%ofPediatricCardiologistssurveyedcitedalackof qualifiedACHDproviders. Currentlytherearemanypa?entswithtoo fewspecialistsandprogramstotakecareof them. ACHD-Evolu)on of Specialty Care “Bethesda32”2000:ACCconcludestheU.S.isnotmee>ngthe needsofadultswithCHD.RecommendsACHDCenters. ACCGuidelines2008:Specificpersonnelandservicesrecommended forACHDCenters.Diseasespecificguidelinesforthecareofadults withCHD. ABIMOctober2015:FirstofferingofBoardExaminACHD. ABIMhasappliedtoACGMEforaccredita&onofpostgraduate trainingprogramsintheU.S. ACHA2015:beginprocessofaccredi&ngACHDCenters. Adult Congenital Heart Disease Defini>on Examples Demographics Evolu>onofSpecialtyCare UniqueMedicalandNon-medicalConcerns Unique Concerns for ACHD Congenitalsyndromes. Endocardi>s,brainabscess. Endocardi>sprophylaxis Secondaryerythropoiesiswithirondeficiency. Noncardiacsurgeryrisk. Depressionandanxiety. Insurance. Medicalrecords. Careerchoice. Finances. Transi>onofCare Hemostasis. Renalfunc>on. Gallstones. Pulmonaryvasculardisease. Restric>velungdisease. Orthopedic/rheumatologicdisease. Varicoseveins. Hepa>cconges>on/cirrhosis. Thromboembolicdisease. Proteinlosingenteropathy. ?Opera>on,re-opera>on,interven>on,transplant. Mortality PregnancyandContracep>on ExerciseandSports ArrhythmiasandriskofSCD Exercise and Sports Symptomsaccountforonly30%ofallbarrierstoexercise. Otherbarriers:lackofexperiencewithexerciseinchildhood,fear, coexis>ngdisabili>es,culturalattudes. Providershouldemphasizewhattodoforexerciseandde-emphasize restric>ons. “Bethesda36”Guidelinesavailableforcompe>>veathle>cs. Compe>>onmayhinderprudentrecogni>onofsymptoms. Nosuchguidelinesfornoncompe>>veexercise. Regular, Moderate, Symptom-Limited Exercise (Braunwald) ReducescardiovascularmorbidityandmortalityinCADpa>ents. Improvesfunc>onalcapacity,qualityoflifeandriskfactorsin pa>entswithHTN,valvularheartdiseaseandchronicheartfailure. Mostindividualswithstructuralheartdiseasecansafelypar>cipatein prescribedphysicalac>vity. Exercise “Progressgraduallyandpaya^en>ontoyoursymptoms.” Stopifchestdiscomfort,lightheaded,heartracing,orshort ofbreathtopointyoucan’ttalk. Goal30+minuteseverydayofmoderatesymptomlimited exercise. Caveatsfor:Marfan’s,cyano>cCHD,aor>cstenosis, coarcta>on,devices.(Avoidanceofisometricexercise,high intensitysportsandcontactsports). Arrhythmias Symptoma>carrhythmiasarethemostfrequentreasonfor hospitaladmissioninadultswithCHD. Hemodynamicstress,structuralabnormali>es,scars, patches,andaccessorypathwaysallcontributetothehigh incidenceoftachyandbradyarrhythmias. Arrhythmias: IART Intra-AtrialRe-entrantTachycardia(IART)isseeninupto50%of pa>entsinlongtermfollowupacersurgeryinvolvingtheRA and/orLAduetomacroreentrantcircuits. 170-250bpm(vs300bpmfortypicalatrialflu^er).Canconduct 1:1toventriclesandcausesyncopeorevenSCD. PharmacologicRxdisappoin>ng.ConsiderATP,atrialICD, abla>on. Typical Atrial FluXer with 2:1 AV Conduc)on A rate = 300, V rate = 150 IART (s/p atrial switch) with 1:1 AV Conduc)on A rate = 190, V rate = 190 Bradyarrhythmias in ACHD Sinusnodedamageacersurgeriesinvolvingtheatria. AVBlockcomplica>ngsurgery(VSDrepair,LVOTrepair,AVR). CongenitalAVBlock(CCTGA,AVSD). Pacemakerindica>onsgenerallyfollowconven>onalguidelines. Transient Complete Heart Block (Septum Primum Atrial Septal Defect) Arrhythmias: VT 35%ofrepairedTOFpa>entshavePVC’sorNSVT.Approximate6% riskofsustainedVTorlateSCDduringlongtermfollowup. Clinicalpredictors(imperfect)acerrepairedTOF:RVdilata>on,QRS 180msorgreater,ventricularectopyonHoltermonitor,PES. Nogenerallyacceptedschemeforrhythmsurveillancein asymptoma>cpa>ents. Symptomsshouldpromptathoroughinves>ga>on. Arrhythmias: Sustained VT, SCD Echo,cath,EPS.Ifsurgeryindicatedforstructuralheart indica>onthenconsiderintra-opera>veVTmappingand abla>on. Ifnosurgeryindicatedthenconsidercatheterabla>onofVT (recurrencemaybe20%orgreaterinlongtermfollowup). Cardiacarrest,hemodynamicallysignificantVTandsustained VTareClassIIaindica>onsforICDplacement. Op>mal>mingofICDplacementrepresentsacrucial researchgap. Arrhythmias: Device Concerns Venousreturntoheartocenabnormal. Incyano>cpa>entswithRtoLshuntthereisariskofleadrelatedsystemicembolism. Considerepicardialleadsorsubcutaneousdefibrillator. Abdominalorsubmusculargeneratorplacementanop>on forcosme>cpurposes. Subcutaneous ICD Leadless Pacemaker Adult Congenital Heart Disease • Growingpopula>on. • EvolvingNewSpecialty. • Uniqueconcerns.