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Adult Congenital Heart Disease
� Defini&on� Examples� Demographics� Evolu>onofSpecialtyCare� UniqueMedicalandNon-medicalConcerns
Adult Congenital Heart Disease
� Structuralheartabnormalitypresentatbirth.� RangefromSimpletoModeratelyComplextoHighlyComplex.
� RepairedorUnrepaired.� Cyano>corNon-cyano>c.
Noonan Syndrome
� SimilartoTurnerSyndromebutwithnormalcomplementofchromosomes.MutatedPTPN11orKRASgene.Autosomaldominant.
� 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).
� MajorityofisolatedcasesofCHDhavenoapparentcause.
Adult Congenital Heart Disease
� Defini>on
� Examples� Demographics� Evolu>onofSpecialtyCare� UniqueMedicalandNon-medicalConcerns
Repaired Secundum ASD Followup
� PercutaneousClosure:3monthsto1yearthen“periodically”thereacer.Watchfor:migra>on,erosion,thrombosis.
� SurgicalClosure:Indefinitefollowupifadultat>meofsurgery,pulmonaryHTN,arrhythmias,RVdysfunc>onorassociatedlesions.
Repaired TOF Pa)ent: Long Term Followup
� Re-opera>onforpulmonicregurgita>on,residualRVOTobstruc>onoraor>cregurgita>on?
� Heritablecause(eg:22q11dele>on)?� Arrhythmias?� IncreasedriskofSCD.� Favorablelongtermprognosis(86%30yearspostop)but:
� RepairedTOFisnotcuredTOF.
ACHD-Highly Complex: VSD with Eisenmenger Physiology
� VSDcorrectedbeforeEisenmengerphysiologydevelopshasanexcellentlongtermoutlook.
VSD with Eisenmenger Physiology
� Ini>alLtoRshuntleadstomedialthickeningofpulmonaryvasculature.
� Resultantincreaseinpulmonaryvascularresistanceul>matelyleadstoRtoLshunt.
� O2-unresponsivehypoxemiaresults.
VSD with Eisenmenger Physiology
� WhenPVRexceeds70%ofSVRduetoirreversiblechangesinthepulmonaryvasculature,theriskofsurgicalrepairoftheVSDbecomesprohibi>veduetothelikelihoodofpostopera>vedeathfromRVfailure.
VSD with Eisenmenger Physiology
� Progressivedyspneaonexer>on.� Secondaryerythrocytosisandirondeficiencycanleadtohyperviscosityproblems(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.
ACHD-Highly Complex: Dextrocardia, DORV, VSD, L-TGA, Pulmonary Atresia
� Infant:LGlennShunt� Infant:RBlalock-TaussigShunt� 11y.o.:Rsidedunifocaliza>onsurgerywithbovinepericardialgrac.� 12y.o.:PatchclosureofmorphologicRAVvalve+excisionofinteratrialseptum.
� 23y.o.:Successfulpregnancy.� 27y.o.:Pulmonaryarterystent+coilingofGlenn“pop-off”collaterals.
� 30y.o.:CoilingofnewGlenncollaterals.
AlthoughVivienThomas(MosDef),ablackmaninthe1930s,isoriginallyhiredasajanitor,heproveshimselfadeptatassis>ngthe''BlueBabydoctor,''AlfredBlalock(AlanRickman),withhismedicalresearch.WhenBlalockinsiststhatThomasfollowhimtoJohnsHopkinsUniversity,theymustfindawaytoskirtaracistsystemtocon>nuetheirstudyofinfantheartdisease.ThomasisindispensabletoBlalock'sprogress,butBlalockistheonlyonewhoisallowedtoreceivetheacclaim.
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.
Adult Congenital Heart Disease � Defini>on� Examples� Demographics
� Evolu&onofSpecialtyCare� UniqueMedicalandNon-medicalConcerns
ACHD-Evolu)on of Specialty Care
� Es>mated>50%ofCHDpa>entsarelosttofollow-upaceradolescence.Only10%receivesubspecialtycare.
� AdultCardiologyFellowshipsrequireonly6hoursoflecturetraininginCHD.
� In2012,76%ofPediatricCardiologistssurveyedcitedalackofqualifiedACHDproviders.
� Currentlytherearemanypa?entswithtoofewspecialistsandprogramstotakecareofthem.
ACHD-Evolu)on of Specialty Care
� “Bethesda32”2000:ACCconcludestheU.S.isnotmee>ngtheneedsofadultswithCHD.RecommendsACHDCenters.
� ACCGuidelines2008:SpecificpersonnelandservicesrecommendedforACHDCenters.DiseasespecificguidelinesforthecareofadultswithCHD.
� ABIMOctober2015:FirstofferingofBoardExaminACHD.� ABIMhasappliedtoACGMEforaccredita&onofpostgraduatetrainingprogramsintheU.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.
� Hemostasis.
� Renalfunc>on.
� Gallstones.
� Pulmonaryvasculardisease.
� Restric>velungdisease.
� Orthopedic/rheumatologicdisease.
� Varicoseveins.� Hepa>cconges>on/cirrhosis.
� Thromboembolicdisease.
� Proteinlosingenteropathy.
� ?Opera>on,re-opera>on,interven>on,transplant.
� Insurance.� Medicalrecords.� Careerchoice.� Finances.� Transi>onofCare
� Mortality� PregnancyandContracep>on� ExerciseandSports� ArrhythmiasandriskofSCD
Exercise and Sports
� Symptomsaccountforonly30%ofallbarrierstoexercise.� Otherbarriers:lackofexperiencewithexerciseinchildhood,fear,coexis>ngdisabili>es,culturalattudes.
� Providershouldemphasizewhattodoforexerciseandde-emphasizerestric>ons.
� “Bethesda36”Guidelinesavailableforcompe>>veathle>cs.Compe>>onmayhinderprudentrecogni>onofsymptoms.
� Nosuchguidelinesfornoncompe>>veexercise.
Regular, Moderate, Symptom-Limited Exercise (Braunwald)
� ReducescardiovascularmorbidityandmortalityinCADpa>ents.� Improvesfunc>onalcapacity,qualityoflifeandriskfactorsinpa>entswithHTN,valvularheartdiseaseandchronicheartfailure.
� Mostindividualswithstructuralheartdiseasecansafelypar>cipateinprescribedphysicalac>vity.
Exercise
� “Progressgraduallyandpaya^en>ontoyoursymptoms.”� Stopifchestdiscomfort,lightheaded,heartracing,orshortofbreathtopointyoucan’ttalk.
� Goal30+minuteseverydayofmoderatesymptomlimitedexercise.
� Caveatsfor:Marfan’s,cyano>cCHD,aor>cstenosis,coarcta>on,devices.(Avoidanceofisometricexercise,highintensitysportsandcontactsports).
Arrhythmias
� Symptoma>carrhythmiasarethemostfrequentreasonforhospitaladmissioninadultswithCHD.
� Hemodynamicstress,structuralabnormali>es,scars,patches,andaccessorypathwaysallcontributetothehighincidenceoftachyandbradyarrhythmias.
Arrhythmias: IART � Intra-AtrialRe-entrantTachycardia(IART)isseeninupto50%ofpa>entsinlongtermfollowupacersurgeryinvolvingtheRAand/orLAduetomacroreentrantcircuits.
� 170-250bpm(vs300bpmfortypicalatrialflu^er).Canconduct1:1toventriclesandcausesyncopeorevenSCD.
� PharmacologicRxdisappoin>ng.ConsiderATP,atrialICD,abla>on.
Bradyarrhythmias in ACHD
� Sinusnodedamageacersurgeriesinvolvingtheatria.
� AVBlockcomplica>ngsurgery(VSDrepair,LVOTrepair,AVR).
� CongenitalAVBlock(CCTGA,AVSD).
� Pacemakerindica>onsgenerallyfollowconven>onalguidelines.
Arrhythmias: VT
� 35%ofrepairedTOFpa>entshavePVC’sorNSVT.Approximate6%riskofsustainedVTorlateSCDduringlongtermfollowup.
� Clinicalpredictors(imperfect)acerrepairedTOF:RVdilata>on,QRS180msorgreater,ventricularectopyonHoltermonitor,PES.
� Nogenerallyacceptedschemeforrhythmsurveillanceinasymptoma>cpa>ents.
� Symptomsshouldpromptathoroughinves>ga>on.
Arrhythmias: Sustained VT, SCD
� Echo,cath,EPS.Ifsurgeryindicatedforstructuralheartindica>onthenconsiderintra-opera>veVTmappingandabla>on.
� Ifnosurgeryindicatedthenconsidercatheterabla>onofVT(recurrencemaybe20%orgreaterinlongtermfollowup).
� Cardiacarrest,hemodynamicallysignificantVTandsustainedVTareClassIIaindica>onsforICDplacement.
� Op>mal>mingofICDplacementrepresentsacrucialresearchgap.
Arrhythmias: Device Concerns
� Venousreturntoheartocenabnormal.� Incyano>cpa>entswithRtoLshuntthereisariskoflead-relatedsystemicembolism.
� Considerepicardialleadsorsubcutaneousdefibrillator.� Abdominalorsubmusculargeneratorplacementanop>onforcosme>cpurposes.