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Adult Congenital HeartDisease for the InternistSaurabh Rajpal, MBBS, MDAssistant ProfessorDepartment of Internal MedicineDivision of Cardiovascular MedicineThe Ohio State University Wexner Medical CenterWhy Should the Internists Care?Objectives To discuss the increasing prevalence ofadult congenital heart disease To discuss the common congenital heartdisease diagnoses encountered in primarycare clinics To discuss strategies for lifelong care ofadult patients with congenital heartdiseaseEXPLOSION of a “Life-Long”Disease BurdenCHD prevalence 12/ 1000 childrenCHD prevalence 6.07 / 1000 adults55-140,000 new pts/yr9% increase/yrHoffman J et al. Am J Cardiol. 1978, 641-647. Fyler D. 1980. 375-461. Ferencz C et al. Am J Epidemiol. 1985:31-6. Congenital heart disease after childhood: an expanding patientpopulation. 22nd Bethesda Conference, Maryland, October 18-19, 1990. J Am Coll Cardiol 1991;18(2):311-342. Warnes CA et al. J Am Coll Cardiol 2001;1170-1175. Warnes CA et al. JAm Coll Cardiol 2008;52(23):1890-1947; Marelli A et al. Am Heart J. 2009, 1-8. Karouache M et al. J Am Coll Cardiol 2013; 61; E505.1
Estimated Probability of SurvivalEstimated Survival- Era EffectACHD Patients- Common IssuesQuality of lifeTransitionBirth ControlPregnancy andCHD Dental Issues Exercise Hep 26Patient ons et al. Circulation 2010 Trends in ACHDRichard A. Krasuski, and Thomas M. Bashore Circulation. 2016;134:110-113Atrial Septal Defect PulmonaryHypertension Heart Failure Arrhythmias Neurocognitiveissues Advance careplanning andadvanced AtrialSeptalDefect-graphic2.html2
Physical Exam – AtrialSeptal Defect EKGHyperdynamic precordiumLoud P2- Pulm HTNSigns of RHF rareWidely split and fixed S2Murmurs in ASD Soft SEM- LUSB Diastolic rumble over LLSB- increased flowTV HSM at LLSB- TRSecundum ASDIncomplete RBBBRight Axis DeviationPrimum ASDIncomplete RBBBLeft Axis trialSeptalDefect-graphic2.htmlASD – Paradoxical EmbolismASD and PulmonaryHypertensionMyocardial Infarction and PE after IVDU3
Associated AnomaliesRV Dilation Anomalous pulmonary veins VSD Mitral regurgitation with primum ASDs dueto cleft valveRVEDVi: 145 ml/m2RVEF: 61%Etiologies of RV DilationLVEDVi: 102 ml/m2LVEF: 56%Qp/Qs : 1.1TR fraction: 49%Inferior Sinus Venosus – ASDTricuspid regurgitationPulmonary regurgitationPulmonary artery hypertensionShunt LesionsMyocardial abnormalities- Uhl’s anomaly- ARVC- Ventricular dysfunction4
ASD- Indications for ClosureASD Closure Significant left-to-right shunt‒ right ventricular volume overload‒ with or without symptoms‒ without pulmonary hypertension* Surgical Transcatheter Orthodeoxia-platypnea Paradoxical embolism At the time of another cardiac surgeryOther Issues with ASDs Patent Foramen OvalePeriodic follow upArrhythmias (also with repaired)Pulmonary hypertensionScuba divingHigh altitude exposureDrawn by: Kjetil Lenes5
PFO - To close or not toclose.Ventricular Septal Defect Patient Factors‒ Hypercoagulable state‒ Atrial Fibrillation‒ ASCVD Risk Factors‒ Presence of devices in the RV PFO factors‒ Shunt size‒ Atrial Septal Aneurysm1. Conoventricular2. Membranous3. Inlet4. MuscularSource: Centers for Disease Control and PreventionPhysical Exam and EKGVSD Smaller the VSD, louder the murmur Holosystolic plateau-shaped murmur atLLSB Majority of patients with isolatedventricular septal defect have normal EKG EKG signs of LAE and LVH – maybe6
Indications for Closure of VSD Symptoms of heart failure Large LV Normal PVRLifelong Follow Up -VSDVSD Closure in Adults Intervention is rarely required Small VSDs do not create a clinicallyimportant shunt Adult patients with large VSDs andirreversible pulmonary vascular disease– Need expert assessmentGerbode Defect More often repair of the VSD‒ Endocarditis‒ Aortic insufficiency‒ Pulmonary insufficiency Surgical repair remains the goldstandard for treatment of VSD7
Patent Ductus ArteriosusSignificant Left to RightShuntEndarteritis‒ Symptoms‒ LA and LVenlargementSource: Patent Ductus Arteriosus (PDA) - American Heart ects/patent-ductus-arteriosus-pdaTetralogy Of Fallot (TOF)UnrepairedCoarctation of Aorta Familial risk Turner syndrome Associated anomalies‒ ASD‒ VSD‒ Bicuspid aortic valve Hypertension Brachial-femoral delay Premature CAD, Stroke Intracranial aneurysms Surgery, Balloon Angioplasty, StentCourtesy: Ohio Fetal Medicine CollaborativeEKG in Repaired TOFRepaired8
Issues in Adults withRepaired TOF Tetralogy Of FallotPulmonary RegurgitationAtrial ArrhythmiasVentricular ArrhythmiasSudden Cardiac DeathResidual VSDLV Dysfunction and left heart failureRight Heart FailureAdults with Repaired TOFEbstein Anomaly EP Procedures Pulmonary Valve Replacement‒ Transcatheter‒ Surgical Lifelong follow up with ACHD9
Ebstein Anomaly Tricuspidregurgitation Right heart failure ASD - O2 desaturation Surgery‒ Tricuspid valvereplacementTransposition of Great Arteries Atrial arrhythmias Atrial fibrillation Atrial flutter WPW Sinus nodedysfunction Sudden cardiac death‒ Cone procedureCourtesy: Ohio Fetal Medicine CollaborativeTransposition of GreatArteries42 year old female with D-TGA s/p atrialswitch w/syncope10
Complex CongenitalHeart DiseaseD-TGA Atrial Switch Eisenmenger Syndrome Unrepaired Cyanotic Congenital HeartDisease Fontan and Single Ventricle Patients Palliated with Systemic toPulmonary Artery Shunts41 Single Ventricle AnatomyVSDLate EndStage20 U87%67%16 U74%HLHSTADORVSurgicalShunts DILV UnbalancedAVCPA97%PVR SVRQp:Qs 0.9:1EbsteinEisenmengerSyndrome BT Central Waterston PottsCourtesy: Ohio Fetal Medicine CollaborativeCourtesy : Curt Daniels, MD11
Complex CongenitalHeart Disease 6 monthly to yearly ACHD follow-upKnow and check O2 saturation regularlyCheck iron stores periodicallyMonitor renal and liver functionMost have restrictive or other lung diseaseDental follow upHigh risk of stroke and brain abscess incyanotic patientsQuality of life Quality of a person’s life is related to howsatisfied they are with their life overall Functional status has to do with a person’sability to do normal daily activities andperform their roles in life Disability paradox Response shift Sense of coherenceACHD PatientsCommon Issues Quality of lifeTransitionBirth ControlPregnancy andCHD Dental Issues Exercise Hep C PulmonaryHypertension Heart Failure Arrhythmias Neurocognitiveissues Advance careplanning andadvanced directivesPregnancy High Risk‒ Aortopathies including Marfansyndrome‒ Severe left sided obstructive lesions‒ Fontan‒ Eisenmenger‒ Pulmonary Hypertension‒ Severe LV Dysfunction12
Birth Control ExerciseHypercoagulable statesLow dose progestin pillsIUDsComplex congenital heart disease patientsshould be evaluated in tertiary centersBilly Rose Theatre Division, The New York Public Library. "Unidentified members exercising" The New York Public Library DigitalCollections. 1930 - 1940. 63aa-0132-0f5b-58d385a7bbd0SBE Prophylaxis Prosthetic heart valvesProsthetic material used for cardiac valve repairPrior history of IEUnrepaired cyanotic congenital heart diseaseRepaired congenital heart disease with residualshunts or valvular regurgitation at the site oradjacent to the site of the prosthetic patch orprosthetic device Repaired congenital heart defects with catheterbased intervention involving an occlusion deviceor stent during the first six months after theprocedure Valve regurgitation due to a structurallyabnormal valve in a transplanted heartAdvance Care Planning andAdvance DirectivesThe place for these difficult conversationsshould not be in the Intensive Care Unit(ICU)‒ 50% of ACHD patients die in thehospital‒ Of these, two-thirds die in theintensive care setting and almost ahalf were on life support‒ Only 10% of patients in ACHD carehad an end-of-life discussionTobler et al Tobler Am J Cardiol 2012Tobler et al Palliative Medicine 201213
Current ConcernsNeurodevelopmental OutcomesNew Guidelines 60 young adults with arterial switch operationre-evaluated at a mean age of 16.9 1.7years Neurologic impairment in 10% Periventricular leukomalacia was detected in 50%; its severity correlated with the grade ofneurologic impairment Magnetic resonance imaging demonstratedmoderate or severe structural brainabnormalities in 32% of the patientsJACC 2018Heinrichs et al. J Thorac Cardiovasc Surg 2014COACHCOACHColumbus Ohio Adult Congenital Heart ProgramColumbus Ohio Adult Congenital Heart Program14
Congenital heart disease after childhood: an expanding patient population. 22nd Bethesda Conference, Maryland, October 18-19, 1990. J Am Coll Cardiol 1991;18(2):311-342. Warnes CA et al. J Am Coll Cardiol 2001;1170-1175. . Late End Stage 20 U 67% 16 U 74% 87% 97% Eisenmenger
