Management of Adults with Congenital Heart Disease
Key Points
Key Points
- Congenital heart disease (CHD) encompasses a range of structural cardiac abnormalities present before birth attributable to abnormal fetal cardiac development but does not include inherited disorders that may have cardiac manifestations such as Marfan syndrome or hypertrophic cardiomyopathy. Also not included are anatomic variants such as patent foramen ovale.
- The prevalence of ACHD is growing because of the success of pediatric cardiology and congenital cardiac surgery in diagnosing and treating congenital heart defects in children. Improved survival to adulthood is most striking for those with the most severe disease, with survival to age 18 years now expected for 90% of children diagnosed with severe CHD.
- Patients with CHD are not cured of their disease after successful treatment in childhood. Almost all patients with ACHD will have sequelae of either their native CHD or its surgical repair or palliation, although these sequelae can take decades to manifest.
- Patients with ACHD who are cared for in ACHD centers have better outcomes than those cared for in centers without ACHD expertise.
1. Background and Pathophysiology
...ckground and Pathophysiolo...
Table 1. Physiological Variables as Used in ACH...
Treatment
...eatment...
.... ACHD AP Classification (CHD Anatomy +...
...Key Personnel and Services Recommended for A...
...ccess to Care...
...ing smooth transitions for adolescents a...
...moting awareness of the need for lifelong spe...
...livery of Care...
...Patients with ACHD AP classification IB-...
...surgery, catheter-based interventional ca...
.... Delivery of Care: Circumstances Whe...
...4. Evaluation of Suspected and Kno...
...ECGs in ACHD EvaluationHaving trouble viewing tab...
...lectrocardiogram
1. A standard 12-lead electrocardiogram...
...2. Ambulatory electrocardiographic...
...Radiation Principles 1. Strategie...
3.4.3. Echocardiograph...
...perative TEE is recommended to gui...
...ients with ACHD should undergo transthoracic ec...
...4.4. CMR Imagin...
1. In patients with ACHD who have...
...CMR can be useful in the initial eval...
...stances Where CMR, CCT, TEE, and/or Cardiac C...
Table 7. Comparison of Imaging Modalities Useful...
...5. Cardiac Computed Tomography 1. CCT imaging...
...4.6. Cardiac Catheterizatio...
...terization (hemodynamic and/or angiog...
.... In patients with a low or intermediate pretes...
...Exercise Testing...
...In patients with ACHD, cardiopulmonary exercise...
...ymptomatic patients with ACHD, a 6-minute wal...
...nsition Education...
...1. Clinicians caring for patients with CHD...
...xercise and Sports...
...icians should assess activity levels at regular in...
...e useful to guide activity recommendations fo...
...l Health and Neurodevelopmental Issues...
...tients with ACHD should be evaluate...
.... Referral for mental health evaluat...
3.8. Endocarditis PreventionPatients with ACH...
...Syndromes 1. Genetic testing for 2...
...ble 8. Underlying Genetic Syndromes Commonl...
...red Cardiovascular Disease Patien...
.... Noncardiac Medical Issues...
...nts with ACHD at risk for hepatitis C should be sc...
...ncardiac Surgery...
...1. Optimization before and close surveillanc...
...In patients with ACHD AP classifica...
...ACHD Management Issues for Noncardiac Surger...
...gnancy, Reproduction, and Sexual He...
...13.1. Pregna...
...n with CHD should receive prepregnancy co...
.... An individualized plan of care tha...
...th CHD receiving chronic anticoagulati...
4. Women with ACHD AP classificat...
...5. In collaboration with an ACHD...
...and women of childbearing age with CH...
...e testing can be useful for risk assessment i...
...r parent has CHD, it is reasonable to perform f...
...Contraception...
...of childbearing potential with CHD sho...
...Estrogen-containing contraceptives are potentially...
...ility Treatment Menstrual cycle disorders...
...xual Function Sexuality is an important...
...Failure and Transplant...
...Heart Failure 1. Consultation with ACHD and HF...
...ansplant Because of the prevalence of HF amo...
...tiorgan Transplant Recognizing the v...
...Palliative Care...
...ssion of end-of-life issues and advance d...
3.16. Cyano...
...ific Management Practices for Cyanotic CHDH...
...acological Therapy for ACHD Patients with ACHD...
4. Specific Lesions
...ecific Lesions...
...Atrial Septal Defec...
...gnostic...
...ulse oximetry at rest and during exercis...
...R, CCT, and/or TEE are useful to evaluate pul...
...ographic imaging is recommended to...
...herapeutic...
...dults with isolated secundum ASD c...
...lts with primum ASD, sinus venosus defe...
...asymptomatic adults with isolated secundu...
...ical closure of a secundum ASD in adults is...
...cutaneous or surgical closure may be considere...
...e should not be performed in adults with PA systo...
...1. Secondum ASD...
...able 11. ASD: Routine Follow-Up and Te...
...lous Pulmonary Venous Connections...
...gnostic...
...r CTA is recommended for evaluatio...
...Cardiac catheterization can be useful in adu...
Therapeuti...
...Surgical repair is recommended for patie...
...ir of partial anomalous pulmonary venous connec...
...scimitar vein is recommended in adults whe...
...n be useful for right- or left-sided partial a...
...Surgery can be useful for repair...
...Ventricular Septal Defect...
...rapeutic...
...Adults with a VSD and evidence of...
...closure of perimembranous or supracristal VS...
Surgical closure of a VSD may be...
...f a VSD may be considered in the presence...
...SD closure should not be performed in adult...
...Routine Follow-Up and Testing IntervalsHaving tro...
...dynamically Significant Ventricular Level Shunt...
...ioventricular Septal Defect...
...iagnostic...
...atheterization can be useful in adults with...
...erapeutic...
...ery for severe left atrioventricular va...
...or primary repair of atrioventricular septal defec...
...Operation for discrete LVOT obstructi...
...Surgery for primary repair of at...
...Surgery for primary repair of atr...
...AVSD: Routine Follow-Up and Testing I...
....5. Patent Ductus Arteriosus
...iagnostic
...Measurement of oxygen saturation should...
...ddition to the standard diagnostic tools, car...
573
...ure in adults is recommended if left at...
...losure in adults may be considered in the...
...hould not be performed in adults wit...
...le 14. PDA: Routine Follow-Up and Testi...
.... Left-Sided Obstructive Le...
....2.1. Cor Triatriatum...
...gnostic...
...esenting with cor triatriatum sinister sh...
...In adults with prior repair of cor triatriatu...
Therapeutic Surgical repair is indicated for...
...ngenital Mitral Stenosis Adults with congenital...
.... Congenital Mitral Stenosis: Routine Follow-Up an...
4.2.3. Subaortic...
...Stress testing for adults with LVOT obs...
...erapeutic
...rgical intervention is recommended for...
...Surgical intervention is recommended for adults...
...e progression of AR, surgical intervention may...
...Subaortic Stenosis: Routine Follow-Up and Testing...
....2.4. Congenital Valvular Aortic Stenosis...
Diagnos...
...bicuspid aortic valve should be eva...
...It is reasonable to screen first-degr...
...c In adults with bicuspid aortic...
...e 17. Congenital Aortic Stenosis: Routine Fol...
....4.1. Turner Syndrom...
...en with Turner syndrome should be evaluated for...
...Prophylactic replacement of the aortic root...
...2.4.2. Aortopathies Several CHD subtypes and...
4.2.5. Supravalvular Aor...
...agnosti...
...maging using TTE, TEE, CMR, or CTA...
...oronary imaging is recommended in pati...
...erapeuti...
Surgical repair is recommended for adul...
...ry artery revascularization is recom...
...18. Supravalvular Aortic Stenosis: Routine Fol...
4.2.6. Coarctation of t...
...agnostic
...nitial and follow-up aortic imaging using CMR...
...blood pressure should be measured in upper an...
...Ambulatory blood pressure monitoring in...
...intracranial aneurysms by magnetic re...
...ise testing to evaluate for exercise-induced hyper...
...erapeutic...
...air or catheter-based stenting is recom...
...GDMT is recommended for treatment o...
...on angioplasty for adults with native and recu...
...e 19. CoA: Routine Follow-Up and Testing Interv...
4.3. Right-Sided Lesions
...Valvular Pulmonary Steno...
Diagnostic
...dults with moderate or severe valvular...
...ults with moderate or severe valvular pulmon...
In asymptomatic adults with severe v...
...ble 20. Severity of RVOT ObstructionHaving tro...
...1. Valvular PS: Routine Follow-Up and T...
...e 3. Isolated PR After Repair...
...1.1. Isolated PR After Repair of P...
Diagnostic
...tic patients with moderate or greater PR re...
...symptomatic patients with residual P...
...asymptomatic patients with moderate or greater PR...
...3.2. Branch and Peripheral Pulmona...
...For adults with peripheral or branch PS, ongoing...
...herapeutic In adults with peripheral or branc...
...e 22. Branch and Peripheral PS: Routine...
...ouble-Chambered Right Ventricle...
...epair for adults with double-chambered right ventr...
...repair for adults with double-chambered right ven...
...Double-Chambered Right Ventricle: Routine...
...4. Ebstein Anomaly...
...agnostic
...h Ebstein anomaly, CMR can be useful to dete...
...n adults with Ebstein anomaly, TEE...
...ectrophysiological study with or without cathe...
...lts with Ebstein anomaly, electrophysiological s...
...erapeutic
...Surgical repair or reoperation for...
...ablation is recommended for adults wi...
Surgical repair or reoperation for...
...tional superior cavopulmonary (Glenn) ana...
...tein Anomaly: Routine and Follow-Up and Testing...
...Tetralogy of Fallot...
...agnosti...
...ul to quantify ventricular size and...
...Coronary artery compression testing...
...ntricular stimulation can be useful to risk-strat...
...ients with repaired TOF, cardiac catheterizati...
...ry valve replacement (surgical or percutan...
...herapeutic
...Pulmonary valve replacement (surg...
...mary prevention ICD therapy is reasonable in adul...
...cal pulmonary valve replacement may be reas...
...alve replacement, in addition to arrhyt...
...ulmonary Valve Replacement in Patients Wi...
...F: Routine Follow-Up and Testing IntervalsHaving...
...ght Ventricle-to-Pulmonary Artery Conduit...
...gnostic...
...ry compression testing with simultaneous coro...
...In patients with stented right v...
...with right ventricle-to-PA conduit and arrhythmi...
...herapeut...
...ight ventricle-to-PA conduit intervention is r...
...cle-to-PA conduit intervention may be reasonable f...
...e 26. Right Ventricle-to-PA Conduit...
...omplex Lesions...
...ansposition of the Great Arteries With Atrial...
...gnostic...
...atory monitoring for bradycardia or sinus...
...-TGA with atrial switch repair should undergo an...
...t for a communication through the inter...
...peutic GDMT with appropriate attention to t...
...e 27. d-TGA With Atrial Switch: Routine Follow-U...
...nsposition of the Great Arteries Wi...
...gnostic...
...serial imaging with either echocar...
...y revascularization for adults with...
...reasonable to perform anatomic evaluatio...
...ological tests of myocardial perfu...
...easonable to determine the need for...
...erapeutic
...GDMT is reasonable to determine indications...
...Catheter or surgical intervention for PS is r...
...e 28. d-TGA With Arterial Switch: Routine Follow...
...4.1.3. Transposition of the Great Arteries With R...
...ngenitally Corrected Transposition o...
...ostic CMR is reasonable in adults wit...
...herapeut...
...lve replacement is recommended for...
Tricuspid valve replacement is reasonable fo...
Conduit intervention/replacement may be consi...
...e 29. CCTGA: Routine Follow-Up and Testing Inter...
...Palliation of Single Ventricle Physiol...
...iagnostic...
...w presentation of an atrial tachyarrhythmia in...
...ts after Fontan palliation should be evalu...
...Cardiac catheterization should be p...
...or worsening atrial tachyarrhythmias in a...
...with Fontan palliation, it is reasonable to enc...
...ging of the liver (ultrasonography, CMR, CT) and...
...after Fontan palliation, it is reas...
...c catheterization can be useful to evaluate a sym...
...tion for cardiac transplantation is...
...e reasonable to perform catheterization in asym...
...herapeut...
...coagulation with a vitamin K antagonist i...
Catheter ablation can be useful in adult...
...sion surgery, including arrhythmia surgery...
...Pulmonary vasoactive medications can be benefici...
...atelet therapy or anticoagulation with a vitam...
...or intervention for structural/anatomi...
...ontan Palliation: Routine Follow-Up and Test...
....3. Hypoplastic Left Heart Syndrome/Norwood Rep...
...e Norwood repair is the first of 3...
...Truncus Arteriosus...
...rteriosus in the adult has almost invariably b...
.... Double Outlet Right Ventricle
...Double outlet right ventricle is an anatomic des...
...4.6. Severe PAH and Eisenmenger...
...4.6.1. Severe PAH...
...gnostic...
...h ACHD with pulmonary vascular resistance 2.5...
...lts with septal or great artery shunts should u...
...heterization to assess pulmonary vascular hemod...
...th septal or great artery shunts, car...
...-ray, 6-minute walk test, and cardiac catheterizat...
...1. Pulmonary Hypertension and Eisenmenger Synd...
....2. Eisenmenger Synd...
...iagnostic When evaluating adults with presume...
Therapeuti...
...is beneficial in symptomatic adults with Eisenme...
...tomatic adults with Eisenmenger syndrom...
...Bosentan is a reasonable therapy to treat symp...
...It is reasonable to use PDE-5 inhibitors (e.g...
.... Coronary Anomalies...
...actors That May Relate to the Clinical Im...
...ous Coronary Artery Evaluation...
...agnostic...
...ngiography, using catheterization,...
...Anatomic and physiological evaluation shou...
...omalous Aortic Origin of the Corona...
...ous Aortic Origin of Coronary Artery...
...erapeuti...
...recommended for AAOCA from the left si...
...ery is reasonable for anomalous aort...
...for AAOCA is reasonable in the setting of vent...
Surgery or continued observation may be reason...
...us Coronary Artery Arising From the P...
Therapeu...
...ecommended for anomalous left corona...
...omatic adult with anomalous right coronary ar...
...rgery for anomalous right coronary artery...
....4.8. Coronary Artery Fistula...
...Coronary artery fistula is an abnor...