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
...1. Background a...
...ological Variables as Used in ACHD A...
Treatment
...able 2. ACHD AP Classification (CHD Anatomy + Phys...
...e 3. Key Personnel and Services Recommended for AC...
...ccess to Care
...suring smooth transitions for adolescents and youn...
b) promoting awareness of the need fo...
...3.3. Delivery of...
1. Patients with ACHD AP classif...
...2. Cardiac surgery, catheter-based...
...ivery of Care: Circumstances Where ACHD Ex...
...3.4....
...e 5. Use of ECGs in ACHD Evaluation...
...3.4.1....
...A standard 12-lead electrocardiogr...
...ulatory electrocardiographic monitoring sh...
...g Radiation Principles 1. Strategies to lim...
...3.4.3. Echocardio...
...operative TEE is recommended to guide...
...atients with ACHD should undergo transthoracic e...
...3.4.4...
...1. In patients with ACHD who have or who are at r...
...CMR can be useful in the initial evaluat...
...ble 6. Circumstances Where CMR, CCT, TEE,...
...rison of Imaging Modalities Useful in...
...Cardiac Computed Tomography 1. CC...
...3.4...
1. Cardiac catheterization (hemodynamic and/or...
...patients with a low or intermediate pr...
3.4.7. E...
...ts with ACHD, cardiopulmonary exercise testing (C...
...ymptomatic patients with ACHD, a 6...
...3.5. Tr...
...1. Clinicians caring for patients with CHD shou...
...3.6. Exercise and S...
...s should assess activity levels at regul...
...2, CPET can be useful to guide activity recomme...
...3.7. Mental...
...Patients with ACHD should be evaluat...
...2. Referral for mental health evalu...
...Endocarditis PreventionPatients with ACHD have an...
....9. Concomitant Syndromes 1. Genet...
...ble 8. Underlying Genetic Syndromes Commonly A...
3.10. Acquired Cardiovascular Disease P...
...3.11. Noncar...
...atients with ACHD at risk for hepatitis C sh...
...3.12. Noncardiac Surg...
.... Optimization before and close surveill...
...2. In patients with ACHD AP classifi...
...ble 9. ACHD Management Issues for Non...
...3.13. Pregnancy, Reprodu...
...3.13.1. Pregnancy...
...Women with CHD should receive prepregna...
...n individualized plan of care that...
...en with CHD receiving chronic anticoagulation shou...
...with ACHD AP classification IB-D,...
...In collaboration with an ACHD cardiologist to en...
...6. Men and women of childbearing age with CHD...
...testing can be useful for risk assessment i...
...her parent has CHD, it is reasonable to...
...Women of childbearing potential with CHD should b...
...gen-containing contraceptives are potenti...
...fertility Treatment Menstrual cycl...
...al Function Sexuality is an important...
...3.14....
...rt Failure 1. Consultation with...
....14.2. Heart Transplant Because of the p...
...Multiorgan Transplant Recognizing the vuln...
...3.15. Palliative Ca...
...ion of end-of-life issues and advance d...
...3.16. Cyanosi...
...10. Specific Management Practices fo...
...ological Therapy for ACHD Patients...
4. Specific Lesions
...4. S...
...4...
...Diagnostic...
...metry at rest and during exercise is recomm...
..., CCT, and/or TEE are useful to evalu...
...graphic imaging is recommended to gu...
...Therapeutic...
...s with isolated secundum ASD causin...
...th primum ASD, sinus venosus defect or...
...In asymptomatic adults with isolated secu...
...rgical closure of a secundum ASD in adu...
...Percutaneous or surgical closure may be cons...
...should not be performed in adults with PA systol...
...re 1. Secondum ASD...
...Routine Follow-Up and Testing Intervals...
4.1.2. An...
...R or CTA is recommended for evaluation of partial...
...ardiac catheterization can be useful in adul...
...Therapeutic...
...l repair is recommended for patients with...
...Repair of partial anomalous pulmonary v...
...r of a scimitar vein is recommended in...
...can be useful for right- or left-sided p...
...be useful for repair of a scimitar vein in ad...
...4....
...Therapeut...
...th a VSD and evidence of left ventricular...
...cal closure of perimembranous or supracri...
...al closure of a VSD may be reasonable in ad...
...of a VSD may be considered in the prese...
...should not be performed in adults with...
Table 12. VSD: Routine Follow-Up and Test...
...2. Hemodynamically Significant Ve...
...4.1.4. Atrioventric...
...Diagnosti...
...eterization can be useful in adults with...
...Therapeutic...
...Surgery for severe left atrioventr...
Surgery for primary repair of atrioventricular...
...n for discrete LVOT obstruction in ad...
...primary repair of atrioventricular sept...
...Surgery for primary repair of atriov...
...D: Routine Follow-Up and Testing Intervals...
...4.1.5. Patent Ductus...
...Diagnostic
...of oxygen saturation should be performed in...
...dition to the standard diagnostic to...
57...
...PDA closure in adults is recomme...
...closure in adults may be considered...
PDA closure should not be perfor...
...ble 14. PDA: Routine Follow-Up and Testing Int...
...4.2. Left...
...4...
...ults presenting with cor triatriatum sinister...
...In adults with prior repair of cor triatria...
...Surgical repair is indicated for adults with cor t...
....2.2. Congenital Mitral Stenosis Adults...
...able 15. Congenital Mitral Stenosis: Rou...
...4.2.3. Subaortic Steno...
...ress testing for adults with LVOT obstruction...
...Therapeutic...
...rvention is recommended for adults w...
...rgical intervention is recommended for adults with...
...he progression of AR, surgical interventi...
...Subaortic Stenosis: Routine Follow-Up a...
...4.2.4. Congeni...
...Diagnostic
...bicuspid aortic valve should be evaluated...
...s reasonable to screen first-degree relatives of...
...n adults with bicuspid aortic valve stenosis and...
...7. Congenital Aortic Stenosis: Routine Follo...
...agnostic Women with Turner syndrome shou...
...peutic Prophylactic replacement of the aorti...
.... Aortopathies Several CHD subty...
...4.2.5. Supravalvular...
...Diagnostic...
Aortic imaging using TTE, TEE, CMR, or CTA...
...ing is recommended in patients with Williams...
...Therapeut...
...Surgical repair is recommended for adults wit...
...ery revascularization is recommended in symptomati...
.... Supravalvular Aortic Stenosis: Routine Follo...
...4.2.6....
...nitial and follow-up aortic imaging using CMR...
...ng blood pressure should be measured in...
...blood pressure monitoring in adults with coarcta...
...reening for intracranial aneurysms by magnetic r...
...Exercise testing to evaluate for exercise-...
...Therapeutic...
Surgical repair or catheter-based...
...ecommended for treatment of hypertension in patie...
Balloon angioplasty for adults with...
...ble 19. CoA: Routine Follow-Up and Testing...
...4.3. Right...
...4.3.1. Valvular Pul...
...Diagnostic...
...In adults with moderate or severe valvular...
...with moderate or severe valvular pulmonary s...
...ptomatic adults with severe valvular...
...ble 20. Severity of RVOT Obstruction Mi...
...21. Valvular PS: Routine Follow-Up...
...igure 3. Isolated PR After Repai...
...4.3.1.1....
...Diagnost...
...In symptomatic patients with mod...
...mptomatic patients with residual PR resulting...
...tomatic patients with moderate or greater PR resu...
...4.3.2. Branch an...
...c For adults with peripheral or branch PS, ongo...
...c In adults with peripheral or branch PA stenosi...
...and Peripheral PS: Routine Follow-Up a...
...4.3.3. Doubl...
...ir for adults with double-chambered right vent...
...ical repair for adults with double-chambered righ...
...e-Chambered Right Ventricle: Routine Follo...
...4.3.4. Ebstein...
...with Ebstein anomaly, CMR can be useful...
...In adults with Ebstein anomaly, TEE c...
...siological study with or without ca...
...h Ebstein anomaly, electrophysiological study (a...
...Therapeutic...
...Surgical repair or reoperation for ad...
...ter ablation is recommended for adults with...
...gical repair or reoperation for adults...
...ctional superior cavopulmonary (Gl...
...able 24. Ebstein Anomaly: Routine and...
...4.3.5. Tetralog...
...Diagnostic...
...l to quantify ventricular size and func...
...onary artery compression testing is i...
...ogrammed ventricular stimulation can be us...
...tients with repaired TOF, cardiac catheteriz...
...lmonary valve replacement (surgical or percu...
...Therapeutic...
...Pulmonary valve replacement (sur...
...prevention ICD therapy is reasonable in adu...
...Surgical pulmonary valve replacement...
...monary valve replacement, in addition to arrhyt...
...ulmonary Valve Replacement in Patients With TOF...
...able 25. TOF: Routine Follow-Up and T...
4...
...Dia...
...onary artery compression testing with si...
...ts with stented right ventricle-to-P...
...adults with right ventricle-to-PA...
...Therapeutic...
...ht ventricle-to-PA conduit intervention is reason...
...Right ventricle-to-PA conduit intervention may b...
...26. Right Ventricle-to-PA Conduit: Routine...
...4.4. C...
...4.4.1.1. Transpo...
...Diagnostic...
...bulatory monitoring for bradycardi...
...h d-TGA with atrial switch repair should under...
...ssessment for a communication throu...
Therapeutic GDMT with appropriate...
...e 27. d-TGA With Atrial Switch: Routi...
...4.4.1....
...Baseline and serial imaging with eithe...
...revascularization for adults with d-TGA...
...onable to perform anatomic evaluation...
...ysiological tests of myocardial perfusion for adu...
...GDMT is reasonable to determine th...
...Therapeutic...
...sonable to determine indications for...
...Catheter or surgical intervention for P...
...d-TGA With Arterial Switch: Routine...
...osition of the Great Arteries With Rastelli...
...4.4.1.4. Congen...
...CMR is reasonable in adults with...
...Therapeutic...
...icuspid valve replacement is recommended for symp...
...Tricuspid valve replacement is reasonable for as...
...ervention/replacement may be considered...
...CCTGA: Routine Follow-Up and Testing Int...
...4.4.2....
...Diagnostic...
...New presentation of an atrial tac...
...lts after Fontan palliation should be eval...
...atheterization should be performed...
...set or worsening atrial tachyarrhy...
...h Fontan palliation, it is reasonable to encourag...
...f the liver (ultrasonography, CMR, CT)...
...adults after Fontan palliation, it is...
...Cardiac catheterization can be useful to evaluat...
Evaluation for cardiac transplanta...
...It may be reasonable to perform catheteriza...
...ticoagulation with a vitamin K ant...
...Catheter ablation can be useful in adults aft...
...Fontan revision surgery, including arrhythmia...
...Pulmonary vasoactive medications can be b...
...telet therapy or anticoagulation with...
...peration or intervention for structural/anatomic...
.... Fontan Palliation: Routine Follow-Up and...
...repair is the first of 3 steps in palliation fo...
...4.4.4. Truncu...
...eriosus in the adult has almost invar...
...4.4.5. Double Outlet Ri...
...outlet right ventricle is an anatomic descript...
...4.4....
...4.4.6.1. Severe PAH...
Diagn...
...with ACHD with pulmonary vascular...
...Adults with septal or great artery shunts sh...
...Cardiac catheterization to assess pulmona...
...dults with septal or great artery shunts, cardiac...
...BNP, chest x-ray, 6-minute walk test, and...
...monary Hypertension and Eisenmenger Syndr...
...4.4.6.2. Eise...
...When evaluating adults with presumed...
...entan is beneficial in symptomatic adults with E...
...c adults with Eisenmenger syndrome, bosentan...
...Bosentan is a reasonable therapy to...
...s reasonable to use PDE-5 inhibitors (...
...4.4.7. Coronary Anomali...
...32. Factors That May Relate to the Clinica...
...4.4.7....
...Diagnostic...
Coronary angiography, using cathete...
...natomic and physiological evaluati...
...malous Aortic Origin of the Coronary Artery...
...4.4.7.2. Anomalo...
Ther...
...is recommended for AAOCA from the left sinus...
...y is reasonable for anomalous aortic origin of t...
...gery for AAOCA is reasonable in the setting of v...
...rgery or continued observation may be reasonab...
...4.4.7....
...Therapeutic...
...Surgery is recommended for anomalous left corona...
...In a symptomatic adult with anomalous righ...
...for anomalous right coronary artery from t...
...4.4.8. Corona...
...oronary artery fistula is an abnormal communi...