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 and Path...
...logical Variables as Used in ACHD AP Classificati...
Treatment
...Treatment...
...HD AP Classification (CHD Anatomy + Physiologica...
Table 3. Key Personnel and Services Re...
....2. Access to Car...
...a) assuring smooth transitions for...
...b) promoting awareness of the need for lifelong...
...3.3. Delivery of...
.... Patients with ACHD AP classification IB-D...
...c surgery, catheter-based interven...
...4. Delivery of Care: Circumstances Where...
...3.4. Evaluation...
.... Use of ECGs in ACHD EvaluationHaving...
...3.4.1. Ele...
...1. A standard 12-lead electrocardiogram (ECG)...
...electrocardiographic monitoring should be per...
...nizing Radiation Principles 1. Strateg...
...3.4.3. Echocardiography...
...1. Intraoperative TEE is recommended to guide...
.... Patients with ACHD should undergo tr...
...patients with ACHD who have or who are at...
...can be useful in the initial eval...
...le 6. Circumstances Where CMR, CCT, TEE,...
...arison of Imaging Modalities Useful in ACH...
....5. Cardiac Computed Tomography 1....
...3.4.6. Cardiac Cat...
...ardiac catheterization (hemodynamic and/o...
...2. In patients with a low or intermedia...
...3.4.7. Exercise Testing...
1. In patients with ACHD, cardiop...
...In symptomatic patients with ACHD, a...
...3.5. Transition Educa...
...inicians caring for patients with CHD sh...
...3.6...
...Clinicians should assess activity levels at regu...
...can be useful to guide activity recommendations...
...3.7. Mental Healt...
...with ACHD should be evaluated for...
...erral for mental health evaluation and treatment...
...carditis PreventionPatients with ACHD have a...
3.9. Concomitant Syndromes 1. Genetic testing fo...
...able 8. Underlying Genetic Syndromes Com...
....10. Acquired Cardiovascular Disease...
...3.11. Noncardia...
...Patients with ACHD at risk for hep...
...3.12....
...timization before and close surveillance after i...
...s with ACHD AP classification IB-D,...
...ble 9. ACHD Management Issues for Noncardiac...
...3.13. Pregnan...
...3.13.1. P...
...Women with CHD should receive prepregn...
...n individualized plan of care that a...
...omen with CHD receiving chronic anticoagulation sh...
...with ACHD AP classification IB-D, IIA-D, and II...
...5. In collaboration with an ACHD cardiologis...
...Men and women of childbearing age with CHD sho...
...cise testing can be useful for risk assessment i...
...parent has CHD, it is reasonable to per...
...3.13.2. Contracepti...
...1. Women of childbearing potential...
.... Estrogen-containing contraceptive...
...ity Treatment Menstrual cycle disorders...
...Function Sexuality is an important...
...3.14. H...
...1 Heart Failure 1. Consultation w...
.... Heart Transplant Because of the prevalence of...
...3. Multiorgan Transplant Recognizing the vulnera...
...3.15. Palliative...
...ussion of end-of-life issues and advance directive...
...3.16. Cyanosis...
.... Specific Management Practices fo...
...Pharmacological Therapy for ACHD Patients with...
4. Specific Lesions
...4...
...4.1.1. Atrial Septal D...
...Diagnosti...
...Pulse oximetry at rest and during...
...CCT, and/or TEE are useful to evaluate pulmonary v...
...ardiographic imaging is recommended to gu...
...Therapeutic...
...dults with isolated secundum ASD causing impaired...
...th primum ASD, sinus venosus defect...
...ymptomatic adults with isolated secundum ASD, righ...
...al closure of a secundum ASD in adult...
...us or surgical closure may be considered for a...
ASD closure should not be performed in adults...
...1. Secondum ASD...
Table 11. ASD: Routine Follow-Up and Testin...
...4.1.2. Anomalous Pu...
...Diagn...
...A is recommended for evaluation of partial...
...iac catheterization can be useful in...
...Therapeuti...
...pair is recommended for patients w...
...f partial anomalous pulmonary venous conn...
...pair of a scimitar vein is recommended in adul...
...gery can be useful for right- or left-...
...can be useful for repair of a scimitar vein...
...4.1.3....
...Ther...
...Adults with a VSD and evidence of le...
Surgical closure of perimembranous or...
...ical closure of a VSD may be reasonabl...
...Closure of a VSD may be considered in...
...e should not be performed in adults with...
...le 12. VSD: Routine Follow-Up and Testing Interv...
Figure 2. Hemodynamically Significant Ventricula...
...4.1.4. Atrioventri...
...Diagnostic...
...diac catheterization can be useful in adults wi...
...Therapeuti...
...ry for severe left atrioventricular val...
...for primary repair of atrioventricular septal def...
...tion for discrete LVOT obstruction in adu...
...Surgery for primary repair of atrioventricula...
...Surgery for primary repair of atrioventricular s...
...13. AVSD: Routine Follow-Up and T...
...4.1.5. Patent...
...Dia...
...of oxygen saturation should be performed in f...
...In addition to the standard diagno...
...73...
...DA closure in adults is recommended if left atrial...
...ure in adults may be considered in the presen...
PDA closure should not be performed in adult...
...e 14. PDA: Routine Follow-Up and Testing Interv...
...4.2. Left-Sided Ob...
...4.2.1. Cor Triatriatum...
...D...
...Adults presenting with cor triatriatum sini...
...h prior repair of cor triatriatum sinister and rec...
...eutic Surgical repair is indicated...
...nital Mitral Stenosis Adults with conge...
...ongenital Mitral Stenosis: Routine Follow-Up...
4.2.3....
...agnostic Stress testing for adults wi...
...Therapeutic...
...tervention is recommended for adults with subAS,...
...gical intervention is recommended fo...
...To prevent the progression of AR, surgical in...
...baortic Stenosis: Routine Follow-Up and Testing...
...4.2.4. Congenital Valvular...
...Diagnostic...
...ith bicuspid aortic valve should be eva...
...easonable to screen first-degree r...
...tic In adults with bicuspid aortic valve...
...Congenital Aortic Stenosis: Routine Follow-Up and...
...4.2.4.1. Turn...
...en with Turner syndrome should be evaluated for...
...Prophylactic replacement of the...
...rtopathies Several CHD subtypes and/or r...
...4.2.5. Supravalvular...
...imaging using TTE, TEE, CMR, or CT...
...onary imaging is recommended in patients wi...
...ical repair is recommended for adults wi...
...onary artery revascularization is recommended...
.... Supravalvular Aortic Stenosis: Routine Follow-Up...
...4.2.6. Coarctati...
...Diagnostic...
...ollow-up aortic imaging using CMR or CTA is...
...Resting blood pressure should be measure...
...Ambulatory blood pressure monitori...
...r intracranial aneurysms by magnetic resonance an...
Exercise testing to evaluate for exercise-induc...
...Therap...
...epair or catheter-based stenting is r...
GDMT is recommended for treatment of h...
Balloon angioplasty for adults with nati...
Table 19. CoA: Routine Follow-Up and T...
...4.3.1. Valvul...
...Diagnostic...
In adults with moderate or severe valvular...
...n adults with moderate or severe valvu...
...asymptomatic adults with severe valvul...
...able 20. Severity of RVOT ObstructionHa...
...Valvular PS: Routine Follow-Up and Testing I...
...solated PR After Repair of PS...
...4.3.1.1. Isola...
...Diagnos...
In symptomatic patients with moderate or...
...asymptomatic patients with residual PR re...
...ptomatic patients with moderate or greater PR...
...4.3.2....
...For adults with peripheral or branch PS, on...
...adults with peripheral or branch PA...
...ch and Peripheral PS: Routine Follo...
...4.3.3. Double-Cham...
...pair for adults with double-chambered right ven...
...al repair for adults with double-chambered right...
...le 23. Double-Chambered Right Ventricle: Routine F...
...4.3.4. Ebs...
...Diagnos...
...dults with Ebstein anomaly, CMR can be...
...adults with Ebstein anomaly, TEE can be useful...
Electrophysiological study with or with...
...In adults with Ebstein anomaly, electrophy...
...Therapeutic...
...l repair or reoperation for adults with Ebste...
...atheter ablation is recommended for adults w...
...repair or reoperation for adults with Ebstei...
...tional superior cavopulmonary (Glenn) anastomo...
...stein Anomaly: Routine and Follow-Up and...
...4.3.5. Tetralo...
...Diagnos...
...CMR is useful to quantify ventricular size and...
...y artery compression testing is indicated b...
...Programmed ventricular stimulation can be us...
...ts with repaired TOF, cardiac cathete...
...valve replacement (surgical or percutaneou...
...Th...
...lve replacement (surgical or percutaneous)...
...ary prevention ICD therapy is reasonable in adu...
...al pulmonary valve replacement may be reasonable...
...monary valve replacement, in addition to...
...ure 4. Pulmonary Valve Replacement in Pati...
...TOF: Routine Follow-Up and Testing IntervalsH...
...4.3.6. Right Ventric...
...Diagnostic...
Coronary artery compression testing wit...
...ients with stented right ventricle-to-PA cond...
...adults with right ventricle-to-PA condui...
...Right ventricle-to-PA conduit intervention is re...
...ht ventricle-to-PA conduit intervention may b...
...ht Ventricle-to-PA Conduit: Routine Fo...
...4.4....
...bulatory monitoring for bradycardia...
...ts with d-TGA with atrial switch repair should un...
...Assessment for a communication through t...
...erapeutic GDMT with appropriate attention to...
...GA With Atrial Switch: Routine Follow-Up and Test...
...4.4.1.2. Transposit...
...Diagnostic...
...Baseline and serial imaging with eit...
...nary revascularization for adults with d-T...
...sonable to perform anatomic evaluation of co...
...al tests of myocardial perfusion for adults with d...
...asonable to determine the need for coronary revasc...
Therap...
...nable to determine indications for aortic valv...
...r or surgical intervention for PS...
...-TGA With Arterial Switch: Routine Follow-Up...
...1.3. Transposition of the Great Arteries With...
...4.4.1.4. Con...
...CMR is reasonable in adults with CCTGA to d...
...Therapeutic...
...id valve replacement is recommended fo...
...id valve replacement is reasonable for a...
...t intervention/replacement may be consi...
...9. CCTGA: Routine Follow-Up and Testi...
...4.4.2....
...Di...
...tion of an atrial tachyarrhythmia in adults with...
...Fontan palliation should be evaluat...
...diac catheterization should be performed i...
...t or worsening atrial tachyarrhythmias in...
...In adults with Fontan palliation, it is re...
...e liver (ultrasonography, CMR, CT) an...
In adults after Fontan palliation, it...
...rdiac catheterization can be useful...
...r cardiac transplantation is reasonable in adults...
...It may be reasonable to perform catheterization...
...Therapeutic...
...ion with a vitamin K antagonist is recommended...
...ablation can be useful in adults af...
...sion surgery, including arrhythmia surgery as ind...
...Pulmonary vasoactive medications can be benefic...
...Antiplatelet therapy or anticoagulation...
...ation or intervention for structural/anatom...
...ntan Palliation: Routine Follow-Up and Testin...
...4.4.3. Hypoplastic L...
...repair is the first of 3 steps in palliation f...
...4....
...s arteriosus in the adult has almost invar...
...4.4.5. Dou...
...let right ventricle is an anatomic descriptor t...
...4.4.6. Sever...
...4.4.6.1. Se...
...Diagnost...
...s with ACHD with pulmonary vascular resistanc...
...s with septal or great artery shunts should...
...iac catheterization to assess pulmonary vascular...
...adults with septal or great artery shunts,...
...chest x-ray, 6-minute walk test, and car...
...ary Hypertension and Eisenmenger Syn...
...When evaluating adults with presumed Eisenme...
...Therapeutic...
...Bosentan is beneficial in sympto...
In symptomatic adults with Eisen...
...tan is a reasonable therapy to treat symptom...
...is reasonable to use PDE-5 inhibit...
...4.4.7. Coronary An...
...e 32. Factors That May Relate to the...
...4.4....
...Diagnostic...
...angiography, using catheterization, CT, or...
...and physiological evaluation should be pe...
...e 5. Anomalous Aortic Origin of the Coronary Art...
...4...
...Therapeutic...
...recommended for AAOCA from the left sinus or AAOC...
...y is reasonable for anomalous aortic...
...or AAOCA is reasonable in the setting of ventr...
...continued observation may be reason...
...4.4.7.3. Anoma...
...The...
...gery is recommended for anomalous left...
...mptomatic adult with anomalous right...
...ery for anomalous right coronary artery from the P...
...4.4.8. Coronary Ar...
...rtery fistula is an abnormal communication betwee...