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
.... Background and Pathoph...
...iological Variables as Used in ACHD AP Cl...
Treatment
...reatme...
...able 2. ACHD AP Classification (CHD Anatomy +...
...rsonnel and Services Recommended for ACHD Progr...
3.2. Access to...
...assuring smooth transitions for adolesc...
...omoting awareness of the need for lifelon...
...Delivery of Care...
...s with ACHD AP classification IB-D,...
...2. Cardiac surgery, catheter-based inte...
...4. Delivery of Care: Circumstances Where ACHD Exp...
...valuation of Suspected and Known CHD...
...ble 5. Use of ECGs in ACHD EvaluationHa...
...lectrocardiogram...
...1. A standard 12-lead electrocardiogram...
...ulatory electrocardiographic monitoring sh...
...izing Radiation Principles 1. Strategies...
...Echocardiography...
.... Intraoperative TEE is recommended to guide sur...
...Patients with ACHD should undergo transthoracic ec...
....4.4. CMR Imaging
...patients with ACHD who have or who are...
...useful in the initial evaluation and serial...
...le 6. Circumstances Where CMR, CCT, TE...
Table 7. Comparison of Imaging Mod...
...rdiac Computed Tomography 1. CCT imaging can...
...Cardiac Catheterization...
...Cardiac catheterization (hemodynamic and/or ang...
.... In patients with a low or intermedi...
.... Exercise Testin...
...nts with ACHD, cardiopulmonary exer...
...matic patients with ACHD, a 6-minute walk te...
...ransition Education...
1. Clinicians caring for patients with...
...xercise and Sports
...nicians should assess activity levels at...
...be useful to guide activity recommendat...
...Mental Health and Neurodevelopmental...
...atients with ACHD should be evaluated for dep...
...l for mental health evaluation and...
...ditis PreventionPatients with ACHD hav...
.... Concomitant Syndromes 1. Genetic testing for...
...8. Underlying Genetic Syndromes Commonly Associat...
...cquired Cardiovascular Disease Pa...
...ncardiac Medical Issues...
1. Patients with ACHD at risk for hepati...
....12. Noncardiac S...
...ion before and close surveillance afte...
...atients with ACHD AP classification IB-D, I...
...D Management Issues for Noncardiac Surg...
...ncy, Reproduction, and Sexual Health...
...3.1. Pregnancy...
...with CHD should receive prepregna...
...ndividualized plan of care that addresses...
Women with CHD receiving chronic an...
...h ACHD AP classification IB-D, IIA-D,...
...ion with an ACHD cardiologist to ensure accurate...
...omen of childbearing age with CHD should be couns...
...e testing can be useful for risk assess...
...n either parent has CHD, it is reasonable to perfo...
3.13.2. Contra...
...1. Women of childbearing potential with...
...trogen-containing contraceptives are potentia...
.... Infertility Treatment Menstrual cycle disorder...
...unction Sexuality is an important element of Q...
...4. Heart Failure and Tr...
...Failure 1. Consultation with ACHD and HF spec...
...4.2. Heart Transplant Because of the prev...
....14.3. Multiorgan Transplant Recognizing the v...
.... Palliative Care...
...ssion of end-of-life issues and adv...
....16. Cyanosis
...le 10. Specific Management Practices...
...rmacological Therapy for ACHD Pati...
4. Specific Lesions
...pecific Lesions...
...rial Septal Defect...
...gnostic
...y at rest and during exercise is recommended...
.../or TEE are useful to evaluate pulmonary venous c...
...Echocardiographic imaging is recommended to gui...
...erapeutic...
...with isolated secundum ASD causing impa...
...th primum ASD, sinus venosus defect or corona...
...atic adults with isolated secundum ASD...
...Surgical closure of a secundum ASD in adults...
...or surgical closure may be considered for adults w...
...should not be performed in adults with PA systol...
...gure 1. Secondu...
...Routine Follow-Up and Testing IntervalsHaving...
...ous Pulmonary Venous Connection...
...agnostic
...or CTA is recommended for evaluatio...
...terization can be useful in adults wi...
...rapeutic...
...Surgical repair is recommended for patients...
...Repair of partial anomalous pulmonary...
...a scimitar vein is recommended in adults when...
...urgery can be useful for right- or left-s...
...Surgery can be useful for repair of a sc...
....1.3. Ventricular Septa...
...herapeutic...
...Adults with a VSD and evidence of left v...
...gical closure of perimembranous or supr...
...closure of a VSD may be reasonable in adult...
...f a VSD may be considered in the presence of a ne...
...ystolic pressure greater than two thir...
...Routine Follow-Up and Testing Intervals...
...igure 2. Hemodynamically Significant...
...trioventricular Septal Defe...
...iagnost...
...rdiac catheterization can be useful in...
...erapeutic...
...Surgery for severe left atrioventric...
...for primary repair of atrioventric...
...or discrete LVOT obstruction in adults...
...Surgery for primary repair of atr...
...or primary repair of atrioventricular sep...
...Routine Follow-Up and Testing Int...
...tent Ductus Arteriosus...
...agnostic...
...ment of oxygen saturation should be...
...ion to the standard diagnostic tools, cardia...
...73
...in adults is recommended if left atrial or...
...PDA closure in adults may be considered in the...
...e should not be performed in adults with a...
...outine Follow-Up and Testing IntervalsHavi...
4.2. Left-Sided Obstructive...
.... Cor Triatriat...
...gnostic...
...lts presenting with cor triatriatum...
...with prior repair of cor triatriatum si...
...peutic Surgical repair is indicated...
...Congenital Mitral Stenosis Adults with...
...5. Congenital Mitral Stenosis: Routine Foll...
...ubaortic Stenosis...
...Stress testing for adults with LVOT...
...rapeutic...
...al intervention is recommended for adults...
...tervention is recommended for adults with subAS a...
...prevent the progression of AR, surgical interven...
...ortic Stenosis: Routine Follow-Up and Testing Inte...
...ital Valvular Aortic Stenosis...
...agnostic...
...th bicuspid aortic valve should be evaluated...
...It is reasonable to screen first-degree relatives...
...erapeutic In adults with bicuspid aortic valve...
...Congenital Aortic Stenosis: Routine Follow-...
....1. Turner Syndrome...
...tic Women with Turner syndrome should...
...apeutic Prophylactic replacement of the a...
.... Aortopathies Several CHD subtypes and/o...
4.2.5. Supravalvular Aortic Ste...
...gnostic
...ng using TTE, TEE, CMR, or CTA is recommended...
...Coronary imaging is recommended in pati...
...erapeutic
...epair is recommended for adults with su...
...Coronary artery revascularization is...
...8. Supravalvular Aortic Stenosis: Routine...
...Coarctation of the Aor...
...agnostic
...tial and follow-up aortic imaging using...
...Resting blood pressure should be measured in...
...ulatory blood pressure monitoring in adults wi...
...Screening for intracranial aneurysms by magnet...
...testing to evaluate for exercise-induced h...
Therapeu...
Surgical repair or catheter-based...
...mended for treatment of hypertension in patient...
...angioplasty for adults with native and rec...
...Routine Follow-Up and Testing Intervals...
...ght-Sided Lesions...
...alvular Pulmonary Stenosis...
...agnostic...
...dults with moderate or severe valv...
...In adults with moderate or severe valv...
...ic adults with severe valvular pulmonary st...
...ty of RVOT ObstructionHaving troubl...
...Valvular PS: Routine Follow-Up and Testin...
...3. Isolated PR After Repair of PS...
4.3.1.1. Isolated PR After Repair of...
Diagnost...
...mptomatic patients with moderate or greater...
...matic patients with residual PR res...
...In asymptomatic patients with moderate or gr...
...Branch and Peripheral Pulmonary Stenosis...
...iagnostic For adults with peripher...
...In adults with peripheral or branch PA st...
...able 22. Branch and Peripheral PS: Routine Follo...
...Double-Chambered Right Ventricle...
...rgical repair for adults with double...
Surgical repair for adults with double-chambe...
...ble-Chambered Right Ventricle: Routi...
...4. Ebstein Anomal...
...iagnost...
...adults with Ebstein anomaly, CMR can be useful...
...s with Ebstein anomaly, TEE can be use...
...hysiological study with or without catheter...
...with Ebstein anomaly, electrophysiological study...
...rapeutic...
...rgical repair or reoperation for ad...
...ation is recommended for adults with Ebstein ano...
Surgical repair or reoperation for ad...
...superior cavopulmonary (Glenn) anast...
...bstein Anomaly: Routine and Follow...
...Tetralogy of Fallot...
...gnostic...
...useful to quantify ventricular size and function,...
Coronary artery compression testing is ind...
...ventricular stimulation can be useful to...
...patients with repaired TOF, cardia...
...ulmonary valve replacement (surgical...
Therapeuti...
...onary valve replacement (surgical or percuta...
Primary prevention ICD therapy is reasonable...
...Surgical pulmonary valve replacement ma...
...alve replacement, in addition to arrhythmia...
...4. Pulmonary Valve Replacement in Patients...
...: Routine Follow-Up and Testing In...
...Ventricle-to-Pulmonary Artery Conduit...
Diagnosti...
...tery compression testing with simultaneous cor...
...patients with stented right ventricle-to-PA co...
...adults with right ventricle-to-PA...
...erapeutic...
...t ventricle-to-PA conduit intervention is re...
...entricle-to-PA conduit intervention may be...
...26. Right Ventricle-to-PA Conduit:...
.... Complex Lesions...
....1. Transposition of the Great Arteries With Atria...
...agnostic...
...monitoring for bradycardia or sinus n...
...dults with d-TGA with atrial switch repair should...
...t for a communication through the interatr...
...GDMT with appropriate attention to the need...
...d-TGA With Atrial Switch: Routine...
4.4.1.2. Transposition of the Great Arter...
...agnostic...
...serial imaging with either echocardiography or C...
...scularization for adults with d-TGA...
...reasonable to perform anatomic evalua...
...hysiological tests of myocardial perfusion for ad...
...MT is reasonable to determine the ne...
Therapeut...
...MT is reasonable to determine indications for...
...theter or surgical intervention for PS is r...
...TGA With Arterial Switch: Routine Fo...
....4.1.3. Transposition of the Great...
...ongenitally Corrected Transposition of the Grea...
Diagnostic CMR is reasonable in adults with...
...rapeutic
...spid valve replacement is recommended for symptoma...
...uspid valve replacement is reasonable for as...
...vention/replacement may be conside...
...29. CCTGA: Routine Follow-Up and Testing Interval...
.... Fontan Palliation of Single Ventr...
Diagnostic
...New presentation of an atrial tachyarrhythmia...
...Fontan palliation should be evaluated annuall...
...Cardiac catheterization should be performed...
...worsening atrial tachyarrhythmias in adul...
...In adults with Fontan palliation, i...
...aging of the liver (ultrasonography, CMR,...
...n adults after Fontan palliation, it...
...Cardiac catheterization can be useful to evaluat...
...for cardiac transplantation is reasonable in adu...
...asonable to perform catheterization in asymptoma...
...erapeutic
...on with a vitamin K antagonist is recommend...
...ter ablation can be useful in adults after Font...
...an revision surgery, including arrhythmia surg...
...ulmonary vasoactive medications can...
...therapy or anticoagulation with a vitamin K...
...Reoperation or intervention for structu...
...ontan Palliation: Routine Follow-Up and Te...
...lastic Left Heart Syndrome/Norwood Repair...
...The Norwood repair is the first of 3 steps in pa...
...runcus Arteriosus...
Truncus arteriosus in the adult has almost inva...
....5. Double Outlet Right Ventricle...
...e outlet right ventricle is an anatomic d...
.... Severe PAH and Eisenmenger Syndrome...
....1. Severe PAH...
Diagnos...
...ith ACHD with pulmonary vascular resistance 2.5...
...ith septal or great artery shunts should...
...heterization to assess pulmonary vas...
...h septal or great artery shunts, cardiac ca...
...chest x-ray, 6-minute walk test, and c...
...monary Hypertension and Eisenmenger Syndrome:...
...2. Eisenmenger Syndrome...
...gnostic When evaluating adults with presumed Ei...
...erapeutic...
...beneficial in symptomatic adults with Eisenmenge...
...In symptomatic adults with Eisenme...
...Bosentan is a reasonable therapy to treat...
...s reasonable to use PDE-5 inhibitors (e...
....7. Coronary Anomali...
...32. Factors That May Relate to the Clinical Impor...
...alous Coronary Artery Evaluation...
...gnostic...
...oronary angiography, using catheterization...
...c and physiological evaluation should be perfor...
...e 5. Anomalous Aortic Origin of the...
...2. Anomalous Aortic Origin of Coronary Artery...
...rapeutic...
...Surgery is recommended for AAOCA f...
...rgery is reasonable for anomalous aortic o...
...Surgery for AAOCA is reasonable in the se...
...rgery or continued observation may be reasonab...
....7.3. Anomalous Coronary Artery Arising From the...
...herapeut...
...ecommended for anomalous left coronary a...
...matic adult with anomalous right coron...
...for anomalous right coronary arter...
.... Coronary Artery Fistul...
...nary artery fistula is an abnormal co...