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
...ound and Pathophysiology...
...1. Physiological Variables as Used in ACH...
Treatment
...reatme...
...Classification (CHD Anatomy + Phys...
...3. Key Personnel and Services Recommended for A...
...2. Access to Care...
...) assuring smooth transitions for a...
...awareness of the need for lifelong specialized...
...Delivery of Ca...
...1. Patients with ACHD AP classificatio...
2. Cardiac surgery, catheter-based...
...y of Care: Circumstances Where ACHD Expertise...
...n of Suspected and Known CHD...
...f ECGs in ACHD EvaluationHaving tr...
....1. Electrocardiog...
1. A standard 12-lead electrocardiogram (ECG)...
...y electrocardiographic monitoring should b...
...ng Radiation Principles 1. Strategies to limi...
3.4.3. Echocardio...
...1. Intraoperative TEE is recommended to...
...2. Patients with ACHD should underg...
....4.4. CMR Imagin...
...ts with ACHD who have or who are at risk of de...
...MR can be useful in the initial evalua...
...tances Where CMR, CCT, TEE, and/or...
.... Comparison of Imaging Modalities Useful i...
...omputed Tomography 1. CCT imaging can...
...diac Catheterization...
...terization (hemodynamic and/or angiographic) i...
...In patients with a low or intermediate prete...
.... Exercise Testing...
...1. In patients with ACHD, cardiopulmonar...
...n symptomatic patients with ACHD, a 6-minute...
...ansition Education...
...s caring for patients with CHD should deliv...
...ercise and Sports...
...inicians should assess activity leve...
2, CPET can be useful to guide a...
.... Mental Health and Neurodevelopmental I...
...1. Patients with ACHD should be evaluated for dep...
...or mental health evaluation and treatment is r...
...arditis PreventionPatients with ACHD have...
3.9. Concomitant Syndromes 1. Gen...
...ing Genetic Syndromes Commonly Associ...
...Cardiovascular Disease Patients with ACHD c...
...cardiac Medical Issues...
...with ACHD at risk for hepatitis C s...
.... Noncardiac Surger...
1. Optimization before and close surveillance...
...nts with ACHD AP classification IB-D, IIA-...
...e 9. ACHD Management Issues for Noncardiac Su...
...cy, Reproduction, and Sexual Health...
....13.1. Pregnancy
...with CHD should receive prepregnancy counsel...
...2. An individualized plan of care that add...
...3. Women with CHD receiving chronic anticoagul...
...omen with ACHD AP classification IB-D, IIA-D, an...
...oration with an ACHD cardiologist to ensure...
...6. Men and women of childbearing age with CHD...
...xercise testing can be useful for risk ass...
...When either parent has CHD, it is re...
....2. Contraception...
...1. Women of childbearing potential with CHD...
...ogen-containing contraceptives are potentiall...
....13.3. Infertility Treatment Menstrual cyc...
...3.4. Sexual Function Sexuality is an importan...
3.14. Heart Failure and Tran...
...Heart Failure 1. Consultation with ACHD and...
...eart Transplant Because of the prevalence of...
...Multiorgan Transplant Recognizing the vulner...
...15. Palliative C...
...ssion of end-of-life issues and adv...
...16. Cyanosis...
Table 10. Specific Management Practices f...
...7. Pharmacological Therapy for ACHD...
4. Specific Lesions
...pecific Lesions...
...Atrial Septal Defec...
...agnostic...
...se oximetry at rest and during exercise is rec...
...R, CCT, and/or TEE are useful to evaluate pulmon...
...Echocardiographic imaging is recommended to...
...herapeut...
...ults with isolated secundum ASD cau...
...primum ASD, sinus venosus defect or cor...
...ptomatic adults with isolated secundum ASD,...
...urgical closure of a secundum ASD in...
...or surgical closure may be considered for adu...
...osure should not be performed in adults w...
...e 1. Secondum ASD...
...ASD: Routine Follow-Up and Testing IntervalsHavi...
...nomalous Pulmonary Venous Connections...
Diagnost...
...CTA is recommended for evaluation...
...catheterization can be useful in...
Therapeutic
...rgical repair is recommended for pa...
...of partial anomalous pulmonary venous conn...
...air of a scimitar vein is recommended in adults...
...Surgery can be useful for right- or left...
...ery can be useful for repair of a sci...
...Ventricular Septal Defect
...rapeutic...
...ith a VSD and evidence of left ventricular v...
...al closure of perimembranous or supracristal V...
...urgical closure of a VSD may be reasonab...
...VSD may be considered in the presence of a...
...VSD closure should not be performed in adults wit...
...outine Follow-Up and Testing Interval...
...ynamically Significant Ventricular Level Shunt...
....1.4. Atrioventricular Septal Defect
Diagnostic
...rdiac catheterization can be useful i...
...herapeut...
...Surgery for severe left atrioventricu...
...gery for primary repair of atrioventricular sep...
...r discrete LVOT obstruction in adults wit...
...y for primary repair of atrioventricul...
...primary repair of atrioventricular septal...
...Routine Follow-Up and Testing Inte...
...5. Patent Ductus Arteriosus...
...gnostic...
...ement of oxygen saturation should be p...
...In addition to the standard diagnosti...
...73
...closure in adults is recommended if left atrial...
...PDA closure in adults may be considered i...
...hould not be performed in adults with a...
...DA: Routine Follow-Up and Testing IntervalsH...
...Left-Sided Obstructive Les...
...2.1. Cor Triatriat...
...gnostic...
...resenting with cor triatriatum sinist...
...ts with prior repair of cor triatriatum si...
...tic Surgical repair is indicated for adu...
...nital Mitral Stenosis Adults with congenital...
...15. Congenital Mitral Stenosis: Routine Fo...
....3. Subaortic Stenosis...
...ess testing for adults with LVOT o...
Therapeu...
...rvention is recommended for adults with...
...intervention is recommended for adults...
...To prevent the progression of AR, surgical...
...16. Subaortic Stenosis: Routine Fo...
...2.4. Congenital Valvular Aor...
Diagnosti...
...s with bicuspid aortic valve should...
...able to screen first-degree relatives of p...
...utic In adults with bicuspid aortic valve...
...ongenital Aortic Stenosis: Routine Follow-Up...
...4.1. Turner Syndrome...
...Women with Turner syndrome should be evalu...
...c Prophylactic replacement of the ao...
...Aortopathies Several CHD subtype...
....5. Supravalvular Aortic Stenosis
...gnostic
...rtic imaging using TTE, TEE, CMR, or CTA is...
...ary imaging is recommended in patients with W...
...herapeutic
...epair is recommended for adults with suprav...
...artery revascularization is recommende...
...e 18. Supravalvular Aortic Stenosis: Routine...
...arctation of the Aorta...
...iagnostic...
...d follow-up aortic imaging using CMR...
Resting blood pressure should be measur...
...blood pressure monitoring in adults with coarctat...
...ing for intracranial aneurysms by m...
...Exercise testing to evaluate for exercis...
...rapeutic...
...ir or catheter-based stenting is recomme...
...s recommended for treatment of hype...
...angioplasty for adults with native and rec...
...e 19. CoA: Routine Follow-Up and Testing Inter...
...Right-Sided Lesi...
4.3.1. Valvular Pulmonary...
...iagnostic...
...ith moderate or severe valvular pulmonary ste...
...th moderate or severe valvular pul...
...In asymptomatic adults with severe va...
...20. Severity of RVOT ObstructionHaving trouble...
...lar PS: Routine Follow-Up and Testing Inter...
...e 3. Isolated PR After Repair of...
...Isolated PR After Repair of Pulmonary...
Diagnos...
...In symptomatic patients with moderate or greater...
...atic patients with residual PR resulting from tr...
...In asymptomatic patients with moderate...
...Branch and Peripheral Pulmonary Stenosi...
...tic For adults with peripheral or branch PS, o...
...In adults with peripheral or branch PA stenosi...
...and Peripheral PS: Routine Follow-Up and Te...
...ble-Chambered Right Ventricle...
...Surgical repair for adults with double-cha...
...urgical repair for adults with double-chambe...
...le-Chambered Right Ventricle: Routine Fol...
4.3.4. Ebstein Anoma...
...agnostic...
...th Ebstein anomaly, CMR can be useful to de...
...In adults with Ebstein anomaly, T...
...ophysiological study with or without cathe...
...h Ebstein anomaly, electrophysiologi...
Therapeu...
...repair or reoperation for adults with E...
...r ablation is recommended for adults with Ebst...
...air or reoperation for adults with E...
...directional superior cavopulmonary (Gle...
...4. Ebstein Anomaly: Routine and Follow-Up a...
...5. Tetralogy of Fallo...
...agnostic...
...ul to quantify ventricular size and func...
...ry compression testing is indicated...
Programmed ventricular stimulation can be...
...ients with repaired TOF, cardiac cathe...
...Pulmonary valve replacement (surgical or per...
...herapeut...
...Pulmonary valve replacement (surgical or percu...
...y prevention ICD therapy is reasonable in adul...
...Surgical pulmonary valve replacemen...
...ve replacement, in addition to arrhyt...
...monary Valve Replacement in Patients Wi...
...outine Follow-Up and Testing IntervalsHaving troub...
...ntricle-to-Pulmonary Artery Conduit...
Diagnosti...
...artery compression testing with simultaneous coron...
...patients with stented right ventricle-t...
...ts with right ventricle-to-PA conduit an...
...erapeutic...
...le-to-PA conduit intervention is reasonable f...
...ventricle-to-PA conduit intervention...
...ight Ventricle-to-PA Conduit: Routine Follow-U...
...Complex Lesions...
...osition of the Great Arteries With Atrial S...
...gnostic...
...ulatory monitoring for bradycardia or...
...lts with d-TGA with atrial switch repai...
...ssment for a communication through the in...
...GDMT with appropriate attention to the need...
...With Atrial Switch: Routine Follow-Up and T...
...4.1.2. Transposition of the Great Ar...
...agnostic...
...serial imaging with either echocardiograp...
...ry revascularization for adults with d-TGA with...
...asonable to perform anatomic evaluation...
...al tests of myocardial perfusion for adults w...
...nable to determine the need for coronary reva...
...erapeutic...
...reasonable to determine indications for aortic...
...surgical intervention for PS is reasonable in a...
...-TGA With Arterial Switch: Routine Follow-Up and...
...sposition of the Great Arteries With Rastelli Typ...
...itally Corrected Transposition of th...
...nostic CMR is reasonable in adult...
Therapeuti...
...pid valve replacement is recommended for...
...lve replacement is reasonable for asymptoma...
...tervention/replacement may be considered f...
...A: Routine Follow-Up and Testing Int...
...Fontan Palliation of Single Ventricle Phy...
...iagnostic...
New presentation of an atrial tach...
...r Fontan palliation should be evaluated annuall...
...diac catheterization should be performed...
...New onset or worsening atrial tachyarrhythmias i...
...h Fontan palliation, it is reasonable to encourage...
...ing of the liver (ultrasonography, CMR, C...
...adults after Fontan palliation, it is reasonable...
...catheterization can be useful to ev...
...ion for cardiac transplantation is reasonab...
...may be reasonable to perform catheteriz...
Therapeuti...
...nticoagulation with a vitamin K antagonist is reco...
...ation can be useful in adults after Fon...
...revision surgery, including arrhythmia surgery...
...vasoactive medications can be beneficial to impr...
...atelet therapy or anticoagulation with a vit...
...n or intervention for structural/anatomic a...
...an Palliation: Routine Follow-Up and Testing Int...
...lastic Left Heart Syndrome/Norwood Repair...
...rwood repair is the first of 3 steps in palliatio...
...Truncus Arteriosus...
...cus arteriosus in the adult has almo...
...ouble Outlet Right Ventricle
...ble outlet right ventricle is an anatomic descr...
...Severe PAH and Eisenmenger Syndro...
...1. Severe PAH...
...gnostic...
...ACHD with pulmonary vascular resistan...
...with septal or great artery shunts should un...
...diac catheterization to assess pulmonary vas...
...adults with septal or great artery shunts...
...P, chest x-ray, 6-minute walk test, and car...
...monary Hypertension and Eisenmenger Syndrome: Rou...
4.4.6.2. Eisenmenger Syn...
...When evaluating adults with presumed Eisenmeng...
...erapeutic...
...Bosentan is beneficial in symptomatic...
...mptomatic adults with Eisenmenger syndrome, b...
...a reasonable therapy to treat sympt...
...sonable to use PDE-5 inhibitors (e.g., s...
....4.7. Coronary Anom...
...tors That May Relate to the Clinical Importa...
....1. Anomalous Coronary Artery Ev...
...gnostic...
...angiography, using catheterization, CT, or CM...
...d physiological evaluation should be per...
...malous Aortic Origin of the Coronary Ar...
...alous Aortic Origin of Coronary Artery...
Therapeuti...
...rgery is recommended for AAOCA from the lef...
...rgery is reasonable for anomalous aortic origi...
...gery for AAOCA is reasonable in the setti...
Surgery or continued observation may be re...
...omalous Coronary Artery Arising From the PA...
Therapeuti...
...ecommended for anomalous left coronary artery f...
In a symptomatic adult with anomalous right...
...Surgery for anomalous right corona...
...4.8. Coronary Artery Fis...
...ronary artery fistula is an abnorm...