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
...kground and Pathophysiology...
...siological Variables as Used in ACHD AP...
Treatment
...eatment...
...e 2. ACHD AP Classification (CHD Anatomy...
Table 3. Key Personnel and Services Recommende...
.... Access to Care...
...ssuring smooth transitions for adolescents and y...
...omoting awareness of the need for lif...
...Delivery of Care...
...1. Patients with ACHD AP classification IB-D,...
...surgery, catheter-based interventional ca...
...Delivery of Care: Circumstances Where A...
...aluation of Suspected and Kno...
...le 5. Use of ECGs in ACHD Evaluation...
...4.1. Electrocardi...
...ndard 12-lead electrocardiogram (ECG) is...
...Ambulatory electrocardiographic monitoring sh...
...Radiation Principles 1. Strategies...
...Echocardiography...
...ative TEE is recommended to guide...
2. Patients with ACHD should under...
...CMR Imaging...
...patients with ACHD who have or who are at ris...
...can be useful in the initial evaluation and seri...
Table 6. Circumstances Where CMR, CCT,...
...rison of Imaging Modalities Useful...
...5. Cardiac Computed Tomography 1. CCT imagi...
3.4.6. Cardiac Cathet...
...terization (hemodynamic and/or angiograp...
.... In patients with a low or intermediat...
3.4.7. Exercise Te...
...1. In patients with ACHD, cardiopulmonary exerci...
...In symptomatic patients with ACHD, a 6-...
3.5. Transition E...
...inicians caring for patients with...
.... Exercise and Spo...
...Clinicians should assess activity levels at...
...2, CPET can be useful to guide activit...
....7. Mental Health and Neurodevelopment...
...1. Patients with ACHD should be eva...
...for mental health evaluation and treatment...
...8. Endocarditis PreventionPatients with ACH...
...t Syndromes 1. Genetic testing f...
...lying Genetic Syndromes Commonly Associated With...
...Acquired Cardiovascular Disease...
...ncardiac Medical Issues...
...tients with ACHD at risk for hepatitis C should...
...oncardiac Surgery...
...1. Optimization before and close s...
...ts with ACHD AP classification IB-D, II...
...CHD Management Issues for Noncardiac...
...regnancy, Reproduction, and Sexual Health...
...1. Pregnancy...
...Women with CHD should receive prepregnancy co...
...dividualized plan of care that addres...
...th CHD receiving chronic anticoagulation should b...
...with ACHD AP classification IB-D,...
...llaboration with an ACHD cardiologist to...
...en of childbearing age with CHD should be cou...
...se testing can be useful for risk assessment...
...arent has CHD, it is reasonable to...
....13.2. Contracept...
...1. Women of childbearing potenti...
...Estrogen-containing contraceptives...
...13.3. Infertility Treatment Menstrua...
3.13.4. Sexual Function Sexuality is...
...Failure and Transplant...
...Failure 1. Consultation with ACHD and HF spec...
...art Transplant Because of the prevalence of...
...4.3. Multiorgan Transplant Recognizing the vu...
...15. Palliative Car...
1. Discussion of end-of-life issues and...
...16. Cyanosi...
...ecific Management Practices for Cyanoti...
...rmacological Therapy for ACHD Patient...
4. Specific Lesions
...ecific Lesions...
....1.1. Atrial Septal Defec...
...iagnostic...
Pulse oximetry at rest and during exercise is r...
...and/or TEE are useful to evaluate pulmona...
...Echocardiographic imaging is recommended to guide...
Therapeut...
...ith isolated secundum ASD causing impaire...
...ts with primum ASD, sinus venosus d...
In asymptomatic adults with isolated...
...urgical closure of a secundum ASD i...
Percutaneous or surgical closure may be consid...
...ASD closure should not be performed in adults w...
...gure 1. Secondum...
...e 11. ASD: Routine Follow-Up and Testing Interval...
....1.2. Anomalous Pulmonary Venous Con...
...iagnostic
...CMR or CTA is recommended for evaluati...
...terization can be useful in adults with par...
...rapeutic...
...l repair is recommended for patients with part...
...partial anomalous pulmonary venous connection i...
...scimitar vein is recommended in adults when func...
...Surgery can be useful for right- or left-sided...
...be useful for repair of a scimitar vein i...
....1.3. Ventricular Septal Defe...
...rapeutic...
...Adults with a VSD and evidence of left ventr...
...ure of perimembranous or supracris...
...rgical closure of a VSD may be reasonable in...
...a VSD may be considered in the pre...
...th PA systolic pressure greater than two th...
Table 12. VSD: Routine Follow-Up and Test...
...Hemodynamically Significant Ventric...
.... Atrioventricular Septal D...
Diagnost...
...ardiac catheterization can be usef...
...rapeutic...
...Surgery for severe left atrioventricular val...
...ry for primary repair of atrioventricular septa...
Operation for discrete LVOT obs...
...primary repair of atrioventricular septal defect o...
...Surgery for primary repair of atr...
...3. AVSD: Routine Follow-Up and Testing Intervals...
....1.5. Patent Ductus Ar...
...iagnosti...
...nt of oxygen saturation should be performed in fee...
...o the standard diagnostic tools, cardi...
573
...n adults is recommended if left at...
...PDA closure in adults may be considered in th...
...e should not be performed in adults with a net rig...
...DA: Routine Follow-Up and Testing IntervalsHa...
...-Sided Obstructive Lesions
...Cor Triatriatum...
...gnostic...
...ting with cor triatriatum sinister shoul...
...adults with prior repair of cor tri...
...Surgical repair is indicated for adu...
...Congenital Mitral Stenosis Adul...
...15. Congenital Mitral Stenosis: Ro...
....2.3. Subaortic Stenosis
...ess testing for adults with LVOT obs...
...erapeutic...
...rvention is recommended for adults with subAS, a...
Surgical intervention is recommend...
...To prevent the progression of AR, surgical in...
...ortic Stenosis: Routine Follow-Up and Testing Int...
4.2.4. Congenital Valvular Aortic Steno...
...agnostic...
...bicuspid aortic valve should be evaluate...
...nable to screen first-degree relat...
...ic In adults with bicuspid aortic valve sten...
...17. Congenital Aortic Stenosis: Routine F...
....2.4.1. Turner Sy...
...iagnostic Women with Turner syndrome should be...
...rapeutic Prophylactic replacement of the aort...
4.2.4.2. Aortopathies Several CHD sub...
...Supravalvular Aortic Stenosis...
...iagnost...
...tic imaging using TTE, TEE, CMR, o...
...oronary imaging is recommended in patien...
...erapeutic
Surgical repair is recommended fo...
...ronary artery revascularization is recomme...
Table 18. Supravalvular Aortic Steno...
...oarctation of the Aorta...
Diagnostic
...Initial and follow-up aortic imagi...
...esting blood pressure should be measured in...
...blood pressure monitoring in adults with coarctat...
...Screening for intracranial aneurysms by...
...Exercise testing to evaluate for exercise-induce...
...erapeutic...
...air or catheter-based stenting is reco...
...is recommended for treatment of hypertension...
...ioplasty for adults with native and recurrent coa...
...19. CoA: Routine Follow-Up and Testing...
....3. Right-Sided Lesions...
...Valvular Pulmonary Stenosis...
...agnostic...
...s with moderate or severe valvular pulmonary sten...
...ts with moderate or severe valvular pulmo...
...tomatic adults with severe valvula...
...verity of RVOT ObstructionHaving trouble viewing...
...alvular PS: Routine Follow-Up and Tes...
...3. Isolated PR After Repair of PS
...ed PR After Repair of Pulmonary Stenosis...
...gnostic...
...tomatic patients with moderate or great...
...For asymptomatic patients with residual PR...
...asymptomatic patients with moderate...
...2. Branch and Peripheral Pulmonary Steno...
...stic For adults with peripheral or branch PS,...
...utic In adults with peripheral or branch PA s...
...Branch and Peripheral PS: Routine Follo...
....3.3. Double-Chambered Right Ven...
...urgical repair for adults with double-c...
...air for adults with double-chambered right ventri...
...-Chambered Right Ventricle: Routin...
....3.4. Ebstein Anoma...
...iagnostic
...In adults with Ebstein anomaly, CMR ca...
...adults with Ebstein anomaly, TEE c...
Electrophysiological study with or without...
...s with Ebstein anomaly, electrophysi...
...herapeut...
...epair or reoperation for adults with Ebstein anom...
...Catheter ablation is recommended for adults wit...
...Surgical repair or reoperation for adu...
...nal superior cavopulmonary (Glenn) a...
...24. Ebstein Anomaly: Routine and...
...3.5. Tetralogy of Fallo...
...iagnost...
...ul to quantify ventricular size and fu...
...onary artery compression testing is indicat...
...ventricular stimulation can be us...
...nts with repaired TOF, cardiac cath...
...y valve replacement (surgical or percutane...
Therapeut...
...lve replacement (surgical or percutan...
...rimary prevention ICD therapy is re...
...pulmonary valve replacement may be reasonable f...
...nary valve replacement, in addition...
...e 4. Pulmonary Valve Replacement in Patients W...
...: Routine Follow-Up and Testing IntervalsHaving t...
...ntricle-to-Pulmonary Artery Conduit...
...iagnostic
...ry compression testing with simultaneous corona...
...patients with stented right ventricle-to-PA cond...
...lts with right ventricle-to-PA conduit and arrhyth...
...erapeuti...
...entricle-to-PA conduit intervention...
...entricle-to-PA conduit intervention m...
...Right Ventricle-to-PA Conduit: Rout...
4.4. Complex Les...
....4.1.1. Transposition of the Great Arteries With A...
...agnostic...
...onitoring for bradycardia or sinus node dysfunc...
...Adults with d-TGA with atrial switch repair...
...for a communication through the interatrial baf...
...herapeutic GDMT with appropriate attent...
...d-TGA With Atrial Switch: Routine Follow-Up and T...
....2. Transposition of the Great Arteries Wi...
...gnostic...
...aseline and serial imaging with eithe...
...y revascularization for adults with...
...easonable to perform anatomic evaluation of co...
...ogical tests of myocardial perfusion for adul...
...asonable to determine the need for...
...rapeutic...
...reasonable to determine indications fo...
...r surgical intervention for PS is r...
...8. d-TGA With Arterial Switch: Routine Follow-...
....3. Transposition of the Great Arteries...
...Congenitally Corrected Transposit...
...CMR is reasonable in adults with CC...
Therapeuti...
...valve replacement is recommended for sy...
...Tricuspid valve replacement is reasonab...
...t intervention/replacement may be co...
...29. CCTGA: Routine Follow-Up and...
...Fontan Palliation of Single Ventricl...
...agnostic...
...w presentation of an atrial tachyarrhyth...
...s after Fontan palliation should be...
...rdiac catheterization should be performed in adult...
New onset or worsening atrial tachyarrhy...
...lts with Fontan palliation, it is re...
...ging of the liver (ultrasonography, CMR, CT) an...
...dults after Fontan palliation, it is reas...
...catheterization can be useful to evaluate...
...Evaluation for cardiac transplantati...
...ay be reasonable to perform catheterizat...
...herapeuti...
...icoagulation with a vitamin K antagoni...
...heter ablation can be useful in adults after F...
...evision surgery, including arrhythmia s...
...soactive medications can be beneficial to impro...
...atelet therapy or anticoagulation with a vitam...
...ation or intervention for structural/anatomic a...
.... Fontan Palliation: Routine Follow-Up and...
...oplastic Left Heart Syndrome/Norwood Rep...
...orwood repair is the first of 3 steps in pall...
.... Truncus Arteriosus
...iosus in the adult has almost invariably been re...
...le Outlet Right Ventricle...
...outlet right ventricle is an anatomic descri...
...e PAH and Eisenmenger Syndrome...
....4.6.1. Severe...
...iagnost...
...th ACHD with pulmonary vascular resi...
...th septal or great artery shunts s...
...eterization to assess pulmonary vascular hemod...
...In adults with septal or great artery shunt...
...est x-ray, 6-minute walk test, and cardiac cathete...
...nary Hypertension and Eisenmenger Synd...
...6.2. Eisenmenger Synd...
...en evaluating adults with presumed Eisen...
...herapeuti...
...an is beneficial in symptomatic adults with...
...In symptomatic adults with Eisenmenge...
...Bosentan is a reasonable therapy to trea...
...It is reasonable to use PDE-5 inh...
.... Coronary Anomalies...
...le 32. Factors That May Relate to the Cli...
...ous Coronary Artery Evaluation...
...gnostic...
...ngiography, using catheterization, CT,...
...c and physiological evaluation should be perf...
.... Anomalous Aortic Origin of the Coronary Artery...
...nomalous Aortic Origin of Coronary Artery...
...rapeutic...
...recommended for AAOCA from the left si...
...ery is reasonable for anomalous aortic origin...
...Surgery for AAOCA is reasonable in the...
...urgery or continued observation may be reasonable...
...us Coronary Artery Arising From the...
...erapeuti...
...s recommended for anomalous left coronary artery f...
...tomatic adult with anomalous right corona...
...for anomalous right coronary artery from the...
...4.8. Coronary Artery Fistul...
...Coronary artery fistula is an abnormal...