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 Pathoph...
...le 1. Physiological Variables as Used in ACHD AP...
Treatment
...atment...
...Classification (CHD Anatomy + Phys...
Table 3. Key Personnel and Services Recommended...
...2. Access to Care...
a) assuring smooth transitions for a...
...promoting awareness of the need for li...
...elivery of Care...
...atients with ACHD AP classification IB-D, IIA-D,...
...Cardiac surgery, catheter-based inter...
Table 4. Delivery of Care: Circums...
...4. Evaluation of Suspected and K...
...of ECGs in ACHD EvaluationHaving tro...
...Electrocardiogram...
...1. A standard 12-lead electrocardiogra...
...mbulatory electrocardiographic monit...
...2. Ionizing Radiation Principles 1....
...4.3. Echocardiograph...
.... Intraoperative TEE is recommended to gui...
...with ACHD should undergo transthoracic echocardi...
3.4.4. CMR Imag...
...tients with ACHD who have or who are at risk of...
...useful in the initial evaluation and...
...le 6. Circumstances Where CMR, CCT, TEE, and...
...rison of Imaging Modalities Useful in ACHD E...
...Cardiac Computed Tomography 1. CCT imaging...
...Cardiac Catheterization...
...atheterization (hemodynamic and/or a...
...s with a low or intermediate pretest probabil...
3.4.7. Exercise Test...
.... In patients with ACHD, cardiopulm...
...matic patients with ACHD, a 6-minute walk t...
...ansition Education
...1. Clinicians caring for patients...
...Exercise and Sports
...1. Clinicians should assess activity level...
...be useful to guide activity recommenda...
...Health and Neurodevelopmental Issue...
...nts with ACHD should be evaluated fo...
...or mental health evaluation and treatment is r...
3.8. Endocarditis PreventionPatients with AC...
...nt Syndromes 1. Genetic testing for 22q11...
...rlying Genetic Syndromes Commonly Associated...
...quired Cardiovascular Disease Patients with ACH...
3.11. Noncardiac Medical...
...1. Patients with ACHD at risk for hepati...
...12. Noncardiac Surger...
...zation before and close surveillance aft...
.... In patients with ACHD AP classification IB-D, I...
...ACHD Management Issues for Noncardiac...
...Reproduction, and Sexual Health...
....1. Pregnancy...
...Women with CHD should receive prepregnancy counsel...
...An individualized plan of care that addresses e...
...3. Women with CHD receiving chronic anticoagulat...
...ACHD AP classification IB-D, IIA-D, and IIIA...
...ration with an ACHD cardiologist to ensure accu...
...women of childbearing age with CHD...
7. Exercise testing can be useful for risk ass...
...en either parent has CHD, it is reasonab...
...2. Contraception...
...hildbearing potential with CHD should be...
...containing contraceptives are potential...
3.13.3. Infertility Treatment Menstrual cycle d...
...al Function Sexuality is an important element of...
...Heart Failure and Transplan...
...art Failure 1. Consultation with ACHD and H...
...2. Heart Transplant Because of t...
....14.3. Multiorgan Transplant Recognizing the...
....15. Palliative...
...1. Discussion of end-of-life issues and...
.... Cyanosis...
...ic Management Practices for Cyanotic CHDHavin...
...harmacological Therapy for ACHD Pati...
4. Specific Lesions
4. Specific Lesions
....1. Atrial Septal Defect...
...agnostic
...oximetry at rest and during exercise is...
.../or TEE are useful to evaluate pulmona...
...Echocardiographic imaging is recommended t...
...erapeutic...
...s with isolated secundum ASD causing im...
...h primum ASD, sinus venosus defect or coronary...
...tic adults with isolated secundum ASD...
...Surgical closure of a secundum ASD in adults is...
...Percutaneous or surgical closure may be cons...
...e should not be performed in adult...
...1. Secondum ASD...
...ASD: Routine Follow-Up and Testing Interv...
...2. Anomalous Pulmonary Venous...
...gnostic
...is recommended for evaluation of partial...
...iac catheterization can be useful in a...
Therapeutic
...ir is recommended for patients with partial anomal...
...artial anomalous pulmonary venous con...
...epair of a scimitar vein is recommended in ad...
...an be useful for right- or left-sided...
...ery can be useful for repair of a sci...
...Ventricular Septal Defect...
...rapeutic
...s with a VSD and evidence of left ventric...
...sure of perimembranous or supracristal VSD...
...ical closure of a VSD may be reasonable in adult...
...osure of a VSD may be considered in the...
...VSD closure should not be performed in adult...
...VSD: Routine Follow-Up and Testing IntervalsHaving...
...2. Hemodynamically Significant Ventricular...
....4. Atrioventricular Septal D...
Diagnos...
...catheterization can be useful in adult...
Therapeuti...
...r severe left atrioventricular valve regurgita...
...gery for primary repair of atrioventricular...
...on for discrete LVOT obstruction in adults wit...
...for primary repair of atrioventricular...
...Surgery for primary repair of atrioventricula...
...ble 13. AVSD: Routine Follow-Up and Testing Interv...
...Patent Ductus Arteriosus...
...gnostic...
...of oxygen saturation should be performed in feet...
...to the standard diagnostic tools,...
573
...n adults is recommended if left atrial or LV enlar...
...DA closure in adults may be consider...
...closure should not be performed in adults wi...
...Routine Follow-Up and Testing IntervalsHav...
...ided Obstructive Lesions...
...2.1. Cor Triatri...
...iagnosti...
...Adults presenting with cor triatriatum...
...lts with prior repair of cor triatr...
...eutic Surgical repair is indicated for adults...
....2.2. Congenital Mitral Stenosis A...
...Congenital Mitral Stenosis: Routin...
...2.3. Subaortic Stenosi...
...ic Stress testing for adults with LVOT obs...
Therapeu...
...urgical intervention is recommended for adults...
...Surgical intervention is recommended f...
...event the progression of AR, surgical interventio...
...Subaortic Stenosis: Routine Follow-Up and Te...
...nital Valvular Aortic Stenosis...
Diagnosti...
...bicuspid aortic valve should be evaluated...
...s reasonable to screen first-degree...
...peutic In adults with bicuspid ao...
...le 17. Congenital Aortic Stenosis: Routine Foll...
4.2.4.1. Turner Syndrome
...nostic Women with Turner syndrome...
...ophylactic replacement of the aortic r...
...Aortopathies Several CHD subtypes a...
...ravalvular Aortic Stenosis...
...gnostic...
...Aortic imaging using TTE, TEE, CMR, or CTA is re...
...maging is recommended in patients with Williams...
Therapeuti...
...Surgical repair is recommended for adults wi...
...onary artery revascularization is recommended i...
...ravalvular Aortic Stenosis: Routine Follow-...
....2.6. Coarctation of the A...
...agnostic
...Initial and follow-up aortic imagi...
...Resting blood pressure should be measure...
...y blood pressure monitoring in adults wit...
...or intracranial aneurysms by magnetic resonance a...
...rcise testing to evaluate for exercise-...
Therapeuti...
...Surgical repair or catheter-based stenting is rec...
...ecommended for treatment of hypertension in pati...
...angioplasty for adults with native and...
...CoA: Routine Follow-Up and Testing IntervalsH...
.... Right-Sided Lesion...
...lvular Pulmonary Stenosis...
...agnostic...
...s with moderate or severe valvular pulmonary...
...adults with moderate or severe valvular pulmonar...
...tomatic adults with severe valvular pulm...
...Severity of RVOT ObstructionHaving tro...
...le 21. Valvular PS: Routine Follow-Up and Testing...
...solated PR After Repair of PS...
...3.1.1. Isolated PR After Repair of Pulmonar...
...gnostic...
...In symptomatic patients with moderate or greate...
...matic patients with residual PR resulting from tre...
...ymptomatic patients with moderate or greater PR re...
.... Branch and Peripheral Pulmonary St...
Diagnostic For adults with peripheral or...
...apeutic In adults with peripheral or b...
...h and Peripheral PS: Routine Follow-Up...
...ouble-Chambered Right Ventricle...
Surgical repair for adults with double-chamb...
...rgical repair for adults with double-chambered r...
...3. Double-Chambered Right Ventricle...
...4. Ebstein Anomaly...
Diagnost...
...h Ebstein anomaly, CMR can be useful...
...with Ebstein anomaly, TEE can be useful...
...hysiological study with or without catheter ablat...
...s with Ebstein anomaly, electrophysiological...
...herapeuti...
...cal repair or reoperation for adults wi...
...blation is recommended for adults with Ebstein an...
...Surgical repair or reoperation for...
...superior cavopulmonary (Glenn) anasto...
...Ebstein Anomaly: Routine and Follow-Up and Tes...
...3.5. Tetralogy of...
...iagnosti...
...is useful to quantify ventricular...
...onary artery compression testing is indi...
...med ventricular stimulation can be u...
...In patients with repaired TOF, cardiac...
...valve replacement (surgical or percutan...
...rapeutic...
...Pulmonary valve replacement (surgica...
...ention ICD therapy is reasonable in adults wi...
...cal pulmonary valve replacement may be reaso...
...ulmonary valve replacement, in addition to arr...
...ary Valve Replacement in Patients With TOF...
...able 25. TOF: Routine Follow-Up and Testing In...
...ght Ventricle-to-Pulmonary Artery Conduit...
Diagnos...
Coronary artery compression testing...
...nts with stented right ventricle-to-PA conduits an...
...dults with right ventricle-to-PA condui...
...herapeuti...
...ight ventricle-to-PA conduit intervention is rea...
...Right ventricle-to-PA conduit intervention...
...e 26. Right Ventricle-to-PA Conduit: Routi...
...Complex Lesions...
...Transposition of the Great Arteries With Atrial...
...agnostic
...monitoring for bradycardia or sinus...
...with d-TGA with atrial switch repair sho...
...for a communication through the in...
...rapeutic GDMT with appropriate atte...
...27. d-TGA With Atrial Switch: Routine Foll...
...ansposition of the Great Arteries...
...iagnostic
...ne and serial imaging with either echocard...
...ry revascularization for adults with d-TGA wit...
...reasonable to perform anatomic eva...
...Physiological tests of myocardial perfusion...
...nable to determine the need for coronary...
...erapeutic
...easonable to determine indications...
...heter or surgical intervention for P...
...d-TGA With Arterial Switch: Routine Foll...
...ansposition of the Great Arteries With...
...Congenitally Corrected Transposition of...
...is reasonable in adults with CCTGA to deter...
...erapeutic...
...uspid valve replacement is recommended for s...
...d valve replacement is reasonable for asymp...
...rvention/replacement may be consid...
...29. CCTGA: Routine Follow-Up and Test...
4.4.2. Fontan Palliation of Single Ventricle...
...gnostic...
New presentation of an atrial tachyarrhythmia i...
...Adults after Fontan palliation should be...
...ac catheterization should be performed...
...New onset or worsening atrial tachyarrhythmias i...
...h Fontan palliation, it is reasona...
...ing of the liver (ultrasonography, CMR, CT) and l...
...after Fontan palliation, it is reasonable...
...iac catheterization can be useful to ev...
...or cardiac transplantation is reas...
...It may be reasonable to perform catheteriza...
Therapeuti...
...lation with a vitamin K antagonist is...
...heter ablation can be useful in adult...
...Fontan revision surgery, including arrhy...
...nary vasoactive medications can be beneficia...
...therapy or anticoagulation with a vitami...
...Reoperation or intervention for struc...
...ontan Palliation: Routine Follow-Up and Te...
...astic Left Heart Syndrome/Norwood R...
...od repair is the first of 3 steps in palliation f...
...4.4. Truncus Arteri...
...uncus arteriosus in the adult has almost invaria...
4.4.5. Double Outlet Right V...
...Double outlet right ventricle is an...
4.4.6. Severe PAH and Eisenmenger Synd...
...6.1. Severe PAH...
...gnostic...
...tients with ACHD with pulmonary vascular...
...ts with septal or great artery shunts...
...Cardiac catheterization to assess pulmon...
...In adults with septal or great artery...
...hest x-ray, 6-minute walk test, and cardiac...
...1. Pulmonary Hypertension and Eisenmenger Syndro...
.... Eisenmenger Syndrome...
...ic When evaluating adults with presum...
Therapeutic
...Bosentan is beneficial in symptomatic...
...In symptomatic adults with Eisenmenger syndrome,...
...a reasonable therapy to treat symptomatic adul...
...able to use PDE-5 inhibitors (e.g....
....7. Coronary Anomalies...
...Factors That May Relate to the Clinical...
....1. Anomalous Coronary Artery Evaluation
...iagnost...
...giography, using catheterization, C...
...Anatomic and physiological evaluati...
...e 5. Anomalous Aortic Origin of the Coronary...
...lous Aortic Origin of Coronary Artery...
Therapeu...
...s recommended for AAOCA from the left si...
...reasonable for anomalous aortic origin of th...
...ery for AAOCA is reasonable in the...
...Surgery or continued observation may...
...lous Coronary Artery Arising From the PA...
...herapeuti...
...is recommended for anomalous left coronary art...
...tomatic adult with anomalous right coronary art...
...y for anomalous right coronary artery from the...
...8. Coronary Artery Fistu...
...tery fistula is an abnormal communication between...