Management of Adults with Congenital Heart Disease

Publication Date: August 16, 2018

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

...und and Pathophysiology...

...logical Variables as Used in ACHD AP Classificati...


Treatment

...reatment

...Classification (CHD Anatomy + Physiologic...


...3. Key Personnel and Services Recomme...


....2. Access to Care...

...ing smooth transitions for adolescents and...

...ng awareness of the need for lifelong spec...


...Delivery of Care...

...1. Patients with ACHD AP classification IB...

2. Cardiac surgery, catheter-based interventio...


...ery of Care: Circumstances Where ACHD...


...uation of Suspected and Known CHD...

...se of ECGs in ACHD EvaluationHaving troubl...

....1. Electrocardio...

...standard 12-lead electrocardiogram (ECG)...

...2. Ambulatory electrocardiographic m...

...2. Ionizing Radiation Principles 1. Str...

...Echocardiography...

...Intraoperative TEE is recommended to guide s...

...s with ACHD should undergo transthorac...

3.4.4. CMR Imag...

...ents with ACHD who have or who are a...

...be useful in the initial evaluation a...

Table 6. Circumstances Where CMR,...

...parison of Imaging Modalities Useful in ACHD Ev...

...Computed Tomography 1. CCT imaging can be usefu...

.... Cardiac Catheterizati...

...heterization (hemodynamic and/or angiograp...

...In patients with a low or intermediate pretest...

...4.7. Exercise Testi...

...ts with ACHD, cardiopulmonary exercise testing (C...

...2. In symptomatic patients with ACHD, a 6-min...


...ransition Education...

...1. Clinicians caring for patients wit...


...ercise and Sports...

...1. Clinicians should assess activity...

...n be useful to guide activity recommend...


...l Health and Neurodevelopmental Issues...

.... Patients with ACHD should be evaluated for...

...Referral for mental health evaluati...


...is PreventionPatients with ACHD ha...


...9. Concomitant Syndromes 1. Genetic testing...

...rlying Genetic Syndromes Commonly Associate...


...Cardiovascular Disease Patients with ACHD can a...


...oncardiac Medical Issues...

...1. Patients with ACHD at risk for...


...2. Noncardiac Sur...

...1. Optimization before and close surveillance...

...2. In patients with ACHD AP classification IB-D,...

...le 9. ACHD Management Issues for Nonc...


...Pregnancy, Reproduction, and Sexual Heal...

3.13.1. Pregna...

...Women with CHD should receive prepreg...

...idualized plan of care that address...

...with CHD receiving chronic anticoagulation sh...

...4. Women with ACHD AP classification I...

.... In collaboration with an ACHD cardiologist...

...n and women of childbearing age with CHD shoul...

...7. Exercise testing can be useful...

...arent has CHD, it is reasonable to perform...

....13.2. Contraception

...omen of childbearing potential with CHD should b...

...ogen-containing contraceptives are potentially...

...ertility Treatment Menstrual cycle disord...

...13.4. Sexual Function Sexuality is an...


...rt Failure and Transplant

...lure 1. Consultation with ACHD and...

...art Transplant Because of the prevalence of HF a...

...ultiorgan Transplant Recognizing the vulne...


....15. Palliative Care...

...scussion of end-of-life issues and advance d...


....16. Cyanos...

...le 10. Specific Management Practices...


...logical Therapy for ACHD Patients w...


4. Specific Lesions

...ecific Lesions...

.... Atrial Septal Defe...

...iagnostic

Pulse oximetry at rest and durin...

..., CCT, and/or TEE are useful to evalua...

...diographic imaging is recommended to guide per...

...erapeuti...

...with isolated secundum ASD causing...

...dults with primum ASD, sinus venosus defe...

...omatic adults with isolated secundum A...

...gical closure of a secundum ASD in adul...

...utaneous or surgical closure may be considered for...

...ASD closure should not be performed...

...e 1. Secondum ASD...

Table 11. ASD: Routine Follow-Up and Test...


...us Pulmonary Venous Connections...

Diagnos...

...TA is recommended for evaluation of pa...

...ac catheterization can be useful in adults...

...herapeutic...

...al repair is recommended for patients w...

...pair of partial anomalous pulmonary v...

...Repair of a scimitar vein is recomm...

...an be useful for right- or left-sided par...

...e useful for repair of a scimitar vein i...


....3. Ventricular Septal Defe...

...erapeutic

Adults with a VSD and evidence of le...

...closure of perimembranous or supracristal VSD...

...closure of a VSD may be reasonable in ad...

...Closure of a VSD may be considered i...

...VSD closure should not be performed in adul...

...VSD: Routine Follow-Up and Testing IntervalsHav...

...Hemodynamically Significant Ventricular Level...


...ventricular Septal Defect...

...agnosti...

Cardiac catheterization can be u...

...rapeutic...

...Surgery for severe left atrioventric...

...Surgery for primary repair of atr...

Operation for discrete LVOT obstru...

...ry for primary repair of atrioventricular septal d...

...rgery for primary repair of atrioventri...

Table 13. AVSD: Routine Follow-Up and Testing Int...


...Patent Ductus Arteriosus

...gnostic...

...Measurement of oxygen saturation should be...

...to the standard diagnostic tools, cardiac...

...73...

...in adults is recommended if left atria...

...PDA closure in adults may be considered in the...

...ure should not be performed in adults...

...14. PDA: Routine Follow-Up and Testin...


....2. Left-Sided Obstructive Le...

4.2.1. Cor Triatriatu...

...iagnostic...

...s presenting with cor triatriatum siniste...

...th prior repair of cor triatriatum...

...tic Surgical repair is indicated for adults with...

....2.2. Congenital Mitral Stenosis Adults with c...

...e 15. Congenital Mitral Stenosis: Routine Follow-U...

...ubaortic Stenosis...

...Stress testing for adults with LVOT obstruc...

...herapeutic...

Surgical intervention is recomme...

...ervention is recommended for adult...

...To prevent the progression of AR, sur...

...tic Stenosis: Routine Follow-Up and Tes...

...ital Valvular Aortic Stenosi...

...gnostic...

...bicuspid aortic valve should be ev...

...is reasonable to screen first-degree relatives o...

...tic In adults with bicuspid aortic valve sten...

Table 17. Congenital Aortic Stenosis: Routi...

....2.4.1. Turner Synd...

...Women with Turner syndrome should be evalu...

...c Prophylactic replacement of the aor...

...Aortopathies Several CHD subtype...

4.2.5. Supravalvular Aortic St...

Diagnos...

...g using TTE, TEE, CMR, or CTA is recommended in...

...ry imaging is recommended in patients...

...herapeuti...

Surgical repair is recommended for adults w...

...artery revascularization is recomme...

...Supravalvular Aortic Stenosis: Rou...

...Coarctation of the Aorta...

...agnosti...

...Initial and follow-up aortic imaging using CMR...

...Resting blood pressure should be measured...

...ory blood pressure monitoring in adults with...

...ening for intracranial aneurysms by magne...

...Exercise testing to evaluate for exerc...

...erapeutic

...epair or catheter-based stenting is recommend...

GDMT is recommended for treatment of hy...

...loon angioplasty for adults with native and...

...Routine Follow-Up and Testing Interval...


4.3. Right-Sided Les...

...1. Valvular Pulmonary Stenosis...

...iagnost...

In adults with moderate or severe valvular pulm...

...adults with moderate or severe valvula...

...In asymptomatic adults with severe valvu...

...Severity of RVOT ObstructionHaving troub...

...lvular PS: Routine Follow-Up and Tes...

...ure 3. Isolated PR After Repair of PS...

...ed PR After Repair of Pulmonary Stenosis...

...iagnosti...

...In symptomatic patients with moderate...

...omatic patients with residual PR r...

...In asymptomatic patients with moderate or grea...

....3.2. Branch and Peripheral Pulmonary S...

...agnostic For adults with peripheral or b...

...adults with peripheral or branch PA stenosis, PA...

...Branch and Peripheral PS: Routine F...

...ble-Chambered Right Ventri...

...epair for adults with double-chambered right ve...

...repair for adults with double-chambered r...

.... Double-Chambered Right Ventricle: Routine Foll...

...4. Ebstein Anoma...

...iagnosti...

...ults with Ebstein anomaly, CMR can be useful to de...

...ults with Ebstein anomaly, TEE can be us...

...rophysiological study with or with...

...dults with Ebstein anomaly, electrophysio...

...herapeutic...

...Surgical repair or reoperation for a...

...lation is recommended for adults with Ebste...

...Surgical repair or reoperation for adult...

...rectional superior cavopulmonary (Glenn) an...

...ble 24. Ebstein Anomaly: Routine and Follow-Up...

....3.5. Tetralogy of Fallo...

...agnosti...

...is useful to quantify ventricular size...

...artery compression testing is indicated before...

Programmed ventricular stimulation can be u...

...ents with repaired TOF, cardiac cathet...

...ry valve replacement (surgical or percuta...

...herapeutic

...lmonary valve replacement (surgical...

...ntion ICD therapy is reasonable in adults wit...

...cal pulmonary valve replacement may be rea...

...ve replacement, in addition to arrhythmia man...

...Pulmonary Valve Replacement in Patients With TO...

...25. TOF: Routine Follow-Up and Tes...

...3.6. Right Ventricle-to-Pulmonary Artery C...

...gnostic...

...artery compression testing with simultaneous co...

...ts with stented right ventricle-to-PA...

...In adults with right ventricle-to-PA conduit...

Therapeutic

...ventricle-to-PA conduit interventi...

...ricle-to-PA conduit intervention m...

...t Ventricle-to-PA Conduit: Routine F...


....4. Complex Lesion...

...1.1. Transposition of the Great Arteries With Atri...

...iagnostic...

...ulatory monitoring for bradycardia...

...th d-TGA with atrial switch repair...

...essment for a communication through the intera...

...GDMT with appropriate attention to the n...

...A With Atrial Switch: Routine Follow-Up and...

...ransposition of the Great Arteries With A...

...iagnost...

...serial imaging with either echocardiog...

...nary revascularization for adults wit...

...t is reasonable to perform anatomi...

...Physiological tests of myocardial pe...

...GDMT is reasonable to determine the need fo...

...erapeutic

...T is reasonable to determine indications...

...Catheter or surgical intervention for...

...e 28. d-TGA With Arterial Switch: Routine Foll...

...3. Transposition of the Great Arteries With R...

....1.4. Congenitally Corrected Transposition...

...ic CMR is reasonable in adults with C...

Therapeutic

...valve replacement is recommended for symp...

Tricuspid valve replacement is reasonable for a...

...it intervention/replacement may be considered f...

...CCTGA: Routine Follow-Up and Testing Interval...

...n Palliation of Single Ventricle Physiology (Inc...

...gnostic...

...esentation of an atrial tachyarrhythmia...

...Adults after Fontan palliation should...

Cardiac catheterization should be...

...or worsening atrial tachyarrhythmias in adult...

...ith Fontan palliation, it is reasonable...

...of the liver (ultrasonography, CMR, CT) a...

...ts after Fontan palliation, it is reason...

...theterization can be useful to evaluate a sympt...

...ation for cardiac transplantation is reasonab...

...e reasonable to perform catheteriza...

...erapeutic...

...ulation with a vitamin K antagonist i...

...Catheter ablation can be useful in ad...

...n revision surgery, including arrhythmia surgery...

...ry vasoactive medications can be ben...

...platelet therapy or anticoagulation with...

...ion or intervention for structural/anatomic abnorm...

...Palliation: Routine Follow-Up and T...

...stic Left Heart Syndrome/Norwood Repa...

...The Norwood repair is the first of 3 steps in...

...4. Truncus Arteri...

...Truncus arteriosus in the adult has almo...

....4.5. Double Outlet Right Ventri...

...Double outlet right ventricle is an...

.... Severe PAH and Eisenmenger Syndr...

....4.6.1. Sever...

...gnostic...

...ts with ACHD with pulmonary vascular resistance 2...

...s with septal or great artery shunts...

...diac catheterization to assess pulmonary va...

...th septal or great artery shunts, ca...

...ray, 6-minute walk test, and cardiac catheter...

...Pulmonary Hypertension and Eisenmenger Syndro...

...Eisenmenger Syndrome...

...gnostic When evaluating adults with...

Therapeutic

...eneficial in symptomatic adults with Eisen...

...omatic adults with Eisenmenger syndrom...

...reasonable therapy to treat sympt...

...It is reasonable to use PDE-5 inhibitors (e.g.,...

...oronary Anomalies...

...tors That May Relate to the Clinical Importan...

...nomalous Coronary Artery Evaluation...

...gnostic

...ography, using catheterization, CT,...

...and physiological evaluation should be per...

...ure 5. Anomalous Aortic Origin of t...

...4.7.2. Anomalous Aortic Origin of Coronary...

...herapeuti...

...commended for AAOCA from the left...

...asonable for anomalous aortic origin of...

...AAOCA is reasonable in the setting of ventricular...

...or continued observation may be reas...

...3. Anomalous Coronary Artery Arising From t...

...erapeuti...

...gery is recommended for anomalous left coron...

...symptomatic adult with anomalous right coronar...

...anomalous right coronary artery from the PA is...

.... Coronary Artery Fistula

...Coronary artery fistula is an abnorma...