Management of Adults With Congenital Heart Disease

Publication Date: August 16, 2018

Treatment

3.2. Access to Care

a) assuring smooth transitions for adolescents and young adults from pediatric to adult providers; and
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b) promoting awareness of the need for lifelong specialized care through outreach and educational programs.
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3.3. Delivery of Care

1. Patients with ACHD AP classification IB-D, IIA-D, and IIIA-D should be managed in collaboration with an ACHD cardiologist.
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2. Cardiac surgery, catheter-based interventional cardiac procedures, and electrophysiological procedures involving congenital heart lesions in patients with ACHD should be performed by operators with expertise in CHD procedures and in collaboration with an ACHD cardiologist.
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3.4. Evaluation of Suspected and Known CHD

3.4.1. Electrocardiogram

1. A standard 12-lead electrocardiogram (ECG) is recommended in adults with CHD with serial assessment depending on the specific ACHD AP classification or when symptoms develop or worsen.
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2. Ambulatory electrocardiographic monitoring should be performed in patients with CHD who are at risk of tachyarrhythmia, bradyarrhythmia or heart block, or when symptoms possibly of arrhythmic origin develop.
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3.4.2. Ionizing Radiation Principles

1. Strategies to limit and monitor radiation exposure are recommended during imaging of patients with ACHD, with studies not involving ionizing radiation chosen whenever appropriate.
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3.4.3. Echocardiography

1. Intraoperative TEE is recommended to guide surgical repair of CHD in adults.
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2. Patients with ACHD should undergo transthoracic echocardiography (TTE) for initial assessment, with timing of serial assessment based on anatomic and physiological severity and clinical status.
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3.4.4. CMR Imaging

1. In patients with ACHD who have or who are at risk of developing RV enlargement and dysfunction, serial CMR is recommended for quantitative assessment of RV size and function.
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2. CMR can be useful in the initial evaluation and serial assessment of selected patients with CHD based on anatomic complexity and clinical status.
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3.4.5. Cardiac Computed Tomography

1. CCT imaging can be useful in patients with ACHD when information that cannot be obtained by other diagnostic modalities is important enough to justify the exposure to ionizing radiation.
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3.4.6. Cardiac Catheterization

1. Cardiac catheterization (hemodynamic and/or angiographic) in patients with ACHD AP classification II and III, or interventional cardiac catheterization in patients with ACHD AP classification I to III should be performed by, or in collaboration with, cardiologists with expertise in ACHD. (I, C-LD)
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2. In patients with a low or intermediate pretest probability of coronary artery disease (CAD), use of CT coronary angiography is reasonable to exclude significant obstructive CAD when cardiac catheterization has significant risk or because of patient preference.
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3.4.7. Exercise Testing

1. In patients with ACHD, cardiopulmonary exercise testing (CPET) can be useful for baseline functional assessment and serial testing.
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2. In symptomatic patients with ACHD, a 6-minute walk test can be useful to objectively assess symptom severity, functional capacity, and response to therapy.
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3.5. Transition Education

1. Clinicians caring for patients with CHD should deliver developmentally appropriate transition education to adolescent and young patients with CHD, and to their families/support network.
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3.6. Exercise and Sports

1. Clinicians should assess activity levels at regular intervals and counsel patients with ACHD about the types and intensity of exercise appropriate to their clinical status.
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2, CPET can be useful to guide activity recommendations for patients with ACHD.
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3.7. Mental Health and Neurodevelopmental Issues

1. Patients with ACHD should be evaluated for depression and anxiety.
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2. Referral for mental health evaluation and treatment is reasonable in patients with ACHD.
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3.9. Concomitant Syndromes

1. Genetic testing for 22q11 deletions is reasonable for patients with conotruncal cardiac defects.
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3.11. Noncardiac Medical Issues

1. Patients with ACHD at risk for hepatitis C should be screened and vaccinated for viral hepatitis and treated as appropriate.
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3.12. Noncardiac Surgery

1. Optimization before and close surveillance after invasive procedures, regardless of the complexity of the anatomic defect or type of procedure is beneficial for patients with ACHD.
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2. In patients with ACHD AP classification IB-D, IIA-D, and IIIA-D noncardiac surgical and interventional procedures should be performed in a hospital with or in consultation with experts in ACHD when possible.
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3.13. Pregnancy, Reproduction, and Sexual Health

3.13.1. Pregnancy

1. Women with CHD should receive prepregnancy counseling with input from an ACHD cardiologist to determine maternal cardiac, obstetrical and fetal risks, and potential long-term risks to the mother.
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2. An individualized plan of care that addresses expectations and contingencies should be developed for and with women with CHD who are pregnant or who may become pregnant and shared with the patient and all caregivers.
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3. Women with CHD receiving chronic anticoagulation should be counseled, ideally before conception, on the risks and benefits of specific anticoagulants during pregnancy.
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4. Women with ACHD AP classification IB-D, IIA-D, and IIIA-Da should be managed collaboratively during pregnancy by ACHD cardiologists, obstetricians, and anesthesiologists experienced in ACHD.
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5. In collaboration with an ACHD cardiologist to ensure accurate assessment of pregnancy risk, patients at high risk of maternal morbidity or mortality, including women with pulmonary arterial hypertension (PAH), Eisenmenger syndrome, severe systemic ventricular dysfunction, severe left-sided obstructive lesions, and/or ACHD AP classification ID, IID, IIIDa should be counseled against becoming pregnant or be given the option of terminating pregnancy.
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6. Men and women of childbearing age with CHD should be counseled on the risk of CHD recurrence in offspring.
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7. Exercise testing can be useful for risk assessment in women with ACHD AP classification IC-D, IIA-D, and IIIA-Da who are considering pregnancy.
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a See Tables 1 and 2 for details on the ACHD AP classification system.
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8. When either parent has CHD, it is reasonable to perform fetal echocardiography. (IIa, B-NR)
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3.13.2. Contraception

1. Women of childbearing potential with CHD should be counseled about the risks associated with pregnancy and appropriate contraceptive options.
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2. Estrogen-containing contraceptives are potentially harmful for women with CHD who are at high risk of thromboembolic events (e.g., cyanosis, Fontan physiology, mechanical valves, prior thrombotic events, PAH).
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3.14. Heart Failure and Transplant

3.14.1 Heart Failure

1. Consultation with ACHD and HF specialists is recommended for patients with ACHD and HF or severe ventricular dysfunction.
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3.14.2. Heart Transplant

  • Because of the prevalence of HF among patients with CHD, heart transplantation is increasingly being considered as a therapeutic option. Although specific criteria for timing of referral for transplantation are desirable, universal recommendations cannot be made based on current data.

3.14.3. Multiorgan Transplant

  • Recognizing the vulnerability of many organ systems in patients with CHD, multiorgan transplantation is often considered, although infrequently performed. Multiorgan transplantation requires a multidisciplinary and comprehensive approach with thoughtful planning and communication among practitioners.

3.15. Palliative Care

1. Discussion of end-of-life issues and advance directives can be beneficial for patients with ACHD or their surrogates.
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4. Specific Lesions

4.1.1. Atrial Septal Defect

Diagnostic

  1. Pulse oximetry at rest and during exercise is recommended for evaluation of adults with unrepaired or repaired ASD with residual shunt to determine the direction and magnitude of the shunt.
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  1. CMR, CCT, and/or TEE are useful to evaluate pulmonary venous connections in adults with ASD.
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  1. Echocardiographic imaging is recommended to guide percutaneous ASD closure.
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Therapeutic

  1. In adults with isolated secundum ASD causing impaired functional capacity, right atrial and/or RV enlargement, and net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., pulmonary–systemic blood flow ratio [Qp:Qs] ≥1.5:1) without cyanosis at rest or during exercise, transcatheter or surgical closure to reduce RV volume and improve exercise tolerance is recommended, provided that systolic PA pressure is less than 50% of systolic systemic pressure and pulmonary vascular resistance is less than one third of the systemic vascular resistance.
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  1. Adults with primum ASD, sinus venosus defect or coronary sinus defect causing impaired functional capacity, right atrial and/or RV enlargement and net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1) without cyanosis at rest or during exercise, should be surgically repaired unless precluded by comorbidities, provided that systolic PA pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one third of the systemic vascular resistance.
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  1. In asymptomatic adults with isolated secundum ASD, right atrial and RV enlargement, and net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs 1.5:1 or greater), without cyanosis at rest or during exercise, transcatheter or surgical closure is reasonable to reduce RV volume and/or improve functional capacity, provided that systolic PA pressure is less than 50% of systemic pressure and pulmonary vascular resistance is less than one third systemic resistance.
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  1. Surgical closure of a secundum ASD in adults is reasonable when a concomitant surgical procedure is being performed and there is a net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs 1.5:1 or greater) and right atrial and RV enlargement without cyanosis at rest or during exercise.
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  1. Percutaneous or surgical closure may be considered for adults with ASD when net left-to-right shunt (Qp:Qs) is 1.5:1 or greater, PA systolic pressure is 50% or more of systemic arterial systolic pressure, and/or pulmonary vascular resistance is greater than one third of the systemic resistance.
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  1. ASD closure should not be performed in adults with PA systolic pressure greater than two thirds systemic, pulmonary vascular resistance greater than two thirds systemic, and/or a net right-to-left shunt.
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4.1.2. Anomalous Pulmonary Venous Connections

Diagnostic

  1. CMR or CTA is recommended for evaluation of partial anomalous pulmonary venous connection.
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  1. Cardiac catheterization can be useful in adults with partial anomalous pulmonary venous connection to further define hemodynamics.
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Therapeutic

  1. Surgical repair is recommended for patients with partial anomalous pulmonary venous connection when functional capacity is impaired and RV enlargement is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemic pressure, and pulmonary vascular resistance is less than one third of systemic resistance.
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  1. Repair of partial anomalous pulmonary venous connection is recommended at the time of closure of a sinus venosus defect or ASD.
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  1. Repair of a scimitar vein is recommended in adults when functional capacity is impaired, evidence of RV volume overload is present, there is a net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1), PA systolic pressure is less than 50% systemic pressure and pulmonary vascular resistance is less than one third systemic.
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  1. Surgery can be useful for right- or left-sided partial anomalous pulmonary venous connection in asymptomatic adults with RV volume overload, net left-to-right shunt sufficiently large to cause physiological sequelae (e.g., Qp:Qs ≥1.5:1), pulmonary pressures less than 50% systemic and pulmonary vascular resistance less than one third systemic.
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  1. Surgery can be useful for repair of a scimitar vein in adults with evidence of RV volume overload, with Qp:Qs 1.5:1 or greater.
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4.1.3. Ventricular Septal Defect

Therapeutic

  1. Adults with a VSD and evidence of left ventricular volume overload and hemodynamically significant shunts (Qp:Qs ≥1.5:1) should undergo VSD closure, if PA systolic pressure is less than 50% systemic and pulmonary vascular resistance is less than one third systemic.
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  1. Surgical closure of perimembranous or supracristal VSD is reasonable in adults when there is worsening aortic regurgitation (AR) caused by VSD.
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  1. Surgical closure of a VSD may be reasonable in adults with a history of IE caused by VSD if not otherwise contraindicated.
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  1. Closure of a VSD may be considered in the presence of a net left-to-right shunt (Qp:Qs ≥1.5:1) when PA systolic pressure is 50% or more than systemic and/or pulmonary vascular resistance is greater than one third systemic.
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  1. VSD closure should not be performed in adults with severe PAH with PA systolic pressure greater than two thirds systemic, pulmonary vascular resistance greater than two thirds systemic and/or a net right-to-left shunt.
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4.1.4. Atrioventricular Septal Defect

Diagnostic

  1. Cardiac catheterization can be useful in adults with atrioventricular septal defect when pulmonary hypertension is suspected.
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Therapeutic

  1. Surgery for severe left atrioventricular valve regurgitation is recommended per GDMT indications for mitral regurgitation.
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  1. Surgery for primary repair of atrioventricular septal defect or closure of residual shunts in adults with repaired atrioventricular septal defect is recommended when there is a net left-to-right shunt (Qp:Qs ≥1.5:1), PA systolic pressure less than 50% systemic and pulmonary vascular resistance less than one third systemic.
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  1. Operation for discrete LVOT obstruction in adults with atrioventricular septal defect is reasonable with a maximum gradient of 50 mm Hg or greater, a lesser gradient if HF symptoms are present, or if concomitant moderate-to-severe mitral or AR are present.
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  1. Surgery for primary repair of atrioventricular septal defect or closure of residual shunts in adults with repaired atrioventricular septal defect may be considered in the presence of a net left-to-right shunt (Qp:Qs ≥1.5:1), if PA systolic pressure is 50% or more systemic, and/or pulmonary vascular resistance is greater than one third systemic.
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  1. Surgery for primary repair of atrioventricular septal defect or closure of residual shunts in adults with repaired atrioventricular septal defect should not be performed with PA systolic pressure greater than two thirds systemic, pulmonary vascular resistance greater than two thirds systemic, or a net right-to-left shunt.
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4.1.5. Patent Ductus Arteriosus

Diagnostic

  1. Measurement of oxygen saturation should be performed in feet and both hands in adults with a PDA to assess for the presence of right-to-left shunting.
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  1. In addition to the standard diagnostic tools, cardiac catheterization can be useful in patients with PDA and suspected pulmonary hypertension.
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  1. PDA closure in adults is recommended if left atrial or LV enlargement is present and attributable to PDA with net left-to-right shunt, PA systolic pressure less than 50% systemic and pulmonary vascular resistance less than one third systemic.
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  1. PDA closure in adults may be considered in the presence of a net left-to-right shunt if PA systolic pressure is 50% or greater systemic, and/or pulmonary vascular resistance is greater than one third systemic.
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  1. PDA closure should not be performed in adults with a net right-to-left shunt and PA systolic pressure greater than two thirds systemic or pulmonary vascular resistance greater than two thirds systemic.
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4.2. Left-Sided Obstructive Lesions

4.2.1. Cor Triatriatum

Diagnostic
  1. Adults presenting with cor triatriatum sinister should be evaluated for other congenital abnormalities, particularly ASD, VSD, and anomalous pulmonary venous connection.
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  1. In adults with prior repair of cor triatriatum sinister and recurrent symptoms, it is reasonable to evaluate for pulmonary vein stenosis.
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Therapeutic
  1. Surgical repair is indicated for adults with cor triatriatum sinister for symptoms attributable to the obstruction or a substantial gradient across the membrane.
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4.2.2. Congenital Mitral Stenosis

  1. Adults with congenital mitral stenosis or a parachute mitral valve should be evaluated for other left-sided obstructive lesions.
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4.2.3. Subaortic Stenosis

Diagnostic
  1. Stress testing for adults with LVOT obstruction to determine exercise capacity, symptoms, electrocardiographic changes, or arrhythmias may be reasonable in the presence of otherwise equivocal indications for intervention.
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Therapeutic
  1. Surgical intervention is recommended for adults with subAS, a maximum gradient 50 mm Hg or more and symptoms attributable to the subAS.
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  1. Surgical intervention is recommended for adults with subAS and less than 50 mm Hg maximum gradient and HF or ischemic symptoms, and/or LV systolic dysfunction attributable to subAS.
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  1. To prevent the progression of AR, surgical intervention may be considered for asymptomatic adults with subAS and at least mild AR and a maximum gradient of 50 mm Hg or more.
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4.2.4. Congenital Valvular Aortic Stenosis

Diagnostic
  1. Adults with bicuspid aortic valve should be evaluated for coarctation of the aorta by clinical examination and imaging studies.
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  1. It is reasonable to screen first-degree relatives of patients with bicuspid aortic valve or unicuspid aortic valve with echocardiography for valve disease and aortopathy.
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Therapeutic
  1. In adults with bicuspid aortic valve stenosis and a noncalcified valve with no more than mild AR meeting indications for intervention per GDMT, it may be reasonable to treat with balloon valvuloplasty.
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4.2.4.1. Turner Syndrome
Diagnostic
  1. Women with Turner syndrome should be evaluated for bicuspid aortic valve, coarctation of the aorta, and enlargement of the ascending aorta.
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Therapeutic
  1. Prophylactic replacement of the aortic root or ascending aorta in adults with Turner syndrome is reasonable when the aortic diameter is 2.5 cm/m2 or greater.
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4.2.5. Supravalvular Aortic Stenosis

Diagnostic
  1. Aortic imaging using TTE, TEE, CMR, or CTA is recommended in adults with Williams syndrome or patients suspected of having supravalvular aortic stenosis.
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  1. Coronary imaging is recommended in patients with Williams syndrome and supravalvular aortic stenosis presenting with symptoms of coronary ischemia.
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Therapeutic
  1. Surgical repair is recommended for adults with supravalvular aortic stenosis (discrete or diffuse) and symptoms or decreased LV systolic function deemed secondary to aortic obstruction.
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  1. Coronary artery revascularization is recommended in symptomatic adults with supravalvular aortic stenosis and coronary ostial stenosis.
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4.2.6. Coarctation of the Aorta

Diagnostic
  1. Initial and follow-up aortic imaging using CMR or CTA is recommended in adults with coarctation of the aorta, including those who have had surgical or catheter intervention.
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  1. Resting blood pressure should be measured in upper and lower extremities in all adults with coarctation of the aorta.
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  1. Ambulatory blood pressure monitoring in adults with coarctation of the aorta can be useful for diagnosis and management of hypertension.
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  1. Screening for intracranial aneurysms by magnetic resonance angiography or CTA may be reasonable in adults with coarctation of the aorta.
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  1. Exercise testing to evaluate for exercise-induced hypertension may be reasonable in adults with coarctation of the aorta who exercise.
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Therapeutic
  1. Surgical repair or catheter-based stenting is recommended for adults with hypertension and significant native or recurrent coarctation of the aorta.
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  1. GDMT is recommended for treatment of hypertension in patients with coarctation of the aorta.
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  1. Balloon angioplasty for adults with native and recurrent coarctation of the aorta may be considered if stent placement is not feasible and surgical intervention is not an option.
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4.3. Right-Sided Lesions

4.3.1. Valvular Pulmonary Stenosis

Diagnostic
  1. In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis from interatrial right-to-left communication, and/or exercise intolerance, balloon valvuloplasty is recommended.
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  1. In adults with moderate or severe valvular pulmonary stenosis and otherwise unexplained symptoms of HF, cyanosis, and/or exercise intolerance who are ineligible for or who failed balloon valvuloplasty, surgical repair is recommended.
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  1. In asymptomatic adults with severe valvular pulmonary stenosis, intervention is reasonable.
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4.3.1.1. Isolated PR After Repair of Pulmonary Stenosis
Diagnostic
  1. In symptomatic patients with moderate or greater PR resulting from treated isolated pulmonary stenosis, with RV dilation or RV dysfunction, pulmonary valve replacement is recommended.
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  1. For asymptomatic patients with residual PR resulting from treatment of isolated pulmonary stenosis with a dilated right ventricle, serial follow-up is recommended.
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  1. In asymptomatic patients with moderate or greater PR resulting from treatment of isolated pulmonary stenosis with progressive RV dilation and/or RV dysfunction, pulmonary valve replacement may be reasonable.
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4.3.2. Branch and Peripheral Pulmonary Stenosis

Diagnostic
  1. For adults with peripheral or branch PS, ongoing surveillance is recommended.
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Therapeutic
  1. In adults with peripheral or branch PA stenosis, PA dilation and stenting can be useful.
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4.3.3. Double-Chambered Right Ventricle

  1. Surgical repair for adults with double-chambered right ventricle and moderate or greater outflow obstruction is recommended in patients with otherwise unexplained symptoms of HF, cyanosis, or exercise limitation.
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  1. Surgical repair for adults with double-chambered right ventricle with a severe gradient may be considered in asymptomatic patients.
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4.3.4. Ebstein Anomaly

Diagnostic
  1. In adults with Ebstein anomaly, CMR can be useful to determine anatomy, RV dimensions, and systolic function.
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  1. In adults with Ebstein anomaly, TEE can be useful for surgical planning if TTE images are inadequate to evaluate tricuspid valve morphology and function.
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  1. Electrophysiological study with or without catheter ablation can be useful in the diagnostic evaluation of adults with Ebstein anomaly and ventricular preexcitation but without supraventricular tachycardia.
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  1. In adults with Ebstein anomaly, electrophysiological study (and catheter ablation, if needed) is reasonable before surgical intervention on the tricuspid valve even in the absence of preexcitation or supraventricular tachycardia.
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Therapeutic
  1. Surgical repair or reoperation for adults with Ebstein anomaly and significant TR is recommended when one or more of the following are present: HF symptoms, objective evidence of worsening exercise capacity, progressive RV systolic dysfunction by echocardiography or CMR.
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  1. Catheter ablation is recommended for adults with Ebstein anomaly and high-risk pathway conduction or multiple accessory pathways.
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  1. Surgical repair or reoperation for adults with Ebstein anomaly and significant TR can be beneficial in the presence of progressive RV enlargement, systemic desaturation from right-to-left atrial shunt, paradoxical embolism, and/or atrial tachyarrhythmias.
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  1. Bidirectional superior cavopulmonary (Glenn) anastomosis at time of Ebstein anomaly repair may be considered for adults when severe RV dilation or severe RV systolic dysfunction is present, LV function is preserved, and left atrial pressure and LV end diastolic pressure are not elevated.
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4.3.5. Tetralogy of Fallot

Diagnostic
  1. CMR is useful to quantify ventricular size and function, pulmonary valve function, pulmonary artery anatomy, and left heart abnormalities in patients with repaired TOF.
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  1. Coronary artery compression testing is indicated before right ventricle-to-PA conduit stenting or transcatheter valve placement in repaired TOF.
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  1. Programmed ventricular stimulation can be useful to risk-stratify adults with TOF and additional risk factors for SCD.
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  1. In patients with repaired TOF, cardiac catheterization with angiography, if indicated, is reasonable to assess hemodynamics when adequate data cannot be obtained noninvasively in the setting of an arrhythmia, HF, unexplained ventricular dysfunction, suspected pulmonary hypertension or cyanosis.
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  1. Pulmonary valve replacement (surgical or percutaneous) for relief of symptoms is recommended for patients with repaired TOF and moderate or greater PR with cardiovascular symptoms not otherwise explained.
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Therapeutic
  1. Pulmonary valve replacement (surgical or percutaneous) is reasonable for preservation of ventricular size and function in asymptomatic patients with repaired TOF and ventricular enlargement or dysfunction and moderate or greater PR.
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  1. Primary prevention ICD therapy is reasonable in adults with TOF and multiple risk factors for SCD.
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  1. Surgical pulmonary valve replacement may be reasonable for adults with repaired TOF and moderate or greater PR with other lesions requiring surgical interventions.
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  1. Pulmonary valve replacement, in addition to arrhythmia management, may be considered for adults with repaired TOF and moderate or greater PR and ventricular tachyarrhythmia.
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4.3.6. Right Ventricle-to-Pulmonary Artery Conduit

Diagnostic
  1. Coronary artery compression testing with simultaneous coronary angiography and high-pressure balloon dilation in the conduit is indicated before right ventricle-to-PA conduit stenting or transcatheter valve placement.
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  1. In patients with stented right ventricle-to-PA conduits and worsening PS or PR, evaluation for conduit complications should be performed, including fluoroscopy to evaluate for stent fracture and blood cultures to assess for IE.
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  1. In adults with right ventricle-to-PA conduit and arrhythmia, congestive HF, unexplained ventricular dysfunction or cyanosis cardiac catheterization is reasonable to assess the hemodynamics.
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Therapeutic
  1. Right ventricle-to-PA conduit intervention is reasonable for adults with right ventricle-to-PA conduit and moderate or greater PR or moderate or greater stenosis with reduced functional capacity or arrhythmia.
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  1. Right ventricle-to-PA conduit intervention may be reasonable for asymptomatic adults with right ventricle-to-PA conduit and severe stenosis or severe regurgitation with reduced RV ejection fraction or RV dilation.
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4.4. Complex Lesions

4.4.1.1. Transposition of the Great Arteries With Atrial Switch
Diagnostic
  1. Ambulatory monitoring for bradycardia or sinus node dysfunction is recommended for adults with d-TGA with atrial switch, especially if treated with beta blockers or other rate-slowing agents.
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  1. Adults with d-TGA with atrial switch repair should undergo annual imaging with either echocardiography or CMR to evaluate for common long-term complications of the atrial switch.
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  1. Assessment for a communication through the interatrial baffle or venous stenosis is reasonable for adults with d-TGA with atrial switch, particularly if transvenous pacemaker/ICD implantation is considered or leads are already present.
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Therapeutic
  1. GDMT with appropriate attention to the need for anticoagulation is recommended to promptly restore sinus rhythm for adults with d-TGA with atrial switch repair presenting with atrial arrhythmia.
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4.4.1.2. Transposition of the Great Arteries With Arterial Switch
Diagnostic
  1. Baseline and serial imaging with either echocardiography or CMR should be performed in adults with d-TGA with arterial switch who have neoaortic dilation, valve dysfunction or PA or branch PA stenosis or ventricular dysfunction.
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  1. Coronary revascularization for adults with d-TGA with arterial switch should be planned by surgeons or interventional cardiologists with expertise in revascularization in collaboration with ACHD providers to ensure coronary and pulmonary artery anatomy are understood.
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  1. It is reasonable to perform anatomic evaluation of coronary artery patency (catheter angiography, or CT or MR angiography) in asymptomatic adults with d-TGA with arterial switch.
(IIa, B-NR)
573
  1. Physiological tests of myocardial perfusion for adults with d-TGA after arterial switch can be beneficial for assessing symptoms suggestive of myocardial ischemia.
(IIa, C-EO)
573
  1. GDMT is reasonable to determine the need for coronary revascularization for adults with d-TGA after arterial switch.
(IIa, C-EO)
573
Therapeutic
  1. GDMT is reasonable to determine indications for aortic valve replacement in adults with d-TGA after arterial switch with severe neoaortic valve regurgitation.
(IIa, C-EO)
573
  1. Catheter or surgical intervention for PS is reasonable in adults with d-TGA after arterial switch with symptoms of HF or decreased exercise capacity attributable to PS.
(IIa, C-EO)
573
4.4.1.3. Transposition of the Great Arteries With Rastelli Type Repair

See Section 3.3 for recommendations on who should perform surgeries, cardiac catheterization, and other procedures in these patients; and Section 3.4 for recommendations on diagnostic evaluation.

The Rastelli operation is performed in patients with d-TGA with VSD and PS and forvariations of double outlet right ventricle with PS. The operation consists of 2 main components:

  1. An intracardiac baffle that directs oxygenated blood from the left ventricle via a nonrestrictive VSD to the aorta.
  2. A right ventricle-to-PA conduit, which is usually valved.

The operation is designed to use the morphologic left ventricle as the systemic ventricle and the morphologic right ventricle as the subpulmonic ventricle. Long-term considerations after the Rastelli operation include:

  1. Right ventricle-to-PA conduit dysfunction (Section 4.3.6)
  2. VSD patch leaks/dehiscence (Section 4.1.3)
  3. LV-to-aorta internal baffle stenosis (Section 4.2.3)
  4. Scar-based VT
4.4.1.4. Congenitally Corrected Transposition of the Great Arteries
Diagnostic
  1. CMR is reasonable in adults with CCTGA to determine systemic RV dimensions and systolic function.
(IIa, C-LD)
573
Therapeutic
  1. Tricuspid valve replacement is recommended for symptomatic adults with CCTGA and severe TR, and preserved or mildly depressed systemic ventricular function.
(I, B-NR)
573
  1. Tricuspid valve replacement is reasonable for asymptomatic adults with CCTGA and severe TR with dilation or mild dysfunction of the systemic ventricle.
(IIa, C-LD)
573
  1. Conduit intervention/replacement may be considered for adults with CCTGA and symptomatic subpulmonary left ventricle-to-PA conduit dysfunction, recognizing that unloading the subpulmonary ventricle may have a detrimental impact on systemic atrioventricular valve function.
(IIb, B-NR)
573
4.4.2. Fontan Palliation of Single Ventricle Physiology (Including Tricuspid Atresia and Double Inlet Left Ventricle)
Diagnostic
  1. New presentation of an atrial tachyarrhythmia in adults with Fontan palliation should be managed promptly and include prevention of thromboembolic events and consultation with an electrophysiologist with CHD expertise.
(I, C-LD)
573
  1. Adults after Fontan palliation should be evaluated annually with either echocardiography or CMR.
(I, C-EO)
573
  1. Cardiac catheterization should be performed in adults before initial Fontan surgery or revision of a prior Fontan connection to assess suitability of preintervention hemodynamics for Fontan physiology or revision of a prior Fontan connection.
(I, C-EO)
573
  1. New onset or worsening atrial tachyarrhythmias in adults with single ventricle after Fontan palliation should prompt a search for potential hemodynamic abnormalities, which may necessitate imaging and/or cardiac catheterization.
(I, C-EO)
573
  1. In adults with Fontan palliation, it is reasonable to encourage a regular exercise program appropriate to their abilities.
(IIa, B-NR)
573
  1. Imaging of the liver (ultrasonography, CMR, CT) and laboratory evaluation of liver function for fibrosis, cirrhosis, and/or hepatocellular carcinoma are reasonable in adults after Fontan palliation.
(IIa, C-LD)
573
  1. In adults after Fontan palliation, it is reasonable to perform biochemical and hematological testing on an annual basis especially for liver and renal function.
(IIa, C-EO)
573
  1. Cardiac catheterization can be useful to evaluate a symptomatic adult after Fontan palliation when noninvasive testing is insufficient to guide therapy.
(IIa, C-LD)
573
  1. Evaluation for cardiac transplantation is reasonable in adults with Fontan palliation and signs and symptoms of proteinlosing enteropathy.
(IIa, C-LD)
573
  1. It may be reasonable to perform catheterization in asymptomatic adults after Fontan palliation to evaluate hemodynamics, oxygenation and cardiac function to guide optimal medical, interventional and/or surgical therapy.
(IIb, C-EO)
573
Therapeutic
  1. Anticoagulation with a vitamin K antagonist is recommended for adults with Fontan palliation with known or suspected thrombus, thromboembolic events, or prior atrial arrhythmia, and no contraindications to anticoagulation.
(I, C-EO)
573
  1. Catheter ablation can be useful in adults after Fontan palliation with intra-atrial reentrant tachycardia or focal atrial tachycardia.
(IIa, C-LD)
573
  1. Fontan revision surgery, including arrhythmia surgery as indicated, is reasonable for adults with atriopulmonary Fontan connections with recurrent atrial tachyarrhythmias refractory to pharmacological therapy and catheter ablation who have preserved systolic ventricular function and severe atrial dilation.
(IIa, C-LD)
573
  1. Pulmonary vasoactive medications can be beneficial to improve exercise capacity in adults with Fontan repair.
(IIa, B-R)
573
  1. Antiplatelet therapy or anticoagulation with a vitamin K antagonist may be considered in adults after Fontan palliation without known or suspected thrombus, thromboembolic events, or prior arrhythmia.
(IIb, B-NR)
573
  1. Reoperation or intervention for structural/anatomic abnormalities in a Fontan palliated patient with symptoms or with failure of the Fontan circulation may be considered.
(IIb, C-LD)
573
4.4.3. Hypoplastic Left Heart Syndrome/Norwood Repair
  • The Norwood repair is the first of 3 steps in palliation for hypoplastic left heart syndrome and consists of atrial septectomy, transection, and ligation of the distal main PA with construction of a systemic-to-PA shunt, and anastomosis of the proximal stump of the main PA to the hypoplastic ascending aorta with augmentation of the entire aortic arch from the sinotubular junction to beyond the ductus arteriosus.
  • Hypoplastic left heart syndrome is fatal unless surgical palliation is performed in the neonatal period. Subsequent surgeries include a bidirectional cavopulmonary anastomosis (often performed around 6 months of age), followed finally by a Fontan procedure (often at approximately 2 to 4 years of age).
  • Sequelae of hypoplastic left heart syndrome are largely those of the Fontan palliation, but additional concerns related to the underlying anatomy and the Norwood repair is important in patients with hypoplastic left heart syndrome. These include aortic obstruction related to anastomosis of the PA and aorta, and neoaortic dilation
  • Additionally, native anatomy wherein coronary arteries arise from a small aortic root make coronary ischemia a greater concern than in other underlying disorders managed with Fontan repair. The frequency and spectrum of long-term sequelae specific to the Norwood repair are not yet known.
4.4.4. Truncus Arteriosus
  • Truncus arteriosus in the adult has almost invariably been repaired in childhood, and in the rare circumstances when an adult has unrepaired truncus arteriosus, Eisenmenger physiology is typical. Pulmonary hypertension may be present in repaired patients.
  • The types of operative repairs may involve VSD closure, right ventricle-to-PA conduit placement, reconstruction of the pulmonary arteries, and replacement of the truncal (neoaortic) valve. Unifocalization of the pulmonary arteries may be necessary in very complex cases. The aorta may be dilated.
  • Recommendations regarding assessment and management of truncus arteriosus can generally be inferred in the recommendations for the specific components, including right ventricle-to-PA conduit, VSD, aortic valve disease, and aortopathies.
4.4.5. Double Outlet Right Ventricle
  • Double outlet right ventricle is an anatomic descriptor that includes abnormalities similar to TOF in some patients (when the aorta is closely related to the VSD) and similar to d-TGA with a VSD in others (when the PA is more closely related to the VSD than the aorta). Repairs are predicated on the underlying anatomy and may involve VSD closure with relief of PS, right ventricle-to-PA conduit, or Rastelli-type repair.
  • In severe cases, single-ventricle physiology may be present. Consequently, recommendations for the management of a patient with double outlet right ventricle can generally be inferred in the recommendations for the lesion with the most similar anatomy and physiology (e.g., TOF can reasonably be based on the recommendations in Section 4.4.1, recognizing that a patient with double outlet right ventricle is more likely to have residual LVOT obstruction).
4.4.6. Severe PAH and Eisenmenger Syndrome
4.4.6.1. Severe PAH
Diagnostic
  1. Patients with ACHD with pulmonary vascular resistance 2.5 Wood units or greater (≥4 Wood units × m2) should be assessed collaboratively by an ACHD cardiologist and an expert in pulmonary hypertension to develop a management plan.
(I, B-NR)
573
  1. Adults with septal or great artery shunts should undergo periodic screening for pulmonary hypertension with TTE.
(I, B-NR)
573
  1. Cardiac catheterization to assess pulmonary vascular hemodynamics is recommended for adults with septal or great artery shunts and clinical symptoms, signs, or echocardiographic findings suggestive of pulmonary hypertension.
(I, B-NR)
573
  1. In adults with septal or great artery shunts, cardiac catheterization with hemodynamics (performed before or at time of closure) is beneficial to assess suitability for closure.
(I, B-NR)
573
  1. BNP, chest x-ray, 6-minute walk test, and cardiac catheterization are useful for initial and follow-up evaluation of patients with ACHD with PAH.
(I, C-EO)
573
4.4.6.2. Eisenmenger Syndrome
Diagnostic
  1. When evaluating adults with presumed Eisenmenger syndrome, clinicians should confirm diagnostic imaging and cardiac catheterization data accuracy and exclude other potential contributors to right-to-left shunting or pulmonary hypertension.
(I, C-EO)
573
Therapeutic
  1. Bosentan is beneficial in symptomatic adults with Eisenmenger syndrome with ASD or VSD.
(I, A)
573
  1. In symptomatic adults with Eisenmenger syndrome, bosentan and PDE-5 inhibitors are reasonable in combination if symptomatic improvement does not occur with either medication alone.
(IIa, B-R)
573
  1. Bosentan is a reasonable therapy to treat symptomatic adults with Eisenmenger syndrome with 1 of the following: shunts other than ASD/VSD (e.g., PDA, aortopulmonary window) (Level of Evidence C-EO), or complex congenital heart lesions or Down syndrome (Level of Evidence B-NR).
(IIa, C-EO)
573
  1. It is reasonable to use PDE-5 inhibitors (e.g., sildenafil, tadalafil) to treat symptomatic adults with Eisenmenger syndrome with ASD, VSD, or great artery shunt.
(IIa, B-NR)
573
4.4.7. Coronary Anomalies
4.4.7.1. Anomalous Coronary Artery Evaluation
Diagnostic
  1. Coronary angiography, using catheterization, CT, or CMR, is recommended for evaluation of anomalous coronary artery.
(I, C-LD)
573
  1. Anatomic and physiological evaluation should be performed in patients with anomalous aortic origin of the left coronary from the right sinus and/or right coronary from the left sinus.
(I, C-LD)
573
4.4.7.2. Anomalous Aortic Origin of Coronary Artery
Therapeutic
  1. Surgery is recommended for AAOCA from the left sinus or AAOCA from the right sinus for symptoms or diagnostic evidence consistent with coronary ischemia attributable to the anomalous coronary artery.
(I, B-NR)
573
  1. Surgery is reasonable for anomalous aortic origin of the left coronary artery from the right sinus in the absence of symptoms or ischemia.
(IIa, C-LD)
573
  1. Surgery for AAOCA is reasonable in the setting of ventricular arrhythmias.
(IIa, C-EO)
573
  1. Surgery or continued observation may be reasonable for asymptomatic patients with an anomalous left coronary artery arising from the right sinus or right coronary artery arising from the left sinus without ischemia or anatomic or physiological evaluation suggesting potential for compromise of coronary perfusion (e.g., intramural course, fish-mouth-shaped orifice, acute angle).
(IIb, B-NR)
573
4.4.7.3. Anomalous Coronary Artery Arising From the PA
Therapeutic
  1. Surgery is recommended for anomalous left coronary artery from the PA.
(I, B-NR)
573
  1. In a symptomatic adult with anomalous right coronary artery from the PA with symptoms attributed to the anomalous coronary, surgery is recommended.
(I, C-EO)
573
  1. Surgery for anomalous right coronary artery from the PA is reasonable in an asymptomatic adult with ventricular dysfunction or with myocardial ischemia attributed to anomalous right coronary artery from the PA.
(IIa, C-EO)
573
4.4.8. Coronary Artery Fistula
  • Coronary artery fistula is an abnormal communication between a coronary artery and another cardiovascular structure, which may include a cardiac chamber, coronary sinus, superior vena cava, or PA. The incidence of coronary artery fistula is 0.1% to 0.2% in all patients undergoing coronary angiography. Fistulous communications may be congenital or acquired. Specific management strategies, which can include surgical repair or catheter embolization, have been controversial.
  • In a series of 46 patients treated with surgery, predominant preoperative symptoms included angina and HF. Importantly, postoperative myocardial infarction occurred in 11% because of low flow in the dilated coronary artery proximal to fistula closure. Late survival was also significantly reduced compared with an age-matched population. The presence of coronary artery fistula(s) requires review by a knowledgeable team that may include congenital or noncongenital cardiologists and surgeons to determine the role of medical therapy and/or percutaneous or surgical closure.

Recommendation Grading

Disclaimer

Overview

Title

Management of Adults With Congenital Heart Disease

Authoring Organizations

Endorsing Organizations

Publication Month/Year

August 16, 2018

Document Type

Guideline

External Publication Status

Published

Country of Publication

US

Inclusion Criteria

Male, Female, Adolescent, Adult, Child, Infant, Older adult

Health Care Settings

Ambulatory, Hospital, Outpatient, Operating and recovery room

Intended Users

Athletics coaching, nurse, nurse practitioner, physician, physician assistant

Scope

Assessment and screening, Management

Diseases/Conditions (MeSH)

D006330 - Heart Defects, Congenital, D000013 - Congenital Abnormalities

Keywords

congenital heart disease, cardiac defects, arrhythmias, unoperated/repaired heart defect, Congenital heart disease

Source Citation

Stout KK, Daniels CJ, Aboulhosn JA, Bozkurt B, Broberg CS, Colman JM, Crumb SR, Dearani JA, Fuller S, Gurvitz M, Khairy P, Landzberg MJ, Saidi A, Valente AM, Van Hare GF. 2018 AHA/ACC Guideline for the Management of Adults With Congenital Heart Disease: Executive Summary: A Report of the American College of Cardiology/American Heart Association Task Force on Clinical Practice Guidelines. J Am Coll Cardiol. 2019 Apr 2;73(12):1494-1563. doi: 10.1016/j.jacc.2018.08.1028. Epub 2018 Aug 16. Erratum in: J Am Coll Cardiol. 2019 May 14;73(18):2361. PMID: 30121240.

Supplemental Methodology Resources

Data Supplement, Systematic Review Document

Methodology

Number of Source Documents
758
Literature Search Start Date
April 1, 2014
Literature Search End Date
November 30, 2014
Specialties Involved
Cardiology, Family Medicine, Internal Medicine General, Medical Genetics And Genomics, Pediatrics, Sports Medicine, Pediatric Cardiology, Pediatric Sports Medicine, Pediatrics, Pediatrics
Percentage of Authors Reporting COI
100