The American Heart Association/American College of Cardiology (ACC/AHA) has released a major update to its 2008 guidelines for the management of Adults With Congenital Heart Disease (ACHD). The guideline is published in the Journal of the American College of Cardiology.
Access to Care
- Physicians caring for patients with ACHD should support access to care by:
- assuring smooth transitions for adolescents and young adults from pediatric to adult providers.
- promoting awareness of the need for lifelong specialized care through outreach and educational programs.
Delivery of Care
- Patients with ACHD anatomic and physiological (AP) classification IB-D, IIA-D, and IIIA-D should be managed in collaboration with an ACHD cardiologist.
- 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.
- 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.
- 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.
Ionizing Radiation Principles
- Strategies to limit and monitor radiation exposure are recommended during imaging of patients with ACHD, with studies not involving ionizing radiation chosen whenever appropriate.
- Every effort should be made to use tests without radiation whenever possible or to select protocols with the lowest possible doses of radiation compatible with securing the needed clinical information.
- Intraoperative TEE is recommended to guide surgical repair of CHD in adults.
- Patients with ACHD should undergo transthoracic echocardiography (TTE) for initial assessment, with the timing of serial assessment based on anatomic and physiological severity and clinical status.
- 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.
- CMR can be useful in the initial evaluation and serial assessment of selected patients with CHD based on anatomic complexity and clinical status.
Cardiac Computed Tomography
- 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.
- 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.
- 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.
- In patients with ACHD, cardiopulmonary exercise testing (CPET) can be useful for baseline functional assessment and serial testing.
- In symptomatic patients with ACHD, a 6-minute walk test can be used to objectively assess symptom severity, functional capacity, and response to therapy.
Exercise and Sports
- 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.
- CPET can be useful to guide activity recommendations for patients with ACHD.
- Cardiac rehabilitation can be useful to increase exercise capacity in patients with ACHD.
Mental Health and Neurodevelopmental Issues
- Patients with ACHD should be evaluated for depression and anxiety.
- Neurodevelopmental or neuropsychological testing may be considered in some patients with ACHD to guide therapies that enhance academic, behavioral, psychosocial, and adaptive functioning.
- Referral for mental health evaluation and treatment is reasonable in patients with ACHD.
Noncardiac Medical Issues
- Patients with ACHD at risk for hepatitis C should be screened and vaccinated for viral hepatitis and treated as appropriate.
- Optimization before and close surveillance after invasive procedures regardless of the complexity of the anatomic defect or type of procedure is beneficial.
- 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.
- 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.
- 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.
- Women with CHD receiving chronic anticoagulation should be counseled, ideally before conception, on the risks and benefits of specific anticoagulants during pregnancy.
- 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, IIID should be counseled against becoming pregnant or be given the option of terminating pregnancy.
- Men and women of childbearing age with CHD should be counseled on the risk of CHD recurrence in offspring.
- Exercise testing can be useful for risk assessment in women with ACHD AP classification IC-D, IIA-D, and IIIA-D* who are considering pregnancy.
- When either parent has CHD, it is reasonable to perform fetal echocardiography.
- Women of childbearing potential with CHD should be counseled about the risks associated with pregnancy and appropriate contraceptive options.
- Estrogen-containing contraceptives are potentially harmful for women with CHD who are at high risk of thromboembolic events.
- Consultation with ACHD and HF specialists is recommended for patients with ACHD and HF or severe ventricular dysfunction.
Atrial Septal Defect
- 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.
- CMR, CCT, and/or TEE are useful to evaluate pulmonary venous connections in adults with ASD.
- Echocardiographic imaging is recommended to guide percutaneous ASD closure.
- 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
- Adults with 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 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.
- 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.
- A 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.
Anomalous Pulmonary Venous Connections
- CMR or CTA is recommended for evaluation of partial anomalous pulmonary venous connection.
- Cardiac catheterization can be useful in adults with a partial anomalous pulmonary venous connection to further define hemodynamics.
- Surgical repair is recommended for patients with the 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, PA systolic pressure is less than 50% systemic pressure, and pulmonary vascular resistance is less than one-third of systemic resistance.
- 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.
- Repair of partial anomalous pulmonary venous connection is recommended at the time of closure of a sinus venosus defect or ASD.
Ventricular Septal Defect
- 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.
- Surgical closure of perimembranous or supracristal VSD is reasonable in adults when there is worsening aortic regurgitation (AR) caused by VSD.
- 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.