The following are key points to remember from the 2018 American Heart Association/American College of Cardiology (AHA/ACC) Guideline for the Management of Adults With Congenital Heart Disease (ACHD):

1. This guideline is a major update to the ACC/AHA 2008 guidelines for the management of ACHD. The 2018 guideline reflects the new format of presenting guidelines, including tables of related recommendations, a brief synopsis, recommendation-specific supportive text, and flow diagrams. This review will emphasize areas of difference between the new and the previous guidelines as well as lesion-specific recommendations considered to be of interest to practitioners.
2. The guideline development process prompted the commissioning of two independent evidence review committees (ERCs) to address two significant clinical questions. The first ERC addressed the issue of the benefit of intervention for asymptomatic patients with secundum atrial septal defects (ASDs) and right ventricular (RV) dilatation. The second ERC addressed the issue of the role of medical therapy, including angiotensin-converting enzyme inhibitors, angiotensin receptor blockers, beta-blockers, and aldosterone antagonists in adults with systemic right ventricles.
3. The guideline presents a new classification system for ACHD. The adult congenital heart disease anatomic and physiological (ACHD AP) classification system uses both anatomic complexity and as well as physiologic status. Anatomic classification includes Class I (simple), Class II (moderate complexity), and Class III (great complexity). The physiological classification is divided into stages A-D and is overall similar to the AHA heart failure classification. The physiological classification system takes into account a patient’s functional status as well as other factors including presence of valve disease, pulmonary hypertension, arrhythmias, aortic dilatation, end-organ function, and cyanosis.
4. The need for such a classification system is based on data that allow better refinement of patient classification that uses physiologic variables in addition to anatomic categorization. For example, a patient with repaired tetralogy of Fallot with no significant pulmonary regurgitation, no symptoms, and no other sequelae would be ACHD AP classification IIA, whereas another patient with tetralogy of Fallot but with severe pulmonary valve regurgitation and exercise intolerance would be ACHD AP classification IIC. This distinction allows patients to be followed prospectively in a fashion more reflective of the interplay between their congenital abnormality and physiologic impact.
5. The ACHD AP classification system provides the basis for making lesion-specific recommendations regarding interval of clinical follow-up and testing modalities such as electrocardiograms, transthoracic echocardiography, cardiovascular magnetic resonance, and exercise testing.
6. Two recommendations related to closure of secundum ASD were informed by a systemic review. In adults with secundum ASD and impaired functional capacity, right atrial and/or RV enlargement, Qp:Qs ≥1.5:1, and absence of cyanosis at rest or during exercise, device or surgical closure is recommended if systolic pulmonary artery pressure is <50% of systolic systemic pressure and pulmonary vascular resistance is less than one-third of the systemic vascular resistance (Class of Recommendation [COR] I, Level of Evidence [LOE] B-NR). For asymptomatic patients, closure is reasonable (COR IIa, LOE C-LD).
7. Patients with bicuspid aortic valve should be evaluated for coarctation of the aorta with clinical exam and imaging studies (COR I, LOE B-NR). It is reasonable to screen first-degree relatives of patients with bicuspid or unicuspid aortic valve for aortic valve disease or aortopathy using echocardiography (COR IIa, LOE B-NR).
8. Screening for intracranial aneurysms by magnetic resonance (MR) angiography or computed tomography (CT) angiography may be reasonable in adults with coarctation of the aorta (COR IIb, LOE B-NR).
9. Pulmonary valve replacement (PVR) for relief of symptoms is recommended for patients with repaired tetralogy of Fallot and moderate or greater pulmonary regurgitation with cardiovascular symptoms not otherwise explained (COR I, LOE B-NR). PVR is reasonable for preservation of ventricular size and function in asymptomatic patients with tetralogy of Fallot and ventricular enlargement or dysfunction and moderate or greater pulmonary regurgitation (COR IIa, LOE B-NR). Specifically, PVR is reasonable in patients with ≥2 of the following: mild or greater RV or left ventricular (LV) dysfunction, severe RV dilatation (RV end-diastolic volume index ≥160 ml/m², RV end-systolic volume index ≥80 ml/m²), RV end-diastolic volume ≥2x LV end-diastolic volume, RV systolic pressure ≥2/3 systemic pressure, and/or progressive reduction in objective exercise capacity.
10. Primary prevention implantable cardioverter-defibrillator therapy is reasonable in adults with tetralogy of Fallot and multiple risk factors for sudden cardiac death (SCD) (COR IIa, LOE B-NR). Risk factors for SCD include LV systolic or diastolic dysfunction, nonsustained ventricular tachycardia, QRS duration ≥180 ms, extensive RV scarring, and inducible sustained ventricular tachycardia at electrophysiological study.
11. The systemic review conducted regarding impact of medical therapy in patients with systemic right ventricles demonstrated that medical therapy for systolic ventricular dysfunction remains largely uncertain. As a result, no recommendations regarding medical therapy for systolic dysfunction of systemic right ventricles were made.
12. It is reasonable to perform anatomic evaluation of coronary artery patency, i.e., catheter angiography or CT or MR angiography in asymptomatic adults with dextro-transposition of the great arteries (d-TGA) with arterial switch (COR IIa, LOE B-NR). Physiologic tests of myocardial perfusion for adults with d-TGA after arterial switch can be beneficial for assessing symptoms of myocardial ischemia (COR IIa, LOE C-EO).
13. Pulmonary vasoactive medications can be beneficial to improve exercise capacity in adults with Fontan repair (COR IIa, LOE B-R).
14. At the conclusion of the document, the writing committee describes evidence gaps in the management of ACHD and proposes future directions to address these issues.

Clinical Topics: Arrhythmias and Clinical EP, Cardiac Surgery, Congenital Heart Disease and Pediatric Cardiology, Diabetes and Cardiometabolic Disease, Heart Failure and Cardiomyopathies, Invasive Cardiovascular Angiography and Intervention, Noninvasive Imaging, Prevention, Pulmonary Hypertension and Venous Thromboembolism, Valvular Heart Disease, Implantable Devices, SCD/Ventricular Arrhythmias, Atrial Fibrillation/Supraventricular Arrhythmias, Aortic Surgery, Cardiac Surgery and Arrhythmias, Cardiac Surgery and CHD and Pediatrics, Cardiac Surgery and Heart Failure, Cardiac Surgery and VHD, Congenital Heart Disease, CHD and Pediatrics and Arrhythmias, CHD and Pediatrics and Imaging, CHD and Pediatrics and Interventions, CHD and Pediatrics and Prevention, CHD and Pediatrics and Quality Improvement, Acute Heart Failure, Heart Transplant, Pulmonary Hypertension, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Echocardiography/Ultrasound, Exercise, Hypertension

Keywords: Aortic Coarctation, Aortic Diseases, Arrhythmias, Cardiac, Cardiac Catheterization, Cardiac Imaging Techniques, Cardiovascular Surgical Procedures, Cyanosis, Death, Sudden, Cardiac, Defibrillators, Implantable, Delivery of Health Care, Delivery of Health Care, Integrated, Diagnostic Imaging, Dilatation, Echocardiography, Electrocardiography, Endocarditis, Exercise, Exercise Test, Functional Residual Capacity, Genetic Diseases, Inborn, Heart Defects, Congenital, Heart Failure, Heart Septal Defects, Atrial, Heart Transplantation, Heart Valve Diseases, Hypertension, Pulmonary, Motor Activity, Myocardial Ischemia, Organization and Administration, Palliative Care, Patient Care Team, Pregnancy, Primary Prevention, Sports Nutritional Sciences, Surgical Procedures, Operative, Tachycardia, Ventricular, Tetralogy of Fallot

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