The following are key points to remember from the 2020 European Society of Cardiology guidelines for the management of adult congenital heart disease. This summary focuses on “what’s new” in the guidelines as well as areas that may contrast with guidelines from other societies.

1. In low- and intermediate-risk patients with repaired simple lesions and precapillary pulmonary hypertension, initial oral combination therapy or sequential combination therapy is recommended, and high-risk patients should be treated with initial combination therapy including parenteral prostanoids (Class I).
2. In Eisenmenger patients with reduced exercise capacity (6-minute hall walk distance <450 m), a treatment strategy with initial endothelin receptor antagonist monotherapy should be considered followed by combination therapy if patients fail to improve (Class IIa).
3. The following are recommendations for atrial septal defect (ASD) closure in patients with Qp:Qs >1.5:1 based on calculated pulmonary vascular resistance:
   • <3 Wood unit (WU): Class I for ASD, ventricular septal defect (VSD), and patent ductus arteriosus (PDA)
   • 3-5 WU: Class IIa for ASD, VSD, and PDA
   • >5 WU but decreasing to <5 WU after targeted pulmonary arterial hypertension (PAH) treatment: Class IIb for ASD (fenestrated closure only)
   • >5 WU for VSD and PDA (careful individual decision in expert centers, Class IIb)
   • ≥5 WU despite targeted treatments for PAH, Class III for ASD closure
4. In patients with lesions associated with pulmonary valve regurgitation and no native outflow tract, catheter intervention should be performed if anatomically feasible.
5. Pulmonary valve replacement should be considered in asymptomatic patients with severe pulmonary regurgitation and/or right ventricular (RV) outflow tract obstruction, in the presence of progressive RV dilatation to RV end-systolic volume index ≥80 ml/m², and/or RV end-diastolic volume index ≥160 ml/m², and/or progression of tricuspid regurgitation (TR) to at least moderate.
6. The following are recommendations for tricuspid valve replacement for severe TR in congenitally corrected transposition of the great arteries:
   • In symptomatic patients and preserved or mildly impaired RV systolic function (ejection fraction [EF] >40%), tricuspid valve (TV) replacement is indicated (Class I).
   • In asymptomatic patients and progressive RV dilatation and/or mildly impaired RV systolic function (EF >40%), TV replacement should be considered (Class IIa).
   • In symptomatic patients with severe TR and more than mildly reduced systemic RV systolic function (EF ≤40%), TV replacement may be considered (Class IIb).
7. Anticoagulation for patients with Fontan circulation is indicated in the presence, or with a history, of atrial thrombus, atrial arrhythmias, or thromboembolic events (Class I).
8. It is recommended that women with a Fontan circulation and any complication are counseled against pregnancy (Class I).
9. Regular liver imaging (ultrasound, computed tomography, cardiac magnetic resonance [CMR]) should be considered for patients with Fontan circulation (Class IIa).
10. Endothelin receptor antagonists and phosphodiesterase-5 inhibitors may be considered in selected patients with Fontan circulation and elevated pulmonary pressures/resistance in the absence of elevated ventricular end-diastolic pressure (Class IIa).
11. Routine imaging to assess for cerebral aneurysms in asymptomatic patients with coarctation of the aorta was not recommended in these guidelines.
12. Consistent with the 2018 American Heart Association/American College of Cardiology Guidelines for Adults With Congenital Heart Disease, it was felt there was not sufficient evidence to make a recommendation regarding medical therapy for systemic RV dysfunction in patients after atrial switch procedures.
13. Implantable cardioverter-defibrillator (ICD) implantation should be considered in selected TOF patients with multiple risk factors for sudden cardiac death, including left ventricular dysfunction, nonsustained, symptomatic ventricular tachycardia (VT), QRS duration >180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation (Class IIa).
14. ICD implantation may be considered in patients with advanced single or systemic RV dysfunction (EF systemic RV <35%) in the presence of additional risk factors. Although data are lacking, additional risk factors may include VT, New York Heart Association class II/III, severe atrioventricular valve regurgitation, and QRS >140 ms for patients with transposition of the great arteries (Class IIb).

Clinical Topics: Anticoagulation Management, 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, 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, Pulmonary Hypertension, Interventions and Imaging, Interventions and Structural Heart Disease, Interventions and Vascular Medicine, Hypertension

Keywords: ESC Congress, ESC20, Anticoagulants, Arrhythmias, Cardiac, Defibrillators, Implantable, Diagnostic Imaging, Ductus Arteriosus, Patent, Eisenmenger Complex, Fontan Procedure, Heart Defects, Congenital, Heart Septal Defects, Atrial, Heart Septal Defects, Ventricular, Hypertension, Pulmonary, Pregnancy, Pulmonary Valve Insufficiency, Thromboembolism, Tricuspid Valve Insufficiency, Tachycardia, Ventricular

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