Home > Cardiology > ESC 2020 > 2020 ESC Clinical Practice Guidelines > 2020 Adult Congenital Heart Disease Guidelines

2020 Adult Congenital Heart Disease Guidelines

Presented by
Prof. Helmut Baumgartner, University Hospital Münster, Germany
ESC 2020
The spectrum of adult congenital disease is so broad that the European Society of Cardiology 2020 guidelines for the management of adult congenital heart disease has a particularly challenging scope. Prof. Helmut Baumgartner (University Hospital Münster, Germany) presented the new guidelines, which were simultaneously published online in the European Heart Journal [1,2].

Below are the 10 most relevant changes in the 2020 version of these guidelines.

      1. The 2020 guidelines recommend that for low- and intermediate-risk patients with repaired simple lesions and precapillary pulmonary hypertension, treatment should include initial oral combination therapy or sequential combination therapy. High-risk patients should receive initial combination therapy including parenteral prostanoids (Class I).
      2. Initial endothelin receptor antagonist monotherapy should be considered for Eisenmenger patients with reduced exercise capacity (i.e. 6-minute hall walk distance <450 m), followed by step-up combination therapy if patients do not improve (Class IIa).
      3. There are new recommendations for atrial septal defect (ASD) closure in patients with Qp:Qs >1.5:1 based on calculated pulmonary vascular resistance, stratified by Wood units.
      4. Individuals with lesions associated with pulmonary valve regurgitation and no native outflow tract should receive catheter intervention if anatomically feasible (Class I).
      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 to at least moderate (Class IIa).
      6. There are new updated recommendations for tricuspid valve replacement for severe tricuspid regurgitation in congenitally corrected transposition of the great arteries.
      7. Patients with Fontan circulation should receive regular liver imaging (i.e. ultrasound, computed tomography, cardiac magnetic resonance) (Class IIa). Anticoagulation is a recommended addition to treatment when the individual has, or has had, atrial thrombus, atrial arrhythmias, or thromboembolic events (Class I). Furthermore, women with a Fontan circulation and any complication are counselled against pregnancy (Class I).
      8. 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).
      9. Selected tetralogy of Fallot patients with multiple risk factors for sudden cardiac death (i.e. left ventricular dysfunction, non-sustained, symptomatic ventricular tachycardia [VT], QRS duration >180 ms, extensive RV scarring on CMR, or inducible VT at programmed electrical stimulation) should be considered as candidates for an implantable cardioverter-defibrillator (Class IIa).
      10. An implantable cardioverter-defibrillator may also be considered in patients with advanced single or systemic RV dysfunction (ejection fraction systemic RV <35%) in the presence of additional risk factors (Class IIb).

In conclusion, substantial practice-changing changes have been implemented into the new guidelines for many manifestations of adult congenital heart disease.


    1. Baumgartner H, et al. 2020 ESC Guidelines on adult congenital heart disease. 2020 New ESC Guidelines. ESC Congress 2020, 1 Sept.
    2. Baumgartner H, et al. Eur Heart J. 2020;ehaa554.DOI:10.1093/eurheartj/ehaa554.


Posted on