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.
- 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).
- 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).
- 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.
- Individuals with lesions associated with pulmonary valve regurgitation and no native outflow tract should receive catheter intervention if anatomically feasible (Class I).
- 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).
- There are new updated recommendations for tricuspid valve replacement for severe tricuspid regurgitation in congenitally corrected transposition of the great arteries.
- 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).
- 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).
- 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).
- 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.
- Baumgartner H, et al. 2020 ESC Guidelines on adult congenital heart disease. 2020 New ESC Guidelines. ESC Congress 2020, 1 Sept.
- Baumgartner H, et al. Eur Heart J. 2020;ehaa554.DOI:10.1093/eurheartj/ehaa554.
« SGLT2 inhibitor improves cardiovascular outcomes in heart failure patients Next Article
2020 Sports Cardiology and Exercise in Cardiovascular Patients Guidelines »