• Francisco Javier Ruperti-Repilado, Corina Thomet, and Markus Schwerzmann.
• Zentrum für angeborene Herzfehler, Medizinalbereich Herz und Gefässe, Universitätsklinik Inselspital und Universität Bern, Freiburgstraße 15, 3010, Bern, Schweiz.
• Herz. 2021 Feb 1; 46 (1): 14-27.

AbstractThe new guidelines of the European Society of Cardiology (ESC) on treatment of adult congenital heart disease (ACHD) were published in August 2020. The previous recommendations from 2010 were adapted to reflect the diagnostic and therapeutic progress made in the past 10 years. The recommendations are nearly exclusively based on an evidence level C (consensus of opinion of experts or knowledge from small studies, retrospective studies or registries). This is not surprising considering the heterogeneous patient population with a multitude of cardiac defects and repair strategies performed in the past. The cohort of ACHD patients is steadily growing in numbers and is becoming older due to reduced perioperative morbidity and mortality and further medical progress. Therefore, the current guidelines do not focus solely on the acute treatment of cardiac problems but also address the importance of a comprehensive longitudinal follow-up for a chronic, lifelong disorder. On a defect-specific level, progress in the past decade in arrhythmia diagnosis and management, percutaneous interventions and the treatment of pulmonary arterial hypertension have led to many revised or new recommendations. Finally, the 2020 guidelines also address for the first time the management of coronary anomalies.

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