The ESC Clinical Practice Guidelines for the Management of Adult Congenital Heart Disease 2020
Helmut Baumgartner, Julie De Backer
Abstract
Since the previous version of the guidelines on the management of grown-up congenital heart disease was published in 2010, new data and experience have accumulated, particularly on percutaneous interventional techniques and on risk stratification with regard to timing of surgery and catheter intervention. Medical treatment options have also emerged for selected indications. This made a revision of the recommendations necessary. Since adult patients with congenital heart disease (CHD) now present in increasing numbers at advanced ages, including the elderly, the term ‘grown-up congenital heart disease’ no longer appeared appropriate and was therefore—in accordance with international literature—replaced with adult congenital heart disease (ACHD) throughout the document. The document focuses on decision-making in ACHD which involves accurate diagnosis, the timing of intervention, risk assessment, and selection of the most suitable type of intervention. In addition, specific aspects of medical treatment such as for heart failure, pulmonary hypertension, and anticoagulation are addressed. Compared to the 2010 version new concepts in the present guidelines include a disease complexity classification, staffing requirements for ACHD expert centres, the emerging role of biomarkers in the follow-up of ACHD, detailed and specific recommendations on arrhythmia management, more specific and adjusted recommendations for pulmonary arterial hypertension (PAH) treatment, recommendations for the use of anticoagulants, consideration of aging and advance care planning, categories of high-risk pregnancies, expansion of the Marfan Syndrome section to inheritable thoracic aortic disease, Turner’s Syndrome and bicuspid aortic disease, and the emerging role of catheter intervention in ACHD. In addition, there is now also a section on coronary anomalies.