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2020 Adult Congenital Heart Disease (previously Grown-Up Congenital Heart Disease) (Management of) Guidelines

ESC Clinical Practice Guidelines

Since the previous version of the Guidelines on the management of grown-up congenital heart disease (CHD) was published in 2010, new evidence has accumulated for this patient group, particularly on percutaneous interventional techniques and risk stratification with regard to timing of surgery and catheter intervention, as well asmedical treatment. This made a revision of the recommendations necessary.


Decision making in ACHD involves accurate diagnosis, timing of intervention, risk assessment, and selection of the most suitable type of intervention. In addition, specific aspects of medical treatment for conditions such as heart failure, pulmonary hypertension (PH), and anticoagulation are addressed.

Congenital Heart Disease and Pediatric Cardiology
Pediatric Cardiology
Pediatric Cardiology
Adult Congenital Heart Disease
CV Surgery - Congenital Heart Disease
Guidelines version available to download
Published in 2020
Reference doi:10.1093/eurheartj/ehaa554
Published in 2020
Reference TOC ACHD Pocket Guidelines
App
Published in 2020
Reference Pocket Guidelines App
Published in 2020
Reference 108 Slides on ACHD
Table of contents: Full Text (ESC Clinical Practice Guidelines)

Preamble      

Introduction

  • Why do we need new Guidelines on the management of adult congenital heart disease?
  • Content of these Guidelines
  • New format of the Guidelines
  • How to use these Guidelines
  • What is new in the 2020 Guidelines?

General aspects        

  • Prevalence of adult congenital heart disease
  • Organization of care
  • Diagnostic work-up
    • Echocardiography
    • Cardiovascular magnetic resonance imaging
    • Cardiovascular computed tomography
    • Cardiopulmonary exercise testing
    • Cardiac catheterization
    • Biomarkers
  • Therapeutic considerations
    • Heart failure
    • Arrhythmias and sudden cardiac death
    • Pulmonary hypertension
    • Surgical treatment
    • Catheter intervention
    • Infective endocarditis
    • Antithrombotic treatment
    • Management of cyanotic patients
  • Additional considerations
    • Sex differences
    • Adult congenital heart disease at more advanced age
    • Advance care planning and end-of-life care
  • Insurance and employment
    • Exercise and sports
    • Non-cardiac surgery
    • Pregnancy, contraception, and genetic counselling

 Specific lesions          

  • Atrial septal defect and anomalous pulmonary venous connection
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Specific aspects of isolated anomalous pulmonary venous connections
    • Follow-up recommendations
    • Additional considerations
  • Ventricular septal defect
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Atrioventricular septal defect
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Patent ductus arteriosus
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Left ventricular outflow tract obstruction
    • Valvular aortic stenosis
    • Supravalvular aortic stenosis
    • Subaortic stenosis
  • Coarctation of the aorta
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Aortopathies
    • Marfan syndrome and related heritable thoracic aortic diseases
    • Bicuspid aortic disease
    • Turner syndrome
  • Right ventricular outflow tract obstruction
    • Introduction and background 46
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Ebstein anomaly
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Tetralogy of Fallot
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up of repaired patients
    • Late surgical/catheter interventional treatment
    • Indications for electrophysiological testing & implantable cardio defibrillator
    • Follow-up recommendations
    • Additional considerations
  • Pulmonary atresia with ventricular septal defect
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Transposition of the great arteries
    • Introduction and background
    • Atrial switch operation
    • Arterial switch operation
    • Rastelli-type operation
    • Follow-up recommendations (irrespective of type of repair)
    • Additional considerations (irrespective of type of repair)
  • Congenitally corrected transposition of the great arteries
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Medical treatment
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Right ventricular to pulmonary artery conduit
    • Introduction and background
    • Diagnostic work-up
    • Surgical/catheter interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Univentricular heart
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Conservative management
    • Follow-up recommendations
    • Additional considerations
  • Patients after Fontan operation
    • Introduction and background
    • Clinical presentation and natural history
    • Diagnostic work-up
    • Medical treatment
    • Surgical/interventional treatment
    • Follow-up recommendations
    • Additional considerations
  • Coronary anomalies
    • Introduction and background
    • Diagnostic evaluation
    • Surgical treatment

Quality Indicators    

Gaps in evidence

  • General aspects
    • Organization of care and patient evaluation
    • Heart failure
    • Arrhythmia
    • Pulmonary arterial hypertension
    • Cyanotic patients
  • Specific lesions
    • Shunt lesions
    • Left ventricular outflow tract obstruction & coarctation
    • Aortopathies
    • Right ventricular outflow tract obstruction
    • Ebstein anomaly
    • Tetralogy of Fallot
    • Transposition of the great arteries
    • Congenitally corrected transposition of the great arteries
    • Univentricular heart and Fontan operation
    • Coronary anomalies

Key messages            

  • General aspects
  • Organization of care and patient evaluation
  • Heart failure
  • Arrhythmia
  • Pulmonary arterial hypertension
  • Cyanotic patients
  • Specific lesions
    • Shunt lesions
    • Left ventricular outflow tract obstruction
    • Aortic coarctation
    • Aortopathies
    • Right ventricular outflow tract obstruction
    • Ebstein anomaly
    • Tetralogy of Fallot
    • Transposition of the great arteries
    • Congenitally corrected transposition of the great arteries
    • Univentricular heart and Fontan operation
    • Coronary anomalies

‘What to do’ and ‘what not to do’ messages          

Appendix

References   

Previous version available to download
Published in 2010
Reference European Heart Journal (2010) 31, 2915–2957; doi:10.1093/eurheartj/ehq249