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