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.

Topic(s):

Congenital Heart Disease and Pediatric Cardiology

Pediatric Cardiology

Adult Congenital Heart Disease

CV Surgery - Congenital Heart Disease

Guidelines version available to download

Full text

Published in 2020

Reference doi:10.1093/eurheartj/ehaa554

Pocket guidelines

Published in 2020

Reference TOC ACHD Pocket Guidelines

App

Published in 2020

Reference Pocket Guidelines App

Slide-set

Published in 2020

Reference 108 Slides on ACHD

Declarations of interest

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

Full text

Published in 2010

Reference European Heart Journal (2010) 31, 2915–2957; doi:10.1093/eurheartj/ehq249

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

ESC Clinical Practice Guidelines