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2020 ESC Guidelines for Adult Congenital Heart Disease

Clinical Quick Reference — Diagnosis, Management, and Intervention

Published: European Heart Journal (2021) 42: 563–645
Societies: European Society of Cardiology (ESC), AEPC, ISACHD
DOI: 10.1093/eurheartj/ehaa554
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ACHD Complexity Classification

Three-level stratification to guide care intensity and expertise requirements.

MILD Complexity

  • Isolated aortic/mitral valve disease, bicuspid aortic valve
  • Mild pulmonary stenosis (infundibular, valvular, supravalvular)
  • Small ASD, VSD, or PDA
  • Repaired secundum ASD, sinus venosus defect, VSD, PDA without sequelae

MODERATE Complexity

  • Anomalous pulmonary venous/coronary connections
  • Aortic stenosis, AVSD, coarctation, Ebstein, Marfan syndrome
  • Moderate PDA, double-chambered RV
  • Repaired ToF, TGA (arterial switch)

SEVERE Complexity

  • Any cyanotic CHD with pulmonary vascular disease
  • Unoperated or palliated cyanotic defects
  • Fontan, TGA (non-arterial switch), truncus, DORV, univentricular heart
  • Pulmonary atresia, interrupted aortic arch

Infectious Endocarditis Prophylaxis

High-risk: prosthetic valves, previous IE, cyanotic lesions, repaired defects with residual shunts.

Prophylaxis Strategy

  • Antibiotic regimens per 2015 ESC IE Guidelines
  • Dental: amoxicillin 2 g PO 30–60 min before (or cephalexin/clindamycin if allergic)
  • GI/respiratory procedures: antibiotic cover for high-risk patients
  • Lifelong counseling on endocarditis risk; regular dental care; avoid piercings/tattoos

Arrhythmia Management

Arrhythmias are common in ACHD (AF, IART, VT). Risk varies by lesion type and surgical history.

Risk Stratification by Lesion

CHD Type SVT Risk AF Risk VT/SCD Bradycardia
ASD Low Very High Low Low
VSD Low Low Low Low
Ebstein Very High Moderate Low–Mod Moderate
ToF Moderate Moderate Moderate Low
TGA (A-switch) Very High Very High Moderate Low
Fontan Moderate Moderate Moderate Moderate

Treatment Approach

Symptomatic Arrhythmias

Referral to ACHD centre with EP expertise mandatory. 1
Catheter ablation: Recommended in mild CHD (SVT, VT) if performed in experienced centres. 2a
Medical therapy: Beta-blockers, class IC drugs, or amiodarone if ablation not feasible.
ICD consideration: Primary prevention in severe RV dysfunction + sustained VT or high SCD risk; secondary prevention after cardiac arrest.

Heart Failure in ACHD

Affects 20–50% of ACHD population; leading cause of death. Due to ventricular dysfunction, arrhythmias, or cyanosis.

HF Management

Comprehensive evaluation: Echo (RV/LV function, valves), CMR (volumes, EF, scar). 1
ACE-I / ARB + beta-blockers for systemic LV/RV dysfunction. 2a
Diuretics, aldosterone antagonists, device therapy (CRT, ICD), transplant evaluation in expert centres.

Risk calculator: MAGGIC HF Risk

Pulmonary Hypertension in ACHD

Major risk factor for adverse outcomes, especially in cyanotic lesions and Eisenmenger syndrome.

PH Classification

Type Haemodynamic Definition Clinical Setting
PH Mean PAP ≥20 mmHg All ACHD
Pre-capillary PH PAP ≥20 + PAWP ≤15 + PVR ≥3 WU Shunt lesions, Eisenmenger, complex CHD
Post-capillary PH PAP ≥20 + PAWP >15 + PVR <3 WU Venous obstruction, AV valve disease
Combined PAP ≥20 + PAWP >15 + PVR ≥3 WU Shunt + LV dysfunction, Fontan + PH

PAH-CHD Treatment

Recommendations

  • Counsel against pregnancy; assess 6-minute walk, O₂ sat. 1
  • Low/intermediate-risk: oral combination therapy (PDE-5i + ERA). 1
  • Eisenmenger (6MWT <450 m): initial ERA monotherapy, then combination if needed. 2a

Calculate PVR: PVR Calculator | Cardiac Output (Fick)

Atrial Septal Defect (ASD)

Most common simple lesion. Management depends on RV volume overload, PAH, paradoxical embolism, LV disease.

Closure Decision

RV volume overload? Device closure indicated when feasible. 1
Paradoxical embolism (stroke)? Closure recommended. 2a
PAH (PVR ≥3 WU)? Consider closure after catheter assessment & balloon testing if Qp:Qs >1.5. 2a
Severe PAH (PVR ≥5 WU) without significant L→R shunt? Not recommended. 3

Follow-up

Echo at 3, 6, 12 months, then annually. Assess residual shunt, thrombus, arrhythmias, RV remodeling.

Ventricular Septal Defect (VSD)

Often closes spontaneously in childhood. Adult concerns: endocarditis risk, LV dilation, PAH, aortic regurgitation.

Closure Framework

LV volume overload? Surgical or transcatheter closure indicated. 1
No significant shunt, normal LV/RV? Conservative management; lifelong endocarditis prophylaxis counseling.
Residual shunt post-repair (Qp:Qs >1.5)? Consider transcatheter closure. 2a
Desaturation + PAH or severe restrictive VSD? Not recommended. 3

Atrioventricular Septal Defect (AVSD)

Central defect involvement; repaired AVSD may have residual AV regurgitation, arrhythmias, LV dysfunction.

Management

  • Surgical repair: Recommended in children with RV overload and/or PAH. 1
  • AV valve repair: Preferred over replacement. 1
  • Adult reoperations: Congenital surgeon expertise essential. 1
Progressive AV regurgitation: Most common long-term problem. Monitor every 1–3 years. Valve replacement indicated if severe + symptomatic or LVEDD >50 mm.

Tetralogy of Fallot (ToF)

Most common cyanotic defect reaching adulthood. Post-repair concerns: pulmonary regurgitation (PR), RV dilation, arrhythmias, sudden cardiac death.

SCD Risk Markers

  • QRS >180 ms: RV dilation and arrhythmia risk marker
  • Severe PR (RVEDI >160 mL/m²): RV dysfunction and VT risk
  • RVEF <35%: Advanced RV dysfunction
  • Sustained VT/VF history: High SCD risk; ICD consideration

Pulmonary Valve Replacement

Indications

Severe PR + RV dilation (RVEDI >170 mL/m²) + symptoms or dysfunction? Replacement indicated. 1
Asymptomatic, severe PR, progressive RV dilation? Consider replacement to prevent deterioration. 2a

Transposition of Great Arteries (TGA)

Arterial switch, Rastelli, or atrial switch (older). Long-term complications: coronary insufficiency, neoaortic regurgitation, arrhythmias, baffle obstruction.

Post-Arterial Switch Complications

  • Coronary anomalies: Noninvasive imaging (CMR stress, PET) recommended; surgical revision if symptomatic
  • Neoaortic regurgitation: Progressive; monitor echo. Valve replacement if severe + symptoms/LV dysfunction
  • Arrhythmias (post-A-switch): Rare

Post-Atrial Switch Complications

  • Baffle obstruction/leaks: Balloon angioplasty or re-do surgery
  • IART/AF: Very high incidence; EP management essential
  • Systemic RV dysfunction: Progressive

Fontan Circulation

Systemic vein → pulmonary artery direct connection for univentricular repair. Long-term complications: arrhythmias, hepatic dysfunction, ventricular deterioration, thromboembolism.

Fontan Follow-up

Annual: Clinical, ECG, echo (ventricular function, flow). 1
Every 2–3 years: CMR (volumes, EF, scar); cardiac cath if declining function.
Arrhythmia: EP evaluation; ablation or pacing may be considered. 2a
Anticoagulation: Aspirin or warfarin based on thromboembolic risk and rhythm.
Fontan-associated liver disease (FALD): Progressive hepatic fibrosis. Screen with elastography annually; assess for varices if cirrhosis present.

Coarctation of the Aorta

Native or repaired coarctation: hypertension, LV hypertrophy, premature CAD, aortic aneurysm, cerebral aneurysms (Turner syndrome).

Long-term Management

  • Hypertension: Present in 80% repaired; aggressive management (ACE-I, ARB)
  • Aortic aneurysm: Monitor CMR/CT every 3–5 years
  • Bicuspid aortic valve: Assess for stenosis/regurgitation
  • Turner syndrome: Intracranial aneurysm screening MRI recommended

Re-intervention Indications

Catheter or Surgery

  • Native/recoarctation with peak gradient ≥20 mmHg + symptoms/hypertension. 1
  • Stent placement (hybrid) for complex anatomy or high surgical risk. 2a

Left Ventricular Outflow Tract (LVOT) Obstruction

Valvular, subvalvular, supravalvular aortic stenosis (AS). Progressive nature may necessitate intervention.

AS Severity Grading

Grade Vmax (m/s) Mean Gradient (mmHg) AVA (cm²)
Mild 2.6–2.9 <20 >1.5
Moderate 3.0–3.9 20–39 1.0–1.5
Severe ≥4.0 ≥40 <1.0

Intervention Decisions

Management

Severe AS (Vmax ≥4.0) + symptoms? Surgery indicated. 1
Severe AS (Vmax ≥4.0) + asymptomatic + abnormal exercise test? Consider intervention. 2a
Moderate AS (Vmax 3.0–3.9)? Conservative management; exercise testing for risk stratification.

Cyanotic Congenital Heart Disease

Specialized management to prevent thromboembolism, stroke, endocarditis, and organ dysfunction.

Risk Reduction Strategies

Complication Prevention
Thromboembolism Avoid dehydration; VKA (INR 2–2.5); air filters; compression stockings
Stroke / Abscess Endocarditis prophylaxis; iron supplementation; avoid hypoxia
Iron Deficiency Monitor ferritin; supplement if deficient (avoid overload)
Erythrocytosis Venesection only if Hct >65% + symptoms OR if iron replete; target 50–55%
Arrhythmias Multidisciplinary EP management; ablation in specialized centres

Eisenmenger Syndrome

Recommendations

  • PAH-targeted therapy (PDE-5i ± ERA) if symptomatic or declining 6MWT. 1
  • Pregnancy contraindicated; maternal mortality 40–100%. 1
  • Counsel against strenuous exercise; moderate activity in asymptomatic patients acceptable.

Annual Monitoring

  • Blood: haematocrit, iron studies, urate, coagulation function
  • Renal: proteinuria, creatinine (common abnormalities)
  • Hyper-viscosity: headache, blurred vision → venesection if symptomatic
  • Gout: allopurinol prophylaxis if recurrent

Pregnancy, Contraception, Genetics

Variable risk depending on disease complexity. Multidisciplinary pre-conception counseling essential.

High-Risk Pregnancy Categories

Significantly Increased Risk (19–27%) Extremely High Risk (40–100%)
Unoperated cyanotic CHD, moderate LV impairment (EF 30–45%), systemic RV, moderate AS/MS PAH, severe LV impairment (EF <30%), severe mitral/aortic stenosis, severe aortic dilation (>45 mm), Fontan + complications

Contraception Counseling

  • Combined oral contraceptives: Acceptable in low-risk ACHD; avoid cyanosis/thrombophilia
  • Progestin-only: Safe for most
  • IUDs: Generally safe; consider antibiotic prophylaxis if high endocarditis risk
  • Permanent methods: Tubal ligation/vasectomy in very high-risk patients

Genetic Counseling & Recurrence Rates

Recurrence varies by lesion and parental gender. Genetic testing recommended for syndromes (Marfan, Turner, DiGeorge, Down). Prenatal counseling and detailed genetic consultation essential.
Lesion Women (%) Men (%)
ASD4–61.5–3.5
VSD6–102–3.5
AVSD11.5–141–4.5
PDA3.5–42–2.5
Coarctation4–6.52–3.5
ToF2–2.51.5

Exercise, Sports, Non-cardiac Surgery

Exercise Recommendations

General Approach

  • Pre-participation exercise testing recommended for moderate/severe ACHD. 1
  • Mild ACHD: moderate activity well-tolerated; no restrictions unless symptoms develop.
  • Complex ACHD (Fontan, cyanosis, PAH): individualized assessment; typically low-intensity recommended.

Non-cardiac Surgery

Perioperative risk: ACHD patients have increased mortality, especially complex lesions. Surgery should occur in specialized centres with ACHD expertise. Anaesthetic planning crucial: avoid hypoxia, maintain haemodynamic stability, prevent paradoxical embolism.

Estimate surgical risk: EuroSCORE II

Related Calculators

Specialized tools for ACHD risk assessment and haemodynamic estimation:

EuroSCORE II

Cardiac surgery risk. Adapted for ACHD patients.

CHA₂DS₂-VASc

Stroke risk in AF. Useful for ACHD with atrial arrhythmias.

HAS-BLED

Bleeding risk on anticoagulation. Essential for Fontan and cyanotic patients on VKA.

CKD-EPI eGFR

Renal function estimation. Critical for cyanotic ACHD nephropathy.

Pulmonary Vascular Resistance

Calculate PVR from cath data. Essential for PAH-CHD decisions.

Corrected QT (QTc)

QTc interval calculation. Monitor on antiarrhythmic drugs.

MAGGIC Heart Failure

Mortality in HF. Applicable to systemic/RV dysfunction.

Cardiac Output (Fick)

Calculate CO from oxygen consumption. Useful in cath lab.

TAPSE & RV PAP

RV function and PAP from echo. Quick ACHD follow-up assessment.