Skip to main content

Health condition

Adult congenital heart disease, from repaired ASDs to Fontan circulations.

A cardiac repair in childhood is not the end of the story. Lifelong specialist follow-up keeps late complications small and manageable.

Jump to treatment
A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Editorial team

    Written by our editorial team from the UK sources listed on each page.

  • 02

    Sourced from guidance

    Checked against NICE, ESC, ISACCD Bethesda and specialist ACHD centre standards you can see at the end.

  • 03

    Current for 2026

    Reflects modern UK ACHD care including catheter valve options, ICDs and pre-conception counselling.

Key facts

Adult congenital heart disease at a glance.

The essentials, in plain English. What ACHD is, why it needs specialist care, and what a lifetime of follow-up actually looks like.

  • What it is

    Structural heart defects present from birth and now managed into adulthood, thanks to decades of paediatric cardiac surgery.

  • A growing population

    There are more adults living with congenital heart disease in the UK than children, and the numbers keep rising.

  • Simple to complex

    Ranges from small ASD, VSD, PDA or bicuspid aortic valve to repaired tetralogy of Fallot, coarctation, Ebstein and Fontan circulations.

  • Lifelong follow-up

    Even a well-repaired heart is not a cured heart. Specialist ACHD follow-up is for life, not for a discharge letter.

  • Specialist centres

    Complex care is concentrated in around ten UK ACHD centres with dedicated imaging, electrophysiology and surgery.

  • Pregnancy needs planning

    The modified WHO (mWHO) class guides risk. Pre-conception counselling is essential, especially for moderate and complex disease.

Why this guide matters

Repaired is not cured.

Adults with congenital heart disease now outnumber children. The three ideas below shape everything else on this page.

  • Lifelong specialist follow-up

    A well-repaired heart still needs periodic imaging, rhythm checks and exercise testing at a network ACHD centre. Do not discharge yourself.

  • Arrhythmia drives outcomes

    Atrial and ventricular arrhythmias are the commonest late complications after Fontan, tetralogy repair and transposition surgery. New palpitations deserve prompt review.

  • Plan pregnancies in advance

    The mWHO classification stratifies risk. Pre-conception counselling and joint obstetric-cardiac delivery planning are essential in moderate and complex disease.

How the assessment is made

From surgical history to a clear ACHD plan.

The steps a UK ACHD centre will normally follow, in order, so you know what to expect and why.

  1. 01

    Assessing

    History and lesion mapping

    A careful review of the original diagnosis, every operation and catheter procedure, and current symptoms including exercise tolerance.

  2. 02

    Assessing

    Examination and functional class

    Saturations, heart sounds, scars, NYHA class and any signs of cyanosis, arrhythmia or heart failure.

  3. 03

    Assessing

    ECG and Holter

    Baseline rhythm, conduction and QRS duration. Ambulatory monitoring picks up atrial and ventricular arrhythmias.

  4. 04

    Confirming

    Echocardiography with ACHD protocol

    Dedicated views for repaired anatomy, residual shunts, valve function, ventricular size and pulmonary pressures.

  5. 05

    Confirming

    Cardiac MRI and CT

    MRI is the gold standard for right ventricular volumes, pulmonary regurgitation and Fontan flows. CT for coronaries, stents and coarctation.

  6. 06

    Confirming

    CPEX and biomarkers

    Cardiopulmonary exercise testing quantifies functional capacity. BNP or NT-proBNP flags decompensation. Haemoglobin and haematocrit for cyanotic patients.

  7. 07

    Preparing

    Specialist ACHD centre referral

    Every moderate and complex lesion belongs in a network centre for imaging, electrophysiology, intervention and multi-disciplinary planning.

Typical timeline: a first specialist visit to a full ACHD plan in a few weeks.

Symptoms

What late complications actually look like.

The everyday signs that a repaired ACHD circulation is starting to strain, and the features that need urgent specialist review.

  • Reduced exercise tolerance

    Breathlessness, fatigue or leg heaviness on exertion is often the first sign that the circulation is under strain.

  • Palpitations

    Atrial arrhythmias are the commonest complication of repaired ACHD. Any new palpitation deserves a Holter and specialist review.

  • Cyanosis and clubbing

    Blue lips, blue nail beds and finger clubbing suggest a right-to-left shunt or Eisenmenger physiology.

  • Peripheral oedema

    Ankle and abdominal swelling can reflect right heart failure, protein-losing enteropathy or Fontan-related venous congestion.

  • Chest and sternal scars

    A midline sternotomy or thoracotomy scar is a prompt to ask about the original repair and any residual lesions.

  • Syncope or presyncope

    Blackouts in an ACHD patient are ventricular arrhythmia until proven otherwise and need urgent specialist assessment.

  • Progressive heart failure

    Orthopnoea, weight gain and reduced appetite may signal systemic or subpulmonary ventricular dysfunction.

  • Red flag - endocarditis features

    Persistent fever, night sweats and new murmur in a patient with prosthetic material or residual shunt needs urgent blood cultures and echo.

Treatment

How ACHD is managed in the UK.

Lifelong specialist review, selective catheter or surgical intervention, arrhythmia and heart failure care, and dedicated pathways for pregnancy and transplant.

  • Specialist ACHD follow-up

    Lifelong review at a network centre with periodic imaging and CPEX. Frequency scales with lesion complexity. Never fully discharged.

  • Catheter closure of shunts

    Selected secundum ASDs, PDAs and some VSDs are closed percutaneously with dedicated devices, avoiding open surgery.

  • Catheter valve intervention

    Balloon or stent for pulmonary stenosis and coarctation. Melody or Sapien valves for pulmonary regurgitation. TAVI for selected aortic disease.

  • Redo cardiac surgery

    Pulmonary valve replacement after tetralogy repair, tricuspid surgery in Ebstein, conduit revision and complex reoperations in specialist theatres.

  • Arrhythmia management

    Catheter ablation for atrial tachycardia and flutter, antiarrhythmic drugs, ICDs for high-risk ventricular arrhythmia and CRT in selected heart failure.

  • Heart failure medical therapy

    ACE inhibitors, beta-blockers, MRAs and SGLT2 inhibitors are tailored to the systemic ventricle. Loop diuretics for congestion.

  • Pregnancy and contraception plan

    Pre-conception counselling using mWHO class, safe contraception advice and joint obstetric-cardiac delivery planning at a tertiary centre.

  • Transplant and Fontan pathways

    Heart or heart-lung transplant for end-stage disease, and dedicated pathways for failing Fontan physiology and Eisenmenger syndrome.

What this guide is based on

The sources behind every claim on this page.

UK national standards, European Society of Cardiology and ISACCD Bethesda guidance, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your ACHD team knows your specific anatomy and history and can tell you which parts apply to you. If in doubt, contact your specialist centre.

  • NICE. Congenital heart disease standards and specification for ACHD services.

  • European Society of Cardiology (ESC). Guidelines for the management of adult congenital heart disease.

  • ISACCD Bethesda Conference. Care of the adult with congenital heart disease.

  • NHS England. Service specification for adult congenital heart disease.

  • British Cardiovascular Society and BCCA. Standards for ACHD networks in the UK.

Red flags

When ACHD needs urgent attention.

Most ACHD is managed through planned follow-up. These are the situations that break out of the schedule and need a specialist review the same day.

  • New syncope

    A blackout in a patient with repaired tetralogy, Fontan or transposition is high risk for ventricular arrhythmia. Same-day specialist review.

  • Sustained palpitations

    New atrial flutter, atrial tachycardia or AF in ACHD tolerates poorly and often needs urgent rate or rhythm control.

  • Rising cyanosis

    Falling saturations or worsening blue discolouration warrants urgent ACHD assessment and haemoglobin review.

  • Fever with prosthetic material

    Any unexplained fever in a patient with a conduit, patch, prosthetic valve or shunt is endocarditis until proven otherwise.

  • Neurological deficit

    Stroke or TIA in ACHD raises the risk of paradoxical embolism, arrhythmia and endocarditis and needs urgent imaging.

  • Haemoptysis

    Coughing up blood in Eisenmenger or a Fontan circulation is a serious sign and needs immediate hospital review.

  • Rapid weight gain

    Two to three kilos in a week in a Fontan or single-ventricle circulation suggests decompensation and needs urgent diuretic review.

  • Pregnancy in complex disease

    Unplanned pregnancy in mWHO class III or IV disease is high risk and needs urgent specialist counselling.

  • Loss to follow-up

    Patients who fell out of specialist care as teenagers frequently present late with arrhythmia or heart failure. Re-engagement is a priority.

Living with it

A lifelong condition, with a clear plan.

Four habits that make the biggest difference: keeping specialist appointments, staying active within tailored advice, planning pregnancies and travel, and looking after your teeth.

A quiet reminder

Stay connected to your ACHD centre.

Late problems are much easier to manage when they are picked up early on planned imaging, rather than late in an emergency department.

  1. 01 Follow-up

    Never miss a specialist review

    Even if you feel completely well, keep your ACHD appointments. The imaging and rhythm checks pick up problems before symptoms do.

  2. 02 Activity

    Stay active within advice

    Most patients benefit from regular moderate aerobic exercise. Your ACHD team will tailor guidance to your lesion and rhythm.

  3. 03 Planning

    Plan pregnancies and travel

    Discuss contraception, pregnancy and long-haul travel with your ACHD team. Altitude and prolonged immobility carry specific risks.

  4. 04 Dental

    Look after your teeth

    Good oral hygiene reduces endocarditis risk. Ask your ACHD team whether you need antibiotic cover before dental work.

Frequently asked

Everything we get asked about ACHD.

Quick answers on follow-up, complications, specialist centres, pregnancy and reoperation.

  • What is adult congenital heart disease?

    It is the umbrella term for structural heart defects that were present at birth and are now being managed in adulthood. It covers everything from a small atrial septal defect through to complex single-ventricle circulations after Fontan surgery. Modern paediatric cardiac care means most children with congenital heart disease now reach adulthood.

  • Do I really need lifelong follow-up if my repair went well?

    Yes. Even the best repair is a repair, not a cure. Late problems such as arrhythmia, valve regurgitation, ventricular dysfunction and pulmonary hypertension can appear decades later without symptoms. Regular imaging, rhythm monitoring and exercise testing at a specialist ACHD centre pick these up early.

  • Why does my care need to be in a specialist centre?

    ACHD combines unusual anatomy with adult cardiology problems. Only around ten UK centres have the imaging, electrophysiology, cardiac surgery and multi-disciplinary teams needed for moderate and complex disease. Local shared care is fine for simple lesions, but complex care belongs in a network centre.

  • What are the biggest complications to watch for?

    Arrhythmias, particularly atrial flutter and atrial tachycardia after Fontan and ventricular arrhythmia after tetralogy repair, are the commonest. Progressive valve disease, ventricular dysfunction, pulmonary hypertension, heart failure, endocarditis and, rarely, sudden cardiac death are the others we plan against.

  • Can I have children safely?

    Many women with ACHD can have safe pregnancies, but it depends on your lesion, function and rhythm. The modified WHO (mWHO) classification stratifies risk. Pre-conception counselling at a specialist centre is essential, and delivery is usually planned in a joint obstetric-cardiac unit.

  • Will I need more operations?

    Some patients do. Pulmonary valve replacement after tetralogy repair, catheter or surgical valve replacement, coarctation stenting, arrhythmia ablation and, occasionally, transplant are all part of long-term ACHD care. Your team will time any intervention against clear imaging and functional criteria rather than symptoms alone.

Related content

Keep reading.