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Educational draft · awaiting clinical reviewUse Rapid for revision, not patient-care decisions. Check current national and local guidance and the BNF or BNFC before acting.

Congenital heart disease in adults

Cardiology · UK scope · Sources checked 25 Aug 2026 · Clinical review pending

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New cyanosis, syncope, sustained arrhythmia, decompensated heart failure, chest pain, haemoptysis, neurological deficit or suspected endocarditis in an adult with congenital heart disease warrants urgent hospital assessment and early contact with the regional ACHD centre. Sudden blue/grey colour with breathing difficulty, chest pain, confusion, dizziness or rapid/irregular heartbeat is a 999 emergency.

Synopsis

Recognise repaired and unrepaired congenital heart disease, identify ACHD emergencies and pregnancy risk, and route lesion-specific surveillance and intervention through specialist services.

  • Never assume childhood repair equals cure: residual shunts, valve disease, ventricular dysfunction, aortopathy, pulmonary hypertension and scar-related arrhythmia may emerge decades later.
  • Identify the exact anatomy, operations/interventions, ventricular physiology, shunts/conduits, devices, baseline saturation and usual ECG before interpreting acute findings.
  • Transthoracic echo is first-line; CMR is central for right-ventricular volumes, flows and extracardiac anatomy, while CT helps with coronary arteries, conduits, calcification and stent detail.
  • Moderate or complex ACHD and clinically significant residual lesions need lifelong specialist follow-up; involve ACHD expertise before invasive procedures, pregnancy or major non-cardiac surgery.
  • An ASD is closed for significant right-ventricular volume overload when pulmonary vascular resistance permits; device closure is preferred for suitable secundum anatomy.
  • Do not close a shunt blindly in pulmonary vascular disease. PVR at least 5 Wood units or desaturation/Eisenmenger physiology requires expert catheter assessment and may make closure harmful.
  • Coarctation clues include upper-limb hypertension, radiofemoral delay and an arm-leg systolic gradient at least 20 mmHg; surveillance continues after repair because recoarctation and aortic complications recur.
  • In repaired tetralogy of Fallot, falling exercise capacity, arrhythmia, worsening pulmonary regurgitation and progressive RV enlargement can trigger pulmonary-valve replacement assessment.
  • Routine antibiotic prophylaxis is not recommended by NICE solely because congenital heart disease is present. Maintain excellent oral health and investigate infection promptly; for people at high risk of infective endocarditis, the March 2026 SDCEP pathway offers prophylaxis for dental extraction/oral surgery and uses shared decision-making for procedures that manipulate gingiva or the periapical region.
  • Pregnancy may be extremely high risk in pulmonary arterial hypertension/Eisenmenger syndrome, severe ventricular dysfunction or major aortopathy; pre-conception ACHD–maternal medicine review is essential.

    • Key red flags

    Acute deterioration

    New cyanosis below baseline, syncope, sustained tachyarrhythmia/bradyarrhythmia, pulmonary oedema, low-output state, haemoptysis, focal neurology or fever with possible endocarditis.

    Repaired tetralogy of Fallot risk

    Palpitations, syncope, NSVT, QRS prolongation, declining exercise capacity, RV dilation/dysfunction or severe pulmonary regurgitation can precede ventricular arrhythmia or heart failure.

    Eisenmenger/hyperviscosity

    Central cyanosis, clubbing, erythrocytosis, headache, visual disturbance, dizziness, thrombosis or bleeding. First exclude dehydration and iron deficiency before attributing symptoms to hyperviscosity.

    High-risk pregnancy

    Pregnancy or pregnancy planning with pulmonary arterial hypertension/Eisenmenger physiology, severe ventricular dysfunction, Fontan failure or significant aortopathy needs urgent specialist counselling; PAH is a very high maternal-risk state.

    Investigation priorities

    01
    Retrieve operative/interventional records, baseline ECG and saturationFirst step

    Define anatomy and the patient's normal physiology before labelling a new abnormality.

    02
    Transthoracic echocardiographyFirst line

    First-line assessment of anatomy, ventricular function, valves, gradients, shunts and estimated pulmonary pressure.

    Management branches

    Emergency / protocol-dependentThe unwell ACHD patient

    New cyanosis, syncope, arrhythmia, heart failure, chest pain, haemoptysis, neurological deficit or sepsis.

    1. Use ABCDE, monitoring, 12-lead ECG, IV access and syndrome-directed resuscitation while contacting the regional ACHD centre early; retrieve the anatomy/operation record and baseline saturation.
    2. Use meticulous air-free IV technique and filters when a right-to-left shunt is possible. Avoid abrupt preload loss or systemic vasodilation in Fontan/cyanotic physiology without expert advice.
    3. Treat immediately reversible standard emergencies, but discuss cardioversion, pacing, ventilation, vasoactive drugs and invasive procedures with ACHD/anaesthesia teams because anatomy and access may be non-standard.
    4. Transfer to an ACHD-capable centre when complex physiology, sustained arrhythmia, decompensation, endocarditis or a procedure requiring specialist anatomy is present.

    Key medicines

    Aspirin after ASD device closure75 mg orally once daily for at least 6 months after device closure, unless the interventional ACHD plan specifies otherwise.Active bleeding, aspirin hypersensitivity, major GI risk and interacting antithrombotics; duration and combination therapy are device/procedure dependent.
    Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.Initiate only through a PAH-experienced service. Contraindicated in pregnancy and moderate/severe hepatic impairment; teratogenic, hepatotoxic and may cause anaemia/fluid retention. Hormonal contraception alone may be ineffective.
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    Sources and review status8 sources · checked 25 Aug 2026 · clinical review pending+
    Sources

    Sources and review status

    National guidance is shown before implementation-dependent detail. Typical adult dose examples remain subject to patient factors, contraindications and the live BNF or specialist protocol. Source check completed 25 Aug 2026; clinical approval remains outstanding.

    • 2020 ESC Guidelines for adult congenital heart diseasePrimary lesion-specific ACHD source where current NICE guidance is absent.
    • NHS: Congenital heart diseaseUK patient-facing recognition and emergency advice.
    • NHS England: Specialised services clinical network specificationsUK specialist-network context for congenital cardiac care.
    • NICE CG64: Prophylaxis against infective endocarditis
    • SDCEP: Antibiotic prophylaxis against infective endocarditis, second editionMarch 2026 UK dental pathway for high-risk patients, including procedures offered prophylaxis and those requiring shared decision-making.
    • Bosentan tablets SmPC
    • Sildenafil 20 mg tablets SmPC
    • Aspirin 75 mg gastro-resistant tablets SmPC

    Editorial contact: Doctor’s Passport editorial team. Source-check date is separate from clinical approval. Report a content problem · Content changes

    Authoring stateRapid draftClinical stateAwaiting reviewJurisdictionUnited Kingdom