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.
Key red flags
New cyanosis below baseline, syncope, sustained tachyarrhythmia/bradyarrhythmia, pulmonary oedema, low-output state, haemoptysis, focal neurology or fever with possible endocarditis.
Investigation priorities
Define anatomy and the patient's normal physiology before labelling a new abnormality.
First-line assessment of anatomy, ventricular function, valves, gradients, shunts and estimated pulmonary pressure.
Management branches
New cyanosis, syncope, arrhythmia, heart failure, chest pain, haemoptysis, neurological deficit or sepsis.
- 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.
- 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.
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.
Bosentan62.5 mg orally twice daily for 4 weeks, then 125 mg twice daily if tolerated.