Paediatric Brugada syndrome: fever-triggered arrhythmias

Brugada syndrome is an inherited disturbance of cardiac electrical activity, most often associated with reduced sodium-channel function. It predisposes to ventricular tachycardia, ventricular fibrillation and sudden cardiac arrest. In younger patients, signs may be subtle, and the first major event can occur during a febrile illness. Fever is therefore more than a background symptom: it is a clinically important trigger that deserves a specific response plan.

Children may present with syncope, seizure-like episodes or palpitations rather than a recognisable arrhythmia. A normal resting ECG does not exclude the condition, while a fever-related ECG change may be the only clue. Distinguishing an inherited arrhythmic disorder from seizures, structural heart disease, long QT syndrome and other causes of collapse must not delay urgent care.

Care should combine paediatric cardiology, inherited arrhythmia expertise and a family-centred plan. Acute ventricular arrhythmias, fever control, risk assessment, device decisions and screening of relatives all matter. Evidence specific to children remains limited, so adult recommendations require careful adaptation to age, symptoms, ECG findings and family history.

Recognise the pattern in a feverish child

A presentation often begins in an emergency department. Australian parents may describe a child who was “hot and flustered”, then pale, sweaty and “passed out” for a few seconds. Timing matters: collapse during fever, unusual breathlessness, a seizure after previously normal activity or sustained palpitations should prompt urgent assessment and an ECG. These episodes should not be labelled benign fainting or epilepsy without review.

Record a 12-lead ECG as soon as practical, particularly during fever, and compare it with previous tracings. Place the right precordial leads in the second, third and fourth intercostal spaces as well as standard positions when experienced staff are available. Brugada changes can be intermittent, and fever can unmask them. A tracing obtained after recovery remains useful but should not replace assessment during the acute episode.

Ask about palpitations, chest pain, breathlessness, syncope and seizures, including whether they occurred in a hot bath, following exertion or during infection. Medication history matters because sodium-channel-blocking drugs can provoke the phenotype. A child with collapse during fever needs emergency paediatric review even if the ECG appears normal; reassurance without a documented rhythm and specialist plan can miss a life-threatening diagnosis.

Confirm the diagnosis and assess the family

Diagnosis rests on the ECG pattern, clinical context and family history, with supportive genetic testing. A spontaneous type 1 pattern shows coved ST-segment elevation of at least 2 mm in a right precordial lead, usually V1 or V2. It is more informative when standard leads are supplemented by high right precordial recordings. Fever alone can produce a Brugada-like pattern, so a paediatric electrophysiologist should interpret the combination rather than apply one tracing in isolation.

Specialist drug testing with ajmaline, flecainide or procainamide may reveal a concealed pattern when suspicion remains high. These agents can provoke malignant arrhythmias and should be used only under continuous monitoring with resuscitation capability. A drug-induced ECG change does not automatically mean a child needs an implantable cardioverter-defibrillator; symptoms and family history still guide risk assessment.

Family evaluation should include ECG screening and, where appropriate, genetic counselling and testing for a pathogenic variant in SCN5A or another relevant gene. A variant can clarify inheritance even when a relative has few symptoms. Relatives should be told that an ECG may be normal, that fever can alter its appearance, and that new collapse or palpitations requires prompt medical review.

Assess risk in the individual child

Risk stratification is individual. Important features include a spontaneous type 1 ECG, previous ventricular arrhythmia or syncope, a family history of premature sudden death, and the circumstances of any event. Fever-provoked and drug-unmasked patterns require separate interpretation from a spontaneous type 1 pattern. The absence of an event does not eliminate inherited susceptibility, but it can materially lower short-term risk in a young asymptomatic child.

Most children without previous arrhythmia or cardiac arrest are managed without prophylactic defibrillator implantation. Ventricular fibrillation carries a different weight from an isolated fever-related blackout, and recurrent polymorphic ventricular tachycardia may shift management towards intensive treatment. Decisions should involve a team experienced in paediatric inherited arrhythmia, with age, growth and competing device risks considered from the outset.

Conduction disease is not required for Brugada syndrome, and bradycardia should prompt assessment for other structural or genetic causes. If symptomatic atrioventricular block develops, device selection becomes separate from preventing ventricular fibrillation. A useful case-based discussion of conduction system pacing shows how anatomy and growth influence pacing feasibility in young patients.

Treat a fever-triggered electrical storm

Recurrent polymorphic ventricular tachycardia or ventricular fibrillation during fever constitutes an electrical storm and requires immediate resuscitation and paediatric intensive care. Defibrillation is used for ventricular fibrillation or unstable sustained ventricular tachycardia according to emergency protocols. Continuous ECG monitoring, intravenous access and blood sampling are essential while the team identifies an infection, corrects fever and suppresses recurrent arrhythmias.

Isoproterenol or quinidine may be used under specialist supervision to reduce recurrent ventricular arrhythmias in Brugada electrical storms. Supportive treatment includes treating the infection source, giving weight-appropriate antipyretics when oral treatment is not possible, and maintaining careful fluid balance. Paracetamol and ibuprofen can help fever, but dose, hydration status and the underlying illness determine safety; aspirin should not be used as a general fever medicine in children.

Transfer to a centre with paediatric electrophysiology and inherited arrhythmia surgery matters when shocks continue. Catheter ablation of the triggering right ventricular outflow tract substrate, often performed epicardially in experienced hands, can reduce recurrences in refractory storms. Ablation is technically demanding in small children and may require repeat procedures. It complements medication and device management rather than automatically replacing them.

Choose devices and procedures carefully

For a child who has survived ventricular fibrillation, an implantable cardioverter-defibrillator is often indicated. The device can terminate a lethal rhythm, yet it does not prevent ventricular tachycardia, and shocks may cause pain, fear and lasting psychological harm. Repeated shocks, infection, lead fracture, growth-related displacement and eventual battery depletion are especially important concerns in a body that is still developing.

Prophylactic implantation in an asymptomatic child is generally avoided unless the specialist team identifies a compelling indication. When recurrent storms persist despite medication, ablation and device optimisation may be needed. A subcutaneous defibrillator should not be assumed to solve the problems of a small child, because size, lead durability and the need for prolonged support must be considered. Follow-up must include psychological care for the child, parents and siblings.

Technical problems can require removal of infected hardware or revision of damaged leads, particularly as the child grows. Lead and generator infection can threaten bloodstream infection and device function. Specialist expertise in abandoned lead management is relevant when definitive extraction is unsafe or only part of a lead can be retrieved, because retained fragments require imaging, infection surveillance and an individualised plan.

Build an Australian response plan

The family should leave with a written fever and arrhythmia plan that any treating clinician can follow. It should specify whom to call, where to attend and which symptoms require an ambulance. Collapse, a seizure associated with fever, severe breathlessness, chest pain or sustained palpitations warrants urgent assessment; call 000 if the child is unresponsive or not breathing normally. Antipyretics should be given according to weight and local medical advice, with prompt treatment of ear, urinary, skin or respiratory infections.

School and childcare staff need a medical summary, emergency contacts and clear instructions about early escalation. Teachers should know that a child who becomes pale, confused or collapses needs immediate medical attention and cardiopulmonary resuscitation if unresponsive. Activity advice should be individualised, especially after previous events, while avoiding unnecessary exclusion from ordinary play. Travel plans should include the fever plan, medication list, specialist contacts and access to paediatric care.

Australian families usually enter specialist care through a general practitioner and a paediatric cardiology referral. Public paediatric cardiac clinics often bulk-bill consultations, while private electrophysiology services may charge more than the Medicare rebate, leaving a gap. Major inherited arrhythmia teams at the Royal Children’s Hospital in Melbourne and Sydney Children’s Hospital coordinate complex care; the Queensland Children’s Hospital in Brisbane provides another paediatric cardiac referral option.

Regional and remote care requires a nominated paediatric cardiologist, telehealth review and accessible ECG records to prevent repeated testing. The Royal Flying Doctor Service can provide aeromedical retrieval when a child becomes critically unwell in remote parts of Northern Territory, South Australia or Western Australia. A handover summary should state previous ventricular arrhythmias, medications, implanted devices and outstanding results, rather than simply recording “Brugada syndrome”.

Arrange an urgent inherited arrhythmia referral for any child with syncope or a seizure during fever, request paediatric ECG interpretation and provide a written fever plan before discharge. Make the plan available to the family, general practitioner, school and ambulance service, and arrange relative screening with genetic counselling. Consistent documentation and a rehearsed escalation pathway give the child the best chance of avoiding a recurrent lethal arrhythmia.