Paeds · cardiology
Ventricular arrhythmias and sudden cardiac death
Also known as Ventricular tachycardia · VT · Ventricular fibrillation · VF · Sudden cardiac death · SCD in the young · Sudden arrhythmic death syndrome · SADS · Commotio cordis · Idiopathic ventricular fibrillation
Fellowship guide to ventricular arrhythmias and sudden cardiac death in children: distinguishing benign ventricular ectopy from sustained monomorphic and polymorphic VT and VF, the four cause families of SCD in the young (structurally normal heart, cardiomyopathy, congenital heart disease, acquired), the acute resuscitation of pulseless VT and VF with defibrillation and amiodarone, the synchronised cardioversion versus pharmacological termination of stable sustained VT, post-arrest targeted temperature management, ICD therapy and its paediatric-specific complications, the molecular autopsy and cascade screening, and the AHA/ACC/HRS and ESC guideline positions.
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Target exams
Red flags
- Any child with pulseless ventricular tachycardia or ventricular fibrillation is in cardiac arrest — defibrillate at 4 J/kg immediately and follow the shockable-rhythm loop of the paediatric advanced life support algorithm without waiting for drug access
- Sustained VT with a pulse and haemodynamic compromise (shock, altered consciousness, heart failure) requires synchronised DC cardioversion at 0.5–1 J/kg, escalating to 2 J/kg — do not delay for an intravenous antiarrhythmic in the unstable child
- The collapse of a previously well child or young athlete demands an urgent twelve-lead ECG, echocardiogram, electrolyte panel, and toxicology screen before any assumption of a primary neurological cause — inherited arrhythmia syndromes and cardiomyopathies are the missed diagnoses that kill
- A blow to the chest wall from a projectile (baseball, hockey puck, cricket ball) producing instantaneous collapse, with no structural cardiac injury, is commotio cordis — the impact lands on the vulnerable repolarisation window and triggers VF; survival requires immediate CPR and early defibrillation
- A child resuscitated from cardiac arrest must not be declared 'stable' simply because the rhythm has normalised — the first twenty-four hours carry the highest risk of re-arrest, and continuous cardiac monitoring in a high-dependency or PICU bed is mandatory
Life stages
Care settings
Clinical exam formats
Board mappings
- Cardiology
- Ventricular arrhythmias
- Sudden cardiac death in the young
- Paediatric Cardiology — ventricular tachycardia and fibrillation
- General Paediatrics learning goal — recognise and resuscitate the collapsed child
- ICD therapy and post-arrest care in children
- Clinical Applications
- Ventricular arrhythmias and SCD prevention
- Long Cases
- Short Cases
- Resuscitation scenarios
- Cardiology: recognises and manages ventricular arrhythmias
- Resuscitation: shockable and non-shockable rhythms
- Sudden cardiac death — prevention, screening and family follow-up
- Foundation of Practice (FOP)
- Applied Knowledge in Practice (AKP)
- Ventricular arrhythmias and sudden cardiac death
- Clinical
- History
- Communication
- Cardiovascular examination, ECG interpretation and resuscitation
- General Pediatrics Content Outline — Domain 9: Cardiology
- Subspecialty: Pediatric Cardiology — ventricular arrhythmias
- Sudden cardiac death risk stratification and prevention
- Patient Care 1: History and Physical Examination
- Patient Care 4: Clinical Reasoning and Resuscitation
- Medical Knowledge 1: Clinical Knowledge of ventricular arrhythmias and SCD
- Systems-Based Practice 1: multidisciplinary cardiac genetic and device care
- Medical Expert
- Collaborator
- Pediatrics: Core EPA — recognise and resuscitate ventricular arrhythmia
Overview & Definition
Picture the fourteen-year-old boy who collapses without warning on a soccer pitch. He was sprinting for the ball, there was no contact, and he is now pulseless and apnoeic. A bystander begins CPR and the automated external defibrillator advises a shock. That child carries the whole story of ventricular arrhythmias and sudden cardiac death in the young: a previously apparently well child who has a clinically silent substrate — a cardiomyopathy, a channelopathy, or a coronary anomaly — and in whom an electrical storm of ventricular tachycardia or ventricular fibrillation strikes without prodrome, often during exertion, and produces cardiac arrest. Whether he survives depends entirely on what happens in the next three minutes, and on whether the underlying cause is subsequently identified and treated. [1] [3]
Ventricular arrhythmias are abnormal rhythms originating below the atrioventricular node in the His-Purkinje system or the ventricular myocardium. They span a spectrum from premature ventricular complexes (isolated ectopic beats that are usually benign in the structurally normal heart), through non-sustained ventricular tachycardia (three or more consecutive ventricular beats lasting less than thirty seconds), to sustained ventricular tachycardia (lasting more than thirty seconds or producing haemodynamic compromise) and ventricular fibrillation (chaotic, disorganised electrical activity producing no effective cardiac output). The clinical urgency escalates along this spectrum, and the management divides sharply at the presence or absence of a pulse. [2] [4]
Sudden cardiac death is defined as an unexpected death from a cardiac cause occurring within one hour of symptom onset (or unwitnessed, within twenty-four hours of last being seen alive), in a person without a prior condition that would appear fatal. In children and young adults, the annual incidence is approximately one to three per hundred thousand, which makes it rare in absolute terms but one of the leading medical causes of death in this age group — and because a substantial proportion of cases have a heritable substrate, every sudden death in a young person carries implications for the surviving relatives. [1]
References12ShowHide
- [1]Bagnall RD, Weintraub RG, Ingles J, Duflou J, Yeates L, et al. A prospective study of sudden cardiac death among children and young adults. N Engl J Med, 2016.PMID 27332903
- [2]Priori SG, Blomström-Lundqvist C, Mazzanti A, Blom N, Borggrefe M, et al. 2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Europace, 2015.PMID 26318695
- [3]Al-Khatib SM, Stevenson WG, Ackerman MJ, Bryant WJ, Callans DJ, et al. 2017 AHA/ACC/HRS Guideline for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death. J Am Coll Cardiol, 2018.PMID 29097296
- [4]Zeppenfeld K, Tfelt-Hansen J, de Riva M, Winkel BG, Behr ER, et al. 2022 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death. Eur Heart J, 2022.PMID 36017572
- [5]Maron BJ, Doerer JJ, Haas TS, et al. Commotio cordis and the epidemiology of sudden death in competitive lacrosse. Pediatrics, 2009.PMID 19706581
- [6]Madias C, Maron BJ, Alsheikh-Ali AA, et al. Commotio cordis. Indian Pacing Electrophysiol J, 2007.PMID 17957272
- [7]Topjian AA, Telford R, Holubkov R, et al. Association of early postresuscitation hypotension with survival to discharge after pediatric cardiac arrest. JAMA Pediatr, 2018.PMID 29228147
- [8]Abrams DJ, Macrae CA Long QT syndrome. Circulation, 2014.PMID 24709866
- [9]Moss AJ, Zareba W, Hall WJ, Schwartz PJ, Crampton RS, et al. Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. Circulation, 2000.PMID 10673253
- [10]Conte G, Belhassen B, Lambiase P, et al. Out-of-hospital cardiac arrest due to idiopathic ventricular fibrillation in patients with normal electrocardiograms: results from a multicentre long-term registry. Europace, 2019.PMID 31504477
- [11]Schwartz PJ, Priori SG, Cerrone M, Spazzolini C, Odero A, et al. Left cardiac sympathetic denervation in the management of high-risk patients affected by the long-QT syndrome. Circulation, 2004.PMID 15051644
- [12]Priori SG, Wilde AA, Horie M, Cho Y, Behr ER, et al. HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes. Heart Rhythm, 2013.PMID 24011539