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Incessant infant ventricular tachycardia
Incessant infant ventricular tachycardia is a rare type of ventricular tachycardia (VT) characterized by the presence of tachycardia originating from the ventricles, observed for more than 10% of a 24 hour monitoring period. Patients are either asymptomatic or present congestive heart failure.
ORPHA:45453Classification level: Disorder
- Synonym(s): -
- Prevalence: 1-9 / 100 000
- Inheritance: Not applicable
- Age of onset: Infancy, Childhood
- ICD-10: I47.2
- OMIM: -
- UMLS: C0340487
- MeSH: -
- GARD: -
- MedDRA: -
The incidence of incessant infant VT is estimated to be 1/333,300 live births in the United Kingdom. Males are more commonly affected than females.
This type of VT is diagnosed in infancy or early childhood, usually presenting at between 3 and 30 months of age. The rate of VT ranges from 170 to 440 (mean 260) beats/min occurring more than 10% of the day. The most common clinical presentation is VT with congestive heart failure.
The physiopathology of this type of VT is unknown but it has been suggested that microscopic tumors such as myocardial hamartomas, also known as histiocytoid cardiomyopathy (see this term), are responsible.
The electrocardiogram (ECG) usually shows a type of right bundle branch block morphology and a superior axis, predicting a tachycardia origin in the posterior or inferior left ventricle. There is usually clear evidence of ventriculo-atrial block with dissociated P waves, or capture beats or fusion beats. The finding of wide QRS tachycardia with retrograde block confirms the diagnosis of ventricular tachycardia. No structural abnormalities are found on the echocardiogram but there is often poor ventricular function at presentation.
The differential diagnosis includes other types of ventricular tachycardia and supraventricular tachycardia with a wide QRS.
Management and treatment
Immediate treatment involves control of tachycardia and general support or resuscitation as required. Intravenous lidocaine (1-2 mg/kg) will usually slow or stop the tachycardia leading to rapid symptomatic improvement. Intravenous amiodarone is an alternative. Direct current (DC) cardioversion is usually ineffective. Once sinus rhythm has been restored, drugs such as amiodarone and flecainide are usually effective in suppressing the arrhythmia, sometimes in combination with a beta-blocker. Digoxin and verapamil are best avoided as they may worsen the tachycardia and lead to cardiovascular collapse.
Tachycardia usually resolves before the age of five years and drug treatment can be withdrawn. Late recurrence is unusual.
- Summary information
- Russian (2013, pdf)