Challenging indication of cardioverter defibrillator implantation after sudden cardiac arrest in the very young: a case series of catecholaminergic polymorphic ventricular tachycardia secondary to de novo calmodulin p.Asn98Ser
Résumé
Background
Calmodulinopathy is an emerging group of primary electrical disease with various, severe, and early onset phenotype. Sudden cardiac arrest (SCA)/death can be the first symptom and current medical management seems insufficient to prevent recurrences. Implantable cardioverter-defibrillator (ICD) in the young is challenging and can be harmful.
Case summary
We report the management of two very young boys (aged 3.5 and 5.5 years old) who survived an SCA due to calmodulin mutation responsible of a catecholaminergic polymorphic ventricular tachycardia phenotype. In both case, SCA had an adrenergic trigger. Despite SCA, ICD implantation was denied by the parents. After thorough discussion with the family, the patients were managed with solely betablocker treatment and loop recorder implantation. At last follow-up of 30 and 23 months, respectively, there were no recurrence of any cardiac event.
Discussion
The benefits of ICD implantation at a very young age must be weighed against the risk complication. In the youngest, whom recreative activities are under constant supervision, the decision, jointly made with the parents, could be to postpone ICD.
Mots clés
Calmodulin
Catecholaminergic polymorphic ventricular tachycardia
Sudden cardiac death
Implantable cardioverter-defibrillator
Shared decision-making
Case report
sudden cardiac death
mutation
implantable defibrillators
cardiac event
calmodulin
follow-up
parent
precipitating factors
implantable defibrillator insertion
catecholaminergic polymorphic ventricular tachycardia
professional supervision
Domaines
Médecine humaine et pathologieOrigine | Publication financée par une institution |
---|