Documenting the descent – remote monitoring and adult-onset Catecholaminergic Polymorphic Ventricular Tachycardia associated with ventricular fibrillation and bradycardia

2021 ◽  
pp. 1-3
Author(s):  
Laura F. Halperin ◽  
Andrew D. Krahn ◽  
Zachary W. Laksman
Keyword(s):  
Ventricular Tachycardia ◽  
Ventricular Fibrillation ◽  
Sinus Rhythm ◽  
Remote Monitoring ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Implantable Loop Recorder ◽  
Adult Onset ◽  
Loop Recorder ◽  
Inherited Arrhythmia

Abstract This image highlights a 38-year-old female with ventricular fibrillation and spontaneous return to sinus rhythm found on an implantable loop recorder inserted for recurrent syncope. Ultimately, she was diagnosed with catecholaminergic polymorphic ventricular tachycardia, a rare inherited arrhythmia disorder.

scholarly journals Catecholaminergic polymorphic ventricular tachycardia in pregnancy: a case report

2020 ◽  
Vol 14 (1) ◽  
Author(s):  
Amy Schumer ◽  
Stephen Contag
Keyword(s):  
Ventricular Tachycardia ◽  
Motor Vehicle ◽  
Motor Vehicle Accident ◽  
Genetic Disorder ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Case Presentation ◽  
Vehicle Accident ◽  
Inherited Arrhythmia ◽  
In Pregnancy

Abstract Introduction Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a genetic disorder that can cause fatal tachyarrhythmias brought on by physical or emotional stress. There is little reported in the literature regarding management of CPVT in pregnancy much less during labor. Case presentation A gravida 2, para 1 presented to our high-risk clinic at 15 weeks gestation with known CPVT. The Caucasian female patient had been diagnosed after experiencing a cardiac arrest following a motor vehicle accident and found to have a pathogenic cardiac ryanodine receptor mutation. An implantable cardioverter defibrillator was placed at that time. Her pregnancy was uncomplicated, and she was medically managed with metoprolol, flecainide, and verapamil. Her labor course and successful vaginal delivery were uncomplicated and involved a multidisciplinary team comprising specialists in electrophysiology, maternal fetal medicine, anesthesiology, general obstetrics, lactation, and neonatology. Conclusions CPVT is likely underdiagnosed and, given that cardiovascular disease is a leading cause of death in pregnancy, it is important to bring further awareness to the diagnosis and management of this inherited arrhythmia syndrome in pregnancy.

scholarly journals 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 mutations

2021 ◽  
Author(s):  
Alice Maltret ◽  
Fatima Azzahrae Benaich ◽  
John Rendu ◽  
Véronique Fressart ◽  
Nathalie Roux-Buisson ◽  
...  
Keyword(s):  
Cardiac Arrest ◽  
Ventricular Tachycardia ◽  
De Novo ◽  
Sudden Cardiac Arrest ◽  
Case Series ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Icd Implantation ◽  
Loop Recorder ◽  
Cardioverter Defibrillator

Abstract Background Calmodulopathie is an emerging group of primary electrical disease with various, severe and early onset phenotype. Sudden cardiac arrest/death can be the first symptom and current medical management seams insufficient to prevent recurrences. Cardioverter defibrillator implantation (ICD) in the young is challenging and can be harmful. Case Summary We report the management of 2 very young boys (aged 3.5 and 5.5 years old) who survived a sudden cardiac arrest (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.

scholarly journals Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular Tachycardia in Mice and in Patients

2013 ◽  
Vol 112 (4) ◽  
pp. 689-697 ◽  
Author(s):  
Michela Faggioni ◽  
Hyun Seok Hwang ◽  
Christian van der Werf ◽  
Ineke Nederend ◽  
Prince J. Kannankeril ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Sinus Rhythm ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia

scholarly journals Pediatric Catecholaminergic Polymorphic Ventricular Tachycardia: A Translational Perspective for the Clinician-Scientist

2021 ◽  
Vol 22 (17) ◽  
pp. 9293
Author(s):  
Dania Kallas ◽  
Avani Lamba ◽  
Thomas M. Roston ◽  
Alia Arslanova ◽  
Sonia Franciosi ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Disease Onset ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Future Research ◽  
Gene Encoding ◽  
Pathogenic Variants ◽  
Inherited Arrhythmia ◽  
Clinician Scientist ◽  
Long Term Prognosis

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare and potentially lethal inherited arrhythmia disease characterized by exercise or emotion-induced bidirectional or polymorphic ventricular tachyarrhythmias. The median age of disease onset is reported to be approximately 10 years of age. The majority of CPVT patients have pathogenic variants in the gene encoding the cardiac ryanodine receptor, or calsequestrin 2. These lead to mishandling of calcium in cardiomyocytes resulting in after-depolarizations, and ventricular arrhythmias. Disease severity is particularly pronounced in younger individuals who usually present with cardiac arrest and arrhythmic syncope. Risk stratification is imprecise and long-term prognosis on therapy is unknown despite decades of research focused on pediatric CPVT populations. The purpose of this review is to summarize contemporary data on pediatric CPVT, highlight knowledge gaps and present future research directions for the clinician-scientist to address.

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