scholarly journals Clinical features and antiarrhythmic therapy in patients with catecholaminergic polymorphic ventricular tachycardia

2021 ◽  
Vol 28 (4) ◽  
pp. 62-69
Author(s):  
V. V. Bereznitskaya ◽  
E. K. Kulbachinskaya ◽  
M. A. Shkolnikova
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blocker ◽  
Diagnostic Methods ◽  
Antiarrhythmic Therapy ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Supraventricular Arrhythmias ◽  
Cardiac Events ◽  
Icd Implantation ◽  
High Prevalence

Aims. To evaluate the long-term efficacy of antiarrhythmic therapy in patients with catecholaminergic polymorphic ventricular tachycardia (CPVT).Methods. CPVT was diagnosed in 11 patients between the ages of 3-12 years with a minimum follow-up of 10 years. The data analyzed was obtained from existing medical records that included symptoms, family screenings, treadmill tests, electrocardiography, echocardiography, implanted cardioverter-defibrillator data (ICD), and medical treatments.Results. Cardiac events were registered in 75% of patients on beta-blocker therapy. Supraventricular arrhythmias such as atrial and atrioventicular nodal tachycardia, atrial fibrillation and atrial flutter were detected using various ECG diagnostic methods in all patients, which is significantly higher than reported in similar studies. A combination of anti-arrhythmic therapy and beta-blocker treatment reduced the number of cardiac events by 50% as compared to only beta-blocker treatment.Conclusion. Multiple supraventricular arrhythmias have a high prevalence in patients with CPVT and can trigger ventricular arrhythmia. Combined antiarrhythmic therapy is effective because it prevents cardiac events in patients with CPVT. Combined antiarrhythmic therapy improves the prognosis of patients with CPVT and may help to avoid or postpone ICD implantation.

P6581Association of neuro-psycho-behavioral troubles to catecholaminergic polymorphic ventricular tachycardia: a more severe arrhythmic phenotype?

2019 ◽  
Vol 40 (Supplement_1) ◽  
Author(s):  
L Placide ◽  
F Sacher ◽  
P Maury ◽  
V Probst ◽  
J L Pasquie
Keyword(s):  
Cardiac Arrest ◽  
Ventricular Tachycardia ◽  
Symptom Onset ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Cardiac Events ◽  
Icd Implantation ◽  
Familial History ◽  
Time To Diagnosis ◽  
History Of

Abstract Catecholaminergic polymorphic ventricular tachycardia (CPVT) is defined by bidirectional or polymorphic ventricular tachycardia during adrenergic situations and is associated to a poor long-term prognosis. Clinical cases suggest an association between epilepsy and/or neuro-psycho-behavioral troubles (NPBT) to cardiac channelopathies. Methods This is a retrospective observational study based on case analysis from the INTEGRALIS database of the referral center for inherited cardiac arrythmias in Nantes. Epidemiological and clinical-biological features of the population have been studied. Patients with Presence or Absence of NPBT were compared. Results:From 8306 pts in the whole database, 533 presented with VT and 71 pts were diagnosed with CPVT and genotyped. Symptom onset occurred at a medium age of 17.1±13.5 years. Median LVEF was 65% (IQR 9,8%) and median corrected QT interval (QTc) was 399 ms (IQR 27 ms). 77.5% of pts had fainting and/or syncopes, and there were 28.2% patients with a history of cardiac arrest. Time to diagnosis was below1 year for 44.2% of symptomatic pts. Symptoms occurred during exertion for 42.3% of pts including swimming. The prevalence of NPBT was 23,9%. 74% of NPBT were convulsive seizures, 21% psycho-behavioral troubles and 5% epilepsy proved by EEG. Median age of symptom onset was younger in the group “NPBT” (12.2±4 yo vs 19.2±15.5 yo). The rate of patients with symptoms during exertion was higher in the group “NPBT” (29.4 vs 7.4% P=0.031). A mutation in the gene of Ryanodine receptor-2 was found in 64.8% of pts. Comparisons patients w/wo NPBT NPBT (N=17) Without NPBT (N=54) Familial history of Sudden death 7/17 (41.2%) 24/54 (44.4%) NS Familial history of CPVT 5/17 (29.4%) 29/54 (53.7%) NS Medium age of symptom onset (yo) 12.1±4 19.2±15.5 P=0.021 Time to diagnosis <1 year 4/17 (23.5%) 16/54 (27.8%) NS Malaises and/or syncopes 17/17 (100%) 38/54 (70.4%) P=0.035 Cardiac arrest 9/17 (52.9%) 11/54 (20.4%) P=0.025 ICD Implantation 6/17 (35.3%) 12/54 (22.2%) NS Supraventricular arrhythmias 3/17 (17.7%) 6/54 (11.1%) NS Antiepileptic treatment 5/17 (29.4%) 2/54 (3.7%) P=0.009 Conclusion NPBT appears to be associated to a younger age of symptom onset and a higher rate of serious cardiac events particularly during swimming. This study will serve as preliminary data for further clinical and experimental protocols.

Monogenic and oligogenic cardiovascular diseases: genetics of arrhythmias—catecholaminergic polymorphic ventricular tachycardia

ESC CardioMed ◽  
2018 ◽  
pp. 683-685
Author(s):  
Peter J. Schwartz ◽  
Lia Crotti
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blockers ◽  
Stress Test ◽  
Exercise Stress Test ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Exercise Stress ◽  
Polymorphic Ventricular Tachycardia ◽  
Cascade Screening ◽  
Icd Implantation ◽  
Symptomatic Diagnosis

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder associated with syncope and sudden death manifesting in the young during sympathetic activation. The electrocardiogram is normal and the heart is structurally normal. The diagnosis is usually made with an exercise stress test that shows a typical pattern of onset and offset of adrenergically induced ventricular arrhythmias. Molecular screening of RyR2, the major CPVT gene, is recommended whenever the suspicion of CPVT is high. If a disease-causing mutation is identified, cascade screening allows pre-symptomatic diagnosis among family members. All affected subjects should be treated with beta blockers (nadolol or propranolol). Preliminary data support the association of beta blockers with flecainide. After a cardiac arrest, an implantable cardioverter defibrillator (ICD) should be implanted, but it is accompanied by a disquietingly high incidence of adverse effects. After syncope on beta blocker therapy, left cardiac sympathetic denervation is most effective, preserves quality of life, and does not preclude a subsequent ICD implantation.

scholarly journals Catecholaminergic polymorphic ventricular tachycardia successfuly treated by .BETA. blocker.

2006 ◽  
Vol 95 (1) ◽  
pp. 136-139
Author(s):  
Mayuri Hayashi ◽  
Humiko Okazaki ◽  
Syuichi Igei ◽  
Keiichi Inada ◽  
Seiichirou Matuo ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blocker ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia

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.

Monogenic and oligogenic cardiovascular diseases: genetics of arrhythmias—catecholaminergic polymorphic ventricular tachycardia

2018 ◽  
Author(s):  
Peter J. Schwartz ◽  
Lia Crotti
Keyword(s):  
Ventricular Tachycardia ◽  
Beta Blockers ◽  
Stress Test ◽  
Exercise Stress Test ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Exercise Stress ◽  
Polymorphic Ventricular Tachycardia ◽  
Cascade Screening ◽  
Icd Implantation ◽  
Symptomatic Diagnosis

Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare inherited disorder associated with syncope and sudden death manifesting in the young during sympathetic activation. The electrocardiogram is normal and the heart is structurally normal. The diagnosis is usually made with an exercise stress test that shows a typical pattern of onset and offset of adrenergically induced ventricular arrhythmias. Molecular screening of RyR2, the major CPVT gene, is recommended whenever the suspicion of CPVT is high. If a disease-causing mutation is identified, cascade screening allows pre-symptomatic diagnosis among family members. All affected subjects should be treated with beta blockers (nadolol or propranolol). Preliminary data support the association of beta blockers with flecainide. After a cardiac arrest, an implantable cardioverter defibrillator (ICD) should be implanted, but it is accompanied by a disquietingly high incidence of adverse effects. After syncope on beta blocker therapy, left cardiac sympathetic denervation is most effective, preserves quality of life, and does not preclude a subsequent ICD implantation.

Advances in the diagnosis and treatment of catecholaminergic polymorphic ventricular tachycardia

2017 ◽  
Vol 27 (S1) ◽  
pp. S49-S56 ◽  
Author(s):  
Thomas M. Roston ◽  
Taylor C. Cunningham ◽  
Shubhayan Sanatani
Keyword(s):  
Cardiac Arrest ◽  
Ventricular Tachycardia ◽  
Ryanodine Receptor ◽  
Calcium Release ◽  
Catecholaminergic Polymorphic Ventricular Tachycardia ◽  
Polymorphic Ventricular Tachycardia ◽  
Electrical Storm ◽  
Implantable Cardioverter Defibrillators ◽  
Cardiac Events ◽  
Β Blockers

AbstractSince the sentinel description of exercise-triggered ventricular arrhythmias in 21 children, our recognition and understanding of catecholaminergic polymorphic ventricular tachycardia has improved substantially. A variety of treatments are now available, but reaching a diagnosis before cardiac arrest remains a challenge. Most cases are related to variants in the gene encoding for ryanodine receptor-2 (RyR2), which mediates calcium-induced calcium release. Up to half of cases remain genetically elusive. The condition is presently incurable, but one basic intervention, the universal administration of β-blockers, has improved survival. In the past, implantable cardioverter-defibrillators (ICDs) were frequently implanted, especially in those with a history of cardiac arrest. Treatment limitations include under-dosing and poor compliance with β-blockers, and potentially lethal ICD-related electrical storm. Newer therapies include flecainide and sympathetic ganglionectomy. Limited data have suggested that genotype may predict phenotype in catecholaminergic polymorphic ventricular tachycardia, including a higher risk of life-threatening cardiac events in subjects with variants in the C-terminus of ryanodine receptor-2 (RyR2). At present, international efforts are underway to better understand this condition through large prospective registries. The recent publication of gene therapy in an animal model of the recessive form of the disease highlights the importance of improving our understanding of the genetic underpinnings of the disease.

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