Demographic data, clinical presentation, diagnostic method, and the threshold heart rate of polymorphic ventricular ectopic and ventricular tachycardia of the 10 patients Six patients presented initially with syncope while the other four presented with [hkmj.org]

Patients with CPVT often present with exercise or emotion induced syncope, the first presentation may also be sudden cardiac death. • In addition to avoidance of triggers, treatment with beta blockers is the mainstay of therapy. [journals.elsevier.com]

Within this group, it is estimated that 30% of subjects may present sudden death in the absence of treatment. [admerahealth.com]

[…] clinical presentation of sudden death or aborted cardiac arrest. [cohesionphenomics.com]

Later, the patient presented to us for a second opinion. We diagnosed the patient with CPVT, started him on a beta-blocker, and identified the disease-causing mutation in the RYR2 gene. [ntvg.nl]

• Medication Noncompliance

  Additional contributors included medication noncompliance, a missed dose, or use of metoprolol, which has recently been shown to be inferior to nadolol in a population who received both medications (5). [electrophysiology.onlinejacc.org]

• Tantrums

  Perhaps athlete and nonathlete classifications are less the issue than the largely unmeasured adrenergic state of say a 2-year-old child having a temper tantrum in the middle of a shopping mall or an adolescent playing video games! [electrophysiology.onlinejacc.org]

• Incontinence

   Some misdiagnosed as epilepsy (because CPVT-related syncope may include convulsive movements and urinary or fecal incontinence).  Symptom onset in childhood ( between 7 to 9 yrs)  Classical family history under similar conditions, but not always [slideshare.net]

This diagnostic workup included genetic testing. In this respect, patients 2 and 3 were of particular interest. Patient 2 attended the clinic as an 11-year-old boy with exercise-induced syncope. [revespcardiol.org]

In the presented case all the patients received a thorough diagnostic workup to exclude structural heart disease. [scinapse.io]

(See Workup ). Accelerated idioventricular rhythm, sometimes termed slow VT, is a variant of VT that produces a rate of 60-120 beats/min. [emedicine.medscape.com]

[…] dehydration, while low blood oxygen levels may be seen following the event in those with pulmonary embolism.[1] Routine broad panel laboratory testing detects abnormalities in <2-3% of results and is therefore not recommended.[14] Based on this initial workup [en.wikipedia.org]

• Bidirectional Ventricular Ectopy

  This bidirectional ventricular ectopy terminated abruptly in the immediate recovery period with the onset of SVT (Figure 1(a)). The patient was aware of these extrasystoles and the onset of SVT. [hindawi.com]

What are the treatment options for CPVT? There are several treatments for CPVT. Your child’s exact course of treatment will be based on the rate or severity of his or her arrhythmia. [childrenshospital.org]

We also summarised the treatment modalities, response to treatment, and clinical outcome up to October 2014. [hkmj.org]

Management and treatment Beta blockers (nadolol and propranolol) are the first treatment option for patients with CPVT and the maximum tolerated dose should be administered to control arrhythmias. [orpha.net]

The proband was implanted with a defibrillator due to presyncope with nonsustained ventricular tachycardia during the EST, despite maximum treatment with beta-blockers (before starting the use of the flecainide treatment in this clinical context 1 ). [revespcardiol.org]

Treatments There is no cure for CPVT but treatment can prevent symptoms. If your child does not show symptoms of CPVT, but has tested positive for the condition, doctors advise treatment to avoid sudden cardiac death. [intermountainhealthcare.org]

Prognosis Although CPVT is a severe and often deadly disease, early diagnosis and proper treatment can greatly increase life expectancy. [orpha.net]

CONCLUSIONS: CPVT may arise in certain distinct areas but the prognosis is poor. The onset of CPVT may be an indication for an implanted cardioverter-defibrillator. [ncbi.nlm.nih.gov]

The aim of this article is to offer a perspective on molecular genetics, disease pathogenesis, clinical and genetic diagnosis, therapeutic strategies, and prognosis in CPVT. [heart.bmj.com]

Etiology The two genes responsible for CPVT that have been discovered to date, are the cardiac ryanodine receptor (RyR2) gene, which is the cause of CPVT in approximately 55% to 65% of cases, and the cardiac calsequestrin gene (CASQ2), seen much less [orpha.net]

| Death, Sudden, Cardiac - etiology | Young Adult | Heart Defects, Congenital - complications | Survival Analysis | Biomarkers | Adult | Female | Arrhythmias, Cardiac - diagnosis | Heart Defects, Congenital - mortality | Heart Defects, Congenital - therapy [query.library.utoronto.ca]

IV beta-blockers is considered first choice, similar to VTach storm of other etiologies. Given that it is Polymorphic VTach, it would also be reasonable to administer Magnesium, but this would be aimed at possible Prolonged QTc. [pedemmorsels.com]

This disorder was first described in 1975,1 and its symptoms are usually syncopal episodes triggered by exercise or emotional stress resembling those observed in LQT syndrome.2 The etiological background for the disorder has been described to be the uncontrolled [scinapse.io]

Physicians must remain vigilant and consider LQTS as a possible etiology in patients with a history of syncope. [radcliffecardiology.com]

Summary Epidemiology The prevalence of CPVT in Europe is 1/10,000. Clinical description Typical age of onset of CPVT is between 7 and 9 years of age with no sex difference. [orpha.net]

In the last decade, genetic testing and our understanding of the mechanisms of arrhythmogenesis has significantly improved, novel antiarrhythmic drug strategies have emerged, more epidemiological experience with athletes with inherited channelopathies [acc.org]

"Epidemiological studies on syncope--a register based approach". Danish Medical Journal. 60 (8): B4702. [en.wikipedia.org]

For the last 15 years, significant progress has been made in our understanding of genetic, pathophysiological, and clinical aspects of CPVT. [heart.bmj.com]

BACKGROUND: The pathophysiological background of catecholaminergic polymorphic ventricular tachycardia is well understood, but the clinical features of this stress-induced arrhythmic disorder, especially the incidence and risk factors of arrhythmic events [scinapse.io]

Despite recent advances in genetics, pathophysiology, and clinical characteristics of CPVT, further research in unraveling molecular basis and clinical translation should be performed. [heraldopenaccess.us]

Implantable cardioverter defibrillators (ICDs) are used for prevention of sudden death in patients with CPVT. [austinpublishinggroup.com]

Prevention There is no way known to prevent CPVT. Your child’s CPVT can be managed with medicine, arrhythmia management techniques, and in more extreme cases, a defibrillator,. [intermountainhealthcare.org]

This can prevent adrenaline from stimulating the heart and causing arrhythmias. [childrenshospital.org]

Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death. Heart. 2003 Jan;89(1):66-70. [en.ecgpedia.org]