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Catecholaminergic polymorphic ventricular tachycardia (CPVT) is a rare congenital arrhythmogenic disorder induced by physical or emotional stress. It mainly affects children and younger adults and is characterized by rapid polymorphic and bidirectional ventricular tachycardia. Symptoms can include dizziness, palpitations, and presyncope, which may progress to syncope, hypotonia, convulsive movements, and sudden cardiac death. CPVT is the result of perturbations in Ca ion handling in the sarcoplasmic reticulum of cardiac myocytes. Mutations in the cardiac ryanodine receptor gene and the calsequestrin isoform 2 gene are most commonly seen in familial CPVT patients. Under catecholaminergic-stimulation, either mutation can result in an excess Ca load during diastole resulting in delayed afterdepolarizations and subsequent arrhythmogenesis. The current first-line treatment for CPVT is β-blocker therapy. Other therapeutic interventions that can be used in conjunction with β-blockers include moderate exercise training, flecainide, left cardiac sympathetic denervation, and implantable cardioverter defibrillators. Several potential therapeutic interventions including verapamil, dantrolene, JTV519, and gene therapy are also discussed.
This article was published in the following journal.
Name: Cardiology in review
A 34-year-old man, who was previously fit and healthy, died suddenly on exercise. A post-mortem exam performed by forensic pathologists and a toxicological screening were normal; therefore, the cause ...
Generation of induced pluripotent stem cells (iPSCs) from an infant with catecholaminergic polymorphic ventricular tachycardia carrying the double heterozygous mutations A1855D in RyR2 and Q1362H in SCN10A.
Induced pluripotent stem cells (iPSCs) were generated from peripheral blood mononuclear cells (PBMCs) isolated from the peripheral blood of a 4 month-old boy with catecholaminergic polymorphic ventr...
Co-Phenotype of Left Ventricular Non-Compaction Cardiomyopathy and Atypical Catecholaminergic Polymorphic Ventricular Tachycardia in Association With R169Q, a Ryanodine Receptor Type 2 Missense Mutation.
Left ventricular non-compaction (LVNC) is a cardiomyopathy characterized by prominent trabeculae and intertrabecular recesses. We present the cases of 3 girls with the sameryanodine receptor type 2(RY...
The prevalence and clinical course of atrial fibrillation (AF) in hypertrophic cardiomyopathy (HCM) is well described, though less so for other inherited cardiomyopathies (familial dilated cardiomyopa...
Verapamil-sensitive fascicular ventricular tachycardia (VT) is the most common type of idiopathic left ventricular tachycardia, and it is divided into three types. Upper septal ventricular tachycardia...
The purpose of this study is to test whether the addition of oral flecainide to standard therapy will reduce cardiac events compared to placebo plus standard therapy in patients with Catec...
To test the hypothesis that increasing the sinus node rate with atropine treatment prior to exercise will reduce exercise-triggered ventricular ectopy compared to baseline in patients with...
This is an observational, prospective, multi-center registry, aiming at building a risk stratification for malignant inherited ventricular arrhythmias, including Brugada syndrome(Brs)、Lo...
The BERLIN VT study is designed to evaluate the impact of prophylactic ventricular tachycardia (VT) ablation on all-cause mortality and unplanned hospital admission for congestive heart fa...
This study aims to evaluate the electrophysiological properties of the heart conduction system in patients with unexplained polymorphic ventricular tachycardia (VT) and/or ventricular fibr...
An abnormally rapid ventricular rhythm usually in excess of 150 beats per minute. It is generated within the ventricle below the BUNDLE OF HIS, either as autonomic impulse formation or reentrant impulse conduction. Depending on the etiology, onset of ventricular tachycardia can be paroxysmal (sudden) or nonparoxysmal, its wide QRS complexes can be uniform or polymorphic, and the ventricular beating may be independent of the atrial beating (AV dissociation).
A malignant form of polymorphic ventricular tachycardia that is characterized by HEART RATE between 200 and 250 beats per minute, and QRS complexes with changing amplitude and twisting of the points. The term also describes the syndrome of tachycardia with prolonged ventricular repolarization, long QT intervals exceeding 500 milliseconds or BRADYCARDIA. Torsades de pointes may be self-limited or may progress to VENTRICULAR FIBRILLATION.
Implantable devices which continuously monitor the electrical activity of the heart and automatically detect and terminate ventricular tachycardia (TACHYCARDIA, VENTRICULAR) and VENTRICULAR FIBRILLATION. They consist of an impulse generator, batteries, and electrodes.
Cardiac electrical stimulators that apply brief high-voltage electroshocks to the HEART. These stimulators are used to restore normal rhythm and contractile function in hearts of patients who are experiencing VENTRICULAR FIBRILLATION or ventricular tachycardia (TACHYCARDIA, VENTRICULAR) that is not accompanied by a palpable PULSE. Some defibrillators may also be used to correct certain noncritical dysrhythmias (called synchronized defibrillation or CARDIOVERSION), using relatively low-level discharges synchronized to the patient's ECG waveform. (UMDNS, 2003)
A potentially lethal cardiac arrhythmia characterized by an extremely rapid, hemodynamically unstable ventricular tachycardia (150-300 beats/min) with a large oscillating sine-wave appearance. If untreated, ventricular flutter typically progresses to VENTRICULAR FIBRILLATION.
Congenital conditions are those which are present from birth. They include structural deformities or loss of function in organs such as the <!--LGfEGNT2Lhm-->heart, gut or skeletal system. They can be corrected by <!--LGfEGNT2Lhm-->surgery, m...
Stress is caused by your perception of situations around you and then the reaction of your body to them. The automatic stress response to unexpected events is known as 'fight or flight'. Discovered by Walter Cannon in 1932, it is the release of h...
Pediatrics is the general medicine of childhood. Because of the developmental processes (psychological and physical) of childhood, the involvement of parents, and the social management of conditions at home and at school, pediatrics is a specialty. With ...