Catecholaminergic polymorphic ventricular tachycardia 5
- Synonyms
- CARDIAC ARRHYTHMIA SYNDROME, WITH OR WITHOUT SKELETAL MUSCLE WEAKNESS; Ventricular tachycardia, catecholaminergic polymorphic, 5, with or without muscle weakness
Summary
Excerpted from the GeneReview:Catecholaminergic polymorphic ventricular tachycardia (CPVT) is characterized by episodic syncope occurring during exercise or acute emotion. The underlying cause of these episodes is the onset of fast ventricular tachycardia (bidirectional or polymorphic). Spontaneous recovery may occur when these arrhythmias self-terminate. In other instances, ventricular tachycardia may degenerate into ventricular fibrillation and cause sudden death if cardiopulmonary resuscitation is not readily available. The mean onset of symptoms (usually a syncopal episode) is between age seven and 12 years; onset as late as the fourth decade of life has been reported. If untreated, CPVT is highly lethal, as approximately 30% of affected individuals experience at least one cardiac arrest and up to 80% have one or more syncopal spells. Sudden death may be the first manifestation of the disease.
- Full text of GeneReview (by section):
- Summary
- Diagnosis
- Clinical Characteristics
- Genetically Related (Allelic) Disorders
- Differential Diagnosis
- Management
- Genetic Counseling
- Resources
- Molecular Genetics
- Chapter Notes
- References
- Authors:
- Carlo Napolitano
- Andrea Mazzanti
- Raffaella Bloise
- view full author information
Available tests
50 tests are in the database for this condition.
Clinical tests (50 available)
Clinical features
Help- Abnormality of the cardiovascular system
- Cardiac arrest
Cardiac arrest
- MedGen UID: 5456
- Concept ID: C0018790
- Finding: Finding
Abnormality of the cardiovascular system
- Polymorphic ventricular tachycardia
Polymorphic ventricular tachycardia
- MedGen UID: 138002
- Concept ID: C0344432
- Finding: Disease or Syndrome
Abnormality of the cardiovascular system
- Premature ventricular contraction
Premature ventricular contraction
- MedGen UID: 56236
- Concept ID: C0151636
- Finding: Disease or Syndrome
Abnormality of the cardiovascular system
- Prolonged QT interval
Prolonged QT interval
- MedGen UID: 57494
- Concept ID: C0151878
- Finding: Finding
Abnormality of the cardiovascular system
- Shock
Shock
- MedGen UID: 20738
- Concept ID: C0036974
- Finding: Pathologic Function
Abnormality of the cardiovascular system
- Syncope
Syncope
- MedGen UID: 21443
- Concept ID: C0039070
- Finding: Sign or Symptom
Abnormality of the cardiovascular system
- Cardiac arrest
- Abnormality of the musculoskeletal system
- Proximal muscle weakness
Proximal muscle weakness
- MedGen UID: 113169
- Concept ID: C0221629
- Finding: Finding
Abnormality of the musculoskeletal system
- Proximal muscle weakness
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