Catecholaminergic polymorphic ventricular tachycardia natural history, complications and prognosis: Difference between revisions
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{{Catecholaminergic polymorphic ventricular tachycardia}} | {{Catecholaminergic polymorphic ventricular tachycardia}} | ||
{{CMG}}; {{AE}} | {{CMG}}; {{AE}}{{MRV}} | ||
==Overview== | ==Overview== | ||
If left untreated, [ | If left untreated, approximately 30% of patients experience at least one [[cardiac arrest]] and up to 80% one or more [[syncope|syncopal spells]]. Common complications of catecholaminergic polymorphic ventricular tachycardia include [[ventricular fibrillation]], [[sudden cardiac arrest]], and [[sudden cardiac death]]. Prognosis is generally poor, and the 10-year [[mortality]] of patients with catecholaminergic polymorphic ventricular tachycardia is approximately 40%. | ||
==Natural History, Complications, and Prognosis== | ==Natural History, Complications, and Prognosis== | ||
===Natural History=== | ===Natural History=== | ||
*The symptoms of | *The symptoms of [[CPVT]] usually develop during childhood and adolescence, in the first and second decades of life, and start with symptoms such as episodes of [[syncope]]. | ||
* | *More than 30% of affected individuals will experience symptoms before the age of 10 years and the majority (60% to 80%) of the patients will have one or more symptomatic [[arrhythmia]] episodes before age 40.<ref name="LeenhardtLucet1995">{{cite journal|last1=Leenhardt|first1=Antoine|last2=Lucet|first2=Vincent|last3=Denjoy|first3=Isabelle|last4=Grau|first4=Francis|last5=Ngoc|first5=Dien Do|last6=Coumel|first6=Philippe|title=Catecholaminergic Polymorphic Ventricular Tachycardia in Children|journal=Circulation|volume=91|issue=5|year=1995|pages=1512–1519|issn=0009-7322|doi=10.1161/01.CIR.91.5.1512}}</ref> <ref name="PrioriNapolitano2002">{{cite journal|last1=Priori|first1=Silvia G.|last2=Napolitano|first2=Carlo|last3=Memmi|first3=Mirella|last4=Colombi|first4=Barbara|last5=Drago|first5=Fabrizio|last6=Gasparini|first6=Maurizio|last7=DeSimone|first7=Luciano|last8=Coltorti|first8=Fernando|last9=Bloise|first9=Raffaella|last10=Keegan|first10=Roberto|last11=Cruz Filho|first11=Fernando E.S.|last12=Vignati|first12=Gabriele|last13=Benatar|first13=Abraham|last14=DeLogu|first14=Angelica|title=Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=106|issue=1|year=2002|pages=69–74|issn=0009-7322|doi=10.1161/01.CIR.0000020013.73106.D8}}</ref> <ref name="Sumitomo2003">{{cite journal|last1=Sumitomo|first1=N|title=Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death|journal=Heart|volume=89|issue=1|year=2003|pages=66–70|issn=00070769|doi=10.1136/heart.89.1.66}}</ref> <ref name="Postma2005">{{cite journal|last1=Postma|first1=A V|title=Catecholaminergic polymorphic ventricular tachycardia: RYR2 mutations, bradycardia, and follow up of the patients|journal=Journal of Medical Genetics|volume=42|issue=11|year=2005|pages=863–870|issn=1468-6244|doi=10.1136/jmg.2004.028993}}</ref> | ||
*If left untreated, [ | *However, molecular analysis showed that there is a small group of patients who remain asymptomatic, even after [[exercise stress testing|exercise tests]] suggesting them to be having normal [[phenotype]] [[CPVT]] ([[mutation]] carriers). Some of these [[phenotype|phenotypically]] normal patients with [[CVPT]] do experience [[syncope]] and [[sudden death]].<ref name="HayashiDenjoy2009">{{cite journal|last1=Hayashi|first1=Meiso|last2=Denjoy|first2=Isabelle|last3=Extramiana|first3=Fabrice|last4=Maltret|first4=Alice|last5=Buisson|first5=Nathalie Roux|last6=Lupoglazoff|first6=Jean-Marc|last7=Klug|first7=Didier|last8=Hayashi|first8=Miyuki|last9=Takatsuki|first9=Seiji|last10=Villain|first10=Elisabeth|last11=Kamblock|first11=Joël|last12=Messali|first12=Anne|last13=Guicheney|first13=Pascale|last14=Lunardi|first14=Joël|last15=Leenhardt|first15=Antoine|title=Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=119|issue=18|year=2009|pages=2426–2434|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.108.829267}}</ref> | ||
*If left untreated, [[CPVT]] is highly lethal, as approximately 30% of patients experience at least one [[cardiac arrest]] and up to 80% one or more [[syncope|syncopal spells]].<ref name="LeenhardtLucet1995">{{cite journal|last1=Leenhardt|first1=Antoine|last2=Lucet|first2=Vincent|last3=Denjoy|first3=Isabelle|last4=Grau|first4=Francis|last5=Ngoc|first5=Dien Do|last6=Coumel|first6=Philippe|title=Catecholaminergic Polymorphic Ventricular Tachycardia in Children|journal=Circulation|volume=91|issue=5|year=1995|pages=1512–1519|issn=0009-7322|doi=10.1161/01.CIR.91.5.1512}}</ref><ref name="PrioriNapolitano2002">{{cite journal|last1=Priori|first1=Silvia G.|last2=Napolitano|first2=Carlo|last3=Memmi|first3=Mirella|last4=Colombi|first4=Barbara|last5=Drago|first5=Fabrizio|last6=Gasparini|first6=Maurizio|last7=DeSimone|first7=Luciano|last8=Coltorti|first8=Fernando|last9=Bloise|first9=Raffaella|last10=Keegan|first10=Roberto|last11=Cruz Filho|first11=Fernando E.S.|last12=Vignati|first12=Gabriele|last13=Benatar|first13=Abraham|last14=DeLogu|first14=Angelica|title=Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=106|issue=1|year=2002|pages=69–74|issn=0009-7322|doi=10.1161/01.CIR.0000020013.73106.D8}}</ref> | |||
*The [[polymorphic ventricular tachycardia]] may self-terminate or it may degenerate into [[ventricular fibrillation]], causing [[sudden cardiac death]]. | |||
===Complications=== | ===Complications=== | ||
*Common complications of | *Common complications of catecholaminergic polymorphic ventricular tachycardia include: | ||
**[ | **[[Ventricular fibrillation]] | ||
**[ | **[[Sudden cardiac arrest]] | ||
**[ | **[[Sudden cardiac death]] | ||
===Prognosis=== | ===Prognosis=== | ||
*Prognosis is generally | *[[Prognosis]] is generally poor, and the 10-year [[mortality]] of patients with [[CPVT]] is approximately 40%.<ref name="Sumitomo2003">{{cite journal|last1=Sumitomo|first1=N|title=Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death|journal=Heart|volume=89|issue=1|year=2003|pages=66–70|issn=00070769|doi=10.1136/heart.89.1.66}}</ref> | ||
* | *Studies show that there is a correlation between the age of the first [[syncope]] and the severity of the disease, with a worse prognosis in the case of early occurrence.<ref name="LeenhardtLucet1995">{{cite journal|last1=Leenhardt|first1=Antoine|last2=Lucet|first2=Vincent|last3=Denjoy|first3=Isabelle|last4=Grau|first4=Francis|last5=Ngoc|first5=Dien Do|last6=Coumel|first6=Philippe|title=Catecholaminergic Polymorphic Ventricular Tachycardia in Children|journal=Circulation|volume=91|issue=5|year=1995|pages=1512–1519|issn=0009-7322|doi=10.1161/01.CIR.91.5.1512}}</ref> | ||
* | *If left untreated, patients with [[CPVT]] have a [[mortality rate]] of 30% before age 40.<ref name="PrioriNapolitano2002">{{cite journal|last1=Priori|first1=Silvia G.|last2=Napolitano|first2=Carlo|last3=Memmi|first3=Mirella|last4=Colombi|first4=Barbara|last5=Drago|first5=Fabrizio|last6=Gasparini|first6=Maurizio|last7=DeSimone|first7=Luciano|last8=Coltorti|first8=Fernando|last9=Bloise|first9=Raffaella|last10=Keegan|first10=Roberto|last11=Cruz Filho|first11=Fernando E.S.|last12=Vignati|first12=Gabriele|last13=Benatar|first13=Abraham|last14=DeLogu|first14=Angelica|title=Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=106|issue=1|year=2002|pages=69–74|issn=0009-7322|doi=10.1161/01.CIR.0000020013.73106.D8}}</ref><ref name="HayashiDenjoy2009">{{cite journal|last1=Hayashi|first1=Meiso|last2=Denjoy|first2=Isabelle|last3=Extramiana|first3=Fabrice|last4=Maltret|first4=Alice|last5=Buisson|first5=Nathalie Roux|last6=Lupoglazoff|first6=Jean-Marc|last7=Klug|first7=Didier|last8=Hayashi|first8=Miyuki|last9=Takatsuki|first9=Seiji|last10=Villain|first10=Elisabeth|last11=Kamblock|first11=Joël|last12=Messali|first12=Anne|last13=Guicheney|first13=Pascale|last14=Lunardi|first14=Joël|last15=Leenhardt|first15=Antoine|title=Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation|volume=119|issue=18|year=2009|pages=2426–2434|issn=0009-7322|doi=10.1161/CIRCULATIONAHA.108.829267}}</ref> | ||
==References== | ==References== | ||
Latest revision as of 15:30, 23 July 2020
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mounika Reddy Vadiyala, M.B.B.S.[2]
Overview
If left untreated, approximately 30% of patients experience at least one cardiac arrest and up to 80% one or more syncopal spells. Common complications of catecholaminergic polymorphic ventricular tachycardia include ventricular fibrillation, sudden cardiac arrest, and sudden cardiac death. Prognosis is generally poor, and the 10-year mortality of patients with catecholaminergic polymorphic ventricular tachycardia is approximately 40%.
Natural History, Complications, and Prognosis
Natural History
- The symptoms of CPVT usually develop during childhood and adolescence, in the first and second decades of life, and start with symptoms such as episodes of syncope.
- More than 30% of affected individuals will experience symptoms before the age of 10 years and the majority (60% to 80%) of the patients will have one or more symptomatic arrhythmia episodes before age 40.[1] [2] [3] [4]
- However, molecular analysis showed that there is a small group of patients who remain asymptomatic, even after exercise tests suggesting them to be having normal phenotype CPVT (mutation carriers). Some of these phenotypically normal patients with CVPT do experience syncope and sudden death.[5]
- If left untreated, CPVT is highly lethal, as approximately 30% of patients experience at least one cardiac arrest and up to 80% one or more syncopal spells.[1][2]
- The polymorphic ventricular tachycardia may self-terminate or it may degenerate into ventricular fibrillation, causing sudden cardiac death.
Complications
- Common complications of catecholaminergic polymorphic ventricular tachycardia include:
Prognosis
- Prognosis is generally poor, and the 10-year mortality of patients with CPVT is approximately 40%.[3]
- Studies show that there is a correlation between the age of the first syncope and the severity of the disease, with a worse prognosis in the case of early occurrence.[1]
- If left untreated, patients with CPVT have a mortality rate of 30% before age 40.[2][5]
References
- ↑ 1.0 1.1 1.2 Leenhardt, Antoine; Lucet, Vincent; Denjoy, Isabelle; Grau, Francis; Ngoc, Dien Do; Coumel, Philippe (1995). "Catecholaminergic Polymorphic Ventricular Tachycardia in Children". Circulation. 91 (5): 1512–1519. doi:10.1161/01.CIR.91.5.1512. ISSN 0009-7322.
- ↑ 2.0 2.1 2.2 Priori, Silvia G.; Napolitano, Carlo; Memmi, Mirella; Colombi, Barbara; Drago, Fabrizio; Gasparini, Maurizio; DeSimone, Luciano; Coltorti, Fernando; Bloise, Raffaella; Keegan, Roberto; Cruz Filho, Fernando E.S.; Vignati, Gabriele; Benatar, Abraham; DeLogu, Angelica (2002). "Clinical and Molecular Characterization of Patients With Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation. 106 (1): 69–74. doi:10.1161/01.CIR.0000020013.73106.D8. ISSN 0009-7322.
- ↑ 3.0 3.1 Sumitomo, N (2003). "Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death". Heart. 89 (1): 66–70. doi:10.1136/heart.89.1.66. ISSN 0007-0769.
- ↑ Postma, A V (2005). "Catecholaminergic polymorphic ventricular tachycardia: RYR2 mutations, bradycardia, and follow up of the patients". Journal of Medical Genetics. 42 (11): 863–870. doi:10.1136/jmg.2004.028993. ISSN 1468-6244.
- ↑ 5.0 5.1 Hayashi, Meiso; Denjoy, Isabelle; Extramiana, Fabrice; Maltret, Alice; Buisson, Nathalie Roux; Lupoglazoff, Jean-Marc; Klug, Didier; Hayashi, Miyuki; Takatsuki, Seiji; Villain, Elisabeth; Kamblock, Joël; Messali, Anne; Guicheney, Pascale; Lunardi, Joël; Leenhardt, Antoine (2009). "Incidence and Risk Factors of Arrhythmic Events in Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation. 119 (18): 2426–2434. doi:10.1161/CIRCULATIONAHA.108.829267. ISSN 0009-7322.