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{{Catecholaminergic polymorphic ventricular tachycardia}}
{{Catecholaminergic polymorphic ventricular tachycardia}}
{{CMG}}; {{AE}}
{{CMG}}; {{AE}}{{MRV}}
== Overview ==
== Overview ==
[[Exercise stress testing]] helps in establishing the diagnosis and [[genetic testing]] helps in confirming the diagnosis of catecholaminergic polymorphic ventricular tachycardia.


== Diagnostic Study of Choice ==
== Diagnostic Study of Choice ==
*Catecholaminergic polymorphic ventricular tachycardia is a diagnosis based on reproducing [[ventricular arrhythmias]] during exercise stress testing, [[syncope]] occurring during physical activity and acute emotion, and a history of exercise or emotion-related [[palpitations]] and [[dizziness]] with an absence of structural [[cardiac]] abnormalities.


=== Study of choice ===
=== Study of choice ===
[Name of the investigation] is the gold standard test for the diagnosis of [disease name].
*[[Exercise stress testing]] is the gold standard test for the diagnosis of catecholaminergic polymorphic ventricular tachycardia.<ref name="ImbertiUnderwood2016">{{cite journal|last1=Imberti|first1=Jacopo F.|last2=Underwood|first2=Katherine|last3=Mazzanti|first3=Andrea|last4=Priori|first4=Silvia G.|title=Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Heart, Lung and Circulation|volume=25|issue=8|year=2016|pages=777–783|issn=14439506|doi=10.1016/j.hlc.2016.01.012}}</ref>
 
*[[Genetic testing]] helps in confirming the diagnosis of catecholaminergic polymorphic ventricular tachycardia.<ref name="AckermanPriori2011">{{cite journal|last1=Ackerman|first1=M. J.|last2=Priori|first2=S. G.|last3=Willems|first3=S.|last4=Berul|first4=C.|last5=Brugada|first5=R.|last6=Calkins|first6=H.|last7=Camm|first7=A. J.|last8=Ellinor|first8=P. T.|last9=Gollob|first9=M.|last10=Hamilton|first10=R.|last11=Hershberger|first11=R. E.|last12=Judge|first12=D. P.|last13=Le Marec|first13=H.|last14=McKenna|first14=W. J.|last15=Schulze-Bahr|first15=E.|last16=Semsarian|first16=C.|last17=Towbin|first17=J. A.|last18=Watkins|first18=H.|last19=Wilde|first19=A.|last20=Wolpert|first20=C.|last21=Zipes|first21=D. P.|title=HRS/EHRA Expert Consensus Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies: This document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA)|journal=Europace|volume=13|issue=8|year=2011|pages=1077–1109|issn=1099-5129|doi=10.1093/europace/eur245}}</ref><ref>{{cite web |url=https://www.ncbi.nlm.nih.gov/books/NBK1289/# |title=Catecholaminergic Polymorphic Ventricular Tachycardia - GeneReviews® - NCBI Bookshelf |format= |work= |accessdate=}}</ref>
OR
 
The following result of [gold standard test] is confirmatory of [disease name]:
* [Result 1]
* [Result 2]
 
OR
 
[Name of the investigation] must be performed when:
* The patient presents with [symptom/sign 1], [symptom/sign 2], and [symptom/sign 3].
* A [name of test] is positive for [sign 1], [sign 2], and [sign 3] in the patient.
 
OR
 
[Name of the investigation] is the gold standard test for the diagnosis of [disease name].
 
OR
 
The diagnostic study of choice for [disease name] is [name of the investigation].
 
OR
 
There is no single diagnostic study of choice for the diagnosis of [disease name].  
 
OR
 
There is no single diagnostic study of choice for the diagnosis of [disease name], but [disease name] can be diagnosed based on [name of the investigation 1] and [name of the investigation 2].
 
OR
 
[Disease name] is primarily diagnosed based on the clinical presentation.
 
OR
 
Investigations:
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most specific test for the diagnosis.
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most sensitive test for diagnosis.
* Among the patients who present with clinical signs of [disease name], the [investigation name] is the most efficient test for diagnosis.
 
==== The comparison of various diagnostic studies for [disease name] ====
{|
|- style="background: #4479BA; color: #FFFFFF; text-align: center;"
! style="background: #4479BA; color: #FFFFFF; text-align: center;" | Test
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Sensitivity
! style="background: #4479BA; color: #FFFFFF; text-align: center;" |Specificity
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 1
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|-
! style="background: #696969; color: #FFFFFF; text-align: center;" |Test 2
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
| style="background: #DCDCDC; padding: 5px; text-align: center;" |...%
|}
<small> [Name of test with higher sensitivity and specificity] is the preferred investigation based on the sensitivity and specificity</small>
 
===== Diagnostic results =====
The following finding(s) on performing [investigation name] is(are) confirmatory for [disease name]:
* [Finding 1]
* [Finding 2]
 
===== Sequence of Diagnostic Studies =====
The [name of investigation] must be performed when:
* The patient presented with symptoms/signs 1, 2, and 3 as the first step of diagnosis.
* A positive [test] is detected in the patient, to confirm the diagnosis.
 
OR
 
The various investigations must be performed in the following order:
* [Initial investigation]
* [2nd investigation]
 
=== Name of Diagnostic Criteria ===
 
'''It is recommended that you include the criteria in a table. Make sure you always cite the source of the content and whether the table has been adapted from another source.'''
 
[Disease name] is primarily diagnosed based on clinical presentation. There are no established criteria for the diagnosis of [disease name].
 
OR
 
There is no single diagnostic study of choice for [disease name], though [disease name] may be diagnosed based on [name of criteria] established by [...].
 
OR
 
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
 
OR
 
The diagnosis of [disease name] is based on the [criteria name] criteria, which includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
[Disease name] may be diagnosed at any time if one or more of the following criteria are met:
* Criteria 1
* Criteria 2
* Criteria 3
 
OR
 
'''IF there are clear, established diagnostic criteria'''
 
The diagnosis of [disease name] is made when at least [number] of the following [number] diagnostic criteria are met: [criterion 1], [criterion 2], [criterion 3], and [criterion 4].
 
OR
 
The diagnosis of [disease name] is based on the [criteria name] criteria, which include [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
The diagnosis of [disease name] is based on the [definition name] definition, which includes [criterion 1], [criterion 2], and [criterion 3].
 
OR
 
'''IF there are no established diagnostic criteria'''


There are no established criteria for the diagnosis of [disease name].
=== Diagnostic Criteria ===
The diagnosis of [[CPVT]] is made when at least one of the following four diagnostic criteria are met:<ref name="PrioriWilde2013">{{cite journal|last1=Priori|first1=Silvia G.|last2=Wilde|first2=Arthur A.|last3=Horie|first3=Minoru|last4=Cho|first4=Yongkeun|last5=Behr|first5=Elijah R.|last6=Berul|first6=Charles|last7=Blom|first7=Nico|last8=Brugada|first8=Josep|last9=Chiang|first9=Chern-En|last10=Huikuri|first10=Heikki|last11=Kannankeril|first11=Prince|last12=Krahn|first12=Andrew|last13=Leenhardt|first13=Antoine|last14=Moss|first14=Arthur|last15=Schwartz|first15=Peter J.|last16=Shimizu|first16=Wataru|last17=Tomaselli|first17=Gordon|last18=Tracy|first18=Cynthia|last19=Ackerman|first19=Michael|last20=Belhassen|first20=Bernard|last21=Estes|first21=N. A. Mark|last22=Fatkin|first22=Diane|last23=Kalman|first23=Jonathan|last24=Kaufman|first24=Elizabeth|last25=Kirchhof|first25=Paulus|last26=Schulze-Bahr|first26=Eric|last27=Wolpert|first27=Christian|last28=Vohra|first28=Jitendra|last29=Refaat|first29=Marwan|last30=Etheridge|first30=Susan P.|last31=Campbell|first31=Robert M.|last32=Martin|first32=Edward T.|last33=Quek|first33=Swee Chye|title=Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes|journal=EP Europace|volume=15|issue=10|year=2013|pages=1389–1406|issn=1532-2092|doi=10.1093/europace/eut272}}</ref><ref name="PrioriBlomström-Lundqvist2015">{{cite journal|last1=Priori|first1=Silvia G.|last2=Blomström-Lundqvist|first2=Carina|last3=Mazzanti|first3=Andrea|last4=Blom|first4=Nico|last5=Borggrefe|first5=Martin|last6=Camm|first6=John|last7=Elliott|first7=Perry Mark|last8=Fitzsimons|first8=Donna|last9=Hatala|first9=Robert|last10=Hindricks|first10=Gerhard|last11=Kirchhof|first11=Paulus|last12=Kjeldsen|first12=Keld|last13=Kuck|first13=Karl-Heinz|last14=Hernandez-Madrid|first14=Antonio|last15=Nikolaou|first15=Nikolaos|last16=Norekvål|first16=Tone M.|last17=Spaulding|first17=Christian|last18=Van Veldhuisen|first18=Dirk J.|title=2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death|journal=European Heart Journal|volume=36|issue=41|year=2015|pages=2793–2867|issn=0195-668X|doi=10.1093/eurheartj/ehv316}}</ref>
# [[CPVT]] is diagnosed in the presence of a structurally normal [[heart]], normal [[ECG]], and unexplained exercise or [[catecholamine]]-induced bidirectional [[VT]], polymorphic [[premature ventricular beats|ventricular premature beats]] or [[VT]] in individuals <40 years of age.
# [[CPVT]] is diagnosed in patients (index case or family member) who have a pathogenic [[mutation]].
# [[CPVT]] is diagnosed in family members of a [[CPVT]] index case with a normal [[heart]] who manifest exercise-induced [[premature ventricular contractions]] or bidirectional/ [[polymorphic VT]].
# [[CPVT]] can be diagnosed in the presence of a structurally normal [[heart]] and [[coronary arteries]], normal [[ECG]], and unexplained exercise or [[catecholamine]]-induced bidirectional [[VT]], polymorphic [[premature ventricular beats|ventricular premature beats]] or [[VT]] in individuals >40 years of age.


==References==
==References==

Latest revision as of 15:31, 23 July 2020

Catecholaminergic polymorphic ventricular tachycardia Microchapters

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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

Exercise stress testing helps in establishing the diagnosis and genetic testing helps in confirming the diagnosis of catecholaminergic polymorphic ventricular tachycardia.

Diagnostic Study of Choice

  • Catecholaminergic polymorphic ventricular tachycardia is a diagnosis based on reproducing ventricular arrhythmias during exercise stress testing, syncope occurring during physical activity and acute emotion, and a history of exercise or emotion-related palpitations and dizziness with an absence of structural cardiac abnormalities.

Study of choice

  • Exercise stress testing is the gold standard test for the diagnosis of catecholaminergic polymorphic ventricular tachycardia.[1]
  • Genetic testing helps in confirming the diagnosis of catecholaminergic polymorphic ventricular tachycardia.[2][3]

Diagnostic Criteria

The diagnosis of CPVT is made when at least one of the following four diagnostic criteria are met:[4][5]

  1. CPVT is diagnosed in the presence of a structurally normal heart, normal ECG, and unexplained exercise or catecholamine-induced bidirectional VT, polymorphic ventricular premature beats or VT in individuals <40 years of age.
  2. CPVT is diagnosed in patients (index case or family member) who have a pathogenic mutation.
  3. CPVT is diagnosed in family members of a CPVT index case with a normal heart who manifest exercise-induced premature ventricular contractions or bidirectional/ polymorphic VT.
  4. CPVT can be diagnosed in the presence of a structurally normal heart and coronary arteries, normal ECG, and unexplained exercise or catecholamine-induced bidirectional VT, polymorphic ventricular premature beats or VT in individuals >40 years of age.

References

  1. ↑ Imberti, Jacopo F.; Underwood, Katherine; Mazzanti, Andrea; Priori, Silvia G. (2016). "Clinical Challenges in Catecholaminergic Polymorphic Ventricular Tachycardia". Heart, Lung and Circulation. 25 (8): 777–783. doi:10.1016/j.hlc.2016.01.012. ISSN 1443-9506.
  2. ↑ Ackerman, M. J.; Priori, S. G.; Willems, S.; Berul, C.; Brugada, R.; Calkins, H.; Camm, A. J.; Ellinor, P. T.; Gollob, M.; Hamilton, R.; Hershberger, R. E.; Judge, D. P.; Le Marec, H.; McKenna, W. J.; Schulze-Bahr, E.; Semsarian, C.; Towbin, J. A.; Watkins, H.; Wilde, A.; Wolpert, C.; Zipes, D. P. (2011). "HRS/EHRA Expert Consensus Statement on the State of Genetic Testing for the Channelopathies and Cardiomyopathies: This document was developed as a partnership between the Heart Rhythm Society (HRS) and the European Heart Rhythm Association (EHRA)". Europace. 13 (8): 1077–1109. doi:10.1093/europace/eur245. ISSN 1099-5129.
  3. ↑ "Catecholaminergic Polymorphic Ventricular Tachycardia - GeneReviews® - NCBI Bookshelf".
  4. ↑ Priori, Silvia G.; Wilde, Arthur A.; Horie, Minoru; Cho, Yongkeun; Behr, Elijah R.; Berul, Charles; Blom, Nico; Brugada, Josep; Chiang, Chern-En; Huikuri, Heikki; Kannankeril, Prince; Krahn, Andrew; Leenhardt, Antoine; Moss, Arthur; Schwartz, Peter J.; Shimizu, Wataru; Tomaselli, Gordon; Tracy, Cynthia; Ackerman, Michael; Belhassen, Bernard; Estes, N. A. Mark; Fatkin, Diane; Kalman, Jonathan; Kaufman, Elizabeth; Kirchhof, Paulus; Schulze-Bahr, Eric; Wolpert, Christian; Vohra, Jitendra; Refaat, Marwan; Etheridge, Susan P.; Campbell, Robert M.; Martin, Edward T.; Quek, Swee Chye (2013). "Executive summary: HRS/EHRA/APHRS expert consensus statement on the diagnosis and management of patients with inherited primary arrhythmia syndromes". EP Europace. 15 (10): 1389–1406. doi:10.1093/europace/eut272. ISSN 1532-2092.
  5. ↑ Priori, Silvia G.; Blomström-Lundqvist, Carina; Mazzanti, Andrea; Blom, Nico; Borggrefe, Martin; Camm, John; Elliott, Perry Mark; Fitzsimons, Donna; Hatala, Robert; Hindricks, Gerhard; Kirchhof, Paulus; Kjeldsen, Keld; Kuck, Karl-Heinz; Hernandez-Madrid, Antonio; Nikolaou, Nikolaos; Norekvål, Tone M.; Spaulding, Christian; Van Veldhuisen, Dirk J. (2015). "2015 ESC Guidelines for the management of patients with ventricular arrhythmias and the prevention of sudden cardiac death". European Heart Journal. 36 (41): 2793–2867. doi:10.1093/eurheartj/ehv316. ISSN 0195-668X.

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