Catecholaminergic polymorphic ventricular tachycardia exercise stress testing: Difference between revisions
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==Exercise Stress Testing== | ==Exercise Stress Testing== | ||
*[[CPVT]] 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. | |||
*It has been observed that [[arrhythmias]] in [[CPVT]] often appear in a uniform and reproducible pattern that facilitates the recognition of affected patients.<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> | |||
*[[Exercise Stress Testing]] is the primary [[diagnostic]] test and the most helpful clinical tool in diagnosing [[CPVT]] as it can reproducibly evoke the typical [[ventricular tachycardia]] during acute [[adrenergic]] activation (e.g., exercise, acute emotion). | |||
*It may also be useful in monitoring the response to [[beta-blocker]] therapy of affected individuals in reproducible conditions. | |||
*During [[Exercise Stress Testing|exercise testing]], [[sinus rhythm]] accelerates and beyond a [[heart rate]] of 120-130 [[beats per minute]], isolated and often monomorphic [[Premature ventricular contraction|ventricular premature beats]] ([[Premature ventricular contraction|VPBs]]) typically occur first and then increase with [[heart rate]] to [[quadrigeminy]], [[trigeminy]], and [[Bigeminal rhythm|bigeminy]]. | |||
*Subsequently, the [[Premature ventricular contraction|VPBs]] become polymorphic, and as the exercise increase, they form bursts of non-sustained [[polymorphic ventricular tachycardia]] ([[VT]]). | |||
*If the activity is stopped, the [[arrhythmia]] disappears in the reverse order without clinical symptoms. | |||
*However, when the activity is continued, the [[arrhythmia]] persists and becomes more rapid, eventually assuming the appearance of [[polymorphic ventricular tachycardia]] ([[VT]]), which is very fast, [[fibrillation]]-like and leads to [[syncope]]. | |||
* Of note, in a subset of patients the [[ventricular arrhythmias]] already disappear with ongoing exercise.<ref name="FaggioniHwang2013">{{cite journal|last1=Faggioni|first1=Michela|last2=Hwang|first2=Hyun Seok|last3=van der Werf|first3=Christian|last4=Nederend|first4=Ineke|last5=Kannankeril|first5=Prince J.|last6=Wilde|first6=Arthur A.M.|last7=Knollmann|first7=Björn C.|title=Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular Tachycardia in Mice and in Patients|journal=Circulation Research|volume=112|issue=4|year=2013|pages=689–697|issn=0009-7330|doi=10.1161/CIRCRESAHA.111.300076}}</ref> | |||
* Another type of [[Polymorphic ventricular tachycardia|polymorphic VT]] observed in [[CPVT]] patients is the bidirectional [[VT]], which is a peculiar form of [[polymorphic ventricular tachycardia|polymorphic VT]] characterized by 180° rotation of the [[QRS]] complex from beat to beat | |||
*The occurrence of a bidirectional [[ventricular tachycardia]] ([[VT]]), which is the hallmark sign of [[CPVT]] is highly [[specificty|specific]] but not present in all patients. | |||
*The bidirectional [[VT]] seen in [[CPVT]] are thought to originate from the [[His-Purkinje system]] from the alternating activation of the [[purkinje fibers]] of the two [[ventricles]].<ref name="CerroneNoujaim2007">{{cite journal|last1=Cerrone|first1=Marina|last2=Noujaim|first2=Sami F.|last3=Tolkacheva|first3=Elena G.|last4=Talkachou|first4=Arkadzi|last5=O’Connell|first5=Ryan|last6=Berenfeld|first6=Omer|last7=Anumonwo|first7=Justus|last8=Pandit|first8=Sandeep V.|last9=Vikstrom|first9=Karen|last10=Napolitano|first10=Carlo|last11=Priori|first11=Silvia G.|last12=Jalife|first12=José|title=Arrhythmogenic Mechanisms in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia|journal=Circulation Research|volume=101|issue=10|year=2007|pages=1039–1048|issn=0009-7330|doi=10.1161/CIRCRESAHA.107.148064}}</ref><ref name="HerronMilstein2010">{{cite journal|last1=Herron|first1=Todd J.|last2=Milstein|first2=Michelle L.|last3=Anumonwo|first3=Justus|last4=Priori|first4=Silvia G.|last5=Jalife|first5=José|title=Purkinje cell calcium dysregulation is the cellular mechanism that underlies catecholaminergic polymorphic ventricular tachycardia|journal=Heart Rhythm|volume=7|issue=8|year=2010|pages=1122–1128|issn=15475271|doi=10.1016/j.hrthm.2010.06.010}}</ref><ref name="CerroneColombi2005">{{cite journal|last1=Cerrone|first1=Marina|last2=Colombi|first2=Barbara|last3=Santoro|first3=Massimo|last4=di Barletta|first4=Marina Raffaele|last5=Scelsi|first5=Mario|last6=Villani|first6=Laura|last7=Napolitano|first7=Carlo|last8=Priori|first8=Silvia G|title=Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model Carrier of a Mutation in the Cardiac Ryanodine Receptor|journal=Circulation Research|volume=96|issue=10|year=2005|issn=0009-7330|doi=10.1161/01.RES.0000169067.51055.72}}</ref> | |||
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{{familytree | | | | | | | | | |!| | | | | | | | }} | |||
{{familytree | | | | | | | | | B01 | | | | | |B01=Increase in [[sinus rhythm]]}} | |||
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{{familytree | | | | | | | | | C01 | | | | | |C01=Monomorphic [[premature ventricular contractions]] ([[PVC]]s)}} | |||
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{{familytree | | D01 | | | | | | | | | | | |D02|D01=Polymorphic [[Premature ventricular contraction|PVC]] [[Bigeminy]]|D02=Bidirectional [[Premature ventricular contraction|PVC]] [[Bigeminy]]}} | |||
{{familytree | | |!| | | | | | | | | | | | | |!| }} | |||
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<br> | |||
Revision as of 13:58, 23 July 2020
Overview
Exercise Stress Testing
- CPVT 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.
- It has been observed that arrhythmias in CPVT often appear in a uniform and reproducible pattern that facilitates the recognition of affected patients.[1]
- Exercise Stress Testing is the primary diagnostic test and the most helpful clinical tool in diagnosing CPVT as it can reproducibly evoke the typical ventricular tachycardia during acute adrenergic activation (e.g., exercise, acute emotion).
- It may also be useful in monitoring the response to beta-blocker therapy of affected individuals in reproducible conditions.
- During exercise testing, sinus rhythm accelerates and beyond a heart rate of 120-130 beats per minute, isolated and often monomorphic ventricular premature beats (VPBs) typically occur first and then increase with heart rate to quadrigeminy, trigeminy, and bigeminy.
- Subsequently, the VPBs become polymorphic, and as the exercise increase, they form bursts of non-sustained polymorphic ventricular tachycardia (VT).
- If the activity is stopped, the arrhythmia disappears in the reverse order without clinical symptoms.
- However, when the activity is continued, the arrhythmia persists and becomes more rapid, eventually assuming the appearance of polymorphic ventricular tachycardia (VT), which is very fast, fibrillation-like and leads to syncope.
- Of note, in a subset of patients the ventricular arrhythmias already disappear with ongoing exercise.[2]
- Another type of polymorphic VT observed in CPVT patients is the bidirectional VT, which is a peculiar form of polymorphic VT characterized by 180° rotation of the QRS complex from beat to beat
- The occurrence of a bidirectional ventricular tachycardia (VT), which is the hallmark sign of CPVT is highly specific but not present in all patients.
- The bidirectional VT seen in CPVT are thought to originate from the His-Purkinje system from the alternating activation of the purkinje fibers of the two ventricles.[3][4][5]
| Exercise stress testing | |||||||||||||||||||||||||||||||||||
| Increase in sinus rhythm | |||||||||||||||||||||||||||||||||||
| Monomorphic premature ventricular contractions (PVCs) | |||||||||||||||||||||||||||||||||||
| Polymorphic PVC Bigeminy | Bidirectional PVC Bigeminy | ||||||||||||||||||||||||||||||||||
| Polymorphic VT | Bidirectional VT | ||||||||||||||||||||||||||||||||||
- ↑ 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.
- ↑ Faggioni, Michela; Hwang, Hyun Seok; van der Werf, Christian; Nederend, Ineke; Kannankeril, Prince J.; Wilde, Arthur A.M.; Knollmann, Björn C. (2013). "Accelerated Sinus Rhythm Prevents Catecholaminergic Polymorphic Ventricular Tachycardia in Mice and in Patients". Circulation Research. 112 (4): 689–697. doi:10.1161/CIRCRESAHA.111.300076. ISSN 0009-7330.
- ↑ Cerrone, Marina; Noujaim, Sami F.; Tolkacheva, Elena G.; Talkachou, Arkadzi; O’Connell, Ryan; Berenfeld, Omer; Anumonwo, Justus; Pandit, Sandeep V.; Vikstrom, Karen; Napolitano, Carlo; Priori, Silvia G.; Jalife, José (2007). "Arrhythmogenic Mechanisms in a Mouse Model of Catecholaminergic Polymorphic Ventricular Tachycardia". Circulation Research. 101 (10): 1039–1048. doi:10.1161/CIRCRESAHA.107.148064. ISSN 0009-7330.
- ↑ Herron, Todd J.; Milstein, Michelle L.; Anumonwo, Justus; Priori, Silvia G.; Jalife, José (2010). "Purkinje cell calcium dysregulation is the cellular mechanism that underlies catecholaminergic polymorphic ventricular tachycardia". Heart Rhythm. 7 (8): 1122–1128. doi:10.1016/j.hrthm.2010.06.010. ISSN 1547-5271.
- ↑ Cerrone, Marina; Colombi, Barbara; Santoro, Massimo; di Barletta, Marina Raffaele; Scelsi, Mario; Villani, Laura; Napolitano, Carlo; Priori, Silvia G (2005). "Bidirectional Ventricular Tachycardia and Fibrillation Elicited in a Knock-In Mouse Model Carrier of a Mutation in the Cardiac Ryanodine Receptor". Circulation Research. 96 (10). doi:10.1161/01.RES.0000169067.51055.72. ISSN 0009-7330.