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Torsade-de-pointes syndrome with short coupling interval

MedGen UID:
462201
•Concept ID:
C3150851
•
Disease or Syndrome
Synonym: Torsade de pointes, short-coupled variant
SNOMED CT: Torsade de pointes with short coupling interval syndrome (718104007); Torsade de pointes short coupled variant (718104007)
 
Monarch Initiative: MONDO:0013317
OMIM®: 613600
Orphanet: ORPHA51084

Definition

Torsade de pointes is characterized by an electrocardiographic (ECG) pattern of nonuniform but still-organized electrical activity with progressive changes in morphology, amplitude, and polarity of the QRS complexes, the peaks of which twist around the isoelectric baseline before ending spontaneously. In classic torsade de pointes, the coupling interval of the first beat is long (see LQT1, 192500), whereas in this short-coupled variant, the coupling interval of the first beat is very short (summary by Leenhardt et al., 1994). [from OMIM]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTorsade-de-pointes syndrome with short coupling interval
Follow this link to review classifications for Torsade-de-pointes syndrome with short coupling interval in Orphanet.

Professional guidelines

PubMed

Polymorphic ventricular tachycardia, ischaemic ventricular fibrillation, and torsade de pointes: importance of the QT and the coupling interval in the differential diagnosis.
Rosso R, Hochstadt A, Viskin D, Chorin E, Schwartz AL, Tovia-Brodie O, Laish-Farkash A, Havakuk O, Gepstein L, Banai S, Viskin S
Eur Heart J 2021 Oct 7;42(38):3965-3975. doi: 10.1093/eurheartj/ehab138. PMID: 33693589

Recent clinical studies

Etiology

Polymorphic ventricular tachycardia, ischaemic ventricular fibrillation, and torsade de pointes: importance of the QT and the coupling interval in the differential diagnosis.
Rosso R, Hochstadt A, Viskin D, Chorin E, Schwartz AL, Tovia-Brodie O, Laish-Farkash A, Havakuk O, Gepstein L, Banai S, Viskin S
Eur Heart J 2021 Oct 7;42(38):3965-3975. doi: 10.1093/eurheartj/ehab138. PMID: 33693589
QT Assessment in Early Drug Development: The Long and the Short of It.
Lester RM, Paglialunga S, Johnson IA
Int J Mol Sci 2019 Mar 15;20(6) doi: 10.3390/ijms20061324. PMID: 30884748Free PMC Article
The natural history of fetal long QT syndrome.
Cuneo BF, Strasburger JF, Wakai RT
J Electrocardiol 2016 Nov-Dec;49(6):807-813. Epub 2016 Jul 28 doi: 10.1016/j.jelectrocard.2016.07.023. PMID: 27539165Free PMC Article
Electrocardiographic Parameters Indicating Worse Evolution in Patients with Acquired Long QT Syndrome and Torsades de Pointes.
Kukla P, Jastrzębski M, Fijorek K, Stec S, Bryniarski L, Czarnecka D, Baranchuk A
Ann Noninvasive Electrocardiol 2016 Nov;21(6):572-579. Epub 2016 Mar 28 doi: 10.1111/anec.12355. PMID: 27018992Free PMC Article
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias.
Leenhardt A, Glaser E, Burguera M, Nürnberg M, Maison-Blanche P, Coumel P
Circulation 1994 Jan;89(1):206-15. doi: 10.1161/01.cir.89.1.206. PMID: 8281648

Diagnosis

Polymorphic ventricular tachycardia, ischaemic ventricular fibrillation, and torsade de pointes: importance of the QT and the coupling interval in the differential diagnosis.
Rosso R, Hochstadt A, Viskin D, Chorin E, Schwartz AL, Tovia-Brodie O, Laish-Farkash A, Havakuk O, Gepstein L, Banai S, Viskin S
Eur Heart J 2021 Oct 7;42(38):3965-3975. doi: 10.1093/eurheartj/ehab138. PMID: 33693589
QT Assessment in Early Drug Development: The Long and the Short of It.
Lester RM, Paglialunga S, Johnson IA
Int J Mol Sci 2019 Mar 15;20(6) doi: 10.3390/ijms20061324. PMID: 30884748Free PMC Article
The classical "R-on-T" phenomenon.
Oksuz F, Sensoy B, Sahan E, Sen F, Baser K, Cetin H, Unal S, Ozeke O, Topaloglu S, Aras D
Indian Heart J 2015 Jul-Aug;67(4):392-4. Epub 2015 Apr 27 doi: 10.1016/j.ihj.2015.02.030. PMID: 26304578Free PMC Article
Short QT syndrome: a case report and review of literature.
Lu LX, Zhou W, Zhang X, Cao Q, Yu K, Zhu C
Resuscitation 2006 Oct;71(1):115-21. Epub 2006 Aug 30 doi: 10.1016/j.resuscitation.2006.03.011. PMID: 16942825
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias.
Leenhardt A, Glaser E, Burguera M, Nürnberg M, Maison-Blanche P, Coumel P
Circulation 1994 Jan;89(1):206-15. doi: 10.1161/01.cir.89.1.206. PMID: 8281648

Therapy

Quinidine-Responsive Polymorphic Ventricular Tachycardia in Patients With Coronary Heart Disease.
Viskin S, Chorin E, Viskin D, Hochstadt A, Halkin A, Tovia-Brodie O, Lee JK, Asher E, Laish-Farkash A, Amit G, Havakuk O, Belhassen B, Rosso R
Circulation 2019 May 14;139(20):2304-2314. doi: 10.1161/CIRCULATIONAHA.118.038036. PMID: 30696267
In utero diagnosis of long QT syndrome by magnetocardiography.
Cuneo BF, Strasburger JF, Yu S, Horigome H, Hosono T, Kandori A, Wakai RT
Circulation 2013 Nov 12;128(20):2183-91. doi: 10.1161/CIRCULATIONAHA.113.004840. PMID: 24218437Free PMC Article
Extreme QT prolongation during therapeutic hypothermia after cardiac arrest due to long QT syndrome.
Nishiyama N, Sato T, Aizawa Y, Nakagawa S, Kanki H
Am J Emerg Med 2012 May;30(4):638.e5-8. Epub 2011 Apr 2 doi: 10.1016/j.ajem.2011.02.019. PMID: 21459539
Short QT syndrome: a case report and review of literature.
Lu LX, Zhou W, Zhang X, Cao Q, Yu K, Zhu C
Resuscitation 2006 Oct;71(1):115-21. Epub 2006 Aug 30 doi: 10.1016/j.resuscitation.2006.03.011. PMID: 16942825
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias.
Leenhardt A, Glaser E, Burguera M, Nürnberg M, Maison-Blanche P, Coumel P
Circulation 1994 Jan;89(1):206-15. doi: 10.1161/01.cir.89.1.206. PMID: 8281648

Prognosis

QT Assessment in Early Drug Development: The Long and the Short of It.
Lester RM, Paglialunga S, Johnson IA
Int J Mol Sci 2019 Mar 15;20(6) doi: 10.3390/ijms20061324. PMID: 30884748Free PMC Article
Quinidine-Responsive Polymorphic Ventricular Tachycardia in Patients With Coronary Heart Disease.
Viskin S, Chorin E, Viskin D, Hochstadt A, Halkin A, Tovia-Brodie O, Lee JK, Asher E, Laish-Farkash A, Amit G, Havakuk O, Belhassen B, Rosso R
Circulation 2019 May 14;139(20):2304-2314. doi: 10.1161/CIRCULATIONAHA.118.038036. PMID: 30696267
Electrocardiographic Parameters Indicating Worse Evolution in Patients with Acquired Long QT Syndrome and Torsades de Pointes.
Kukla P, Jastrzębski M, Fijorek K, Stec S, Bryniarski L, Czarnecka D, Baranchuk A
Ann Noninvasive Electrocardiol 2016 Nov;21(6):572-579. Epub 2016 Mar 28 doi: 10.1111/anec.12355. PMID: 27018992Free PMC Article
In utero diagnosis of long QT syndrome by magnetocardiography.
Cuneo BF, Strasburger JF, Yu S, Horigome H, Hosono T, Kandori A, Wakai RT
Circulation 2013 Nov 12;128(20):2183-91. doi: 10.1161/CIRCULATIONAHA.113.004840. PMID: 24218437Free PMC Article
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias.
Leenhardt A, Glaser E, Burguera M, Nürnberg M, Maison-Blanche P, Coumel P
Circulation 1994 Jan;89(1):206-15. doi: 10.1161/01.cir.89.1.206. PMID: 8281648

Clinical prediction guides

QT Assessment in Early Drug Development: The Long and the Short of It.
Lester RM, Paglialunga S, Johnson IA
Int J Mol Sci 2019 Mar 15;20(6) doi: 10.3390/ijms20061324. PMID: 30884748Free PMC Article
Electrocardiographic Parameters Indicating Worse Evolution in Patients with Acquired Long QT Syndrome and Torsades de Pointes.
Kukla P, Jastrzębski M, Fijorek K, Stec S, Bryniarski L, Czarnecka D, Baranchuk A
Ann Noninvasive Electrocardiol 2016 Nov;21(6):572-579. Epub 2016 Mar 28 doi: 10.1111/anec.12355. PMID: 27018992Free PMC Article
In utero diagnosis of long QT syndrome by magnetocardiography.
Cuneo BF, Strasburger JF, Yu S, Horigome H, Hosono T, Kandori A, Wakai RT
Circulation 2013 Nov 12;128(20):2183-91. doi: 10.1161/CIRCULATIONAHA.113.004840. PMID: 24218437Free PMC Article
Classification and mechanism of Torsade de Pointes initiation in patients with congenital long QT syndrome.
Noda T, Shimizu W, Satomi K, Suyama K, Kurita T, Aihara N, Kamakura S
Eur Heart J 2004 Dec;25(23):2149-54. doi: 10.1016/j.ehj.2004.08.020. PMID: 15571831
Short-coupled variant of torsade de pointes. A new electrocardiographic entity in the spectrum of idiopathic ventricular tachyarrhythmias.
Leenhardt A, Glaser E, Burguera M, Nürnberg M, Maison-Blanche P, Coumel P
Circulation 1994 Jan;89(1):206-15. doi: 10.1161/01.cir.89.1.206. PMID: 8281648

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