Catecholaminergic polymorphic ventricular tachycardia differential diagnosis

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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

Catecholaminergic polymorphic ventricular tachycardia must be differentiated from Arrhythmogenic right ventricular dysplasia, Short-coupled ventricular tachycardia (SC-torsade de pointes [TdP]), Long QT syndrome and Andersen-Tawil syndrome.

Differentiating Catecholaminergic polymorphic ventricular tachycardia from other Diseases

Catecholaminergic polymorphic ventricular tachycardia must be differentiated from other diseases that cause syncope, ventricular tachycardia, and sudden cardiac death, such as:

Differentiating Catecholaminergic polymorphic ventricular tachycardia from other diseases on the basis of syncope, sudden cardiac death, and ventricular tachycardia

On the basis syncope, sudden cardiac death, and ventricular tachycardia, Catecholaminergic polymorphic ventricular tachycardia must be differentiated from Arrhythmogenic right ventricular dysplasia, Short-coupled ventricular tachycardia (SC-torsade de pointes TdP), Long QT syndrome, Andersen-Tawil syndrome and Brugada syndrome.

Diseases Cause Clinical manifestations ECG Structural abnormalities
ECG during rest ECG during exercise or stress
Catecholaminergic polymorphic ventricular tachycardia Mutations in RYR2 and CASQ2 genes Symptoms are exercise or emotion related -
Arrhythmogenic right ventricular dysplasia Usually caused by mutations in genes encoding for desmosomal proteins. Symptoms are usually exercise-related

Symptoms and signs related to right ventricular failure may also be seen.

Left bundle branch block pattern during tachycardia It primarily affects the right ventricle (RV). Changes seen are:[1]
  • Fatty infiltration of the RV free wall
  • Thinning of the RV myocardium
  • RV Dilation and Regional Wall Motion Abnormalities
Short QT syndrome Symptoms are not exercise-related or triggered

Physical examination is normal.

- -
Long QT syndrome Mutations in genes encoding for sodium and potassium ion channels in the heart. Symptoms are triggered by exercise, stress, certain drugs, etc
  • Prolongation of the QTc interval (>460 ms)
  • Abnormal T-wave morphology
  • Prolongation of the QTc interval (>460 ms)
  • Abnormal T-wave morphology
-
Andersen-Tawil syndrome Mutation in KCNJ2 gene. Symptoms are not related to adrenergic activation

Other significant findings include:

- -
Brugada syndrome Mutation in SCN5A gene. Symptoms occur predominantly during sleep or at rest

Other findings:

- -
Short-coupled ventricular tachycardia

(SC-torsade de pointes TdP)

Unknown Symptoms are not related to adrenergic stimuli, - -

References

  1. Hundley, W. Gregory; Bluemke, David A.; Finn, J. Paul; Flamm, Scott D.; Fogel, Mark A.; Friedrich, Matthias G.; Ho, Vincent B.; Jerosch-Herold, Michael; Kramer, Christopher M.; Manning, Warren J.; Patel, Manesh; Pohost, Gerald M.; Stillman, Arthur E.; White, Richard D.; Woodard, Pamela K. (2010). "ACCF/ACR/AHA/NASCI/SCMR 2010 Expert Consensus Document on Cardiovascular Magnetic Resonance". Circulation. 121 (22): 2462–2508. doi:10.1161/CIR.0b013e3181d44a8f. ISSN 0009-7322.

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