CPVT

File:ECG000032.jpg

The ECG of a patient with CPVT in rest is normal

File:ECG000033.jpg

The ECG of the same patient with CPVT during exercise. Asterisks mark polymorphic ventricular beats.

Catecholaminergic Polymorphic Ventricular Tachycardia is a congenital disease that leads to exercise induced ventricular arrhythmias and / or syncope and carries an increased risk of sudden death.

Characteristics of CPVT:

• The mean onset of arrhythmias is 7-9 years
• Absence of structural cardiac abnormalities
• Normal resting ECG
• Syncope during physical activity or emotional stress

Diagnosis

• The diagnosis is based on the patient's clinical history (dizziness or syncope induced by exercise or emotional stress and a family history containing syncope or sudden death in young relatives related to similar triggers) and reproducible ventricular arrhythmias during exercise testing. The complexity of these arrhythmias often increases with increasing work load, starting with Ventricular Premature Beats, and ending with bidirectional ventricular tachycardia to polymorphic ventricular tachycardia.
• Two genes have been linked to CPVT. Both lead to a defect in intracellular calcium metabolism:
  • the hRyR2 gene, coding for the cardiac ryanodine receptor: (OMIM™ link 180902) (50-55 % of patients)
  • the CASQ2 gene, coding for the calsequestrine protein: (OMIM™ link 114251) (1-2 % of patients)

Treatment[1]

• Beta-blockers
• ICD (Internal Cardioverter Defibrillator) implantation combined with beta-blockers in CPVT patients who survived a cardiac arrest or patients with syncope and/or documented sustained ventricular tachycardia despite beta-blocker therapy.[2]
• Surgical left cardiac sympathetic denervation in selected patients whose symptoms and/or ventricular arrhythmias are not controlled by pharmacologic therapy [3][4]
• Avoid competitive and other strenuous exercise

References

1. Sumitomo N, Harada K, Nagashima M, Yasuda T, Nakamura Y, Aragaki Y, Saito A, Kurosaki K, Jouo K, Koujiro M, Konishi S, Matsuoka S, Oono T, Hayakawa S, Miura M, Ushinohama H, Shibata T, and Niimura I. Catecholaminergic polymorphic ventricular tachycardia: electrocardiographic characteristics and optimal therapeutic strategies to prevent sudden death. Heart. 2003 Jan;89(1):66-70. DOI:10.1136/heart.89.1.66 |
2. Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M, Gregoratos G, Klein G, Moss AJ, Myerburg RJ, Priori SG, Quinones MA, Roden DM, Silka MJ, Tracy C, Smith SC Jr, Jacobs AK, Adams CD, Antman EM, Anderson JL, Hunt SA, Halperin JL, Nishimura R, Ornato JP, Page RL, Riegel B, Blanc JJ, Budaj A, Dean V, Deckers JW, Despres C, Dickstein K, Lekakis J, McGregor K, Metra M, Morais J, Osterspey A, Tamargo JL, Zamorano JL, American College of Cardiology/American Heart Association Task Force, European Society of Cardiology Committee for Practice Guidelines, European Heart Rhythm Association, and Heart Rhythm Society. ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. Circulation. 2006 Sep 5;114(10):e385-484. DOI:10.1161/CIRCULATIONAHA.106.178233 |
3. Wilde AA, Bhuiyan ZA, Crotti L, Facchini M, De Ferrari GM, Paul T, Ferrandi C, Koolbergen DR, Odero A, and Schwartz PJ. Left cardiac sympathetic denervation for catecholaminergic polymorphic ventricular tachycardia. N Engl J Med. 2008 May 8;358(19):2024-9. DOI:10.1056/NEJMoa0708006 |
4. Collura CA, Johnson JN, Moir C, and Ackerman MJ. Left cardiac sympathetic denervation for the treatment of long QT syndrome and catecholaminergic polymorphic ventricular tachycardia using video-assisted thoracic surgery. Heart Rhythm. 2009 Jun;6(6):752-9. DOI:10.1016/j.hrthm.2009.03.024 |