Quinidine for Brugada syndrome

Quinidine for Brugada syndrome

Brugada syndrome is an inherited cardiac channelopathy with potential for life threatening ventricular arrhythmias and sudden cardiac death [1]. Coved ST segment elevation of 2 mm or more with negative T wave in right precordial leads V1 and V2, either spontaneously or after challenge with sodium channel blocker drug is the characteristic ECG finding. Though implantation of an ICD (implantable cardioverter-defibrillator is the main treatment, quinidine for Brugada syndrome is an option worth it in certain situations [2].

Quinidine is a Class I antiarrhythmic agent which can block I_to and I_Kr currents. It has been shown to prevent phase II re-entry and ventricular fibrillation in experimental studies on Brugada syndrome. When ventricular fibrillation was inducible at baseline electrophysiology studies, quinidine can render it non inducible in 76% to 88% [2]. Quinidine has been reported to be useful in pediatric patients as an alternative to ICD [3]. It may be useful in the setting of arrhythmic storms and to reduce the number of ICD shocks.

Belhassen B et al prospectively evaluated 25 patients with Brugada syndrome, of which 7 were cardiac arrest survivors and 7 had unexplained syncope [4]. In all 25 patients, ventricular fibrillation could be induced at baseline electrophysiological study. Twenty two of the 25  (88%) were non-inducible on while Quinidine. Long term treatment ranging from 6 to 219 months was given to 19 patients. None had arrhythmic events, though 2 had non arrhythmia related syncope. Side effects occurred in 36% which resolved on drug discontinuation.

Side effects of quinidine like QT prolongation with potential for torsades de pointes, thrombocytopenia, diarrhea, esophagitis, allergic reactions and aggravation of sinus node dysfunction have to be kept in mind. Moreover it is not freely available in all countries [2].

Quinine instead of quinidine

Since quinidine is not freely available, people have used quinine which is used as an antimalarial drug [3]. It was successful in controlling electrical storm in a ten year old child with Brugada syndrome. Intravenous quinine, a diastereomer of quinidine was used in this case as quinidine was not available.

References

1. Francis J, Antzelevitch C. Atrial fibrillation and Brugada syndrome. J Am Coll Cardiol. 2008 Mar 25;51(12):1149-53.
2. Brugada J, Campuzano O, Arbelo E, Sarquella-Brugada G, Brugada R. Present Status of Brugada Syndrome: JACC State-of-the-Art Review. J Am Coll Cardiol. 2018 Aug 28;72(9):1046-1059.
3. Gonzalez Corcia MC, de Asmundis C, Chierchia GB, Brugada P. Brugada syndrome in the paediatric population: a comprehensive approach to clinical manifestations, diagnosis, and management. Cardiol Young. 2016 Aug;26(6):1044-55.
4. Belhassen B, Glick A, Viskin S. Efficacy of quinidine in high-risk patients with Brugada syndrome. Circulation. 2004 Sep 28;110(13):1731-7.
5. Sameer Mehrotra, Rajnish Juneja, Nitish Naik, Behzad B Pavri. Successful use of quinine in the treatment of electrical storm in a child with Brugada syndrome. J Cardiovasc Electrophysiol. 2011 May;22(5):594-7.