2024-03-28T13:22:09Zhttp://repisalud.isciii.es/oai/requestoai:repisalud.isciii.es:20.500.12105/104972022-10-11T13:23:28Zcom_20.500.12105_2145com_20.500.12105_2051com_20.500.12105_2144col_20.500.12105_2146
00925njm 22002777a 4500
dc
Mazzanti, Andrea
author
Maragna, Riccardo
author
Vacanti, Gaetano
author
Kostopoulou, Anna
author
Marino, Maira
author
Monteforte, Nicola
author
Bloise, Raffaella
author
Underwood, Katherine
author
Tibollo, Valentina
author
Pagan, Eleonora
author
Napolitano, Carlo
author
Bellazzi, Riccardo
author
Bagnardi, Vincenzo
author
Priori, Silvia G.
author
2017-12-19
Short QT syndrome (SQTS) is a rare and life-threatening arrhythmogenic syndrome characterized by abbreviated repolarization. Hydroquinidine (HQ) prolongs the QT interval in SQTS patients, although whether it reduces cardiac events is currently unknown.
This study investigated whether long-term treatment with HQ reduces the occurrence of life-threatening arrhythmic events (LAE) (cardiac arrest or sudden cardiac death) in SQTS patients.
In this cohort study on consecutive SQTS patients, 2 analyses were performed: 1) a matched-period analysis for the occurrence of LAE in 17 SQTS patients who received long-term HQ; and 2) a comparison of the annual incidence of LAE off- and on-HQ in 16 SQTS patients who survived a cardiac arrest.
A total of 17 patients (82% male, age 29 ± 3 years, QTc before treatment 331 ± 3 ms) received HQ therapy (584 ± 53 mg/day). Therapy was stopped in 2 cases (12%) due to gastrointestinal intolerance, and 15 patients continued treatment for 6 ± 1 year. QTc prolongation was observed in all patients (by 60 ± 6 ms; p < 0.001). We compared the occurrence of LAE during 6 ± 1 years before and after HQ, observing that patients on HQ experienced a reduction in both the rate of LAE from 40% to 0% (p = 0.03) and the number of LAE per patient from 0.73 ± 0.3 to 0 (p = 0.026). Furthermore, the annual rate of LAE in the 16 patients with a previous cardiac arrest dropped from 12% before HQ to 0 on therapy (p = 0.028).
We demonstrated for the first time that treatment with HQ was associated with a lower incidence of LAE in SQTS patients. These data point to the importance that quinidine, that in several countries has been removed from the market, remains available worldwide for patients with SQTS. In the present study, therapy with HQ has been proven to be safe, with a relatively low rate of side effects.
J Am Coll Cardiol. 2017; 70(24):3010-3015
http://hdl.handle.net/20.500.12105/10497
29241489
10.1016/j.jacc.2017.10.025
1558-3597
Journal of the American College of Cardiology
Hydroquinidine Prevents Life-Threatening Arrhythmic Events in Patients With Short QT Syndrome.