Hydroquinidine Prevents Life-Threatening Arrhythmic Events in Patients With Short QT Syndrome
| Autor: | Silvia G. Priori, Riccardo Bellazzi, Riccardo Maragna, Gaetano Vacanti, Andrea Mazzanti, Valentina Tibollo, Katherine Underwood, Carlo Napolitano, Maira Marino, Anna Kostopoulou, Nicola Monteforte, Eleonora Pagan, Raffaella Bloise, Vincenzo Bagnardi |
|---|---|
| Přispěvatelé: | Italian Ministry of Health, Mazzanti, A, Maragna, R, Vacanti, G, Kostopoulou, A, Marino, M, Monteforte, N, Bloise, R, Underwood, K, Tibollo, V, Pagan, E, Napolitano, C, Bellazzi, R, Bagnardi, V, Priori, S, Ministero della Salute (Italia) |
| Rok vydání: | 2017 |
| Předmět: |
0301 basic medicine
Adult Male medicine.medical_specialty Adolescent 030204 cardiovascular system & hematology arrhythmia QT interval sudden cardiac death Follow-Up Studie Sudden cardiac death 03 medical and health sciences Electrocardiography Young Adult 0302 clinical medicine Heart Conduction System Heart Rate Internal medicine Heart rate medicine Humans Survival rate short QT syndrome drug repurposing medicine.diagnostic_test business.industry Incidence Short QT syndrome Arrhythmias Cardiac medicine.disease Quinidine Survival Rate 030104 developmental biology Death Sudden Cardiac hydroquinidine Italy Anti-Arrhythmia Agent Ventricular fibrillation Ventricular Fibrillation Cardiology Female Electrical conduction system of the heart Cardiology and Cardiovascular Medicine business Anti-Arrhythmia Agents Human Follow-Up Studies |
| Zdroj: | Repisalud Instituto de Salud Carlos III (ISCIII) |
| Popis: | 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. This work was supported by the Ricerca Corrente Funding scheme of the Italian Ministry of Health. Dr. Bellazzi is a shareholder of Biomeris; and is a stakeholder of Engenome. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Pedro Brugada, MD, served as Guest Editor for this paper. Sí |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|