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Background: 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.Objectives: 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.Methods: 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.Results: 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).Conclusions: 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. [ABSTRACT FROM AUTHOR]

Background: Risk stratification in asymptomatic patients remains by far the most important yet unresolved clinical problem in the Brugada syndrome (BrS).Objectives: This study sought to analyze the usefulness of electrocardiographic parameters as markers of sudden cardiac death (SCD) in BrS.Methods: This study analyzed data from 347 consecutive patients (78.4% male; mean age 45 ± 13.1 years) with spontaneous type 1 BrS by ECG parameters but with no history of cardiac arrest (including 91.1% asymptomatic at presentation, 5.2% with a history of atrial fibrillation [AF], and 4% with a history of arrhythmic syncope). Electrocardiographic characteristics at the first clinic visit were analyzed to predict ventricular fibrillation (VF)/SCD during follow-up.Results: During the follow-up (48 ± 38 months), 276 (79.5%) patients remained asymptomatic, 39 (11.2%) developed syncope, and 32 (9.2%) developed VF/SCD. Patients who developed VF/SCD had a lower prevalence of SCN5A gene mutations (p = 0.009) and a higher prevalence of positive electrophysiological study results (p < 0.0001), a family history of SCD (p = 0.03), and AF (p < 0.0001). The most powerful marker for VF/SCD was a significant S-wave (≥0.1 mV and/or ≥40 ms) in lead I. In the multivariate analysis, the duration of S-wave in lead I ≥40 ms (hazard ratio: 39.1) and AF (hazard ratio: 3.7) were independent predictors of VF/SCD during follow-up. Electroanatomic mapping in 12 patients showed an endocardial activation time significantly longer in patients with an S-wave in lead I, mostly because of a significant delay in the anterolateral right ventricular outflow tract.Conclusions: The presence of a wide and/or large S-wave in lead I was a powerful predictor of life-threatening ventricular arrhythmias in patients with BrS and no history of cardiac arrest at presentation. However, the prognostic value of a significant S-wave in lead I should be confirmed by larger studies and by an independent confirmation cohort of healthy subjects. [ABSTRACT FROM AUTHOR]

Introduction: Serum uric acid (SUA) may contribute to the increased cardiovascular damage through direct injury to the endothelium and alteration of cardiovascular function. Aim: To evaluate the association of SUA with the presence of the most recurrent electrographic alterations and with the length of the main ECG intervals in a large sample of general population. Methods: For this study, on the database of the Brisighella Heart Study, we evaluated the available data of 790 men and 849 women, excluding subjects affected by gout or taking antihyperuricemic agents, those taking drug increasing the QT interval and those using beta-blockers or non-dihydropyridine calcium channel blockers at the moment of the ECG registration. Multiple ascending stepwise regression analyses were carried out to determine the independent predictors of the predefined ECG alterations. Results: The prevalence of predefined ECG alterations was comparable between genders, with the exception of sinus bradicardia, left-anterior fascicular block, atrio-ventricular blocks and left ventricular hypertrophy (LVH), which appeared to be more frequent in men. The multivariate analysis revealed that SUA was associated to ischaemic alterations, LVH, sinus tachycardia and tachyarrhytmias. Age was associated to all evaluated ECG alterations beyond sinus tachycardia and LVH. Male sex was associated to sinus bradicardia, atrio-ventricular blocks, anterior-left fascicular block and LVH. Blood pressure was associated to different ECG alterations, but with clinically relevant OR with ischaemic alterations and LVH. Conclusion: SUA level is related the prevalence of both organic and rhythm ECG alterations in a wide sample of general population. [ABSTRACT FROM AUTHOR]