SQTS carries a high risk of sudden death in all age groups. Symptomatic patients have a high risk of recurrent arrhythmic events. HQ is effective in preventing ventricular tachyarrhythmia induction and arrhythmic events during long-term follow-up.

SQTS carries a high risk of sudden death and may be a cause of death in early infancy. ICD is the first choice therapy; hydroquinidine may be proposed in children and in the patients who refuse the implant.

The short QT syndrome, a recently discovered ion channel disorder, combines shortened repolarization, a predisposition to atrial and ventricular fibrillatory arrhythmias, and a risk of sudden death. Few cases have been reported, but the prevalence may be underestimated. This syndrome might account for some cases of unexplained ventricular fibrillation in patients with otherwise healthy hearts. Patients have abnormally short QT intervals and refractory periods, and atrial/ventricular fibrillation can be triggered during investigations. Gain-of-function mutations have been detected in three genes encoding potassium channels. Treatment is based on defibrillator implantation, sometimes as a preventive measure. Quinidine may be beneficial in certain cases.

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Tissue Doppler echocardiography is accurate for predicting decompensated HF with preserved LV systolic function and may be used as a diagnostic complement to inconclusive BNP level in this setting.

Objective: To evaluate the effect of pre-procedural acute oral administration of trimetazidine (TMZ) on percutaneous coronary intervention (PCI)-induced myocardial injury. Design: Single-centre, prospective, randomised evaluation study. Setting: Patients with stable angina pectoris and single-vessel disease undergoing PCI. Patients: 582 patients were prospectively randomised. Patients who underwent more than one inflation during PCI were excluded, resulting in 266 patients randomly assigned to 2 groups. Interventions: Patients were randomly assigned to receive or not an acute loading dose of 60 mg of TMZ prior to intervention. Main outcome: The frequency and the increase in the level of cardiac troponin Ic (cTnI) after successful PCI. cTnI levels were measured before and 6, 12, 18 and 24 h after PCI. Results: 136 patients were assigned to the TMZ group and 130 to the control group. Although no statistically significant difference was observed in the frequency of cTnI increase between the two groups, post-procedural cTnI levels were significantly reduced in the TMZ group at all time points (6 h: mean (SD) 4.2 (0.8) vs 1.7 (0.2), p,0.001; 12 h: 5.5 (1.5) vs 2.3 (0.4), p,0.001; 18 h: 9 (2.3) vs 3 (0.5), p,0.001; and 24 h: 3.2 (1.2) vs 1 (0.5), p,0.001). Moreover, the total amount of cTnI released after PCI, as assessed by the area under the curve of serial measurement, was significantly reduced in the TMZ group (p,0.05). Conclusion: Pre-procedural acute oral TMZ administration significantly reduces PCI-induced myocardial infarction.A symptomatic minor post-procedural myocardial necrosis does have an important prognostic signification after percutaneous coronary intervention (PCI). The magnitude of increase in the level of cardiac troponin Ic (cTnI) directly correlates with irreversible myocardial injury assessed by cardiovascular MRI. 1 2 Trimetazidine (TMZ; 1-[2,3,4-trimethoxybenzyl] piperazine) is a cellular anti-ischaemic agent that selectively inhibits the activity of the final enzyme of the fatty acid oxidation pathway, 3-ketoacylcoenzyme A thiolase. Administration of this drug leads to a switch in preference of the energy substrate, resulting in partial inhibition of fatty acid oxidation and increased glucose oxidation. Clinical studies have shown that TMZ has cardioprotective effects in the setting of myocardial ischaemia including acute myocardial infarction.3-8 However, although Kober et al 9 have demonstrated that TMZ reduces preprocedural myocardial cell ischaemia as assessed by the duration and amplitude of ST elevation during PCI, whether its cytoprotective effects translate into a reduction of myocardial necrosis is unknown. The aim of this study was to evaluate the protective effect of an acute oral loading dose of TMZ (60 mg) on post-procedural myocardial infarction as assessed by the frequency and level of cTnI release. METHODS Patient population and study protocolA single-centre, prospective, randomised evaluation study was undertaken after approval by the local research ethics committee. The stud...

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