Administration of specific drugs may occasionally induce acquired long QT syndrome (aLQTS), a disorder that predisposes to ventricular arrhythmias, typically of the torsade de pointes (TdP) type, and sudden cardiac death. "Forme fruste" mutations in congenital LQTS (cLQTS) genes have been reported repeatedly as the underlying cause of aLQTS, and are therefore considered as an important risk factor. We evaluated the impact of genetic susceptibility for aLQTS through mutations in cLQTS genes. Five cLQTS genes ( KCNH2, KCNQ1, SCN5A, KCNE1, KCNE2) were thoroughly screened for genetic variations in 32 drug-induced aLQTS patients with confirmed TdP and 32 healthy individuals. Missense forme frust mutations were identified in four aLQTS patients: D85N in KCNE1 (two cases), T8A in KCNE2, and P347S in KCNH2. Three other missense variations were found both in patients and controls, and are thus unlikely to significantly influence aLQTS susceptibility. In addition, 13 silent and six intronic variations were detected, four of which were found in a single aLQTS patient but not in the controls. We conclude that missense mutations in the examined cLQTS genes explain only a minority of aLQTS cases.
The recurrence rate of paroxysmal atrial fibrillation after successful radiofrequency ablation of accessory pathways shows an age-related increase, being low in patients younger than 50 years of age (12%) and high in the older patients: 35% in patients older than 50 years and 55% in patients older than 60. These results have significant therapeutic implications concerning the decisions on pharmacological therapy after successful ablation in patients older than 50 years. Furthermore, these data will help physicians advise older patients properly about their risk of recurrence of atrial fibrillation after ablation.
Background: Patients resuscitated from ventricular tachyarrhythmias benefit from implantable cardioverter-defibrillators (ICDs) as opposed to medical treatment. Patients with increased QRS duration receiving an ICD in the presence of heart failure are at greatest risk of cardiac death and benefit most from ICD therapy. Objective: To determine whether an increased QRS duration predicts cardiac mortality in ICD recipients. Design: Consecutive patients with heart failure in New York Heart Association functional class III were grouped according to QRS duration (< 150 ms, n = 139, group 1; v > 150 ms, n = 26, group 2) and followed up for (mean (SD)) 23 (20) months. Patients: 165 patients were studied (80% men, 20% women); 73% had coronary artery disease and 18% had dilated cardiomyopathy. Their mean age was 62 (10) years and mean ejection fraction (EF) was 33 (14)%. They presented either with ventricular tachycardia (VT) or ventricular fibrillation (VF). Main outcome measures: Overall and cardiac mortality; recurrence rates of VT, fast VT, or VF. Results: Mean left ventricular EF did not differ between group 1 (33 (13)%) and group 2 (31 (15)%). Forty patients died (34 cardiac deaths). There was no difference in survival between patients with EF > 35% and < 35%. Cardiac mortality was significantly higher in group 2 than in group 1 (31.3% at 12 months and 46.6% at 24 months, v 9.5% at 12 months and 18.2% at 24 months, respectively; p = 0.04). The recurrence rate of VT was similar in both groups. Conclusions: Within subgroups at highest risk of cardiac death, QRS duration-a simple non-invasive index-predicts outcome in ICD recipients in the presence of heart failure.
The relation between lipoprotein(a) [Lp(a)] as an independent risk factor for coronary atherosclerosis and the severity and extension of angiographically detectable coronary atherosclerotic lesions has not been systematically evaluated. In 118 male patients (54.3±7.4 years) with suspected coronary artery disease and without a history of myocardial infarction undergoing coronary angiography, the relation between plasma Lp(a) levels and other lipoproteins and the severity and extension of coronary lesions was studied. The coronary angiograms were evaluated in a blinded manner according to three scores: vessel score (0 to 3 points for 0 to 3 vessels with stenoses a 70%), stenosis score (0 to 32 points; number and severity of coronary stenoses or lesions), and extent score (0 to 100 points; length-extension of all coronary lesions in relation to the total coronary vessel length). The score values obtained
The prevalence of coronary artery disease with at least one critical stenosis in subjects aged 40-70 years with an average cholesterol level of 238+/-42 mg. dl(-1)is 7.3%.
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