Summary: Purpose: Because several reports have described the relation between epilepsy and cardiac arrhythmias and suggest that changes in autonomic neural control of the heart could be involved in the pathogenesis of sudden unexplained death in patients with epilepsy, the aim of this study was to evaluate cardiac function in patients with temporal lobe epilepsy.Methods: Sixty-five patients with epilepsy were evaluated by simultaneous ambulatory 24-h EEG-ECG monitoring, and in 30 of these, power spectral analysis of relative-risk (RR) variability also was carried out, both in the supine position and in a passive tilt position at 60". The power spectrum of RR variability, the two major spectral components detectable at low frequency (LF) and at high frequency (HF), respectively, and the LF/HF ratio were calculated.Results: By EEG-ECG monitoring, we recorded six partial seizures, and in four cases, discharges were associated with sinus tachycardia. However, interictally the occurrence of ventricular and supraventricular arrhythmias was not different from that in normal subjects. The spectral analysis of RR variability, on the other hand, demonstrated in patients with epilepsy a significant decrease in the total RR variability and in both of its components (LF and HF) in the supine position, and of the LF/HF ratio in orthostatic position.Conclusions: These findings suggest that the spectral analysis of RR variability may detect disorders of autonomic cardiac control in patients with epilepsy, even in the absence of abnormal findings during ECG monitoring. This alteration, which is more severe in cases with right EEG focus, could play a role in the pathogenesis of cardiac arrhythmias. Key Words: Temporal lobe epilepsy-Sudden unexplained death in patients with epilepsy-ECG monitoring-Heart rate variabilityAutonomic disorders.Sudden unexplained death in patients with epilepsy (SUDEP) is a well-recognized phenomenon (l), although its pathogenesis is not yet completely understood. In a recent cohort study (2), the maximal overall incidence of SUDEP observed was 1.35 cases/l,OOO person-years, which was similar to values described in previous studies (3,4). In particular, the incidence of SUDEP is increased in males and is correlated with the number of antiepileptic drugs (AEDs) prescribed and with treatment. with psychotropic drugs. It is also associated with alcohol abuse and slightly with mental retardation.With regard to the pathogenesis of SUDEP, the hypothesis of an acute disturbance of the cardiac rhythm as a possible mechanism is supported by various clinical case reports of patients with temporal lobe epilepsy (5,6) Accepted September 13, 1996. Address correspondence and reprint requests to Dr. L. Mum at Department of Neurosciences, Institute of Neurology, Via Roma, 67, 56126 Pisa, Italy and by the finding that both simple and complex partial seizures (CPSs) are consistently associated with tachycardia (7,s) and rarely with bradycardia (9,lO). On the other hand, certain data obtained interictally provide only par...

Background-Effective myocardial reperfusion after primary percutaneous coronary intervention (PCI) may be limited by distal embolization. We tested the safety, feasibility, and efficacy of the FilterWire-Ex (FW), a distal embolic protection device, as an adjunct to primary PCI.

This paper reviews the existing epidemiological and clinical evidence about the relationships of non-diabetic microalbuminuria with cardiovascular risk factors such as elevated blood pressure (BP), systolic particularly, cardiac hypertrophy, adverse metabolic status, smoking habits, elevated angiotensin II levels, endothelial dysfunction, acute and perhaps subclinical inflammation. Because of that unique property of reflecting the influence of so many clinically relevant parameters, microalbuminuria may legitimately be defined as an integrated marker of cardiovascular risk, an unique profile among the several prognostic predictors available to

BackgroundThe purpose of our study was to investigate the potential contribution of germline mutations in NOTCH1, GATA5 and TGFBR1 and TGFBR2 genes in a cohort of Italian patients with familial Bicuspid Aortic Valve (BAV).MethodsAll the coding exons including adjacent intronic as well as 5′ and 3′ untranslated (UTR) sequences of NOTCH1, GATA5, TGFBR1 and TGFBR2 genes were screened by direct gene sequencing in 11 index patients (8 males; age = 42 ± 19 years) with familial BAV defined as two or more affected members.ResultsTwo novel mutations, a missense and a nonsense mutation (Exon 5, p.P284L; Exon 26, p.Y1619X), were found in the NOTCH1 gene in two unrelated families. The mutations segregated with the disease in these families, and they were not found on 200 unrelated chromosomes from ethnically matched controls. No pathogenetic mutation was identified in GATA5, TGFBR1 and TGFBR2 genes.ConclusionsTwo novel NOTCH1 mutations were identified in two Italian families with BAV, highlighting the role of a NOTCH1 signaling pathway in BAV and its aortic complications. These findings are of relevance for genetic counseling and clinical care of families presenting with BAV. Future studies are needed in order to unravel the still largely unknown genetics of BAV.

Abstract-To identify the biological covariates of microalbuminuria (albuminuria Ն15 g/min) in nondiabetic subjects, brachial blood pressure, echocardiographic left ventricular mass, and other cardiovascular and metabolic parameters were evaluated in 211 untreated males (38 normal controls, 109 uncomplicated stage 1 to 3 essential hypertensives, and 64 patients with clinically stable atherosclerotic peripheral vascular disease either with [nϭ44] or without [nϭ20] essential hypertension) with normal cardiac and renal function. Compared with normoalbuminuric subjects, microalbuminuric subjects (nϭ67) were characterized by higher systolic blood pressure, comparable diastolic blood pressure, and, therefore, wider pulse pressure. Greater prevalence of hypertension, peripheral vascular disease, left ventricular hypertrophy, and reduced HDL cholesterol values further distinguished microalbuminuric from normoalbuminuric subjects in univariate comparisons. The risk of microalbuminuria increased by ascending pulse pressure quintiles in age-corrected logistic regression models, in which pulse pressure was more predictive than systolic pressure and was independent of mean pressure. When microalbuminuric status was regressed against a series of dichotomous (vascular and active smoker status) and continuous (age, pulse and mean pressure, left ventricular mass index, and HDL and LDL cholesterol) variables, only pulse pressure, left ventricular mass index, and smoking status were independent predictors. The association of increased albuminuria with wider pulse pressure, a correlate of the pulsatile hemodynamic load and conduit vessel stiffness as well as an important cardiovascular risk factor, may explain why microalbuminuria predicts cardiovascular events in nondiabetic subjects. The independence from concomitant vascular disease also suggests that wider pulse pressure, rather than representing a simple marker for atherosclerotic disease, influences albuminuria directly. To specify to a better extent the biological covariates of microalbuminuria in nondiabetic subjects, we have evaluated cross-sectionally a series of cardiovascular and metabolic parameters in a large group of normal control subjects, subjects with essential hypertension (EH), and patients with atherosclerotic vascular disease. Methods SubjectsThe study was carried out on 211 eligible sedentary subjects referred to our unit from 1996 to 1998 for screening and treatment of hypertension and related cardiovascular risk factors. Inclusion criteria required male gender, negative urine dipstick test, normal urinary sediment and renal ultrasound, plasma glucose Ͻ7.8 mmol/L (120 mg/dL), serum creatinine Ͻ110 mol/L (1.4 mg/dL), total serum cholesterol Ͻ7.8 mmol/L (300 mg/dL), body mass index (BMI) Ͻ27 kg/m 2 , and preserved systolic cardiac function (ejection fraction Ͼ50%) (see Table 1 for the overall demographic and clinical characteristics). One hundred nine patients had stage 1 to 3 uncomplicated EH, 20 had atherosclerotic peripheral vascular disease (PVD), and 44 w...