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  • 1
    Digitale Medien
    Digitale Medien
    How to Find the High-Risk Patient Among Individuals with a Brugada Syndrome-Type Electrocardiogram (2005)
    350 Main Street , Malden , MA 02148-5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 16 (2005), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2005.04723.x
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  • 2
    Digitale Medien
    Digitale Medien
    Is Restoration of Intracellular Trafficking Clinically Feasible in the Long QT Syndrome?: (2003)
    350 Main Street , Malden , MA 02148 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 14 (2003), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2003.02363.x
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  • 3
    Digitale Medien
    Digitale Medien
    Heart Rate Variability by Triangular Index in Infants Exposed Prenatally to Cocaine (2002)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of noninvasive electrocardiology 7 (2002), S. 0 
    Details
    ISSN: 1542-474X
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Background: In adults, heart rate variability triangular index (HRVi) is a highly reproducible measure of heart rate variability (HRV), which makes it more suitable for use in longitudinal studies. Although normative data have been published for newborns, studies in infants with pathological conditions are lacking.Methods: From 1997 to 2000, within the first 4 days of life, we prospectively evaluated HRVi in cocaine-exposed asymptomatic newborns (N = 97) by Holter monitoring. Their data were compared with infants from two control groups (one with no in utero drug exposure, N = 102; the other with exposure to alcohol, nicotine, or marijuana but no cocaine, N = 111).Results: In assessing concordance between and within operators for HRVi, the intraclass correlations were 0.983 (95% Cl: 0.958, 0.994) and 0.997 (95% Cl: 0.984, 0.999), respectively. Infants with in utero cocaine exposure had significantly (P 〈 0.0001) lower HRVi than those exposed to other drugs and to no drugs in utero. If abnormal HRVi is defined as 〈 fifth percentile for the no drug exposed group (HRVi 〈 8), 10% of the cocaine-exposed newborns, in contrast to 2% in each of the control groups (P = 0.003) had abnormal values.Conclusion: HRVi is a reliable measure to study heart rate variability in newborns. Asymptomatic infants with in utero cocaine exposure have lower HRVi. Our study supports the clinical use of an abnormal HRVi as a value 〈 8 for newborn infants. A.N.E. 2002;7(4):374–378
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1542-474X.2002.tb00188.x
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  • 4
    Digitale Medien
    Digitale Medien
    Variable Expression of Long QT Syndrome Among Gene Carriers from Families with Five Different HERG Mutations (2001)
    Oxford, UK : Blackwell Publishing Ltd
    Annals of noninvasive electrocardiology 7 (2001), S. 0 
    Details
    ISSN: 1542-474X
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Objectives: This study assessed the phenotypic variability of LQTS in carriers with the same and with different mutations in the LQT2 gene.Background: Mutations of ion-channel genes are known to cause the long QT syndrome (LQTS), a disorder associated with distinctive genotypic-specific electrocardiographic patterns and variable clinical expression.Methods: Clinical and electrocardiographic characteristics were assessed in five large LQTS families, each with a different mutation of the HERG gene (LQT2; n = 469, 69% genotyped, 102 carriers). One mutation was located on the N-terminus and the other four on the C-terminus of the HERG channel protein.Results: The QTc duration and the frequency of cardiac events (syncope and LQTS-related cardiac arrest/deatht were similar among carriers with the five HERG mutations. QTc was as variable in carriers of the same mutation as it was among carriers with different HERG mutations (P = 0.19). Qualitative assessment of the electrocardiograms revealed extensive intra-and interfamilial variability in T-vvave morphology. Among carriers with multiple electrocardiograms extending over 2 to 7 years, variation in QTc over time was minimal. A strong association was found between QTc and the occurrence of cardiac events in carriers of all five mutations.Conclusions: The clinical expression of LQTS was equally variable in carriers from families with the same or different HERG mutations. These findings highlight the complexity of the clinical phenotype in this Mendelian dominant disorder and suggest that one or more modifier genes contribute to the variable expression of this syndrome. A.N.E. 2002;7(1):40–46
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1542-474X.2001.tb00137.x
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  • 5
    Digitale Medien
    Digitale Medien
    Repolarization Dynamics in Patients with Long QT Syndrome (2002)
    Oxford, UK : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 13 (2002), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Repolarization Dynamics in LQTS. Introduction: Dynamics of ventricular repolarization may contribute to cardiac arrhythmias in subjects with the long QT syndrome (LQTS). The aim of the present study was to assess the dynamics of repolarization duration and the dynamics of repolarization complexity in LQTS patients and their unaffected family members. Methods and Results: Twelve-lead 24-hour ambulatory ECG recordings were obtained from LQTS patients (n = 38) and unaffected family members (n = 20). The 24-hour dynamics of the QT interval, T wave complexity (TWC) index measured by principal component analysis, and the RR interval were analyzed using standard deviation (SD) and square root of the mean squared differences of successive values of the parameters (RMSSD). QT variability, mean TWC, and TWC variability were increased in the LQTS patients compared with unaffected family members (QT-SD: 38 ± 20 msec vs 19 ± 7 msec, P = 0.0001; QT-RMSSD: 36 ± 20 msec vs 14 ± 8 msec, P = 0.0001; TWC: 27.7% ± 11.1% vs 20.4% ± 6.7%, P = 0.003; TWC-SD: 6.7% ± 2.8% vs 4.6% ± 1.8%, P = 0.003; TWC-RMSSD: 5.3% ± 2.8% vs 3.7% ± 1.2%, P = 0.004, respectively). At the same time, the measures of heart rate variability were similar between the affected and unaffected LQTS subjects (SD of normal-to-normal RR intervals [SDNN]: 94 ± 25 msec vs 89 ± 37 ms, P = 0.56; RMSSD: 49 ± 26 msec vs 49 ± 34 ms, P = 0.97, respectively). Conclusion: Despite similar heart rate variability, QT variability and the variability of TWC are significantly increased in LQTS patients compared with unaffected family members, suggesting that disturbances in temporal dynamics of repolarization and repolarization complexity in LQTS patients possibly increase vulnerability to arrhythmias.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2002.00651.x
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  • 6
    Digitale Medien
    Digitale Medien
    Evaluation of Suspected Arrhythmic Syncope: Leaning Away from Tilting (2005)
    350 Main Street , Malden , MA 02148-5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 16 (2005), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1111/j.1540-8167.2005.50243.x
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  • 7
    Digitale Medien
    Digitale Medien
    Saving Lives in Congenital Long QT Syndrome: Who Benefits from Implantable Cardioverter Defibrillator Therapy? (2003)
    350 Main Street , Malden , MA 02148 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 14 (2003), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2003.03069.x
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  • 8
    Digitale Medien
    Digitale Medien
    Location of Mutation in the KCNQ1 and Phenotypic Presentation of Long QT Syndrome (2003)
    350 Main Street , Malden , MA 02148-5018 , USA , and 9600 Garsington Road , Oxford OX4 2DQ , UK . : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 14 (2003), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Introduction: Recent data showed that long QT syndrome (LQTS) patients with mutations in the pore region of the HERG (LQT2) gene have significantly higher risk of cardiac events than subjects with mutations in the non-pore region. The aim of this study was to determine whether there is an association between the location of mutations in the KCNQ1 gene and cardiac events in LQT1 patients. Methods and Results: The study population consisted of 294 LQT1 patients with KCNQ1 gene mutations. Demographic, clinical, and follow-up information was compared among subjects with different locations of KCNQ1 mutations defined as pre-pore region including N-terminus (1–278), pore region (279–354), and post-pore region including C-terminus (〉354). Cardiac events observed during follow-up from birth until age of last contact or age 40 years were defined as syncope, cardiac arrest, or sudden death. There were 164 (56%) LQT1 patients with pre-pore mutations, 101 (34%) with pore mutations, and 29 (10%) with post-pore mutations. QTc duration did not differ significantly among the three subgroups (mean QTc = 494, 487, and 501 ms, respectively). There was no significant difference between groups with regard to the risk of cardiac events by age 40 years. Conclusion: There are no significant differences in clinical presentation, ECG parameters, and cardiac events among LQT1 patients with different locations of KCNQ1 mutations. These findings indicate that factors other than location of mutation influence clinical phenotype in patients with LQT1 mutations. (J Cardiovasc Electrophysiol, Vol. 14, pp. 1149-1153, November 2003)
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2003.03177.x
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  • 9
    Digitale Medien
    Digitale Medien
    Electrocardiographic Prediction of Abnormal Genotype in Congenital Long QT Syndrome: Experience in 101 Related Family Members (2001)
    Oxford, UK : Blackwell Science Inc
    Journal of cardiovascular electrophysiology 12 (2001), S. 0 
    Details
    ISSN: 1540-8167
    Quelle: Blackwell Publishing Journal Backfiles 1879-2005
    Thema: Medizin
    Notizen: Prediction of Congenital Long QT Syndrome. Introduction: Previous studies showed that diagnosing congenital long QT syndrome (LQTS) is difficult due to variable penetrance and genetic heterogeneity, especially when subjects from multiple families with diverse mutations are combined. We hypothesized that a combination of clinical and ECG techniques could identify gene carriers within a single family with congenital LQTS. Methods and Results: One hundred one genotyped members of a family with LQTS, including 26 carriers of a HERG mutation, underwent history and ECG analysis. Forty-eight family members also underwent exercise testing with QT and T wave alternans (TWA) analysis and 24-hour Holter monitoring with QT and heart rate variability analysis. A logistic regression model, which included age, gender, QTc, and QTc by age, provided the best prediction of gene carrier status, although there was substantial overlap (78%) of QTc among subjects with and without the mutation. QTc was not helpful as a discriminator in children ≤ 13 years. TWA (observed infrequently) did not add significantly to the model's ability to predict abnormal genotype. Conclusion: Even in this homogeneous LQTS population, the phenotype was so variable that clinical and detailed ECG analyses did not permit an accurate diagnosis of gene carrier status, especially in children. Sustained microvolt TWA was a specific (100%) but insensitive (18%) marker for LQTS. Its ability to predict risk of arrhythmia in this population remains to be determined. Genetic testing serves an essential role in screening for carriers of LQTS.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1046/j.1540-8167.2001.00455.x
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  • 10
    Digitale Medien
    Digitale Medien
    Efficiency of energy delivery systems: II. Estimating energy costs of capital equipment (1979)
    Springer
    Environmental management 3 (1979), S. 505-510 
    Details
    ISSN: 1432-1009
    Quelle: Springer Online Journal Archives 1860-2000
    Thema: Energietechnik
    Notizen: Abstract Procedures are developed and summarized that allow the calculation of the energy required to manufacture and install capital equipment, such as the material needed for a coalfired power plant. Three methods are available, each with certain strengths and weaknessess The first uses both literature value and national manufacturing statistics, the second uses a previously developed technique that disaggregates energy use according to how much each industry buys from all other industries, and the third technique is based on the average national relation between energy use and dollar flow. The most important differences between estimates of energy use by the different techniques relates to the inclusion or exclusion of labor and indirect expenditures rather than to differences in the data bases of the three techniques.
    Materialart: Digitale Medien
    URL: http://dx.doi.org/10.1007/BF01866319
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