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4 results on '"Hofman N"'

1. Age and gender related differences in corrected QT interval in children and adolescents with LQT1 and LQT2

Author

Vink, A, Clur, S, Geskus, R, Blank, A, De Kezel, C, Loontjens, R, Bruijn, M, Hofman, N, Wilde, A, and Blom, N

Abstract

Children and adolescents with congenital Long QT Syndrome (LQTS) are an important risk group with age, gender and genotype related differences in cardiac events pre and post onset of puberty. The extent of the prolongation of the QT interval corrected for the heart rate (QTc) is associated with an increased risk for cardiac events. The magnitude of age, gender and genotype influences on QTc interval are limited to the comparison of baseline measurements between categorical age groups, rather than age related trends in QTc intervals.

Published
2018

4. Clinical Aspects of Type 3 Long-QT Syndrome

Author

Derick R. Peterson, Marielle Alders, Connie R. Bezzina, Wataru Shimizu, Takeshi Aiba, Michael J. Ackerman, Bronislava Polonsky, Coeli M. Lopes, Yoshihiro Miyamoto, Arthur J. Moss, David J. Tester, Jørgen K. Kanters, Carla Spazzolini, Jennifer L. Robinson, Ming Qi, Jeffrey A. Towbin, Arthur A.M. Wilde, Wojciech Zareba, Ilan Goldenberg, Elizabeth S. Kaufman, Jesaia Benhorin, Nynke Hofman, Mark L. Andrews, Lia Crotti, Peter J. Schwartz, Scott McNitt, Shiro Kamakura, Cardiology, Human Genetics, Wilde, A, Moss, A, Kaufman, E, Shimizu, W, Peterson, D, Benhorin, J, Lopes, C, Towbin, J, Spazzolini, C, Crotti, L, Zareba, W, Goldenberg, I, Kanters, J, Robinson, J, Qi, M, Hofman, N, Tester, D, Bezzina, C, Alders, M, Aiba, T, Kamakura, S, Miyamoto, Y, Andrews, M, Mcnitt, S, Polonsky, B, Schwartz, P, and Ackerman, M

Subjects
Male, Pediatrics, cardiac event, risk stratification, 030204 cardiovascular system & hematology, Sodium Channels, Electrocardiography, 0302 clinical medicine, Cardiac Conduction System Disease, Registries, Young adult, Child, Sex Characteristics, medicine.diagnostic_test, clinical manifestation, Long QT Syndrome, Child, Preschool, Risk stratification, Female, long qt syndrome type 3, Cardiology and Cardiovascular Medicine, Risk assessment, Adult, medicine.medical_specialty, Adolescent, medicine.drug_class, Long QT syndrome, Adrenergic beta-Antagonists, Risk Assessment, Syncope, Article, Young Adult, 03 medical and health sciences, Physiology (medical), medicine, Humans, In patient, Intensive care medicine, Beta blocker, Genetic testing, therapy, business.industry, Infant, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, medicine.disease, Heart Arrest, Scn5a, Genetic Testing, Long Qt Syndrome, Risk Stratification, Sudden Cardiac Death, Arrhythmia, beta blocker, Multicenter study, business, 030217 neurology & neurosurgery
Abstract

Background: Risk stratification in patients with type 3 long-QT syndrome (LQT3) by clinical and genetic characteristics and effectiveness of β-blocker therapy has not been studied previously in a large LQT3 population. Methods: The study population included 406 LQT3 patients with 51 sodium channel mutations; 391 patients were known to be event free during the first year of life and were the focus of our study. Clinical, electrocardiographic, and genetic parameters were acquired for patients from 7 participating LQT3 registries. Cox regression analysis was used to evaluate the independent contribution of clinical, genetic, and therapeutic factors to the first occurrence of time-dependent cardiac events (CEs) from age 1 to 41 years. Results: Of the 391 patients, 118 (41 males, 77 females) patients (30%) experienced at least 1 CE (syncope, aborted cardiac arrest, or long-QT syndrome–related sudden death), and 24 (20%) suffered from LQT3-related aborted cardiac arrest/sudden death. The risk of a first CE was directly related to the degree of QTc prolongation. Cox regression analysis revealed that time-dependent β-blocker therapy was associated with an 83% reduction in CEs in females ( P =0.015) but not in males (who had many fewer events), with a significant sex × β-blocker interaction ( P =0.04). Each 10-ms increase in QTc duration up to 500 ms was associated with a 19% increase in CEs. Prior syncope doubled the risk for life-threatening events ( P Conclusions: Prolonged QTc and syncope predispose patients with LQT3 to life-threatening CEs. However, β-blocker therapy reduces this risk in females; efficacy in males could not be determined conclusively because of the low number of events.

Published
2016

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