Long QT Syndrome and the Risk of Sudden Death in Adult Patients: An In-depth Analysis of Clinical Correlates and Prognostic Factors
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Abstract
Background
Long QT Syndrome (LQTS) is a hereditary or acquired cardiac disorder characterized by delayed ventricular repolarization, predisposing affected individuals to life-threatening arrhythmias. While extensively studied in the pediatric population, there is a paucity of comprehensive research focusing on the clinical implications and prognostic markers of LQTS in adults. This article aims to elucidate the association between Long QT Syndrome and the heightened risk of sudden death in adult patients, exploring pertinent clinical correlates and potential prognostic factors.
A systematic literature review was conducted to identify relevant studies published between 2000 and 2024, utilizing databases such as PubMed, MEDLINE, and Cochrane Library. Eligible articles were critically appraised for methodological quality and relevance to adult populations with Long QT Syndrome. Data extraction encompassed demographics, genetic predispositions, clinical presentations, electrocardiographic findings, and outcomes, focusing on the incidence of sudden death.
Preliminary findings underscore the significance of a prolonged QT interval as a critical marker for adverse cardiovascular events in adults with Long QT Syndrome. Additionally, the study examines the impact of genotype-phenotype correlations, gender-related differences, and the influence of comorbidities on the risk of sudden death. Furthermore, potential therapeutic interventions and risk stratification strategies will be discussed in light of recent advancements in the field.
This review aims to bridge the existing knowledge gap regarding Long QT Syndrome in the adult population, emphasizing the need for heightened clinical awareness, risk stratification, and targeted interventions to mitigate the risk of sudden death in affected individuals. The synthesis of current evidence will contribute to a more nuanced understanding of the complex interplay between Long QT Syndrome and adverse outcomes in adults, facilitating the development of tailored therapeutic approaches and improved patient outcomes
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References
I. Goldenberg I, Zareba W, Moss AJ. Long QT syndrome. Curr Probl Cardiol, 2008; 33:629–94.
II. Goldenberg I, Moss AJ. Long QT syndrome. J Am Coll Cardiol, 2008; 51:2291–2300.
III. Moss AJ, W. Zareba W and Benhorin J et al. ECG T-wave patterns in genetically distinct forms of the hereditary long QT syndrome. Circulation, 1995; 92:2929–2934.
IV. Schwartz PJ, Moss AJ, Vincent GM and Crampton RS. Diagnostic criteria for the long QT syndrome: an update. Circulation, 1993; 88:782–784.
V. Goldenberg, Moss AJ and Zareba W. QT interval: how to measure it and what is “normal.” J Cardiovasc Electrophysiol, 2006; 17:333–336.
VI. Moss AJ, Kass RS. Long QT syndrome: from channels to cardiac arrhythmias. J Clin Invest, 2005; 115:2018–2024.
VII. Moss AJ, Shimizu W, Wilde AA et al. Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene. Circulation, 2007; 115:2481–2489.
VIII. Goldenberg I, Moss AJ, Zareba W, McNitt S, Robinson JL, Qi M, Towbin JA, Ackerman MJ, Murphy L. Clinical course and risk stratification of patients affected with the Jervell and LangeNielsen syndrome. J Cardiovasc, Electrophysiol, 2006; Nov:17(11):1161–8.
IX. Vincent GM. The molecular genetics of the long QT syndrome: genes causing fainting and sudden death. Ann Rev Med, 1998; 49:263–274.
X. Lehnart SE, Ackerman MG, Benson DW Jr. et al. Inherited arrhythmias: a National Heart, Lung, and Blood Institute and Office of Rare Diseases workshop consensus report about the diagnosis, phenotyping, molecular mechanisms, and therapeutic approaches for primary cardiomyopathies of gene mutations affecting ion channel function. Circulation, 2007; 116(20):2325–45.
XI. Napolitano C, Priori SG, Schwartz PJ et al. Genetic Testing in the Long QT Syndrome: Development and Validation of an Efficient Approach to Genotyping in Clinical Practice. JAMA, 2005; 294(23):2975-2980.