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  1. Article: Congenital and acquired long QT syndromes.

    Morphet, John

    The Canadian journal of cardiology

    2003  Volume 19, Issue 8, Page(s) 949; author reply 949

    MeSH term(s) Electrocardiography ; Heart Conduction System/abnormalities ; Heart Conduction System/pathology ; Humans ; Long QT Syndrome/congenital ; Long QT Syndrome/etiology ; Long QT Syndrome/pathology ; Torsades de Pointes/congenital ; Torsades de Pointes/etiology ; Torsades de Pointes/pathology
    Language English
    Publishing date 2003-07
    Publishing country England
    Document type Comment ; Letter
    ZDB-ID 632813-1
    ISSN 1916-7075 ; 0828-282X
    ISSN (online) 1916-7075
    ISSN 0828-282X
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  2. Article: Congenital and acquired long QT syndromes.

    Walker, Bruce D / Krahn, Andrew D / Klein, George J / Skanes, Allan C / Wang, Jian / Hegele, Robert A / Yee, Raymond

    The Canadian journal of cardiology

    2003  Volume 19, Issue 1, Page(s) 76–87

    Abstract: Exploration into the underlying genetic causes of congenital long QT syndrome (LQTS) has opened ... of congenital and acquired LQTS. ... from gene and channel studies that have used the congenital syndrome as a springboard for directing research ...

    Abstract Exploration into the underlying genetic causes of congenital long QT syndrome (LQTS) has opened the door to our understanding of repolarization disorders. Expression of LQTS mutations has led to an improved understanding of the mechanisms of arrhythmogenesis, clinical diagnostic tools and channel specific therapy. Further insight into the mechanisms underlying the more common acquired LQTS is emerging from gene and channel studies that have used the congenital syndrome as a springboard for directing research to improve understanding. This review summarizes the clinical, genetic and electrophysiological understanding of congenital and acquired LQTS.
    MeSH term(s) Action Potentials/physiology ; Anti-Arrhythmia Agents/adverse effects ; Anti-Arrhythmia Agents/therapeutic use ; Cardiac Catheterization ; Electrophysiology ; Genetic Linkage ; Heart Conduction System/abnormalities ; Heart Conduction System/drug effects ; Humans ; Long QT Syndrome/congenital ; Long QT Syndrome/genetics ; Long QT Syndrome/therapy ; Sodium Channels/physiology ; Torsades de Pointes/etiology
    Chemical Substances Anti-Arrhythmia Agents ; Sodium Channels
    Language English
    Publishing date 2003-01
    Publishing country England
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 632813-1
    ISSN 1916-7075 ; 0828-282X
    ISSN (online) 1916-7075
    ISSN 0828-282X
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  3. Article: Molecular basis of congenital and acquired long QT syndromes.

    Ackerman, Michael J

    Journal of electrocardiology

    2004  Volume 37 Suppl, Page(s) 1–6

    MeSH term(s) Electrocardiography ; Genotype ; Humans ; Ion Channels/genetics ; Long QT Syndrome/congenital ; Long QT Syndrome/genetics ; Long QT Syndrome/physiopathology ; Molecular Biology ; Mutation/genetics ; Phenotype
    Chemical Substances Ion Channels
    Language English
    Publishing date 2004-09-03
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 410286-1
    ISSN 1532-8430 ; 0022-0736
    ISSN (online) 1532-8430
    ISSN 0022-0736
    DOI 10.1016/j.jelectrocard.2004.08.002
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  4. Article: Drug induced QT prolongation: lessons from congenital and acquired long QT syndromes.

    Walker, Bruce D / Krahn, Andrew D / Klein, George J / Skanes, Allan C / Yee, Raymond

    Current drug targets. Cardiovascular & haematological disorders

    2003  Volume 3, Issue 4, Page(s) 327–335

    Abstract: ... of congenital long QT syndrome have shed new light in the area of repolarization disorders and their resultant ... long QT syndrome, though the genetic basis of this has not been elucidated in the majority of cases ... cardiac arrhythmias. Drug induced or acquired QT prolongation often represents a latent form of congenital ...

    Abstract Recent developments regarding the underlying genetic and intracardiac ion channel causes of congenital long QT syndrome have shed new light in the area of repolarization disorders and their resultant cardiac arrhythmias. Drug induced or acquired QT prolongation often represents a latent form of congenital long QT syndrome, though the genetic basis of this has not been elucidated in the majority of cases. Understanding this has lead to a new concept of repolarization reserve, a measure of inherent susceptibility to repolarization-mediated arrhythmias. The majority of pharmacologic agents that cause significant QT prolongation have potassium channel blocking characteristics, predominantly affecting the rapidly activating current I(Kr). The list of agents known to affect I(Kr)continues to grow, best monitored through several websites that collate reports of drug-induced QT prolongation and arrhythmias. Discontinuation of the offending agent and supportive care are often all that is necessary when clinical arrhythmias arise.
    MeSH term(s) Anti-Arrhythmia Agents/adverse effects ; Anti-Arrhythmia Agents/therapeutic use ; Female ; Humans ; Long QT Syndrome/chemically induced ; Long QT Syndrome/congenital ; Long QT Syndrome/genetics ; Middle Aged ; Potassium Channels/genetics ; Potassium Channels/physiology ; Torsades de Pointes/chemically induced
    Chemical Substances Anti-Arrhythmia Agents ; Potassium Channels
    Language English
    Publishing date 2003-11-11
    Publishing country Netherlands
    Document type Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 2064870-4
    ISSN 1875-5895 ; 1568-0061
    ISSN (online) 1875-5895
    ISSN 1568-0061
    DOI 10.2174/1568006033481393
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  5. Article: Mechanisms and management of congenital and acquired long QT syndromes.

    Lazzara, R

    Archives des maladies du coeur et des vaisseaux

    1996  Volume 89 Spec No 1, Page(s) 51–55

    Abstract: The long QT syndromes can be divided into congenital and acquired forms. Early afterdepolarizations ... have been identified as triggering mechanisms for both congenital and acquired QT syndromes but reentry ... which enhances K+ current, and Ca2+ blockers or Mg, also a Ca2+ blocker. In the congenital long QT syndromes ...

    Abstract The long QT syndromes can be divided into congenital and acquired forms. Early afterdepolarizations have been identified as triggering mechanisms for both congenital and acquired QT syndromes but reentry may play a role in the perpetuation of the ventricular tachycardia, torsade de pointes. Studies of the ionic mechanisms of early afterdepolarizations have implicated L-type Ca2+ current, persisting Na+ current, and Na+:Ca2+ exchange current related to Ca2+ loading. Different ionic mechanisms may be operative in early afterdepolarizations occuring at different levels of membrane potential in the setting of prolonged repolarization by blocking K+ currents or maintaining non-inactivating Ca2+ or Na+ currents or in early afterdepolarizations due to adrenergic stimulation. In the congenital long QT syndromes, two mutations have recently been discovered in the genes SCN5A and HERG which encode respectively the Na+ channel and a K+ channel conducting the current IKr. It is postulated that the SCN5A mutation leads to a problem with inactivation of Na+ current. In the case of the HERG mutation, the K+ current appears to be diminished. In the case of the acquired long QT syndromes, the therapeutic challenge is to maintain the prolonged repolarization but to interrupt the arrhythmogenic cascade. Current therapies for torsades de pointes include speeding of the heart rate, which enhances K+ current, and Ca2+ blockers or Mg, also a Ca2+ blocker. In the congenital long QT syndromes, therapy in the past has been directed toward reducting adrenergic influence either by betablockade or left cardiac sympathectomy. Recent discoveries open other possibilities such as Na+ channel blockers and methods to increase IKr such as elevation of extracellular K+.
    MeSH term(s) Adrenergic beta-Antagonists/therapeutic use ; Anti-Arrhythmia Agents/pharmacology ; Anti-Arrhythmia Agents/therapeutic use ; Heart Conduction System/physiopathology ; Humans ; Long QT Syndrome/congenital ; Long QT Syndrome/physiopathology ; Long QT Syndrome/therapy ; Mutation ; Pacemaker, Artificial ; Sodium Channels/drug effects ; Sodium Channels/genetics ; Sympathectomy ; Sympathetic Nervous System/physiopathology
    Chemical Substances Adrenergic beta-Antagonists ; Anti-Arrhythmia Agents ; Sodium Channels
    Language English
    Publishing date 1996-02
    Publishing country France
    Document type Journal Article ; Review
    ZDB-ID 211571-2
    ISSN 0003-9683 ; 0301-4525
    ISSN 0003-9683 ; 0301-4525
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  6. Article ; Online: The diagnostic role of T wave morphology biomarkers in congenital and acquired long QT syndrome: A systematic review.

    Tardo, Daniel T / Peck, Matthew / Subbiah, Rajesh N / Vandenberg, Jamie I / Hill, Adam P

    Annals of noninvasive electrocardiology : the official journal of the International Society for Holter and Noninvasive Electrocardiology, Inc

    2022  , Page(s) e13015

    Abstract: ... using the terms "LQTS," "long QT syndrome," "QTc prolongation," "prolonged QT," and "T wave," "T wave ... Introduction: QTc prolongation is key in diagnosing long QT syndrome (LQTS), however 25%-50 ... subtypes but its role in acquired LQTS (aLQTS) is unclear.: Methods: Electronic databases were searched ...

    Abstract Introduction: QTc prolongation is key in diagnosing long QT syndrome (LQTS), however 25%-50% with congenital LQTS (cLQTS) demonstrate a normal resting QTc. T wave morphology (TWM) can distinguish cLQTS subtypes but its role in acquired LQTS (aLQTS) is unclear.
    Methods: Electronic databases were searched using the terms "LQTS," "long QT syndrome," "QTc prolongation," "prolonged QT," and "T wave," "T wave morphology," "T wave pattern," "T wave biomarkers." Whole text articles assessing TWM, independent of QTc, were included.
    Results: Seventeen studies met criteria. TWM measurements included T-wave amplitude, duration, magnitude, Tpeak-Tend, QTpeak, left and right slope, center of gravity (COG), sigmoidal and polynomial classifiers, repolarizing integral, morphology combination score (MCS) and principal component analysis (PCA); and vectorcardiographic biomarkers. cLQTS were distinguished from controls by sigmoidal and polynomial classifiers, MCS, QTpeak, Tpeak-Tend, left slope; and COG x axis. MCS detected aLQTS more significantly than QTc. Flatness, asymmetry and notching, J-Tpeak; and Tpeak-Tend correlated with QTc in aLQTS. Multichannel block in aLQTS was identified by early repolarization (ERD
    Conclusion: Numerous TWM biomarkers which supplement QTc assessment were identified. Their diagnostic capabilities include differentiation of genotypes, identification of concealed LQTS, differentiating aLQTS from cLQTS; and determining multichannel versus hERG channel blockade.
    Language English
    Publishing date 2022-11-07
    Publishing country United States
    Document type Journal Article
    ZDB-ID 1325530-7
    ISSN 1542-474X ; 1082-720X
    ISSN (online) 1542-474X
    ISSN 1082-720X
    DOI 10.1111/anec.13015
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  7. Article ; Online: Sex influences on ventricular repolarization duration in normal subjects and in type 1, 2 and 3 long QT syndrome patients: Different effect in acquired and congenital type 2 LQTS.

    Extramiana, Fabrice / Badilini, Fabio / Denjoy, Isabelle / Vaglio, Martino / Green, Cynthia L / Kligfield, Paul / Leenhardt, Antoine / Maison-Blanche, Pierre

    Journal of electrocardiology

    2020  Volume 62, Page(s) 148–154

    Abstract: Aim: To evaluate the interaction between sex and rate corrected QT interval (QTc) duration ... in normal subjects after drug-induced QT prolongation and in LQTS patients.: Methods: Semi-automated ... and 33 LQT3 patients (15 females)). A sex specific coefficient was calculated in each group and was ...

    Abstract Aim: To evaluate the interaction between sex and rate corrected QT interval (QTc) duration in normal subjects after drug-induced QT prolongation and in LQTS patients.
    Methods: Semi-automated measurements were performed on 875 digital ECGs (200 normal subjects off drugs (100 females), 200 normal subjects on Moxifloxacin (100 females), 259 LQT1 patients (161 females), 183 LQT2 patients (100 females) and 33 LQT3 patients (15 females)). A sex specific coefficient was calculated in each group and was used to calculate group specific corrected QT intervals (QTci).
    Results: The mean sex difference (female minus male) in QTci interval duration was 17 ms 95%CI(12.7; 21.3) in normal subjects, 19 ms (14.5; 23.5) on Moxifloxacin, and 13 ms (4.8; 21.2) in LQT1 patients. The mean difference was 2 ms (-7.9; 11.9) in LQT2 and - 5 ms (-32.2; 22.2) in LQT3 patients (p = 0.0067 for the group and sex interaction). In the subgroup of patients above 15 years and without beta blocker treatment, the sex effect (female minus male) on QTci interval duration was 17 ms (4.1; 29.9) in LQT1 patients. QTc duration was not different between sex in LQT2 and in LQT3 patients (mean difference - 3 ms (-21.6; 15.6) and 12 ms (-28.4; 52.4), respectively) (p = 0.0191 for group and sex interaction).
    Conclusions: The interaction between sex and QTc interval is preserved in type 1 LQTS and drug-induced QTc prolongation but blurred in type 2 LQTS. Further experimental studies are warranted to better understand the interaction of sexual hormones with malfunctioning KCNH2 encoded repolarizing potassium channel.
    MeSH term(s) Adrenergic beta-Antagonists ; Electrocardiography ; Female ; Humans ; Long QT Syndrome/diagnosis ; Male
    Chemical Substances Adrenergic beta-Antagonists
    Language English
    Publishing date 2020-08-26
    Publishing country United States
    Document type Journal Article
    ZDB-ID 410286-1
    ISSN 1532-8430 ; 0022-0736
    ISSN (online) 1532-8430
    ISSN 0022-0736
    DOI 10.1016/j.jelectrocard.2020.08.016
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: The application of root mean square electrocardiography (RMS ECG) for the detection of acquired and congenital long QT syndrome.

    Lux, Robert L / Sower, Christopher Todd / Allen, Nancy / Etheridge, Susan P / Tristani-Firouzi, Martin / Saarel, Elizabeth V

    PloS one

    2014  Volume 9, Issue 1, Page(s) e85689

    Abstract: ... Long QT Syndrome (LQTS).: Methods: RMS ECG signals were derived from high-resolution 24 hour Holter monitor ... automatically measured using custom software and compared to traditional QT measures using lead II.: Results ... of RMS ECG to assess ventricular repolarization in humans in the setting of drug-induced and congenital ...

    Abstract Background: Precise measurement of the QT interval is often hampered by difficulty determining the end of the low amplitude T wave. Root mean square electrocardiography (RMS ECG) provides a novel alternative measure of ventricular repolarization. Experimental data have shown that the interval between the RMS ECG QRS and T wave peaks (RTPK) closely reflects the mean ventricular action potential duration while the RMS T wave width (TW) tracks the dispersion of repolarization timing. Here, we tested the precision of RMS ECG to assess ventricular repolarization in humans in the setting of drug-induced and congenital Long QT Syndrome (LQTS).
    Methods: RMS ECG signals were derived from high-resolution 24 hour Holter monitor recordings from 68 subjects after receiving placebo and moxifloxacin and from standard 12 lead ECGs obtained in 97 subjects with LQTS and 97 age- and sex-matched controls. RTPK, QTRMS and RMS TW intervals were automatically measured using custom software and compared to traditional QT measures using lead II.
    Results: All measures of repolarization were prolonged during moxifloxacin administration and in LQTS subjects, but the variance of RMS intervals was significantly smaller than traditional lead II measurements. TW was prolonged during moxifloxacin and in subjects with LQT-2, but not LQT-1 or LQT-3.
    Conclusion: These data validate the application of RMS ECG for the detection of drug-induced and congenital LQTS. RMS ECG measurements are more precise than the current standard of care lead II measurements.
    MeSH term(s) Adolescent ; Adult ; Cardiotonic Agents/therapeutic use ; Case-Control Studies ; Child ; Child, Preschool ; Data Interpretation, Statistical ; Electrocardiography, Ambulatory/methods ; Female ; Fluoroquinolones/therapeutic use ; Heart Rate ; Humans ; Long QT Syndrome/diagnosis ; Long QT Syndrome/drug therapy ; Long QT Syndrome/physiopathology ; Male ; Middle Aged ; Moxifloxacin ; Randomized Controlled Trials as Topic ; Young Adult
    Chemical Substances Cardiotonic Agents ; Fluoroquinolones ; Moxifloxacin (U188XYD42P)
    Language English
    Publishing date 2014-01-15
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1932-6203
    ISSN (online) 1932-6203
    DOI 10.1371/journal.pone.0085689
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  9. Article ; Online: Molecular aspects of the congenital and acquired Long QT Syndrome: clinical implications.

    Saenen, J B / Vrints, C J

    Journal of molecular and cellular cardiology

    2008  Volume 44, Issue 4, Page(s) 633–646

    Abstract: The Long QT Syndrome (LQTS) is a complex and multi-factorial disorder that predisposes to life ... threatening ventricular arrhythmias. Both hereditary and acquired subforms have been identified over the years ... Recently, it has become clear that the interaction of multiple acquired and genetic aetiologic factors (e.g ...

    Abstract The Long QT Syndrome (LQTS) is a complex and multi-factorial disorder that predisposes to life-threatening ventricular arrhythmias. Both hereditary and acquired subforms have been identified over the years. Recently, it has become clear that the interaction of multiple acquired and genetic aetiologic factors (e.g. disease modifiers) play an important role in differentiating genotype into a continuous spectrum of clinical or subclinical phenotypes. The genotype-phenotype correlation thereby remains very unpredictable in asymptomatic patients, raising important concerns for clinical practice and also for drug development. Therefore, this review aims at providing a comprehensive overview on LQTS highlighting the molecular mechanisms of arrhythmogenesis involved in both the hereditary and the acquired subtypes of the disorder. From this perspective this manuscript then focuses on how the genotype translates into phenotype. A logical overview is provided with the multitude of hereditary and acquired factors that are involved and of the complexity of the interactions that ultimately result in the heterogeneous expressivity and the unpredictability of the phenotype. Based on recent basic and clinical data this review further aims at providing an update on the clinical properties and management of LQT patients including diagnostic work-up and therapy.
    MeSH term(s) Genotype ; Humans ; Long QT Syndrome/congenital ; Long QT Syndrome/diagnosis ; Long QT Syndrome/pathology ; Long QT Syndrome/therapy ; Phenotype
    Language English
    Publishing date 2008-04
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 80157-4
    ISSN 1095-8584 ; 0022-2828
    ISSN (online) 1095-8584
    ISSN 0022-2828
    DOI 10.1016/j.yjmcc.2008.01.006
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  10. Article ; Online: The application of root mean square electrocardiography (RMS ECG) for the detection of acquired and congenital long QT syndrome.

    Robert L Lux / Christopher Todd Sower / Nancy Allen / Susan P Etheridge / Martin Tristani-Firouzi / Elizabeth V Saarel

    PLoS ONE, Vol 9, Iss 1, p e

    2014  Volume 85689

    Abstract: ... Long QT Syndrome (LQTS). METHODS: RMS ECG signals were derived from high-resolution 24 hour Holter monitor ... automatically measured using custom software and compared to traditional QT measures using lead II. RESULTS ... of RMS ECG to assess ventricular repolarization in humans in the setting of drug-induced and congenital ...

    Abstract BACKGROUND: Precise measurement of the QT interval is often hampered by difficulty determining the end of the low amplitude T wave. Root mean square electrocardiography (RMS ECG) provides a novel alternative measure of ventricular repolarization. Experimental data have shown that the interval between the RMS ECG QRS and T wave peaks (RTPK) closely reflects the mean ventricular action potential duration while the RMS T wave width (TW) tracks the dispersion of repolarization timing. Here, we tested the precision of RMS ECG to assess ventricular repolarization in humans in the setting of drug-induced and congenital Long QT Syndrome (LQTS). METHODS: RMS ECG signals were derived from high-resolution 24 hour Holter monitor recordings from 68 subjects after receiving placebo and moxifloxacin and from standard 12 lead ECGs obtained in 97 subjects with LQTS and 97 age- and sex-matched controls. RTPK, QTRMS and RMS TW intervals were automatically measured using custom software and compared to traditional QT measures using lead II. RESULTS: All measures of repolarization were prolonged during moxifloxacin administration and in LQTS subjects, but the variance of RMS intervals was significantly smaller than traditional lead II measurements. TW was prolonged during moxifloxacin and in subjects with LQT-2, but not LQT-1 or LQT-3. CONCLUSION: These data validate the application of RMS ECG for the detection of drug-induced and congenital LQTS. RMS ECG measurements are more precise than the current standard of care lead II measurements.
    Keywords Medicine ; R ; Science ; Q
    Subject code 551
    Language English
    Publishing date 2014-01-01T00:00:00Z
    Publisher Public Library of Science (PLoS)
    Document type Article ; Online
    Database BASE - Bielefeld Academic Search Engine (life sciences selection)

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