Materiały źródłowe

• #1 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Long QT syndrome (LQTS) is a cardiac disorder characterized by prolonged repolarization of the heart’s electrical cycle, which can be observed as an extended QT interval on an electrocardiogram (ECG). The safe and effective management of LQTS often necessitates a multifaceted approach encompassing pharmacological treatment, lifestyle modifications, and, in high-risk cases, the implantation of implantable cardioverter-defibrillators (ICDs). […] Lifestyle modifications, such as avoiding strenuous physical activities and certain medications, play a critical role. […] For individuals diagnosed with LQTS, the management strategy includes a focus on preventing lethal cardiac arrhythmias such as ventricular tachycardia/ventricular fibrillation. Treatment options range from pharmacological interventions to surgical and device-based solutions, tailored according to the patient’s symptoms and risk levels identified through risk stratification.

• #2 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #3 Long QT syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/long-qt-syndrome

  There is no known way to prevent congenital long QT syndrome (LQTS). If someone in your family has LQTS, ask a health professional if genetic screening is right for you. With proper treatment, you can manage and prevent the dangerous heartbeats that can lead to LQTS complications. […] Regular health checkups and good communication with your healthcare professional also may help prevent causes of some types of acquired long QT syndrome. It’s especially important not to take medicines that can affect the heart rhythm and cause a prolonged QT interval.

• #4 Long QT syndrome – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/long-qt-syndrome/

  Congenital long QT syndrome is caused by a genetic mutation. There is no known prevention. However, with proper treatment, you can prevent the dangerous heartbeats that can lead to long QT syndrome complications. […] You may be able to prevent the health conditions that lead to some types of acquired long QT syndrome with regular health checkups and good communication with your doctor. It is especially important to avoid medications that can affect your heart rhythm and cause prolongation of the QT interval.

• #5 Long QT Syndrome Panel Test – PreventionGenetics

  https://www.preventiongenetics.com/testInfo?val=Long-QT-Syndrome-Panel

  Candidates for this test include individuals with long QT syndrome, or a family history of long QT syndrome. […] Advantages of genetic testing for LQTS include confirmation of diagnosis, targeted testing of other family members, and assistance with reproductive planning. An early diagnosis of LQTS followed by early medical treatment (beta-blockers, implantable cardioverter-defibrillators, and left cardiac sympathetic denervation) has been demonstrated to reduce the mortality rate (Rohatgi et al. 2017. PubMed ID: 28728690).

• #6 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #7 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  The decision to implement ICD therapy must be carefully considered, especially given the device’s association with complications such as inappropriate shocks and the psychosocial impact on quality of life. […] The 2022 European Society of Cardiology (ESC) guidelines recommend beta-blockers as the initial therapy for all LQTS patients irrespective of heart rate, though the impact on those with slow heart rates remains unclear. […] LCSD is another non-drug strategy for those with contraindications or intolerance to ICDs; however, its effectiveness and safety on a larger scale remain uncertain, and it is primarily considered an auxiliary therapy.

• #8 Congenital long QT syndrome: Treatment – UpToDate

  https://www.uptodate.com/contents/congenital-long-qt-syndrome-treatment-and-prognosis

  Regardless of genotype, age, and previous symptomatic/asymptomatic status, all patients with congenital LQTS should be advised of simple QT preventive measures and implement them whenever possible. These include avoidance of medications with QT-prolonging potential; replacing electrolytes during vomiting and diarrheal illnesses, as both hypokalemia and hypomagnesemia can be QT aggravating; and lowering fever. […] Like the 2015 Heart Rhythm Society (HRS) guidelines, the 2017 American Heart Association/American College of Cardiology (AHA/ACC) guidelines continue to recommend universal beta-blocker therapy for all patients with congenital LQTS, whether asymptomatic or symptomatic, in the absence of a contraindication.

• #9 The Clinical Diagnosis and Management of Long QT Syndrome: Insights from the 2022 ESC Guidelines

  https://www.imrpress.com/journal/RCM/24/6/10.31083/j.rcm2406170/htm

  We read the updated 2022 European Society of Cardiology (ESC) guidelines on the management of patients with long QT syndrome (LQTS) to prevent the occurrence of life-threatening ventricular arrhythmia (VA) and/or sudden cardiac death (SCD) (Table 1) [1]. […] General recommendations to prevent SCD […] Beta-blockers, ideally non-selective beta-blockers (nadolol or propranolol), are recommended in LQTS patients with documented QT interval prolongation, to reduce risk of arrhythmic events. […] ICD implantation is recommended in patients with LQTS who are symptomatic while receiving beta-blockers and genotype-specific therapies. […] These guidelines also provide a lifesaving recommendation of non-drug therapeutic strategies for the treatment of VA and prevention of SCD when pharmacotherapy fails [1]. Use of implantable cardiac defibrillators (ICD) is only recommended in patients with symptomatic LQTS while receiving β-blockers and genotype-specific drugs. […] The guidelines state that genetic analysis of LQTS is crucial for diagnosis, prescription of genotype-specific drugs, and risk stratification.

• #10 Long QT syndrome with mutations in three genes: A rare case | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-long-qt-syndrome-with-mutations-articulo-S217420491500077X

  Genetic testing, which identifies the mutation responsible in 50-70% of cases, is important to confirm the diagnosis, identify asymptomatic carriers, stratify risk for arrhythmic events and provide a basis for genetic counseling. […] There is evidence that nadolol and propanolol are the safest and most effective beta-blockers for these patients.

• #11 The Clinical Diagnosis and Management of Long QT Syndrome: Insights from the 2022 ESC Guidelines

  https://www.imrpress.com/journal/RCM/24/6/10.31083/j.rcm2406170/htm

  We read the updated 2022 European Society of Cardiology (ESC) guidelines on the management of patients with long QT syndrome (LQTS) to prevent the occurrence of life-threatening ventricular arrhythmia (VA) and/or sudden cardiac death (SCD) (Table 1) [1]. […] General recommendations to prevent SCD […] Beta-blockers, ideally non-selective beta-blockers (nadolol or propranolol), are recommended in LQTS patients with documented QT interval prolongation, to reduce risk of arrhythmic events. […] ICD implantation is recommended in patients with LQTS who are symptomatic while receiving beta-blockers and genotype-specific therapies. […] These guidelines also provide a lifesaving recommendation of non-drug therapeutic strategies for the treatment of VA and prevention of SCD when pharmacotherapy fails [1]. Use of implantable cardiac defibrillators (ICD) is only recommended in patients with symptomatic LQTS while receiving β-blockers and genotype-specific drugs. […] The guidelines state that genetic analysis of LQTS is crucial for diagnosis, prescription of genotype-specific drugs, and risk stratification.

• #12 Long QT Syndrome Treatment & Management: Approach Considerations, Beta-Blockers, Pacemakers and ICDs

  https://emedicine.medscape.com/article/157826-treatment

  All patients with LQTS should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium levels. […] Potassium and magnesium deficiency should be corrected. […] Beta-blockers are drugs of choice for patients with LQTS. […] The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. […] Physical activity, swimming, and stress-related emotions frequently trigger cardiac events in patients with long QT syndrome (LQTS). Therefore, discourage patients from participating in competitive sports. […] The triggering effect of exercise and tachycardia, and therefore the protective effect of beta-blockers, varies depending on the type of LQTS. […] Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS).

• #13

  https://link.springer.com/article/10.1007/s11886-000-0033-2

  Life-long therapy is necessary for patients with symptomatic long QT syndrome to prevent arrhythmic death. […] b-blockers remain the mainstay of therapy, but this medication may not be sufficient for cardiac arrest survivors and for those with the LQT3 genotype. […] Genotype-specific therapy, like potassium-channel openers for patients with inadequate potassium outflow (LQT1 and LQT2 genotypes) or sodium-channel blockers for patients with excessive sodium inflow (LQT3), significantly shortens the QT interval, but the effects of these drugs on arrhythmia prevention is less well established. […] Cardiac pacemakers may be especially beneficial for patients with LQT2 or LQT3 and for those with pause-dependent torsade de pointes. […] More important is to recognize that device programming for preventing tachyarrhythmias in patients with long QT differs from the standard pacemaker programming. […] Finally, implantable defibrillators with dual-chamber pacing capability are indicated for patients at high risk for arrhythmic death, including all cardiac arrest survivors.

• #14 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #15

  https://link.springer.com/article/10.1007/s11886-000-0033-2

  Life-long therapy is necessary for patients with symptomatic long QT syndrome to prevent arrhythmic death. […] b-blockers remain the mainstay of therapy, but this medication may not be sufficient for cardiac arrest survivors and for those with the LQT3 genotype. […] Genotype-specific therapy, like potassium-channel openers for patients with inadequate potassium outflow (LQT1 and LQT2 genotypes) or sodium-channel blockers for patients with excessive sodium inflow (LQT3), significantly shortens the QT interval, but the effects of these drugs on arrhythmia prevention is less well established. […] Cardiac pacemakers may be especially beneficial for patients with LQT2 or LQT3 and for those with pause-dependent torsade de pointes. […] More important is to recognize that device programming for preventing tachyarrhythmias in patients with long QT differs from the standard pacemaker programming. […] Finally, implantable defibrillators with dual-chamber pacing capability are indicated for patients at high risk for arrhythmic death, including all cardiac arrest survivors.

• #16 Long QT Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/157826-guidelines

  Left cardiac sympathetic denervation should be considered in patients with symptomatic LQTS when (Level of evidence: C): […] Beta-blockers are ineffective, not tolerated, or contraindicated […] ICD therapy is contraindicated or refused […] Patients on beta-blockers with an ICD experience multiple shocks. […] Consider sodium channel blockers (mexiletine, flecainide or ranolazine) as add-on therapy to shorten the QT interval in LQTS3 patients with a QTc longer than 500 ms. […] Consider an ICD in addition to beta-blocker therapy in asymptomatic carriers of a pathogenic mutation in KCNH2 or SCN5A when the QTc is longer than 500 ms.

• #17 Long QT Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/157826-guidelines

  Left cardiac sympathetic denervation should be considered in patients with symptomatic LQTS when (Level of evidence: C): […] Beta-blockers are ineffective, not tolerated, or contraindicated […] ICD therapy is contraindicated or refused […] Patients on beta-blockers with an ICD experience multiple shocks. […] Consider sodium channel blockers (mexiletine, flecainide or ranolazine) as add-on therapy to shorten the QT interval in LQTS3 patients with a QTc longer than 500 ms. […] Consider an ICD in addition to beta-blocker therapy in asymptomatic carriers of a pathogenic mutation in KCNH2 or SCN5A when the QTc is longer than 500 ms.

• #18 Long QT syndrome – Wikipedia

  https://en.wikipedia.org/wiki/Long_QT_syndrome

  Arrhythmia suppression involves the use of medications or surgical procedures that attack the underlying cause of the arrhythmias associated with LQTS. Since the cause of arrhythmias in LQTS is early afterdepolarizations (EADs), and they are increased in states of adrenergic stimulation, steps can be taken to blunt adrenergic stimulation in these individuals. These include administration of beta receptor blocking agents, which decreases the risk of stress-induced arrhythmias. Nadolol, a powerful non-selective beta blocker, has been shown to reduce the arrhythmic risk in all three main genotypes (LQT1, LQT2, and LQT3). […] Genotype and QT interval duration are independent predictors of recurrence of life-threatening events. […] In patients considered at high risk of life-threatening arrhythmic events, ICD implantation may be considered as a preventive step.

• #19 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #20 Long QT Syndrome secondary prevention – wikidoc

  https://www.wikidoc.org/index.php/Long_QT_Syndrome_secondary_prevention

  Effective measures for the secondary prevention of long QT syndrome include: […] Implantable cardioverter-defibrillator (ICD) in high-risk patients […] Magnesium sulfate for recurrence of long QT-related ventricular ectopic beats or Torsades de pointes […] Beta blockers for patients with congenital LQTS and a history of syncope, seizures, or resuscitated sudden cardiac death.

• #21 The Clinical Diagnosis and Management of Long QT Syndrome: Insights from the 2022 ESC Guidelines

  https://www.imrpress.com/journal/RCM/24/6/10.31083/j.rcm2406170/htm

  We read the updated 2022 European Society of Cardiology (ESC) guidelines on the management of patients with long QT syndrome (LQTS) to prevent the occurrence of life-threatening ventricular arrhythmia (VA) and/or sudden cardiac death (SCD) (Table 1) [1]. […] General recommendations to prevent SCD […] Beta-blockers, ideally non-selective beta-blockers (nadolol or propranolol), are recommended in LQTS patients with documented QT interval prolongation, to reduce risk of arrhythmic events. […] ICD implantation is recommended in patients with LQTS who are symptomatic while receiving beta-blockers and genotype-specific therapies. […] These guidelines also provide a lifesaving recommendation of non-drug therapeutic strategies for the treatment of VA and prevention of SCD when pharmacotherapy fails [1]. Use of implantable cardiac defibrillators (ICD) is only recommended in patients with symptomatic LQTS while receiving β-blockers and genotype-specific drugs. […] The guidelines state that genetic analysis of LQTS is crucial for diagnosis, prescription of genotype-specific drugs, and risk stratification.

• #22

  https://link.springer.com/article/10.1007/s11886-000-0033-2

  Life-long therapy is necessary for patients with symptomatic long QT syndrome to prevent arrhythmic death. […] b-blockers remain the mainstay of therapy, but this medication may not be sufficient for cardiac arrest survivors and for those with the LQT3 genotype. […] Genotype-specific therapy, like potassium-channel openers for patients with inadequate potassium outflow (LQT1 and LQT2 genotypes) or sodium-channel blockers for patients with excessive sodium inflow (LQT3), significantly shortens the QT interval, but the effects of these drugs on arrhythmia prevention is less well established. […] Cardiac pacemakers may be especially beneficial for patients with LQT2 or LQT3 and for those with pause-dependent torsade de pointes. […] More important is to recognize that device programming for preventing tachyarrhythmias in patients with long QT differs from the standard pacemaker programming. […] Finally, implantable defibrillators with dual-chamber pacing capability are indicated for patients at high risk for arrhythmic death, including all cardiac arrest survivors.

• #23 Long QT Syndrome secondary prevention – wikidoc

  https://www.wikidoc.org/index.php/Long_QT_Syndrome_secondary_prevention

  Effective measures for the secondary prevention of long QT syndrome include: […] Implantable cardioverter-defibrillator (ICD) in high-risk patients […] Magnesium sulfate for recurrence of long QT-related ventricular ectopic beats or Torsades de pointes […] Beta blockers for patients with congenital LQTS and a history of syncope, seizures, or resuscitated sudden cardiac death.

• #24 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #25 Long QT Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/157826-guidelines

  Left cardiac sympathetic denervation should be considered in patients with symptomatic LQTS when (Level of evidence: C): […] Beta-blockers are ineffective, not tolerated, or contraindicated […] ICD therapy is contraindicated or refused […] Patients on beta-blockers with an ICD experience multiple shocks. […] Consider sodium channel blockers (mexiletine, flecainide or ranolazine) as add-on therapy to shorten the QT interval in LQTS3 patients with a QTc longer than 500 ms. […] Consider an ICD in addition to beta-blocker therapy in asymptomatic carriers of a pathogenic mutation in KCNH2 or SCN5A when the QTc is longer than 500 ms.

• #26 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  The decision to implement ICD therapy must be carefully considered, especially given the device’s association with complications such as inappropriate shocks and the psychosocial impact on quality of life. […] The 2022 European Society of Cardiology (ESC) guidelines recommend beta-blockers as the initial therapy for all LQTS patients irrespective of heart rate, though the impact on those with slow heart rates remains unclear. […] LCSD is another non-drug strategy for those with contraindications or intolerance to ICDs; however, its effectiveness and safety on a larger scale remain uncertain, and it is primarily considered an auxiliary therapy.

• #27

  https://journals.lww.com/cd/fulltext/2021/09000/clinical_advances_in_congenital_long_qt_syndrome.7.aspx

  The recommended indications of ICD implantation are patients with previous cardiac arrest, patients who experienced syncope and/or ventricular tachycardia while receiving an adequate dose of beta-blockers (and LCSD).[3] However, international guidelines are not always followed, and risk stratification may be based on genotype rather than individual risk profile. A previous research revealed that 30% of Swedish LQTS patients with ICD received the treatment without a strong indication based on international guidelines. LQT3 patients were over-represented among asymptomatic patients, and many LQT1 patients received ICD despite the known effect of beta-blockers.[51] Another recent study with 157 patients showed a trend that most patients had Class II and Class III indications for ICD implantation.[52] Therefore, prudent consideration is needed before deciding to recommend an ICD implantation, and more detailed data regarding the role of ICD for the primary prevention of sudden cardiac death in patients with LQTS is needed. In 2018, Biton et al[53] built a risk score based on Rochester LQTS-ICD registry and each risk variable was assigned for a score roughly reflecting the relative parameter estimate in the model (2 points for QTc 550 ms and 1 point for each of the other variables: QTc 500550 ms, syncope while on -blockers treatment, LQT2 genotype, and multiple mutations). And the results revealed that for patients with a score of 0, 1, 2, and 3, the cumulative probabilities of the first appropriate shock were 0%, 15%, 23%, and 50%, respectively after 10 years, (P 0.001) which provided genetic and clinical criteria to identify high-risk patients for whom ICD provides appropriate and necessary shocks.

• #28 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #29 Long QT Syndrome Guidelines: Guidelines Summary

  https://emedicine.medscape.com/article/157826-guidelines

  Left cardiac sympathetic denervation should be considered in patients with symptomatic LQTS when (Level of evidence: C): […] Beta-blockers are ineffective, not tolerated, or contraindicated […] ICD therapy is contraindicated or refused […] Patients on beta-blockers with an ICD experience multiple shocks. […] Consider sodium channel blockers (mexiletine, flecainide or ranolazine) as add-on therapy to shorten the QT interval in LQTS3 patients with a QTc longer than 500 ms. […] Consider an ICD in addition to beta-blocker therapy in asymptomatic carriers of a pathogenic mutation in KCNH2 or SCN5A when the QTc is longer than 500 ms.

• #30 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #31

  https://link.springer.com/article/10.1007/s11886-000-0033-2

  Life-long therapy is necessary for patients with symptomatic long QT syndrome to prevent arrhythmic death. […] b-blockers remain the mainstay of therapy, but this medication may not be sufficient for cardiac arrest survivors and for those with the LQT3 genotype. […] Genotype-specific therapy, like potassium-channel openers for patients with inadequate potassium outflow (LQT1 and LQT2 genotypes) or sodium-channel blockers for patients with excessive sodium inflow (LQT3), significantly shortens the QT interval, but the effects of these drugs on arrhythmia prevention is less well established. […] Cardiac pacemakers may be especially beneficial for patients with LQT2 or LQT3 and for those with pause-dependent torsade de pointes. […] More important is to recognize that device programming for preventing tachyarrhythmias in patients with long QT differs from the standard pacemaker programming. […] Finally, implantable defibrillators with dual-chamber pacing capability are indicated for patients at high risk for arrhythmic death, including all cardiac arrest survivors.

• #32 Prevention of torsade de pointes in the congenital long QT syndrome: use of a pause prevention pacing algorithm | Heart

  https://heart.bmj.com/content/79/4/417

  Torsade de pointes in the congenital long QT syndrome (LQTS) is often pause dependent. Thus, the main goal of pacemaker treatment in the LQTS may be the prevention of pauses that facilitate the onset of torsade de pointes. A pause prevention pacing algorithm (rate smoothing) was used for arrhythmia prevention in a 14 year old girl with congenital LQTS. By temporarily increasing the pacing rate after spontaneous premature beats, rate smoothing down of 18% prevented postextrasystolic pauses, pause related T-U changes, and recurrence of pause induced torsade de pointes. Rate smoothing is a potentially useful tool that ought to be evaluated for the prevention of torsade de pointes in the LQTS.

• #33 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Long QT syndrome (LQTS) is a cardiac disorder characterized by prolonged repolarization of the heart’s electrical cycle, which can be observed as an extended QT interval on an electrocardiogram (ECG). The safe and effective management of LQTS often necessitates a multifaceted approach encompassing pharmacological treatment, lifestyle modifications, and, in high-risk cases, the implantation of implantable cardioverter-defibrillators (ICDs). […] Lifestyle modifications, such as avoiding strenuous physical activities and certain medications, play a critical role. […] For individuals diagnosed with LQTS, the management strategy includes a focus on preventing lethal cardiac arrhythmias such as ventricular tachycardia/ventricular fibrillation. Treatment options range from pharmacological interventions to surgical and device-based solutions, tailored according to the patient’s symptoms and risk levels identified through risk stratification.

• #34 Management of Long QT Syndrome: A Systematic Review

  https://pmc.ncbi.nlm.nih.gov/articles/PMC11257643/

  Lifestyle modification remains a crucial complement to medical, surgical, or device therapy in managing LQTS. This entails avoiding extreme physical exertion, such as swimming and diving, as well as minimizing exposure to auditory stimuli such as alarm clocks, and being cautious with a wide array of medications listed on QTdrugs.org, to prevent recurrent symptoms. […] The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] Implantable ICDs are important in managing LQTS, particularly for patients with LQT3, who show reduced responsiveness to beta-blockers and often experience cardiac events during rest or sleep.

• #35 Long QT Syndrome Treatment & Management: Approach Considerations, Beta-Blockers, Pacemakers and ICDs

  https://emedicine.medscape.com/article/157826-treatment

  All patients with LQTS should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium levels. […] Potassium and magnesium deficiency should be corrected. […] Beta-blockers are drugs of choice for patients with LQTS. […] The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. […] Physical activity, swimming, and stress-related emotions frequently trigger cardiac events in patients with long QT syndrome (LQTS). Therefore, discourage patients from participating in competitive sports. […] The triggering effect of exercise and tachycardia, and therefore the protective effect of beta-blockers, varies depending on the type of LQTS. […] Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS).

• #36 Long QT syndrome: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/long-qt-syndrome

  It is not possible to prevent inherited LQTS. However, a person can prevent triggers or conditions that cause acquired LQTS. […] The following lifestyle changes may help reduce a persons risk of fainting and sudden cardiac death associated with LQTS: avoiding swimming alone, avoiding strenuous activities and exercises, including competitive sports, avoiding startling sounds, avoiding stressful situations and managing emotions, rehydrating, especially after an illness, taking potassium supplements and increasing intake of potassium-rich foods like vegetables and bananas, discussing with a doctor before taking new medications or supplements or making any other changes to your treatment plan.

• #37 Long-QT Syndrome and Competitive Sports | AER Journal

  https://www.aerjournal.com/articles/long-qt-syndrome-and-competitive-sports?language_content_entity=en

  The ESC recommendations for competitive sport participation, published in 2005, are the most restrictive. These state that congenital LQTS is a contraindication for any type of sports, even without documented major arrhythmic events. […] The more recent US guidelines on suitability and disqualification recommendations for competitive athletes in cardiac channelopathies, proposed in 2015, are less restrictive. […] For an athlete with symptomatic LQTS or an ECG with manifest LQTS, competitive sports participation (except competitive swimming in a previously symptomatic person with LQT1) may be considered after treatment has been implemented and appropriate precautionary measures taken, assuming the athlete has been asymptomatic on treatment for at least 3 months. […] In asymptomatic genotype-positive/phenotype-negative athletes, the experts concluded that it was reasonable for them to participate in all competitive sports as long as they took precautionary measures. These include the avoidance of QT-prolonging drugs, electrolyte/hydration replenishment and avoidance of dehydration, avoidance or treatment of training-related heat exhaustion or heat stroke, as well as acquiring a personal automatic external defibrillator (AED) and establishing an emergency action plan with school or team officials. […] As these studies state, optimal treatment is warranted. As the ESC guidelines recommend, beta-blockers are the cornerstone of treatment and are recommended in patients with a diagnosis of LQTS; they should also be considered in carriers of a causative LQTS mutation who have a normal QT interval.

• #38

  https://journals.lww.com/cd/fulltext/2021/09000/clinical_advances_in_congenital_long_qt_syndrome.7.aspx

  Lifestyle change is an essential part of LQTS management, which include avoidance of competitive sports, especially swimming or water sports in LQT1 patients, reduction in exposure to abrupt loud noises (alarm clock, phone ringing, etc) in LQT2 patients, and avoidance of QT-prolonging drugs and identification and correction of electrolyte abnormalities caused by vomiting, diarrhea, metabolic conditions or imbalanced diets for weight loss.[4,14,36] […] Life-threatening ventricular arrhythmias in LQTS patients are usually triggered by increased sympathetic activity, like physical exercise and emotional stress. Beta-blocker therapy is considered a milestone in all high and intermediate-risk patients with LQTS since the mid-1970s. They are recommended in all symptomatic patients and in asymptomatic patients with a resting QTc greater than 470 ms. However, their use is also reasonable in asymptomatic patients with a resting QTc less than 470 ms.[37] A previous study showed that beta-blockers were associated with a significant reduction in cardiac events in LQTS probands and in the affected family members (0.97 1.42 to 0.31 0.86 and 0.26 0.84 to 0.15 0.69 events per year, respectively).[19] However, despite a significant reduction in mortality, about 3% of LQTS patients treated with beta-blockers still experience recurrent cardiac event.[19,38]

• #39 Long QT syndrome: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/long-qt-syndrome

  It is not possible to prevent inherited LQTS. However, a person can prevent triggers or conditions that cause acquired LQTS. […] The following lifestyle changes may help reduce a persons risk of fainting and sudden cardiac death associated with LQTS: avoiding swimming alone, avoiding strenuous activities and exercises, including competitive sports, avoiding startling sounds, avoiding stressful situations and managing emotions, rehydrating, especially after an illness, taking potassium supplements and increasing intake of potassium-rich foods like vegetables and bananas, discussing with a doctor before taking new medications or supplements or making any other changes to your treatment plan.

• #40

  https://journals.lww.com/cd/fulltext/2021/09000/clinical_advances_in_congenital_long_qt_syndrome.7.aspx

  Lifestyle change is an essential part of LQTS management, which include avoidance of competitive sports, especially swimming or water sports in LQT1 patients, reduction in exposure to abrupt loud noises (alarm clock, phone ringing, etc) in LQT2 patients, and avoidance of QT-prolonging drugs and identification and correction of electrolyte abnormalities caused by vomiting, diarrhea, metabolic conditions or imbalanced diets for weight loss.[4,14,36] […] Life-threatening ventricular arrhythmias in LQTS patients are usually triggered by increased sympathetic activity, like physical exercise and emotional stress. Beta-blocker therapy is considered a milestone in all high and intermediate-risk patients with LQTS since the mid-1970s. They are recommended in all symptomatic patients and in asymptomatic patients with a resting QTc greater than 470 ms. However, their use is also reasonable in asymptomatic patients with a resting QTc less than 470 ms.[37] A previous study showed that beta-blockers were associated with a significant reduction in cardiac events in LQTS probands and in the affected family members (0.97 1.42 to 0.31 0.86 and 0.26 0.84 to 0.15 0.69 events per year, respectively).[19] However, despite a significant reduction in mortality, about 3% of LQTS patients treated with beta-blockers still experience recurrent cardiac event.[19,38]

• #41 Long QT Syndrome

  http://library.oumedicine.com/Search/134,233

  You can’t prevent inherited LQTS. But you can do things to reduce your risk for things that can set off arrhythmias and sudden death: […] Take your medicines as prescribed. Don’t take medicines that prolong the QT. […] Follow limits on certain activities, such as strenuous exercise and driving. […] Stay away from high-stress situations that may set off LQTS. […] Ask your healthcare provider if you need a potassium supplement. […] Wear a medical alert bracelet.

• #42 Long QT Syndrome

  https://healthlibrary.uwmedicine.org/Library/DiseasesConditions/Adult/Cardiovascular/134,233

  You can’t prevent inherited LQTS. But you can do things to reduce your risk for things that can set off arrhythmias and sudden death: […] Take your medicines as prescribed. Don’t take medicines that prolong the QT. […] Stay away from high-stress situations that may set off LQTS. […] Ask your healthcare provider if you need a potassium supplement. […] Wear a medical alert bracelet.

• #43 Long QT syndrome: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/long-qt-syndrome

  It is not possible to prevent inherited LQTS. However, a person can prevent triggers or conditions that cause acquired LQTS. […] The following lifestyle changes may help reduce a persons risk of fainting and sudden cardiac death associated with LQTS: avoiding swimming alone, avoiding strenuous activities and exercises, including competitive sports, avoiding startling sounds, avoiding stressful situations and managing emotions, rehydrating, especially after an illness, taking potassium supplements and increasing intake of potassium-rich foods like vegetables and bananas, discussing with a doctor before taking new medications or supplements or making any other changes to your treatment plan.

• #44 Long QT Syndrome

  http://library.oumedicine.com/Search/134,233

  You can’t prevent inherited LQTS. But you can do things to reduce your risk for things that can set off arrhythmias and sudden death: […] Take your medicines as prescribed. Don’t take medicines that prolong the QT. […] Follow limits on certain activities, such as strenuous exercise and driving. […] Stay away from high-stress situations that may set off LQTS. […] Ask your healthcare provider if you need a potassium supplement. […] Wear a medical alert bracelet.

• #45 Long QT Syndrome

  https://livinghealthy.hawaiipacifichealth.org/Search/134,233

  You can’t prevent inherited LQTS. But you can do things to reduce your risk for things that can set off arrhythmias and sudden death: […] See your healthcare provider regularly. […] Take your medicines as prescribed. Don’t take medicines that prolong the QT. […] Follow limits on certain activities, such as strenuous exercise and driving. […] Stay away from high-stress situations that may set off LQTS. […] Ask your healthcare provider if you need a potassium supplement. […] Wear a medical alert bracelet.

• #46 Long QT Syndrome Treatment & Management: Approach Considerations, Beta-Blockers, Pacemakers and ICDs

  https://emedicine.medscape.com/article/157826-treatment

  All patients with LQTS should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium levels. […] Potassium and magnesium deficiency should be corrected. […] Beta-blockers are drugs of choice for patients with LQTS. […] The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. […] Physical activity, swimming, and stress-related emotions frequently trigger cardiac events in patients with long QT syndrome (LQTS). Therefore, discourage patients from participating in competitive sports. […] The triggering effect of exercise and tachycardia, and therefore the protective effect of beta-blockers, varies depending on the type of LQTS. […] Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS).

• #47 Congenital long QT syndrome: Treatment – UpToDate

  https://www.uptodate.com/contents/congenital-long-qt-syndrome-treatment-and-prognosis

  Regardless of genotype, age, and previous symptomatic/asymptomatic status, all patients with congenital LQTS should be advised of simple QT preventive measures and implement them whenever possible. These include avoidance of medications with QT-prolonging potential; replacing electrolytes during vomiting and diarrheal illnesses, as both hypokalemia and hypomagnesemia can be QT aggravating; and lowering fever. […] Like the 2015 Heart Rhythm Society (HRS) guidelines, the 2017 American Heart Association/American College of Cardiology (AHA/ACC) guidelines continue to recommend universal beta-blocker therapy for all patients with congenital LQTS, whether asymptomatic or symptomatic, in the absence of a contraindication.

• #48 Long QT Syndrome Treatment & Management: Approach Considerations, Beta-Blockers, Pacemakers and ICDs

  https://emedicine.medscape.com/article/157826-treatment

  Epinephrine (adrenaline) for local anesthesia or as an asthma medication should be avoided in patients with long QT syndrome (LQTS). […] Antihistamines to be avoided include the following: Terfenadine (Seldane [recalled from US market]), Astemizole (Hismanal [recalled from US market]), Diphenhydramine (Benadryl). […] Heart medications to avoid in patients with LQTS include the following: Quinidine, Procainamide, Disopyramide, Sotalol, Probucol, Bepridil, Dofetilide, Ibutilide. […] Potassium-loss medications to be avoided include the following: Indapamide, Other diuretics, Medications for vomiting and diarrhea.

• #49 Long QT Syndrome – Harvard Health

  https://www.health.harvard.edu/a_to_z/long-qt-syndrome-a-to-z

  There’s no way to prevent inherited long QT syndrome. However, people with the inherited syndrome want to avoid taking medications that can make the QT even longer than it is naturally. Eating enough food and a balanced diet can prevent mineral imbalances that may lengthen the QT interval. […] Everyone starting a new medication should check with a pharmacist or doctor about potential interactions with current medications. This includes people who don’t have any history of long QT intervals. Some drug combinations can significantly prolong the QT interval, which would increase the risk of an arrhythmia.

• #50 Long QT syndrome Guide: Causes, Symptoms and Treatment Options

  https://www.drugs.com/health-guide/long-qt-syndrome.html

  There’s no way to prevent inherited long QT syndrome. However, people with the inherited syndrome want to avoid taking medications that can make the QT even longer than it is naturally. Eating enough food and a balanced diet can prevent mineral imbalances that may lengthen the QT interval. […] Everyone starting a new medication should check with a pharmacist or doctor about potential interactions with current medications. This includes people who don’t have any history of long QT intervals. Some drug combinations can significantly prolong the QT interval, which would increase the risk of an arrhythmia.

• #51 Long QT Syndrome Treatment & Management: Approach Considerations, Beta-Blockers, Pacemakers and ICDs

  https://emedicine.medscape.com/article/157826-treatment

  All patients with LQTS should avoid drugs that prolong the QT interval or that reduce their serum potassium or magnesium levels. […] Potassium and magnesium deficiency should be corrected. […] Beta-blockers are drugs of choice for patients with LQTS. […] The protective effect of beta-blockers is related to their adrenergic blockade, which diminishes the risk of cardiac arrhythmias. […] Physical activity, swimming, and stress-related emotions frequently trigger cardiac events in patients with long QT syndrome (LQTS). Therefore, discourage patients from participating in competitive sports. […] The triggering effect of exercise and tachycardia, and therefore the protective effect of beta-blockers, varies depending on the type of LQTS. […] Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS).

• #52 Congenital long QT syndrome: Treatment – UpToDate

  https://www.uptodate.com/contents/congenital-long-qt-syndrome-treatment-and-prognosis

  Regardless of genotype, age, and previous symptomatic/asymptomatic status, all patients with congenital LQTS should be advised of simple QT preventive measures and implement them whenever possible. These include avoidance of medications with QT-prolonging potential; replacing electrolytes during vomiting and diarrheal illnesses, as both hypokalemia and hypomagnesemia can be QT aggravating; and lowering fever. […] Like the 2015 Heart Rhythm Society (HRS) guidelines, the 2017 American Heart Association/American College of Cardiology (AHA/ACC) guidelines continue to recommend universal beta-blocker therapy for all patients with congenital LQTS, whether asymptomatic or symptomatic, in the absence of a contraindication.

• #53 Long QT Syndrome

  http://library.oumedicine.com/Search/134,233

  You can’t prevent inherited LQTS. But you can do things to reduce your risk for things that can set off arrhythmias and sudden death: […] Take your medicines as prescribed. Don’t take medicines that prolong the QT. […] Follow limits on certain activities, such as strenuous exercise and driving. […] Stay away from high-stress situations that may set off LQTS. […] Ask your healthcare provider if you need a potassium supplement. […] Wear a medical alert bracelet.

• #54 Long QT syndrome – BHF

  https://www.bhf.org.uk/informationsupport/conditions/long-qt-syndrome

  If you have LQTS, you could be given medicines like beta blockers to treat it. Beta blockers, such as Propranolol or Nadolol, help control irregular heartbeats and slow your heart rate to make the prolonged QT interval less likely. […] If you’re at risk of a life-threatening arrhythmia and you need more than just medicine to manage this risk, you may need a pacemaker or an implantable cardioverter defibrillator (ICD) fitted. These are fitted to help control the rhythm and rate of your heart or help correct life-threatening heart rhythms. […] Some people may need surgery to remove certain nerves that affect the heart to help reduce the chance of a sudden cardiac arrest. This is usually only for people who cannot take medicine. […] Others with LQTS may need to be prescribed potassium supplements from their doctor. They might also suggest you increase your uptake of potassium-rich foods like bananas, vegetables and pulses. […] Always tell medical staff you have LQTS.

• #55 Long QT syndrome: Symptoms, causes, and more

  https://www.medicalnewstoday.com/articles/long-qt-syndrome

  It is not possible to prevent inherited LQTS. However, a person can prevent triggers or conditions that cause acquired LQTS. […] The following lifestyle changes may help reduce a persons risk of fainting and sudden cardiac death associated with LQTS: avoiding swimming alone, avoiding strenuous activities and exercises, including competitive sports, avoiding startling sounds, avoiding stressful situations and managing emotions, rehydrating, especially after an illness, taking potassium supplements and increasing intake of potassium-rich foods like vegetables and bananas, discussing with a doctor before taking new medications or supplements or making any other changes to your treatment plan.

• #56 Long QT Syndrome – Harvard Health

  https://www.health.harvard.edu/a_to_z/long-qt-syndrome-a-to-z

  There’s no way to prevent inherited long QT syndrome. However, people with the inherited syndrome want to avoid taking medications that can make the QT even longer than it is naturally. Eating enough food and a balanced diet can prevent mineral imbalances that may lengthen the QT interval. […] Everyone starting a new medication should check with a pharmacist or doctor about potential interactions with current medications. This includes people who don’t have any history of long QT intervals. Some drug combinations can significantly prolong the QT interval, which would increase the risk of an arrhythmia.

• #57 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  Congenital long QT syndrome (LQTS) is a primary genetic and electrical disorder that increases risk for torsades de pointes, syncope, and sudden death. Post-pubertal women with LQTS require specialized multidisciplinary management before, during, and after pregnancy involving cardiology and obstetrics to reduce risk for cardiac events in themselves and their fetuses and babies. […] Treatment of women with LQTS with a preferred -blocker at optimal doses along with close monitoring are indicated throughout pregnancy and during the high-risk postpartum period. […] Most studies show that the risk of LQTS-related cardiac events decreases during pregnancy compared with the time period before a woman’s first conception, but increases significantly in the 9-month postpartum period, and frequency of events returns to pre-pregnancy levels after this period.

• #58 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  Congenital long QT syndrome (LQTS) is a primary genetic and electrical disorder that increases risk for torsades de pointes, syncope, and sudden death. Post-pubertal women with LQTS require specialized multidisciplinary management before, during, and after pregnancy involving cardiology and obstetrics to reduce risk for cardiac events in themselves and their fetuses and babies. […] Treatment of women with LQTS with a preferred -blocker at optimal doses along with close monitoring are indicated throughout pregnancy and during the high-risk postpartum period. […] Most studies show that the risk of LQTS-related cardiac events decreases during pregnancy compared with the time period before a woman’s first conception, but increases significantly in the 9-month postpartum period, and frequency of events returns to pre-pregnancy levels after this period.

• #59 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  The hyper-estrogenic state during pregnancy may provide protection against arrhythmias. […] Once pregnancy is confirmed in a woman with LQTS, a management plan for pregnancy and the 9-month postpartum period should be made in collaboration with the obstetrician and cardiologist with ongoing review and discussion throughout. […] Treatment with a -blocker is indicated to reduce risk of cardiac events and sudden cardiac death. […] Guidelines for management of ventricular arrhythmias and prevention of sudden cardiac death strongly recommend that in women with LQTS, a -blocker should be continued during pregnancy and the postpartum period regardless of symptoms, including while breastfeeding. […] In at least one study, increased risk for cardiac events in the high-risk postpartum period was significantly reduced by -blockers.

• #60 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  The hyper-estrogenic state during pregnancy may provide protection against arrhythmias. […] Once pregnancy is confirmed in a woman with LQTS, a management plan for pregnancy and the 9-month postpartum period should be made in collaboration with the obstetrician and cardiologist with ongoing review and discussion throughout. […] Treatment with a -blocker is indicated to reduce risk of cardiac events and sudden cardiac death. […] Guidelines for management of ventricular arrhythmias and prevention of sudden cardiac death strongly recommend that in women with LQTS, a -blocker should be continued during pregnancy and the postpartum period regardless of symptoms, including while breastfeeding. […] In at least one study, increased risk for cardiac events in the high-risk postpartum period was significantly reduced by -blockers.

• #61 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  Therefore, it is essential for high-risk women with LQTS to continue taking -blockers throughout the postpartum period as first-line, protective therapy for which the benefits outweigh risks of treatment. […] Close cardiac follow-up of women with LQT2 mutation during the postpartum period is recommended with serial ECGs every few weeks after delivery in consultation with a cardiologist experienced in LQTS management. […] Treatment with -blockers at adequate doses is of paramount importance in LQT2 patients postpartum. […] Women with congenital LQTS require a team approach involving cardiology and obstetrics before, during and after pregnancy to optimize care and reduce the risk of potentially life-threatening events in the mother, fetus, and baby.

• #62 Congenital long QT syndrome during and after pregnancy

  https://www.uscjournal.com/articles/management-long-qt-syndrome-women-during-and-after-pregnancy?language_content_entity=en

  Therefore, it is essential for high-risk women with LQTS to continue taking -blockers throughout the postpartum period as first-line, protective therapy for which the benefits outweigh risks of treatment. […] Close cardiac follow-up of women with LQT2 mutation during the postpartum period is recommended with serial ECGs every few weeks after delivery in consultation with a cardiologist experienced in LQTS management. […] Treatment with -blockers at adequate doses is of paramount importance in LQT2 patients postpartum. […] Women with congenital LQTS require a team approach involving cardiology and obstetrics before, during and after pregnancy to optimize care and reduce the risk of potentially life-threatening events in the mother, fetus, and baby.

• #63 Drugs to be avoided in patients with long QT syndrome: Focus on the anaesthesiological management

  https://www.wjgnet.com/1949-8462/full/v5/i4/87.htm

  Long QT syndrome incidence is increasing in general population. A careful pre-, peri- and post-operative management is needed for patients with this syndrome because of the risk of Torsades de Pointes and malignant arrhythmias. […] Actually, there are no definitive guidelines on pre-, peri- and post-operative anesthetic management of congenital long QT syndrome. Our review focuses on anesthetic recommendations for patients diagnosed with congenital long QT syndrome furnishing some key points for preoperative optimization, intraoperative anesthetic agents and postoperative care plan, which could be the best for patients with c-long QT syndrome who undergo surgery. […] A good anaesthesiological preoperative physical examination should be the cornerstone, mostly in childhood and adolescence. Moreover, an ECG at rest is always needed in order to reveal a QT prolongation. Patients treated with beta-blockers should continue their medication throughout the perioperative period until the operating day. Electrolytes should be normalized. Drugs known to induce TdP (Table 1) should be discontinued or the dose should be decreased if it cannot be discontinued. The presence of a pacemaker or implantable cardioverter defibrillator should be checked.

• #64 Drugs to be avoided in patients with long QT syndrome: Focus on the anaesthesiological management

  https://www.wjgnet.com/1949-8462/full/v5/i4/87.htm

  Despite an adequate -blocking, patients with LQTS candidate to surgical or anesthetic procedure have an increased risk of developing perioperative ventricular arrhythmias. The probability of developing these arrhythmias significantly decreases with a careful pre-, intra- and post-operative management. […] Postoperative management of patients with c-LQTS should include the permanence in a postsurgical intensive care unit for at least 24 h, avoiding stimuli that could trigger TdP. An adequate postoperative analgesia and beta-blocking must be guaranteed. […] We speculate that genetic subtyping of patients with LQTS could help tailor anesthetic therapy for these high-risk patients.

• #65 Drugs to be avoided in patients with long QT syndrome: Focus on the anaesthesiological management

  https://www.wjgnet.com/1949-8462/full/v5/i4/87.htm

  Despite an adequate -blocking, patients with LQTS candidate to surgical or anesthetic procedure have an increased risk of developing perioperative ventricular arrhythmias. The probability of developing these arrhythmias significantly decreases with a careful pre-, intra- and post-operative management. […] Postoperative management of patients with c-LQTS should include the permanence in a postsurgical intensive care unit for at least 24 h, avoiding stimuli that could trigger TdP. An adequate postoperative analgesia and beta-blocking must be guaranteed. […] We speculate that genetic subtyping of patients with LQTS could help tailor anesthetic therapy for these high-risk patients.

• #66 Long QT Syndrome | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/heart-disease/long-qt-syndrome/

  When the heart takes longer than it should to “recharge” between heartbeats, the condition is called long QT syndrome. […] It is important that the diagnosis of long QT syndrome is made early in life in order to prevent severe manifestations of the syndrome. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute will likely prescribe medication, diet changes and exercise to help children with long QT syndrome stay healthy. […] Long QT syndrome often can be treated with beta blocker medications. […] For children who are born with long QT syndrome, treatment usually won’t shorten the QT interval. But it can lower the risk of life-threatening abnormal heart rhythms and fainting. […] If your child has long QT syndrome, certain medicines should be avoided. Check with your child’s pediatric cardiologist to find out which medicines are safe.

• #67 Long QT Syndrome | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/heart-disease/long-qt-syndrome/

  When the heart takes longer than it should to “recharge” between heartbeats, the condition is called long QT syndrome. […] It is important that the diagnosis of long QT syndrome is made early in life in order to prevent severe manifestations of the syndrome. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute will likely prescribe medication, diet changes and exercise to help children with long QT syndrome stay healthy. […] Long QT syndrome often can be treated with beta blocker medications. […] For children who are born with long QT syndrome, treatment usually won’t shorten the QT interval. But it can lower the risk of life-threatening abnormal heart rhythms and fainting. […] If your child has long QT syndrome, certain medicines should be avoided. Check with your child’s pediatric cardiologist to find out which medicines are safe.

• #68 Long QT Syndrome | Norton Children’s

  https://nortonchildrens.com/services/cardiology/conditions/heart-disease/long-qt-syndrome/

  When the heart takes longer than it should to “recharge” between heartbeats, the condition is called long QT syndrome. […] It is important that the diagnosis of long QT syndrome is made early in life in order to prevent severe manifestations of the syndrome. […] The board-certified and fellowship-trained specialists at Norton Children’s Heart Institute will likely prescribe medication, diet changes and exercise to help children with long QT syndrome stay healthy. […] Long QT syndrome often can be treated with beta blocker medications. […] For children who are born with long QT syndrome, treatment usually won’t shorten the QT interval. But it can lower the risk of life-threatening abnormal heart rhythms and fainting. […] If your child has long QT syndrome, certain medicines should be avoided. Check with your child’s pediatric cardiologist to find out which medicines are safe.

• #69 Long QT syndrome // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/long-qt-syndrome

  There is no known way to prevent congenital long QT syndrome (LQTS). If someone in your family has LQTS, ask a health professional if genetic screening is right for you. With proper treatment, you can manage and prevent the dangerous heartbeats that can lead to LQTS complications. […] Regular health checkups and good communication with your healthcare professional also may help prevent causes of some types of acquired long QT syndrome. It’s especially important not to take medicines that can affect the heart rhythm and cause a prolonged QT interval.

• #70 Long QT syndrome – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/long-qt-syndrome/

  Congenital long QT syndrome is caused by a genetic mutation. There is no known prevention. However, with proper treatment, you can prevent the dangerous heartbeats that can lead to long QT syndrome complications. […] You may be able to prevent the health conditions that lead to some types of acquired long QT syndrome with regular health checkups and good communication with your doctor. It is especially important to avoid medications that can affect your heart rhythm and cause prolongation of the QT interval.

• #71 Long QT Syndrome: Symptoms, Causes, and Managing LQTS

  https://resources.healthgrades.com/right-care/heart-health/long-qt-syndrome

  It is not possible to prevent the congenital form of LQTS. However, you can reduce symptoms and cardiac events by following your treatment plan. This includes avoiding events that trigger fainting and other symptoms. You will also need to avoid drugs that can aggravate the problem. […] You may be able to prevent the acquired form of LQTS by avoiding drugs that cause it. Treating medical conditions that can cause it is another way to reduce your risk. […] For people with LQTS, it is important to work with your pharmacist. Using the same pharmacy for all of your prescription needs is key. This allows your pharmacist to monitor all of your drugs and find potential problems.

• #72 Long QT syndrome with mutations in three genes: A rare case | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-long-qt-syndrome-with-mutations-articulo-S217420491500077X

  Genetic testing, which identifies the mutation responsible in 50-70% of cases, is important to confirm the diagnosis, identify asymptomatic carriers, stratify risk for arrhythmic events and provide a basis for genetic counseling. […] There is evidence that nadolol and propanolol are the safest and most effective beta-blockers for these patients.

• #73 Long QT syndrome with mutations in three genes: A rare case | Revista Portuguesa de Cardiologia (English edition)

  https://www.revportcardiol.org/en-long-qt-syndrome-with-mutations-articulo-S217420491500077X

  Genetic testing, which identifies the mutation responsible in 50-70% of cases, is important to confirm the diagnosis, identify asymptomatic carriers, stratify risk for arrhythmic events and provide a basis for genetic counseling. […] There is evidence that nadolol and propanolol are the safest and most effective beta-blockers for these patients.

• #74 Long QT Syndrome via the SCN4B Gene Test – PreventionGenetics

  https://www.preventiongenetics.com/testInfo?val=Long-QT-Syndrome-via-the-SCN4B-Gene

  All patients with symptoms suggestive of Long QT syndrome are candidates for this test. […] Long QT syndrome (LQTS) is a heritable channelopathy characterized by an exceedingly prolonged cardiac repolarization that may trigger ventricular arrhythmias (torsade de pointes), recurrent syncopes, seizure, or sudden cardiac death (SCD) (Cerrone et al. 2012). […] Pathogenic variants in SCN4B are associated with Long QT syndrome type 10 (LQT10) and are inherited in an autosomal dominant manner (Yu et al. 2003). […] Up to 70 % of patients with clinical diagnosis of Long QT syndrome have identifiable pathogenic variants (Beckmann et al. 2013). […] The majority of LQTS cases are caused by pathogenic variants in one of three genes: KCNQ1, KCNH2 and SCN5A. […] This test provides full coverage of all coding exons of the SCN4B gene plus 10 bases of flanking noncoding DNA in all available transcripts along with other non-coding regions in which pathogenic variants have been identified at PreventionGenetics or reported elsewhere. […] All patients with symptoms suggestive of Long QT syndrome are candidates for this test.

• #75 Long QT Syndrome Panel Test – PreventionGenetics

  https://www.preventiongenetics.com/testInfo?val=Long-QT-Syndrome-Panel

  Candidates for this test include individuals with long QT syndrome, or a family history of long QT syndrome. […] Advantages of genetic testing for LQTS include confirmation of diagnosis, targeted testing of other family members, and assistance with reproductive planning. An early diagnosis of LQTS followed by early medical treatment (beta-blockers, implantable cardioverter-defibrillators, and left cardiac sympathetic denervation) has been demonstrated to reduce the mortality rate (Rohatgi et al. 2017. PubMed ID: 28728690).

• #76 Long QT syndrome – Genomics Education Programme

  https://www.genomicseducation.hee.nhs.uk/documents/long-qt-syndrome/

  The focus in the management of individuals with LQTS is to identify the subset of individuals at high risk of cardiac events. Risk stratification would include: […] Pre-symptomatic diagnosis of at-risk relatives via clinical screening and targeted variant analysis, followed by treatment, is necessary to prevent syncope and sudden death in those individuals who have inherited a pathogenic variant and/or have ECG findings consistent with LQTS. At-risk family members should be alerted to their risk. […] Management is focused on the prevention of syncope, cardiac arrest and sudden death using: -blockers; implantable cardioverter-defibrillators; left cardiac sympathetic denervation; and sodium channel blockers. […] The postpartum period is associated with increased risk for a cardiac event, especially in individuals with a LQTS2 phenotype. -blocker treatment and close clinical surveillance has been associated with a reduction of events during the nine month postpartum period.

• #77 Therapeutic for prevention and treatment of autoimmune induced Long QT Syndrome | TechLinkTechLinkTechLinkTechLinkTechLinkFacebookTwitterLinkedinDouble Arrow LeftApple iTunesAndroidRSS

  https://techlinkcenter.org/technologies/therapeutic-for-prevention-and-treatment-of-autoimmune-induced-long-qt-syndrome/6cad069c-8fe5-4f2c-ac80-7811917f6a75

  Therapeutic for prevention and treatment of autoimmune induced Long QT Syndrome […] Department of Veterans Affairs scientists under the leadership of Dr. Mohamed Boutjdir have developed a therapeutic biologic that alleviates Long QT syndrome – a rare disease induced by anti-Ro/SSA-antibodies. […] The decoy peptide or polypeptide can block the activity of anti-Ro antibodies by binding to the anti-Ro antibodies. It can also inhibit an anti-Ro antibody binding to the hERG-K channel by binding to the anti-Ro antibodies. […] Pharmaceutical composition prevents and treats QTc prolongation in a patient with autoimmune disease. […] Decoy peptides used in the composition can prevent or reduce the risk of ventricular tachycardia in patients.

• #78 Long QT interval in resting electrocardiogram – and what next? Sports cardiologist’s point of view Part 2. From provocative tests to prevention of cardiac events – Pediatria i Medycyna Rodzinna

  https://pimr.pl/index.php/wydawnictwa/2014-vol-10-no-4-1/long-qt-interval-in-resting-electrocardiogram-and-what-next-sports-cardiologist-s-point-of-view-part-2-from-provocative-tests-to-prevention-of-cardiac-events

  Resting electrocardiogram is a basic diagnostic tool used to identify myocardial diseases caused by a genetic defect of ion channels that results in bioelectric instability of cardiomyocytes and the risk of a complex induction of life-threatening ventricular arrhythmia. Long QT syndrome is the most common genetic ion channel disease affecting the general population. […] Prevention of cardiac events in patients with long QT syndrome involves, among others, the use of beta blockers. Implantation of a cardioverter-defibrillator is recommended in patients who developed cardiac arrest, and left cervicothoracic sympathectomy is also recommended in some cases. In terms of everyday functioning, patients with long QT syndrome should avoid drugs that prolong QT interval and have their electrolyte abnormalities corrected. […] Methods of risk stratification for sudden cardiac events in patients diagnosed with long QT syndrome, current possibilities associated with prevention and treatment as well as recommendations related to sports participation are also discussed.

• #79 Long QT syndrome — Knowledge Hub

  https://www.genomicseducation.hee.nhs.uk/genotes/knowledge-hub/long-qt-syndrome/

  All patients with LQTS should be given lifestyle advice. […] Certain drugs may increase the likelihood of arrhythmia in the setting of LQTS, and patients should be directed to CredibleMeds for a list of drugs to avoid. […] Affected individuals should avoid gene-specific triggers (such as competitive swimming in LQTS type 1). […] The mainstay of treatment is beta blockade (ideally non-selective beta blockers such as nadolol or propranolol). […] Implantable cardioverter-defibrillator (ICD) implantation is recommended in individuals who have survived cardiac arrest, or have arrhythmic syncope or haemodynamically non-tolerated ventricular arrhythmia despite beta blockade and genotype-specific therapy. […] Left sympathetic cardiac denervation (LSCD) is indicated in symptomatic individuals who decline ICD therapy or who have arrhythmic syncope or haemodynamically non-tolerated ventricular arrhythmia despite beta blockade and genotype-specific therapy (with ICD in situ). […] ICD implantation or LCSD should be considered in patients with symptomatic LQTS when beta-blockers and genotype-specific therapies are not tolerated or contraindicated at the therapeutic dose.

• #80 Long QT interval in resting electrocardiogram – and what next? Sports cardiologist’s point of view Part 2. From provocative tests to prevention of cardiac events – Pediatria i Medycyna Rodzinna

  https://pimr.pl/index.php/wydawnictwa/2014-vol-10-no-4-1/long-qt-interval-in-resting-electrocardiogram-and-what-next-sports-cardiologist-s-point-of-view-part-2-from-provocative-tests-to-prevention-of-cardiac-events

  Resting electrocardiogram is a basic diagnostic tool used to identify myocardial diseases caused by a genetic defect of ion channels that results in bioelectric instability of cardiomyocytes and the risk of a complex induction of life-threatening ventricular arrhythmia. Long QT syndrome is the most common genetic ion channel disease affecting the general population. […] Prevention of cardiac events in patients with long QT syndrome involves, among others, the use of beta blockers. Implantation of a cardioverter-defibrillator is recommended in patients who developed cardiac arrest, and left cervicothoracic sympathectomy is also recommended in some cases. In terms of everyday functioning, patients with long QT syndrome should avoid drugs that prolong QT interval and have their electrolyte abnormalities corrected. […] Methods of risk stratification for sudden cardiac events in patients diagnosed with long QT syndrome, current possibilities associated with prevention and treatment as well as recommendations related to sports participation are also discussed.

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