Materiały źródłowe

• #1 Long QT syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/diagnosis-treatment/drc-20352524

  Treatment for long QT syndrome (LQTS) may include: […] The goals of LQTS treatment are to: […] Your healthcare professional talks with you about your treatment options. Treatment depends on your symptoms and your type of long QT syndrome. You may need treatment even if you don’t often have symptoms. […] Some people with acquired long QT syndrome may get fluids or minerals, such as magnesium, through a needle in a vein. […] If medicine causes long QT syndrome (LQTS), stopping the medicine may be all that’s needed to treat the disorder. […] Some people with LQTS need medicines to treat symptoms and prevent life-threatening heart rhythm changes. […] Medicines used to treat long QT syndrome may include: […] Some people with long QT syndrome need surgery or a device to control the heartbeat. […] This surgery may be done if you have long QT syndrome and continuing heart rhythm changes but beta blockers don’t work for you. […] The decision to place an ICD, especially in children, needs to be carefully considered.

• #2 Long QT Syndrome – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK441860/

  The goal of management is the prevention of lethal arrhythmias such as torsade de pointes (TdP). […] The first-line treatment is magnesium sulfate, and the benefit is independent of serum magnesium level. Temporary transvenous overdrive pacing should be considered for those who do not respond to magnesium sulfate. Isoproterenol and Class IB antiarrhythmic drugs, such as lidocaine and phenytoin, may also be used. […] For long-term management in congenital Long QT syndrome, beta-blockers are the first line choice, and they help prevent ventricular arrhythmias by stabilizing ventricular action potential and helping block sympathetic surges associated with arrhythmias. An implantable cardioverter defibrillator (ICD) is recommended in patients with Long QT syndrome who were resuscitated from a cardiac arrest. It is also indicated in those who have beta-blocker-resistant symptoms or have contraindications to beta-blockers.

• #3 Long QT Syndrome: Symptoms & Treatment

  https://my.clevelandclinic.org/health/diseases/17183-long-q-t-syndrome-lqts

  Long QT syndrome treatments include medications, devices or surgery that help you manage symptoms and prevent sudden death. Treatments dont provide a cure, but can keep you safer from abnormal heart rhythms. […] Most people with long QT syndrome (even those without symptoms) take a beta-blocker such as nadolol. Other medications can shorten your QT interval or improve your electrolyte levels. Your provider will discuss which medications are best for you. […] Some people with long QT syndrome also need a device, such as: An implantable cardioverter defibrillator (ICD). People with a history of cardiac arrest or symptoms, even with beta-blocker therapy, may receive an ICD. This device detects life-threatening arrhythmias and automatically shocks your heart to prevent sudden death. […] Some people with long QT syndrome may need left cardiac sympathetic denervation (LCSD) or a sympathectomy operation. This minimally invasive procedure involves removing specific nerves in your sympathetic nervous system.

• #4 Long QT syndrome – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/symptoms-causes/syc-20352518

  Treatment for long QT syndrome includes lifestyle changes and medicines to prevent dangerous heartbeats. Sometimes a medical device or surgery is needed. […] Proper medical treatment and lifestyle changes can help prevent complications of long QT syndrome. […] With proper treatment, you can manage and prevent the dangerous heartbeats that can lead to LQTS complications.

• #5 Long QT syndrome

  https://www.nhs.uk/conditions/long-qt-syndrome/

  Treatment for long QT syndrome depends on the cause, your test results, your symptoms and if you’re at high risk of serious problems. […] You may be offered: […] medicines called beta blockers to help control your heart rhythm recommended for most people with long QT syndrome […] a small device called an implantable cardioverter defibrillator (ICD) that’s put under the skin in your chest, in a similar way to a pacemaker recommended if you’re at high risk of serious heart problems […] surgery to cut certain nerves that affect the heart only recommended in rare cases if other treatments are not suitable. […] You’ll also be given advice about lifestyle changes to make, and you’ll be monitored regularly to check the health of your heart. […] Sometimes long QT syndrome is caused by another health condition or medicine. In these cases, treating the condition or stopping the medicine can improve the symptoms.

• #6 Diagnosis, management and therapeutic strategies for congenital long QT syndrome | Heart

  https://heart.bmj.com/content/108/5/332

  Congenital long QT syndrome (LQTS) is characterised by heart rate corrected QT interval prolongation and life-threatening arrhythmias, leading to syncope and sudden death. […] Growing insights into the genetic background and pathophysiology of LQTS has led to the identification of genotype-phenotype relationships for the most common genetic subtypes, the recognition of genetic and non-genetic modifiers of phenotype, optimisation of risk stratification algorithms and the discovery of gene-specific therapies in LQTS. […] In this review, we summarise the current evidence available on the diagnosis, clinical management and therapeutic strategies in LQTS. […] The cornerstone of management of LQTS patients is -blocker therapy. […] Additional pharmacological therapy consists of blockers of the late sodium inward current (ie, mexiletine, flecainide and ranolazine).

• #7 Long QT syndrome – Wikipedia

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

  Management may include avoiding strenuous exercise, getting sufficient potassium in the diet, the use of beta blockers, or an implantable cardiac defibrillator. […] For people with LQTS who survive cardiac arrest and remain untreated, the risk of death within 15 years is greater than 50%. With proper treatment this decreases to less than 1% over 20 years. […] Those diagnosed with LQTS are usually advised to avoid drugs that can prolong the QT interval further or lower the threshold for TDP, lists of which can be found in public access online databases. […] Arrhythmia suppression involves the use of medications or surgical procedures that attack the underlying cause of the arrhythmias associated with LQTS. […] 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). […] In patients considered at high risk of life-threatening arrhythmic events, ICD implantation may be considered as a preventive step. […] One effective form of arrhythmia termination in individuals with LQTS is placement of an implantable cardioverter-defibrillator (ICD).

• #8 Long QT syndrome | Better Health Channel

  https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/long-qt-syndrome

  Treatments include: […] medications beta blockers are effective for 90 per cent of people with long QT syndrome. […] implantable cardioverter-defibrillators (ICDs) ICDs are devices placed inside the body to detect and correct abnormal heart rhythms. ICDs may be necessary for patients that do not respond to beta blocker therapy.

• #9 Long QT Syndrome | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/long-qt-syndrome

  Treatment for LQTS depends on which type of the condition your child has. […] LQT1 can usually be treated effectively with medications called beta blockers, which help lower the chances of heart problems like arrhythmias and sudden cardiac arrest. […] LQT2 can often be treated well with beta blocker medication, but keeping potassium levels normal can also help. […] Beta blocker medications are less effective in some children with this type of LQTS, and some may need to take additional medication. Some children with LQT3 may need an implantable cardioverter-defibrillator (ICD). […] It’s important that all children with LQTS have access to an AED (automated external defibrillator) at home, school and on the playing field. […] Staying hydrated with water and sports drinks containing electrolytes is very important before, during and after exercise. […] Children with LQTS will need to continue their medication and other treatment for the rest of their lives.

• #10 Long QT Syndrome: Practice Essentials, Background, Etiopathophysiology

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

  No treatment addresses the cause of LQTS. Antiadrenergic therapeutic measures (eg, use of beta-blockers, left cervicothoracic stellectomy) and device therapy (eg, use of pacemakers, implantable cardioverter-defibrillators) aim to decrease the risk and lethality of cardiac events. […] Beta-adrenergic blocking agents are the drugs of choice to treat LQTS and include the following medications: Nadolol, Propranolol, Metoprolol, Atenolol. […] Nadolol, however, is the preferred beta-blocker to be used at a dose of 1-1.5 mg/kg/day (once a day for patients older than 12 years; divided twice a day for younger patients). […] Surgical intervention in patients with LQTS may include the following procedures: Implantation of cardioverter-defibrillators, Placement of a pacemaker, Left cervicothoracic stellectomy.

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

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

  The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. For patients with LQT3, sodium channel blockers such as flecainide and mexiletine are recommended as adjunct therapies to potentially shorten the QT interval, with mexiletine considered a secondary treatment option. […] Treatment strategies tailored to specific genotypes, such as adding mexiletine for LQT3 and left cardiac sympathetic denervation (LCSD) for LQT1, are advised to align with genotype-phenotype correlations. […] Medical therapy for LQTS, particularly types LQT1 and LQT2, relies heavily on high-dose beta-blocker treatment, such as nadolol or propranolol, administered at 2-4 mg/kg/day. Beta-blockers are established as the cornerstone of treatment for these subtypes, effectively reducing syncope and SCD risks. […] Propranolol, known for its sodium channel-blocking properties independent of adrenergic pathways, proves especially beneficial for patients with LQT1 and LQT2 who experience cardiac events under adrenergic stress such as physical or emotional exertion.

• #12 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. […] If you’re taking any new medicine or supplements, always check with your pharmacist that what you’re taking is okay with LQTS. It’s important to drink enough after illness. Always tell medical staff you have LQTS.

• #13 Beta-Blocker Therapy for Long QT Syndrome and Catecholaminergic Polymorphic Ventricular Tachycardia: Are All Beta-Blockers Equivalent? – HRS

  https://www.hrsonline.org/guidance/clinical-resources/beta-blocker-therapy-long-qt-syndrome-and-catecholaminergic-polymorphic-ventricular-tachycardia-are

  Drug therapy with beta-blockers represents the therapeutic mainstay for both LQTS and CPVT. […] Nadolol, when available, has been the preferred beta-blocker utilized by the largest LQTS/CPVT specialty centers throughout the world for the past 25 years. […] More than 70% of respondents (n=109) use nadolol in at least 75% of their patients with LQTS. Thus, the potential lack of availability of nadolol would necessitate a major change in practice patterns.

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

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

  The role of ICD extends beyond the reduction of T-wave alternans (TWA) and peak amplitude, correlating with fewer instances of syncope and TdP. […] Furthermore, propranolol and nadolol surpass other beta-blockers such as metoprolol in preventing cardiac events, with particular efficacy in reducing cardiac repolarization time in patients with markedly prolonged QTc intervals, providing crucial insights for tailored LQTS management. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] While LCSD is effective, it is not considered curative and should be selected for patients after careful risk assessment. […] 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. […] Guidelines suggest ICD placement as a class II indication for LQTS patients experiencing life-threatening arrhythmias despite beta-blocker therapy, highlighting its importance in preventing sudden death among high-risk groups.

• #15 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  Beta Blockade […] Beta blockade should be initiated in those who have had symptoms, and those with a definite long QT interval, particularly in pre-adolescent boys, including infants. Overall reduction of risk of sudden cardiac death in high risk subjects is 67% in LQT 1 males and 71% in LQT 2 females. Beta blockers are also used in long QT 3 although the response may not be as good as in KLQT1 and 2 and additional treatment may be required. Once started, they should not be stopped; there is a period of high risk after stopping beta-blockers due to up-regulation of beta-receptors on treatment. […] Intracardiac Cardioverter-defibrillators (ICD) […] These devices are not without morbidity and patients must be selected carefully. AICDs are considered to be indicated for: […] 1. Resuscitated cardiac arrest,

• #16 Long QT Syndrome – SADS Foundation

  https://sads.org/sads-conditions/long-qt-syndrome/

  LQTS is a highly treatable disorder and, with correct diagnosis and common treatments, most deaths are preventable. […] If you’re symptomatic, you should receive treatment, and most children and young adults should be treated even if they do not have symptoms. […] The usual treatment involves taking beta-blocker medications daily (preferably nadolol or propranolol). This approach is effective for the majority of those with LQTS. It is very important that the medication be taken every day, and not be missed or omitted. […] If you continue to have symptoms despite appropriate doses of beta-blockers, or are not able to tolerate your beta-blocker therapy, you may need additional medications, surgery (left cardiac sympathetic denervation [LCSD]), or device therapy with an implantable cardioverter defibrillator (ICD). […] You should also avoid medications that prolong the QT interval.

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

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

  The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. For patients with LQT3, sodium channel blockers such as flecainide and mexiletine are recommended as adjunct therapies to potentially shorten the QT interval, with mexiletine considered a secondary treatment option. […] Treatment strategies tailored to specific genotypes, such as adding mexiletine for LQT3 and left cardiac sympathetic denervation (LCSD) for LQT1, are advised to align with genotype-phenotype correlations. […] Medical therapy for LQTS, particularly types LQT1 and LQT2, relies heavily on high-dose beta-blocker treatment, such as nadolol or propranolol, administered at 2-4 mg/kg/day. Beta-blockers are established as the cornerstone of treatment for these subtypes, effectively reducing syncope and SCD risks. […] Propranolol, known for its sodium channel-blocking properties independent of adrenergic pathways, proves especially beneficial for patients with LQT1 and LQT2 who experience cardiac events under adrenergic stress such as physical or emotional exertion.

• #18 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

  Long QT syndrome (LQTS) is an uncommon disorder that is characterized by QT prolongation and torsade de pointes leading to sudden cardiac death. […] The updated 2022 European Society of Cardiology (ESC) guidelines aim to define the diagnosis of LQTS and spread its management. […] The guidelines state that β-blockers are recommended as a stand-alone therapy in all LQTS patients regardless of the heart rate. […] Non-selective β-blockers nadolol and propranolol have better efficacy in inhibiting adrenergic activation to reduce arrhythmic risk for inherited LQTS. […] Currently, mexiletine is the only promising alternative to β-blockers for all LQTS patients with markedly prolonged QTc intervals at a slow heart rate. […] These guidelines recommend that mexiletine is only to be given to LQT3 patients with a QTc prolongation.

• #19 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. During a diarrhoea and vomiting illness, affected individuals should keep well hydrated using oral rehydration therapy (such as Dioralyte). 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). Mexiletine is indicated for patients with LQTS type 3 and QT prolongation. Flecainide and/or acetazolamide should be considered in patients with LQTS type 7. 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.

• #20 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. During a diarrhoea and vomiting illness, affected individuals should keep well hydrated using oral rehydration therapy (such as Dioralyte). 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). Mexiletine is indicated for patients with LQTS type 3 and QT prolongation. Flecainide and/or acetazolamide should be considered in patients with LQTS type 7. 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.

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

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

  To date, the safe and effective management of patients with LQTS often necessitates the implantation of an ICD. While ICD therapy is currently recommended for primary prevention of sudden death, its placement may not always be practical or technically feasible. High-risk LQTS patients and those who have experienced cardiac arrest or multiple arrhythmic syncopal episodes should undergo aggressive management with ICD placement, often alongside medical therapy (beta-blockers) to mitigate future episodes, though there are risks of inappropriate treatment due to oversensing of T-waves. […] 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.

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

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

  The role of ICD extends beyond the reduction of T-wave alternans (TWA) and peak amplitude, correlating with fewer instances of syncope and TdP. […] Furthermore, propranolol and nadolol surpass other beta-blockers such as metoprolol in preventing cardiac events, with particular efficacy in reducing cardiac repolarization time in patients with markedly prolonged QTc intervals, providing crucial insights for tailored LQTS management. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] While LCSD is effective, it is not considered curative and should be selected for patients after careful risk assessment. […] 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. […] Guidelines suggest ICD placement as a class II indication for LQTS patients experiencing life-threatening arrhythmias despite beta-blocker therapy, highlighting its importance in preventing sudden death among high-risk groups.

• #23 Long QT Syndrome | Cedars-Sinai

  https://www.cedars-sinai.org/health-library/diseases-and-conditions/l/long-qt-syndrome.html

  For acquired LQTS, correcting any electrolyte problems or changing medicines may be all that’s needed. There is no cure for inherited LQTS. But treatment helps prevent symptoms and lowers the risk for fainting or cardiac arrest. Your healthcare provider may prescribe medicines called beta blockers to reduce arrhythmias. If you continue to have arrhythmias that keep coming back, or have had sudden cardiac arrest, you may need an implantable cardiac defibrillator (ICD). This device is implanted under the skin in the front of the chest wall or on the left side of your chest below the armpit. A wire connects the device to the heart. The ICD keeps track of your heart rhythm. If needed, it can deliver therapy, such as ATP (anti-tachycardia pacing). Or one or more brief electrical shocks to the heart to reset the rhythm. […] Surgery is another choice for some people with inherited LQTS. During surgery, certain nerves in your chest that influence the heart rhythm are removed.

• #24 Long QT syndrome – Diagnosis and treatment – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/long-qt-syndrome/diagnosis-treatment/drc-20352524

  Treatment for long QT syndrome (LQTS) may include: […] The goals of LQTS treatment are to: […] Your healthcare professional talks with you about your treatment options. Treatment depends on your symptoms and your type of long QT syndrome. You may need treatment even if you don’t often have symptoms. […] Some people with acquired long QT syndrome may get fluids or minerals, such as magnesium, through a needle in a vein. […] If medicine causes long QT syndrome (LQTS), stopping the medicine may be all that’s needed to treat the disorder. […] Some people with LQTS need medicines to treat symptoms and prevent life-threatening heart rhythm changes. […] Medicines used to treat long QT syndrome may include: […] Some people with long QT syndrome need surgery or a device to control the heartbeat. […] This surgery may be done if you have long QT syndrome and continuing heart rhythm changes but beta blockers don’t work for you. […] The decision to place an ICD, especially in children, needs to be carefully considered.

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

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

  A longer QT interval increases the risk of life threatening arrhythmias. Treatment aims to shorten these intervals. Treatment may include medications, devices, or surgery to improve electrolyte levels and shorten a persons QT interval. […] Most people with LQTS, even those without symptoms, require medications like beta-blockers. However, some medications may prolong the Qtc intervals. If this happens, discontinuation of the medication under the guidance of a healthcare professional is important. […] People with frequent or severe symptoms may require extra help to manage the rhythm of the heart. Doctors implant the device under the skin of the chest and have wires pass down into the heart to monitor the rhythm. […] An implantable automatic cardioverter-defibrillator (ICD) detects life threatening arrhythmias and sends a shock to the heart to prevent sudden death. Doctors recommend this to high risk individuals, including those previously resuscitated from cardiac arrest.

• #26 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  2. Persistent arrhythmic syncope whilst on beta blockers, […] 3. When beta blockers are contra-indicated and high risk is established. […] A relative indication is the presence of a very long QT interval (QTc>0.55sec) even without symptomatology, particularly adult females and males with LQT 3. Unless inserted for contraindication to beta-blockers, it is important that beta-blockers are continued because of the risk that a defibrillation shock may cause an adrenergic surge and precipitate a further event or electrical storm. […] Left cervical sympathectomy […] Minimally invasive selective left cardiac sympathectomy may be considered for: […] 1. Those with severe disease and in whom beta blockers are contra-indicated or AICD cannot be placed or is refused by the patient. […] 2. Controlling VT storms in those with an AICD,

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

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

  The role of ICD extends beyond the reduction of T-wave alternans (TWA) and peak amplitude, correlating with fewer instances of syncope and TdP. […] Furthermore, propranolol and nadolol surpass other beta-blockers such as metoprolol in preventing cardiac events, with particular efficacy in reducing cardiac repolarization time in patients with markedly prolonged QTc intervals, providing crucial insights for tailored LQTS management. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] While LCSD is effective, it is not considered curative and should be selected for patients after careful risk assessment. […] 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. […] Guidelines suggest ICD placement as a class II indication for LQTS patients experiencing life-threatening arrhythmias despite beta-blocker therapy, highlighting its importance in preventing sudden death among high-risk groups.

• #28 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  3. High risk LQT3 or a personal or family history of events during rest or sleep. […] Some individuals at intermediate risk may choose this option rather than take beta blockers, and it may be considered as a primary prevention, particularly boys with LQT1. Those at high risk should continue beta blockers after the sympathectomy when feasible. […] Asymptomatic Family Members […] All patients with LQTS should avoid medications contra-indicated in LQTS. A list of these drugs can be found on the net under ‘LQT drugs’ Those with a long QT interval (>500ms), especially young males and adult females need to be treated much as someone who has already presented with syncope. Beta blockers should be proposed and sensible limitations placed on sporting activities and particularly swimming. The role of beta blockers in those without symptoms, a normal QT interval and yet a positive genetic diagnosis is controversial, since evidence is not yet strong to establish a reduction in risk. Those with a family history of adrenergic induced cardiac events, or known to have LQT1, are most likely to benefit. It should be remembered that intermittent adherence to beta blocker therapy may carry its own risk; when the medication is stopped, the up-regulated beta-receptors may lead to an increased risk for a few days after stopping.

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

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

  The role of ICD extends beyond the reduction of T-wave alternans (TWA) and peak amplitude, correlating with fewer instances of syncope and TdP. […] Furthermore, propranolol and nadolol surpass other beta-blockers such as metoprolol in preventing cardiac events, with particular efficacy in reducing cardiac repolarization time in patients with markedly prolonged QTc intervals, providing crucial insights for tailored LQTS management. […] Left cardiac sympathetic denervation has become an important treatment strategy for preventing SCD in LQTS, particularly before considering an implantable ICD in younger patients. […] While LCSD is effective, it is not considered curative and should be selected for patients after careful risk assessment. […] 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. […] Guidelines suggest ICD placement as a class II indication for LQTS patients experiencing life-threatening arrhythmias despite beta-blocker therapy, highlighting its importance in preventing sudden death among high-risk groups.

• #30 Diagnosis, management and therapeutic strategies for congenital long QT syndrome | Heart

  https://heart.bmj.com/content/108/5/332

  New pharmacological therapy is emerging, but the target population becomes more and more specific. […] The next level of therapy is left cardiac sympathetic denervation (LCSD). […] Device therapy is the final level of therapy in LQT patients with -blocker and/or LCSD breakthrough cardiac events or in patients with an anticipated high risk for arrhythmic events. […] Pacemaker therapy has not extensively been tested in LQTS but may be beneficial in particular in LQT2 patients where arrhythmias are almost exclusively pause dependent. […] Therapy relies importantly on -blockers and lifestyle measures. […] More patient specific pharmacological and invasive therapies are available and under continuous development.

• #31 Long QT Interval Syndromes – Cardiovascular Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/cardiovascular-disorders/arrhythmogenic-cardiac-disorders/long-qt-interval-syndromes

  Treatment of any VT/VF […] Alleviation of predisposing causes and triggers, especially electrolyte abnormalities and use of certain medications […] Usually beta-blockade […] Sometimes mexiletine […] Sometimes an ICD […] Sometimes left cardiac sympathetic denervation […] Long-term treatment to prevent sudden death includes avoidance of specific triggers (including strenuous exercise in LQTS1 and LQTS2) and QTc-prolonging conditions. […] Beta-blocker therapy is recommended for most patients who have LQTS with QT interval prolongation or who are symptomatic (most effective with LQTS1 or LQTS2). […] When beta-blocker therapy is ineffective at relieving symptoms, mexiletine is added (most effective in LQTS3). […] Permanent pacing to increase the basal ventricular rate and to prevent post-extrasystolic pauses may reduce the probability of recurrent TdeP VT. […] An implantable cardioverter-defibrillator (ICD) is indicated in patients who have been resuscitated after cardiac arrest and in those who have cardiac syncope despite therapy with a beta-blocker. […] Left heart (stellate ganglion) denervation may also be used.

• #32 Long QT Syndrome: Symptoms & Treatment | Mass General Brigham

  https://www.massgeneralbrigham.org/en/patient-care/services-and-specialties/heart/conditions/long-qt-syndrome

  Treatment for acquired long QT syndrome focuses on addressing the underlying cause or stopping the offending agent. […] For congenital long QT syndrome, the common treatment is a beta-blocker, which helps shorten the QT interval and reduce the risk of dangerous arrhythmias like Torsades de Pointes. […] In cases where medication isn’t enough, you may need surgery to either implant a pacemaker or defibrillator to regulate the heart rhythm or remove specific nerves that trigger arrhythmias. […] Acquired long QT syndrome can often be cured by treating the underlying issue, such as correcting electrolyte imbalances or stopping medications that prolong the QT interval. […] Inherited long QT syndrome, however, can’t be cured, but treatment can effectively reduce the risk of life-threatening arrhythmias.

• #33 Long QT Syndrome (QT Prolongation): Causes, Symptoms, Treatment

  https://www.webmd.com/heart-disease/long-qt-syndrome-overview

  Treatment depends on the LQTS type and how severe it is. […] For inherited LQTS, your symptoms could be relieved by something as simple as avoiding intense exercise. More severe LQTS might mean you need to take medications called beta-blockers or antiarrhythmic drugs to control your hearts rhythm. You and your doctor may also consider inserting a pacemaker or implantable cardioverter defibrillator (ICD) that keeps your heart rhythm steady. […] For acquired LQTS, you’ll need to stop taking any medications that trigger it and treat related conditions or shortfalls of any electrolytes. As with inherited LQTS, you might also take antiarrhythmic medications or need a pacemaker or ICD.

• #34 Long QT Syndrome: Symptoms & Treatment | Mass General Brigham

  https://www.massgeneralbrigham.org/en/patient-care/services-and-specialties/heart/conditions/long-qt-syndrome

  Treatment for acquired long QT syndrome focuses on addressing the underlying cause or stopping the offending agent. […] For congenital long QT syndrome, the common treatment is a beta-blocker, which helps shorten the QT interval and reduce the risk of dangerous arrhythmias like Torsades de Pointes. […] In cases where medication isn’t enough, you may need surgery to either implant a pacemaker or defibrillator to regulate the heart rhythm or remove specific nerves that trigger arrhythmias. […] Acquired long QT syndrome can often be cured by treating the underlying issue, such as correcting electrolyte imbalances or stopping medications that prolong the QT interval. […] Inherited long QT syndrome, however, can’t be cured, but treatment can effectively reduce the risk of life-threatening arrhythmias.

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

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

  The management of LQTS emphasizes the use of beta-blockers as the primary treatment option. For patients with LQT3, sodium channel blockers such as flecainide and mexiletine are recommended as adjunct therapies to potentially shorten the QT interval, with mexiletine considered a secondary treatment option. […] Treatment strategies tailored to specific genotypes, such as adding mexiletine for LQT3 and left cardiac sympathetic denervation (LCSD) for LQT1, are advised to align with genotype-phenotype correlations. […] Medical therapy for LQTS, particularly types LQT1 and LQT2, relies heavily on high-dose beta-blocker treatment, such as nadolol or propranolol, administered at 2-4 mg/kg/day. Beta-blockers are established as the cornerstone of treatment for these subtypes, effectively reducing syncope and SCD risks. […] Propranolol, known for its sodium channel-blocking properties independent of adrenergic pathways, proves especially beneficial for patients with LQT1 and LQT2 who experience cardiac events under adrenergic stress such as physical or emotional exertion.

• #36 Long QT Syndrome | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/long-qt-syndrome

  Treatment for LQTS depends on which type of the condition your child has. […] LQT1 can usually be treated effectively with medications called beta blockers, which help lower the chances of heart problems like arrhythmias and sudden cardiac arrest. […] LQT2 can often be treated well with beta blocker medication, but keeping potassium levels normal can also help. […] Beta blocker medications are less effective in some children with this type of LQTS, and some may need to take additional medication. Some children with LQT3 may need an implantable cardioverter-defibrillator (ICD). […] It’s important that all children with LQTS have access to an AED (automated external defibrillator) at home, school and on the playing field. […] Staying hydrated with water and sports drinks containing electrolytes is very important before, during and after exercise. […] Children with LQTS will need to continue their medication and other treatment for the rest of their lives.

• #37 Long QT Syndrome: Practice Essentials, Background, Etiopathophysiology

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

  Patients with LQTS should avoid participation in competitive sports, strenuous exercise, and stress-related emotions. […] These individuals should also avoid the following agents: Anesthetics or asthma medication (eg, epinephrine), Antihistamines (eg, diphenhydramine; terfenadine and astemizole [both recalled from the US market]), Antibiotics (eg, erythromycin, trimethoprim and sulfamethoxazole, pentamidine), Cardiac medications (eg, quinidine, procainamide, disopyramide, sotalol, probucol, bepridil, dofetilide, ibutilide), Gastrointestinal medications (eg, cisapride), Antifungal medications (eg, ketoconazole, fluconazole, itraconazole), Psychotropic medications (eg, tricyclic antidepressants, phenothiazine derivatives, butyrophenones, benzisoxazole, diphenylbutylpiperidine), Potassium-losing medications (eg, indapamide, other diuretics; medications for vomiting/diarrhea).

• #38 Congenital long QT syndrome: Treatment – UpToDate

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

  Congenital long QT syndrome: Treatment […] The treatment of congenital LQTS will be reviewed here. […] 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.

• #39 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  Management of Long QT Syndrome […] Removal of triggers […] All gene carriers must avoid medications which prolong the QT interval, can cause torsade de pointes or lower serum potassium levels. A constantly updated list is available at www.qtdrugs.org. […] Assessing level of risk of sudden cardiac death […] Management must be guided by an assessment of risk. Data from the international long QT registry show that the most important features are: […] 1. Length of the QT interval on serial resting ECGs; […] 2. History of arrhythmic syncope or cardiac arrest; and […] 3. Gender. […] Lifestyle modifications […] With all forms of LQTS, where there is a long QT interval (and not necessarily just gene carriage), some degree of limitation in sporting activity is recommended. The limitation needs to be more severe with LQT1, or those who have already experienced events during exercise, than LQT2 and 3. They should not become professional athletes, and all highly competitive sports are to be discouraged. With LQT 1, and subjects with a history of exercise induced syncope, swimming and diving are contraindicated. With LQT 2, or those with a history of auditory evoked events, remove loud alarm clocks and turn down the volume on the phone at night.

• #40 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. […] If you’re taking any new medicine or supplements, always check with your pharmacist that what you’re taking is okay with LQTS. It’s important to drink enough after illness. Always tell medical staff you have LQTS.

• #41 Long QT Syndrome | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/long-qt-syndrome

  Treatment for LQTS depends on which type of the condition your child has. […] LQT1 can usually be treated effectively with medications called beta blockers, which help lower the chances of heart problems like arrhythmias and sudden cardiac arrest. […] LQT2 can often be treated well with beta blocker medication, but keeping potassium levels normal can also help. […] Beta blocker medications are less effective in some children with this type of LQTS, and some may need to take additional medication. Some children with LQT3 may need an implantable cardioverter-defibrillator (ICD). […] It’s important that all children with LQTS have access to an AED (automated external defibrillator) at home, school and on the playing field. […] Staying hydrated with water and sports drinks containing electrolytes is very important before, during and after exercise. […] Children with LQTS will need to continue their medication and other treatment for the rest of their lives.

• #42 Get Treatment for Long QT Syndrome in Children | Cleveland Clinic Children’s

  https://my.clevelandclinic.org/pediatrics/services/long-qt-syndrome-treatment

  Your child’s pediatric provider may recommend a cardiac device to help regulate their heart rhythm. We implant a full range of pacemakers, implantable cardioverter defibrillators (ICDs) and loop recorders and do more than 50,000 device checks a year in person and remotely. Your child’s care team will go over cardiac device options with you. […] We only rarely treat LQTS with surgery. In most cases, we do it when the condition hasnt responded to other treatments. Left cardiac sympathetic denervation (LCSD) surgery, or sympathectomy, detaches the nerves that cause dangerous heart rhythms and helps your childs heart keep a steady pace. […] Facing a chronic (lifelong) heart condition like long QT syndrome can affect every part of your childs life. Theyll need to commit to regular care and management, so its important to have the right team by their side. Cleveland Clinic Childrens has providers dedicated to treating heart conditions in children like LQTS. Were here to support your child, and you, through this journey and find ways to reduce their symptoms and risk of complications. We want you and your child to feel confident about moving forward in everyday life.

• #43 Long QT Syndrome | Children’s Hospital of Philadelphia

  https://www.chop.edu/conditions-diseases/long-qt-syndrome

  Treatment for LQTS depends on which type of the condition your child has. […] LQT1 can usually be treated effectively with medications called beta blockers, which help lower the chances of heart problems like arrhythmias and sudden cardiac arrest. […] LQT2 can often be treated well with beta blocker medication, but keeping potassium levels normal can also help. […] Beta blocker medications are less effective in some children with this type of LQTS, and some may need to take additional medication. Some children with LQT3 may need an implantable cardioverter-defibrillator (ICD). […] It’s important that all children with LQTS have access to an AED (automated external defibrillator) at home, school and on the playing field. […] Staying hydrated with water and sports drinks containing electrolytes is very important before, during and after exercise. […] Children with LQTS will need to continue their medication and other treatment for the rest of their lives.

• #44 Long QT Syndrome (LQTS) – Seattle Children’s

  https://www.seattlechildrens.org/conditions/long-qt-syndrome/

  The goal of treatment for LQTS is to prevent your childs heart from having dangerous arrhythmias. Treatment does not cure LQTS. […] LQTS can be treated with medicines, like beta-blockers. These slow the heart rate and decrease the chance of having dangerous arrhythmias and sudden cardiac arrest. […] Some children with LQTS need a pacemaker to help control the speed and pattern of their heartbeat or a defibrillator to bring the heart back to a normal rhythm. […] Your childs doctor may also want your child to avoid things that might trigger an arrhythmia. Common triggers include: strenuous exercise, certain medicines, conditions that can lower your childs level of the mineral potassium (like diarrhea and vomiting), loud noises that might startle them during sleep. […] We offer a full range of diagnostic procedures and treatments. Along with using heart medicines, we have deep experience implanting a pacemaker or defibrillator in children who need these devices to correct arrhythmias. […] Doctors at Seattle Childrens are working to advance treatment for long QT syndrome and enhance the quality of life for children with this condition.

• #45 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  3. High risk LQT3 or a personal or family history of events during rest or sleep. […] Some individuals at intermediate risk may choose this option rather than take beta blockers, and it may be considered as a primary prevention, particularly boys with LQT1. Those at high risk should continue beta blockers after the sympathectomy when feasible. […] Asymptomatic Family Members […] All patients with LQTS should avoid medications contra-indicated in LQTS. A list of these drugs can be found on the net under ‘LQT drugs’ Those with a long QT interval (>500ms), especially young males and adult females need to be treated much as someone who has already presented with syncope. Beta blockers should be proposed and sensible limitations placed on sporting activities and particularly swimming. The role of beta blockers in those without symptoms, a normal QT interval and yet a positive genetic diagnosis is controversial, since evidence is not yet strong to establish a reduction in risk. Those with a family history of adrenergic induced cardiac events, or known to have LQT1, are most likely to benefit. It should be remembered that intermittent adherence to beta blocker therapy may carry its own risk; when the medication is stopped, the up-regulated beta-receptors may lead to an increased risk for a few days after stopping.

• #46 Long QT Syndrome – Melbourne Heart Rhythm

  https://www.melbourneheartrhythm.com.au/learn/conditions/53-long-qt-syndrome

  3. High risk LQT3 or a personal or family history of events during rest or sleep. […] Some individuals at intermediate risk may choose this option rather than take beta blockers, and it may be considered as a primary prevention, particularly boys with LQT1. Those at high risk should continue beta blockers after the sympathectomy when feasible. […] Asymptomatic Family Members […] All patients with LQTS should avoid medications contra-indicated in LQTS. A list of these drugs can be found on the net under ‘LQT drugs’ Those with a long QT interval (>500ms), especially young males and adult females need to be treated much as someone who has already presented with syncope. Beta blockers should be proposed and sensible limitations placed on sporting activities and particularly swimming. The role of beta blockers in those without symptoms, a normal QT interval and yet a positive genetic diagnosis is controversial, since evidence is not yet strong to establish a reduction in risk. Those with a family history of adrenergic induced cardiac events, or known to have LQT1, are most likely to benefit. It should be remembered that intermittent adherence to beta blocker therapy may carry its own risk; when the medication is stopped, the up-regulated beta-receptors may lead to an increased risk for a few days after stopping.

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

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

  An alternative treatment is beta blockade in combination with pacemaker implantation and/or stellectomy in some patients. […] Left cervicothoracic stellectomy is another antiadrenergic therapeutic measure used in high-risk patients with long QT syndrome (LQTS), especially in those with recurrent cardiac events despite beta-blocker therapy. […] 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. […] Gene-specific therapy is an area under investigation in the treatment of long QT syndrome (LQTS).

• #48 Sudden Arrhythmia Death Syndrome: Importance of the Long QT Syndrome | AAFP

  https://www.aafp.org/pubs/afp/issues/2003/0801/p483.html

  Beta blockers, potassium supplements, and implantable defibrillators have been used for treatment of long QT syndrome. […] Different ion channel gene mutations are targeted with different medications. Beta blockers and potassium supplements are among the drugs used to treat long QT syndrome. Permanent pacemakers or automatic implantable defibrillators also have been employed. […] The goal of therapy is the resolution of symptoms and, in many cases, abnormalities on ECG normalize after treatment. […] Measures to prevent long QT syndrome include avoiding hypokalemia and medications that lengthen the QT interval. […] The future holds promise of mutation-specific pharmacology. Thus, identification of the type of mutation could be useful in determining optimal therapy and may become more generally applicable when analysis of the long QT syndrome gene moves from the research laboratory into community medicine.

• #49 Long QT syndrome – Wikipedia

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

  Management may include avoiding strenuous exercise, getting sufficient potassium in the diet, the use of beta blockers, or an implantable cardiac defibrillator. […] For people with LQTS who survive cardiac arrest and remain untreated, the risk of death within 15 years is greater than 50%. With proper treatment this decreases to less than 1% over 20 years. […] Those diagnosed with LQTS are usually advised to avoid drugs that can prolong the QT interval further or lower the threshold for TDP, lists of which can be found in public access online databases. […] Arrhythmia suppression involves the use of medications or surgical procedures that attack the underlying cause of the arrhythmias associated with LQTS. […] 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). […] In patients considered at high risk of life-threatening arrhythmic events, ICD implantation may be considered as a preventive step. […] One effective form of arrhythmia termination in individuals with LQTS is placement of an implantable cardioverter-defibrillator (ICD).

• #50

  https://smw.ch/index.php/smw/article/view/1757

  The long QT syndrome (LQTS) is a leading cause of sudden death in the young. […] Extremely effective therapies are available, which makes the existence of undiagnosed affected and symptomatic patients inexcusable. […] Indeed, mortality for properly treated patients has now declined to around 1% over a 10-year period. […] One special point of interest is in the impact of genetics on clinical management and the potential medicolegal consequences of not pursuing genetic screening in the proband and hence in the family members. […] Not all beta-blockers are equal in the management of long QT syndrome types 1 and 2: Higher recurrence of events under metoprolol. […] Effectiveness and limitations of beta-blocker therapy in congenital long-QT syndrome. […] Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers.

• #51 Long QT Syndrome: Symptoms & Treatment | Mass General Brigham

  https://www.massgeneralbrigham.org/en/patient-care/services-and-specialties/heart/conditions/long-qt-syndrome

  With the right treatment and lifestyle adjustments, most people with LQTS can live a mostly normal life. It’s important to follow a doctor-approved exercise routine, avoid certain medications and supplements that prolong the QT interval, and minimize startling situations that can trigger a cardiac event. Regular medical follow-ups and proactive management can help you stay safe while leading an active life.

Zobacz więcej