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| __NOTOC__ | | __NOTOC__ |
| {{Long QT Syndrome}} | | {{Long QT Syndrome}} |
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| {{CMG}}
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| ==Overview== | | ==Overview== |
| Beta-blockers are first line treatment in LQTs along with electrolyte repletion, and avoidance of triggers (drugs, supplements, loud noises). LQTs is one of the few diseases where genetic testing actually can provide important guidance such as who to put a [[AICD]] (defibrillator) in for primary prevention. <ref>Compton SJ, Lux RL, Ramsey MR, Strelich KR, Sanguinetti MC, Green LS, Keating MT, Mason JW. Genetically defined therapy of inherited long-QT syndrome. Correction of abnormal repolarization by potassium. Circulation. 1996 Sep 1;94(5):1018-22. PMID 8790040</ref> Left [[Stellate ganglion|stellectomy]] is not a cure, but is second line therapy to reduce the risk of [[sudden cardiac death]] and is indicated if the patient does not tolerate [[beta blockers]] or breaks through [[beta blockers]], as well as in young patients under the age of 12 where [[beta blockers]] are not deemed protective enough and where the morbidity of an [[AICD]] seems excessive. Patients with [[Long QT syndrome]] should undergo secondary prevention with [[AICD]] implantation for secondary prevention if they sustain an aborted [[cardiac arrest]] or [[sudden cardiac death]].
| | Surgical intervention is not recommended for the management of [disease name]. |
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| ==Primary Prevention==
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| ===Withdrawal of Drugs and Supplements===
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| Certain medications should be avoided in persons with long QT syndrome, to avoid worsening the condition. These medications include certain [[appetite suppressants]], [[decongestants]], and [[antibiotics]] such as [[erythromycin]]. Illicit drugs such as [[cocaine]] and [[amphetamines]] can be even more dangerous in persons with long QT syndrome.
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| ====List of Medications to be Avoided in Congenital Long QT Syndrome====
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| *[[Albuterol]]
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| *[[Alfuzosin]]
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| *[[Amantadine]]
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| *[[Amiodarone]]
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| *[[Amisulpride]]
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| *[[Amitriptyline]]
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| *[[Amphetamine]]
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| *[[Arsenic trioxide]]
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| *[[Artenimol]] + [[piperaquine]]
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| *[[Astemizole]]
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| *[[Atazanavir]]
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| *[[Atomoxetine]]
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| *[[Azithromycin]]
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| *[[Bepridil]]
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| *[[Chloral hydrate]]
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| *[[Chloroquine]]
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| *[[Chlorpromazine]]
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| *[[Ciprofloxacin]]
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| *[[Cisapride]]
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| *[[Citalopram]]
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| *[[Clarithromycin]]
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| *[[Clomipramine]]
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| *[[Clozapine]]
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| *[[Cocaine]]
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| *[[Desipramine]]
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| *[[Dexmethylphenidate]]
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| *[[Diphenhydramine]]
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| *[[Diphenhydramine]]
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| *[[Disopyramide]]
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| *[[Dobutamine]]
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| *[[Dofetilide]]
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| *[[Dolasetron]]
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| *[[Domperidone]]
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| *[[Dopamine]]
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| *[[Doxepin]]
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| *[[Dronedarone]]
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| *[[Droperidol]]
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| *[[Ephedrine]]
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| *[[Epinephrine]]
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| *[[Eribulin]]
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| *[[Erythromycin]]
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| *[[Escitalopram]]
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| *[[Famotidine]]
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| *[[Fenfluramine]]
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| *[[Fingolimod]]
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| *[[Flecainide]]
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| *[[Fluconazole]]
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| *[[Fluoxetine]]
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| *[[Foscarnet]]
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| *[[Fosphenytoin]]
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| *[[Galantamine]]
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| *[[Gatifloxacin]]
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| *[[Gemifloxacin]]
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| *[[Granisetron]]
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| *[[Halofantrine]]
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| *[[Haloperidol]]
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| *[[Ibutilide]]
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| *[[Iloperidone]]
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| *[[Imipramine]]
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| *[[Indapamide]]
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| *[[Isoproterenol]]
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| *[[Isradipine]]
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| *[[Itraconazole]]
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| *[[Ketoconazole]]
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| *[[Lapatinib]]
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| *[[Levalbuterol]]
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| *[[Levofloxacin]]
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| *[[Levomethadyl]]
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| *[[Lisdexamfetamine]]
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| *[[Lithium]]
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| *[[Mesoridazine]]
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| *[[Metaproterenol]]
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| *[[Methadone]]
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| *[[Methylphenidate]]
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| *[[Midodrine]]
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| *[[Mirtazapine]]
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| *[[Moexipril]] / [[HCTZ]]
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| *[[Moxifloxacin]]
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| *[[Nicardipine]]
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| *[[Nilotinib]]
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| *[[Norepinephrine]]
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| *[[Nortriptyline]]
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| *[[Octreotide]]
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| *[[Ofloxacin]]
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| *[[Ondansetron]]
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| *[[Oxytocin]]
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| *[[Paliperidone]]
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| *[[Paroxetine]]
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| *[[Pentamidine]]
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| *[[Perflutren]] lipid microspheres
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| *[[Phentermine]]
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| *[[Phenylephrine]]
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| *[[Phenylpropanolamine]]
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| *[[Pimozide]]
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| *[[Probucol]]
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| *[[Procainamide]]
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| *[[Protriptyline]]
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| *[[Pseudoephedrine]]
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| *[[Quetiapine]]
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| *[[Quinidine]]
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| *[[Ranolazine]]
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| *[[Ritodrine]]
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| *[[Ritonavir]]
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| *[[Roxithromycin]]
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| *[[Salmeterol]]
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| *[[Sertindole]]
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| *[[Sertraline]]
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| *[[Sevoflurane]]
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| *[[Sibutramine]]
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| *[[Solifenacin]]
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| *[[Sotalol]]
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| *[[Sparfloxacin]]
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| *[[Sunitinib]]
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| *[[Tacrolimus]]
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| *[[Tamoxifen]]
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| *[[Telithromycin]]
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| *[[Terbutaline]]
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| *[[Terfenadine]]
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| *[[Thioridazine]]
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| *[[Tizanidine]]
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| *[[Tolterodine]]
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| *[[Trazodone]]
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| *[[Trimethoprim-Sulfamethoxazole]]
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| *[[Trimipramine]]
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| *[[Vandetanib]]
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| *[[Vardenafil]]
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| *[[Venlafaxine]]
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| *[[Voriconazole]]
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| *[[Ziprasidone]]
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| ===Correct Electrolyte Disturbances===
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| Illness that cause [[hypokalemia]] due to [[vomiting]] and [[diarrhea]] can aggravate long QT syndrome. Medications that can lower the levels of [[potassium]] in the blood should also be avoided.
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| ===Postassium Administration===
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| The use of potassium supplementation is experimental and is not evidence based. The hypothesis is that ff the potassium content in the blood rises, the [[action potential]] shortens and it is for this reason that increasing potassium concentration may minimize the occurrence of arrhythmias. It should work best in [[LQT2]] since the HERG channel is especially sensible to potassium concentration, but potassium supplementation is experimental and not evidence based.
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| ===Beta Blockers===
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| Beta blockers are first line therapy in the treatment of Long QT syndrome.
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| 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 after depolarizations, and these after depolarizations are increased in states of adrenergic stimulation, steps can be taken to blunt adrenergic stimulation in these individuals. [[Beta blocker|beta receptor blocking agents]] decrease the risk of stress or catecholamine induced arrhythmias. [[Nadolol]] and [[propranolol]] are recommended, and caution should be used with [[atenolol]].
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| ====Nadolol====
| | OR |
| Nadolol at a dose of 1.0 to 1.5 mg/kg/day or 50 mg/m2/day QD or BID is the dose
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| ====Propranolol====
| | Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either [indication 1], [indication 2], and [indication 3] |
| 3-4 mg/kg/day BID for the long acting form and TID for the liquid. Often preferred in LQT3.
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| ===Mexiletine===
| | OR |
| [[Mexiletine]] is a [[sodium channel]] blocker. In [[LQT3]] the problem is that the sodium channel does not close properly. Mexiletine closes these channels and is believed to be potentially of use when other therapies fail. It should be especially effective in LQT3 but there is limited evidence to support this recommendation.
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| ===AICD Implantation===
| | The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either [indication 1], [indication 2], and/or [indication 3]. |
| Genotype and QT interval duration are independent predictors of recurrence of life-threatening events during beta-blockers therapy. Specifically the presence of QTc >500ms and [[LQT2]] and [[LQT3]] genotype are associated with the highest incidence of recurrence. In these patients primary prevention with ICD (Implantable Cardioverster Defibrilator) implantaion can be considered.<ref>Priori SG, Napolitano C, Schwartz PJ, Grillo M, Bloise R, Ronchetti E, Moncalvo C, Tulipani C, Veia A, Bottelli G, Nastoli J. Association of long QT syndrome loci and cardiac events among patients treated
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| with beta-blockers. JAMA. 2004 Sep 15;292(11):1341-4.[http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=pubmed&cmd=Retrieve&dopt=AbstractPlus&list_uids=15367556&query_hl=26&itool=pubmed_docsumPMID: 15367556]</ref>
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| An [[AICD]] should be implanted if:
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| *The QTc is > 550 ms and if it is not [[LQT1]]
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| *[[LQT2]] in women and the QTc is > 500 ms, with or without symptoms
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| *In infants with 2:1 [[AV block]] (controversial)
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| *In [[JLNS]] (LQTS with deafness) given its malignant propensity (controversial)
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| ===Sympathetic Denervation===
| | The feasibility of surgery depends on the stage of [malignancy] at diagnosis. |
| Videoscopic Left Cardiac Sympathetic Denervation Surgery (left [[Stellate ganglion|stellectomy]]) is not a cure, but reduces the risk of [[sudden cardiac death]] and is indicated if:
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| *The patient does not tolerate [[beta blockers]] or breaks through [[beta blockers]]
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| *The patient [[faints]] while on [[beta blockers]]
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| *There is a history of [[VF]] terminating [[AICD]] shocks
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| *In young patients under the age of 12 where [[beta blockers]] are not deemed protective enough and where the morbidity of an [[AICD]] seems excessive.
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| ==Secondary Prevention==
| | OR |
| Patients with [[Long QT syndrome]] should undergo secondary prevention with [[AICD]] implantation if they sustain an aborted [[cardiac arrest]] or [[sudden cardiac death]].
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| == 2006 ACC/AHA/ESC Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death (DO NOT EDIT) <ref name="pmid16935995">{{cite journal| author=Zipes DP, Camm AJ, Borggrefe M, Buxton AE, Chaitman B, Fromer M et al.| title=ACC/AHA/ESC 2006 Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death: a report of the American College of Cardiology/American Heart Association Task Force and the European Society of Cardiology Committee for Practice Guidelines (writing committee to develop Guidelines for Management of Patients With Ventricular Arrhythmias and the Prevention of Sudden Cardiac Death): developed in collaboration with the European Heart Rhythm Association and the Heart Rhythm Society. | journal=Circulation | year= 2006 | volume= 114 | issue= 10 | pages= e385-484 | pmid=16935995 | doi=10.1161/CIRCULATIONAHA.106.178233 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16935995}}</ref> ==
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| === Recommendations for Long QT Syndrome ===
| | Surgery is the mainstay of treatment for [disease or malignancy]. |
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| {|class="wikitable"
| | ==Indications== |
| |-
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
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| |-
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' Lifestyle modification is recommended for patients with an LQTS diagnosis (clinical and/or molecular). ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |-
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''2.''' [[Beta blockers]] are recommended for patients with an LQTS clinical diagnosis (i.e., in the presence of prolonged QT interval). ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |-
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''3.''' Implantation of an [[ICD]] along with use of [[beta blockers]] is recommended for LQTS patients with previous [[cardiac arrest]] and who have reasonable expectation of survival with a good functional status for more than 1 y. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: A]])''<nowiki>"</nowiki>
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| |}
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| {|class="wikitable"
| | *Surgical intervention is not recommended for the management of [disease name]. |
| |-
| | OR |
| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
| | *Surgery is not the first-line treatment option for patients with [disease name]. Surgery is usually reserved for patients with either: |
| |-
| | **[Indication 1] |
| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Beta blockers]] can be effective to reduce [[Sudden cardiac death|SCD]] in patients with a molecular LQTS analysis and normal [[QT interval]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
| | **[Indication 2] |
| |-
| | **[Indication 3] |
| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Implantation of an [[ICD]] with continued use of [[beta blockers]] can be effective to reduce [[Sudden cardiac death|SCD]] in LQTS patients experiencing [[syncope]] and/or [[VT]] while receiving [[beta blockers]] and who have reasonable expectation of survival with a good functional status for more than 1 y. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
| | *The mainstay of treatment for [disease name] is medical therapy. Surgery is usually reserved for patients with either: |
| |}
| | **[Indication 1] |
| | **[Indication 2] |
| | **[Indication 3] |
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| {|class="wikitable"
| | ==Surgery== |
| |-
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
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| |-
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| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Left cardiac sympathetic neural denervation may be considered for LQTS patients with [[syncope]], [[torsades de pointes]], or [[cardiac arrest]] while receiving [[beta blockers]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |-
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| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Implantation of an [[ICD]] with the use of [[beta blockers]] may be considered for prophylaxis of [[Sudden cardiac death|SCD]] for patients in categories possibly associated with higher risk of [[cardiac arrest]] such as [[LQT2]] and [[LQT3]] and who have reasonable expectation of survival with a good functional status for more than 1 y. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |}
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| === Recommendations for Drug-Induced Long QT Syndrome ===
| | *The feasibility of surgery depends on the stage of [malignancy] at diagnosis. |
| | OR |
| | *Surgery is the mainstay of treatment for [disease or malignancy]. |
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| {|class="wikitable"
| | ==Contraindications== |
| |-
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| | colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
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|
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|
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| | bgcolor="LightGreen"|<nowiki>"</nowiki>'''1.''' In patients with drug-induced LQTS, removal of the offending agent is indicated. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: A]])''<nowiki>"</nowiki>
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| |}
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| {|class="wikitable"
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| |-
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
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| |-
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| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Management with intravenous [[magnesium sulfate]] is reasonable for patients who take QT-prolonging drugs and present with few episodes of [[torsades de pointes]] in which the [[QT]] remains long. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |-
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| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Atrial or [[ventricular pacing]] or [[isoproterenol]] is reasonable for patients taking QT-prolonging drugs who present with recurrent [[torsades de pointes]]. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: B]])''<nowiki>"</nowiki>
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| |}
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| {|class="wikitable"
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| |-
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| | colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
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| |-
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| |bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' [[Potassium]] ion repletion to 4.5 to 5 mmol/L may be reasonable for patients who take QT-prolonging drugs and present with few episodes of [[torsades de pointes]] in whom the [[QT]] remains long. ''([[ACC AHA Guidelines Classification Scheme#Level of Evidence|Level of Evidence: C]])''<nowiki>"</nowiki>
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| |}
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| ==References== | | ==References== |
| | | {{Reflist|2}} |
| {{reflist|2}} | |
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| {{WH}} | | {{WH}} |
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| {{WS}} | | {{WS}} |
| [[Category:Cardiology]] | | [[Category: (name of the system)]] |
| [[Category:Electrophysiology]]
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| [[Category:Channelopathy]]
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| [[Category:Genetic disorders]]
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| [[Category:Syndromes]]
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