Long QT Syndrome medical and device therapy: Difference between revisions

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Latest revision as of 17:34, 18 March 2020

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [2]; Associate Editor(s)-in-Chief:

Overview

There is no treatment for [disease name]; the mainstay of therapy is supportive care.

OR

Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].

OR

The majority of cases of [disease name] are self-limited and require only supportive care.

OR

[Disease name] is a medical emergency and requires prompt treatment.

OR

The mainstay of treatment for [disease name] is [therapy].

OR

The optimal therapy for [malignancy name] depends on the stage at diagnosis.

OR

[Therapy] is recommended among all patients who develop [disease name].

OR

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].

OR

Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].

OR

Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].

OR

Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Medical Therapy

Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].

Disease Name

1 Stage 1 - Name of stage
- 1.1 Specific Organ system involved 1
  - 1.1.1 Adult
    - Preferred regimen (1): drug name 100 mg PO q12h for 10-21 days (Contraindications/specific instructions)
    - Preferred regimen (2): drug name 500 mg PO q8h for 14-21 days
    - Preferred regimen (3): drug name 500 mg q12h for 14-21 days
    - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
    - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
    - Alternative regimen (3): drug name 500 mg PO q6h for 14–21 days
  - 1.1.2 Pediatric
    - 1.1.2.1 (Specific population e.g. children < 8 years of age)
      - Preferred regimen (1): drug name 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name10 mg/kg PO q6h (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
    - 1.1.2.2 (Specific population e.g. 'children < 8 years of age')
      - Preferred regimen (1): drug name 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
      - Alternative regimen (1): drug name 10 mg/kg PO q6h (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
- 1.2 Specific Organ system involved 2
  - 1.2.1 Adult
    - Preferred regimen (1): drug name 500 mg PO q8h
  - 1.2.2 Pediatric
    - Preferred regimen (1): drug name 50 mg/kg/day PO q8h (maximum, 500 mg per dose)

2 Stage 2 - Name of stage
- 2.1 Specific Organ system involved 1
  Note (1):
  
  Note (2):
  
  Note (3):
  - 2.1.1 Adult
    - Parenteral regimen
      - Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
      - Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
      - Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
    - Oral regimen
      - Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
      - Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
      - Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
      - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
      - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
      - Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
  - 2.1.2 Pediatric
    - Parenteral regimen
      - Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
      - Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
      - Alternative regimen (2): drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '(Contraindications/specific instructions)'
    - Oral regimen
      - Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name (for children aged ≥ 8 years) 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
      - Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)
- 2.2 'Other Organ system involved 2'
  Note (1):
  
  Note (2):
  
  Note (3):
  - 2.2.1 Adult
    - Parenteral regimen
      - Preferred regimen (1): drug name 2 g IV q24h for 14 (14–21) days
      - Alternative regimen (1): drug name 2 g IV q8h for 14 (14–21) days
      - Alternative regimen (2): drug name 18–24 MU/day IV q4h for 14 (14–21) days
    - Oral regimen
      - Preferred regimen (1): drug name 500 mg PO q8h for 14 (14–21) days
      - Preferred regimen (2): drug name 100 mg PO q12h for 14 (14–21) days
      - Preferred regimen (3): drug name 500 mg PO q12h for 14 (14–21) days
      - Alternative regimen (1): drug name 500 mg PO q6h for 7–10 days
      - Alternative regimen (2): drug name 500 mg PO q12h for 14–21 days
      - Alternative regimen (3):drug name 500 mg PO q6h for 14–21 days
  - 2.2.2 Pediatric
    - Parenteral regimen
      - Preferred regimen (1): drug name 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
      - Alternative regimen (1): drug name 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
      - Alternative regimen (2): drug name 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
    - Oral regimen
      - Preferred regimen (1): drug name 50 mg/kg/day PO q8h for 14 (14–21) days (maximum, 500 mg per dose)
      - Preferred regimen (2): drug name 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
      - Preferred regimen (3): drug name 30 mg/kg/day PO q12h for 14 (14–21) days (maximum, 500 mg per dose)
      - Alternative regimen (1): drug name 10 mg/kg PO q6h 7–10 days (maximum, 500 mg per day)
      - Alternative regimen (2): drug name 7.5 mg/kg PO q12h for 14–21 days (maximum, 500 mg per dose)
      - Alternative regimen (3): drug name 12.5 mg/kg PO q6h for 14–21 days (maximum,500 mg per dose)

References

Template:WH Template:WS

@@ Line 1: / Line 1: @@
 __NOTOC__
 {{Long QT Syndrome}}
+{{CMG}}; {{AE}}
-{{CMG}}
 ==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]].
+There is no treatment for [disease name]; the mainstay of therapy is supportive care.
-== ACC/AHA/ESC 2006 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> ==
-=== Recommendations for Long QT Syndrome ===
-{|class="wikitable"
-|-
-| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
-|-
-| 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>
-|-
-| 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>
-|-
-| 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>
-|}
-{|class="wikitable"
-|-
-| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
-|-
-|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''1.''' Beta blockers can be effective to reduce 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>
-|-
-|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Implantation of an ICD with continued use of beta blockers can be effective to reduce 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>
-|}
-{|class="wikitable"
+OR
-|-
-| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
-|-
+Supportive therapy for [disease name] includes [therapy 1], [therapy 2], and [therapy 3].
-|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>
-|-
+OR
-|bgcolor="LemonChiffon"|<nowiki>"</nowiki>'''2.''' Implantation of an ICD with the use of beta blockers may be considered for prophylaxis of 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>
-|}
-=== Recommendations for Drug-Induced Long QT Syndrome ===
+The majority of cases of [disease name] are self-limited and require only supportive care.
-{|class="wikitable"
+OR
-|-
-| colspan="1" style="text-align:center; background:LightGreen"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class I]]
-|-
+[Disease name] is a medical emergency and requires prompt treatment.
-| 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>
-|}
-{|class="wikitable"
+OR
-|-
-| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIa]]
-|-
+The mainstay of treatment for [disease name] is [therapy].
-|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>
-|-
+OR
-|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>
-|}
+The optimal therapy for [malignancy name] depends on the stage at diagnosis.
-{|class="wikitable"
+OR
-|-
-| colspan="1" style="text-align:center; background:LemonChiffon"|[[ACC AHA Guidelines Classification Scheme#Classification of Recommendations|Class IIb]]
-|-
+[Therapy] is recommended among all patients who develop [disease name].
-|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>
-|}
-==Primary Prevention==
+OR
-===Withdrawal of Drugs and Supplements===
-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.
-====List of Medications to be Avoided in Congenital Long QT Syndrome====
-*[[Albuterol]]
-*[[Alfuzosin]]
-*[[Amantadine]]
-*[[Amiodarone]]
-*[[Amisulpride]]
-*[[Amitriptyline]]
-*[[Amphetamine]]
-*[[Arsenic trioxide]]
-*[[Artenimol]] + [[piperaquine]]
-*[[Astemizole]]
-*[[Atazanavir]]
-*[[Atomoxetine]]
-*[[Azithromycin]]
-*[[Bepridil]]
-*[[Chloral hydrate]]
-*[[Chloroquine]]
-*[[Chlorpromazine]]
-*[[Ciprofloxacin]]
-*[[Cisapride]]
-*[[Citalopram]]
-*[[Clarithromycin]]
-*[[Clomipramine]]
-*[[Clozapine]]
-*[[Cocaine]]
-*[[Desipramine]]
-*[[Dexmethylphenidate]]
-*[[Diphenhydramine]]
-*[[Diphenhydramine]]
-*[[Disopyramide]]
-*[[Dobutamine]]
-*[[Dofetilide]]
-*[[Dolasetron]]
-*[[Domperidone]]
-*[[Dopamine]]
-*[[Doxepin]]
-*[[Dronedarone]]
-*[[Droperidol]]
-*[[Ephedrine]]
-*[[Epinephrine]]
-*[[Eribulin]]
-*[[Erythromycin]]
-*[[Escitalopram]]
-*[[Famotidine]]
-*[[Fenfluramine]]
-*[[Fingolimod]]
-*[[Flecainide]]
-*[[Fluconazole]]
-*[[Fluoxetine]]
-*[[Foscarnet]]
-*[[Fosphenytoin]]
-*[[Galantamine]]
-*[[Gatifloxacin]]
-*[[Gemifloxacin]]
-*[[Granisetron]]
-*[[Halofantrine]]
-*[[Haloperidol]]
-*[[Ibutilide]]
-*[[Iloperidone]]
-*[[Imipramine]]
-*[[Indapamide]]
-*[[Isoproterenol]]
-*[[Isradipine]]
-*[[Itraconazole]]
-*[[Ketoconazole]]
-*[[Lapatinib]]
-*[[Levalbuterol]]
-*[[Levofloxacin]]
-*[[Levomethadyl]]
-*[[Lisdexamfetamine]]
-*[[Lithium]]
-*[[Mesoridazine]]
-*[[Metaproterenol]]
-*[[Methadone]]
-*[[Methylphenidate]]
-*[[Midodrine]]
-*[[Mirtazapine]]
-*[[Moexipril]] / [[HCTZ]]
-*[[Moxifloxacin]]
-*[[Nicardipine]]
-*[[Nilotinib]]
-*[[Norepinephrine]]
-*[[Nortriptyline]]
-*[[Octreotide]]
-*[[Ofloxacin]]
-*[[Ondansetron]]
-*[[Oxytocin]]
-*[[Paliperidone]]
-*[[Paroxetine]]
-*[[Pentamidine]]
-*[[Perflutren]] lipid microspheres
-*[[Phentermine]]
-*[[Phenylephrine]]
-*[[Phenylpropanolamine]]
-*[[Pimozide]]
-*[[Probucol]]
-*[[Procainamide]]
-*[[Protriptyline]]
-*[[Pseudoephedrine]]
-*[[Quetiapine]]
-*[[Quinidine]]
-*[[Ranolazine]]
-*[[Ritodrine]]
-*[[Ritonavir]]
-*[[Roxithromycin]]
-*[[Salmeterol]]
-*[[Sertindole]]
-*[[Sertraline]]
-*[[Sevoflurane]]
-*[[Sibutramine]]
-*[[Solifenacin]]
-*[[Sotalol]]
-*[[Sparfloxacin]]
-*[[Sunitinib]]
-*[[Tacrolimus]]
-*[[Tamoxifen]]
-*[[Telithromycin]]
-*[[Terbutaline]]
-*[[Terfenadine]]
-*[[Thioridazine]]
-*[[Tizanidine]]
-*[[Tolterodine]]
-*[[Trazodone]]
-*[[Trimethoprim-Sulfamethoxazole]]
-*[[Trimipramine]]
-*[[Vandetanib]]
-*[[Vardenafil]]
-*[[Venlafaxine]]
-*[[Voriconazole]]
-*[[Ziprasidone]]
-===Correct Electrolyte Disturbances===
+Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
-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.
-===Postassium Administration===
+OR
-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.
-===Beta Blockers===
+Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
-Beta blockers are first line therapy in the treatment of 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 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]].
+OR
-====Nadolol====
+Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
-Nadolol at a dose of 1.0 to 1.5 mg/kg/day or 50 mg/m2/day QD or BID is the dose
-====Propranolol====
+OR
--4 mg/kg/day BID for the long acting form and TID for the liquid.  Often preferred in LQT3.
-===Mexiletine===
+Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
-[[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.
-===AICD Implantation===
+==Medical Therapy==
-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
+*Pharmacologic medical therapy is recommended among patients with [disease subclass 1], [disease subclass 2], and [disease subclass 3].
-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>
+*Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
+*Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
+*Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
+===Disease Name===
-An [[AICD]] should be implanted if:
+* '''1 Stage 1 - Name of stage'''
-*The QTc is > 550 ms and if it is not [[LQT1]]
+** 1.1 '''Specific Organ system involved 1'''
-*[[LQT2]] in women and the QTc is > 500 ms, with or without symptoms
+*** 1.1.1 '''Adult'''
-*In infants with 2:1 [[AV block]] (controversial)
+**** Preferred regimen (1): [[drug name]] 100 mg PO q12h for 10-21 days '''(Contraindications/specific instructions)'''
-*In [[JLNS]] (LQTS with deafness) given its malignant propensity (controversial)
+**** Preferred regimen (2): [[drug name]] 500 mg PO q8h for 14-21 days
+**** Preferred regimen (3): [[drug name]] 500 mg q12h for 14-21 days
-===Sympathetic Denervation===
+**** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
-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:
+**** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
-*The patient does not tolerate [[beta blockers]] or breaks through [[beta blockers]]
+**** Alternative regimen (3): [[drug name]] 500 mg PO q6h for 14–21 days
-*The patient [[faints]] while on [[beta blockers]]
+*** 1.1.2 '''Pediatric'''
-*There is a history of [[VF]] terminating [[AICD]] shocks
+**** 1.1.2.1 (Specific population e.g. '''children < 8 years of age''')
-*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.
+***** Preferred regimen (1): [[drug name]] 50 mg/kg PO per day q8h (maximum, 500 mg per dose)
+***** Preferred regimen (2): [[drug name]] 30 mg/kg PO per day in 2 divided doses (maximum, 500 mg per dose)
-==Secondary Prevention==
+***** Alternative regimen (1): [[drug name]]10 mg/kg PO q6h (maximum, 500 mg per day)
-Patients with [[Long QT syndrome]] should undergo secondary prevention with [[AICD]] implantation if they sustain an aborted [[cardiac arrest]] or [[sudden cardiac death]].
+***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
+***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
+****1.1.2.2 (Specific population e.g. '<nowiki/>'''''children < 8 years of age'''''')
+***** Preferred regimen (1): [[drug name]] 4 mg/kg/day PO q12h（maximum, 100 mg per dose）
+***** Alternative regimen (1): [[drug name]] 10 mg/kg PO q6h (maximum, 500 mg per day)
+***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h (maximum, 500 mg per dose)
+***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h (maximum, 500 mg per dose)
+** 1.2 '''Specific Organ system involved 2'''
+*** 1.2.1 '''Adult'''
+**** Preferred regimen (1): [[drug name]] 500 mg PO q8h
+*** 1.2.2  '''Pediatric'''
+**** Preferred regimen (1): [[drug name]] 50 mg/kg/day PO q8h (maximum, 500 mg per dose)
+* 2 '''Stage 2 - Name of stage'''
+** 2.1 '''Specific Organ system involved 1 '''
+**: '''Note (1):'''
+**: '''Note (2)''':
+**: '''Note (3):'''
+*** 2.1.1 '''Adult'''
+**** Parenteral regimen
+***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
+***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
+***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
+**** Oral regimen
+***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
+***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
+***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
+***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
+***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
+***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
+*** 2.1.2 '''Pediatric'''
+**** Parenteral regimen
+***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
+***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
+***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day) '<nowiki/>'''''(Contraindications/specific instructions)''''''
+**** Oral regimen
+***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
+***** Preferred regimen (2): [[drug name]] '''(for children aged ≥ 8 years)''' 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
+***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
+***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
+***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
+***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
+** 2.2  '<nowiki/>'''''Other Organ system involved 2''''''
+**: '''Note (1):'''
+**: '''Note (2)''':
+**: '''Note (3):'''
+*** 2.2.1 '''Adult'''
+**** Parenteral regimen
+***** Preferred regimen (1): [[drug name]] 2 g IV q24h for 14 (14–21) days
+***** Alternative regimen (1): [[drug name]] 2 g IV q8h for 14 (14–21) days
+***** Alternative regimen (2): [[drug name]] 18–24 MU/day IV q4h for 14 (14–21) days
+**** Oral regimen
+***** Preferred regimen (1): [[drug name]] 500 mg PO q8h for 14 (14–21) days
+***** Preferred regimen (2): [[drug name]] 100 mg PO q12h for 14 (14–21) days
+***** Preferred regimen (3): [[drug name]] 500 mg PO q12h for 14 (14–21) days
+***** Alternative regimen (1): [[drug name]] 500 mg PO q6h for 7–10 days
+***** Alternative regimen (2): [[drug name]] 500 mg PO q12h for 14–21 days
+***** Alternative regimen (3):[[drug name]] 500 mg PO q6h for 14–21 days
+*** 2.2.2 '''Pediatric'''
+**** Parenteral regimen
+***** Preferred regimen (1): [[drug name]] 50–75 mg/kg IV q24h for 14 (14–21) days (maximum, 2 g)
+***** Alternative regimen (1): [[drug name]] 150–200 mg/kg/day IV q6–8h for 14 (14–21) days (maximum, 6 g per day)
+***** Alternative regimen (2):  [[drug name]] 200,000–400,000 U/kg/day IV q4h for 14 (14–21) days (maximum, 18–24 million U per day)
+**** Oral regimen
+***** Preferred regimen (1):  [[drug name]] 50 mg/kg/day PO q8h for 14 (14–21) days  (maximum, 500 mg per dose)
+***** Preferred regimen (2): [[drug name]] 4 mg/kg/day PO q12h for 14 (14–21) days (maximum, 100 mg per dose)
+***** Preferred regimen (3): [[drug name]] 30 mg/kg/day PO q12h for 14 (14–21) days  (maximum, 500 mg per dose)
+***** Alternative regimen (1):  [[drug name]] 10 mg/kg PO q6h 7–10 days  (maximum, 500 mg per day)
+***** Alternative regimen (2): [[drug name]] 7.5 mg/kg PO q12h for 14–21 days  (maximum, 500 mg per dose)
+***** Alternative regimen (3): [[drug name]] 12.5 mg/kg PO q6h for 14–21 days  (maximum,500 mg per dose)
 ==References==
+{{Reflist|2}}
-{{reflist|2}}
 {{WH}}
 {{WS}}
-[[Category:Cardiology]]
+[[Category: (name of the system)]]
-[[Category:Electrophysiology]]
-[[Category:Channelopathy]]
-[[Category:Genetic disorders]]
-[[Category:Syndromes]]