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==
+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
-==Overview==
+Pharmacologic medical therapies for [disease name] include (either) [therapy 1], [therapy 2], and/or [therapy 3].
-==Medical Therapy==
+OR
-==Treatment options==
-There are two treatment options in individuals with LQTS: arrhythmia prevention, and arrhythmia termination.
-===Arrhythmia prevention===
+Empiric therapy for [disease name] depends on [disease factor 1] and [disease factor 2].
-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.  These include:
-* Administration of [[beta blocker|beta receptor blocking agents]] which decreases the risk of stress induced arrhythmias. Beta blockers are the first choice in treating Long QT syndrome.
-In 2004 it has been shown that 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
+OR
-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>
-* Potassium supplementation. If the potassium content in the blood rises, the action potential shortens and due to this reason it is believed that increasing potassium concentration could minimize the occurrence of arrhythmias. It should work best in LQT2 since the HERG channel is especially sensible to potassium concentration, but the use is experimental and not evidence based.
+Patients with [disease subclass 1] are treated with [therapy 1], whereas patients with [disease subclass 2] are treated with [therapy 2].
-* [[Mexiletine]]. 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 usable when other therapies fail. It should be especially effective in LQT3 but there is no evidence based documentation.
-* Amputation of the [[cervical sympathetic chain]] (left [[Stellate ganglion|stellectomy]]). This may be used as an add-on therapy to beta blockers but modern therapy mostly favors ICD implantation if beta blocker therapy fails.
-===Arrhythmia termination===
+==Medical Therapy==
-Arrhythmia termination involves stopping a life-threatening arrhythmia once it has already occurred.  The only effective form of arrhythmia termination in individuals with LQTS is placement of an [[implantable cardioverter-defibrillator]] (ICD). ICD are commonly used in patients with syncopes despite beta blocker therapy, and in patients who have experienced a cardiac arrest.
+*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===
-With better knowledge of the genetics underlying the long QT syndrome, more precise treatments will be readily available.<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>
+* '''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 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)
+****1.1.2.2 (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]]