Romano-Ward Syndrome

999 Users are discussing this topic

PatientPlus articles are written by UK doctors and are based on research evidence, UK and European Guidelines. They are designed for health professionals to use, so you may find the language more technical than the condition leaflets.

This page has been archived. It has not been updated since 21/01/2011. External links and references may no longer work.

Synonyms: autosomal-dominant long QT syndrome, long QT syndrome type 1, LQTS1, RWS, Romano-Ward long QT syndrome, Ward-Romano syndrome

Romano-Ward syndrome is an inherited heart disorder characterised by prolongation of the QT interval, often in association with episodes of ventricular tachyarrhythmia, torsades de pointes, syncope and sudden death.[1]

KCNQ1 (formerly called KVLQT1) is a voltage-gated potassium-channel gene responsible for the long QT 1 subtype of long QT syndromes. In general, heterozygous mutations in KCNQ1 cause Romano-Ward syndrome (LQT1 only), while homozygous mutations cause Jervell and Lange-Nielsen syndrome (LQT1 and deafness).[2]

Attacks of ventricular tachyarrhythmia are usually associated with sympathetic stimulation such as exercise, stress or emotion, dependent on genotype.

NEW - log your activity

  • Notes
    Add notes to any clinical page and create a reflective diary
  • Track
    Automatically track and log every page you have viewed
  • Print
    Print and export a summary to use in your appraisal
Click to find out more »
  • The disorder may be sporadic or transmitted as an autosomal-dominant trait.
  • Six different genes have so far been identified as being involved in the development of congenital long QT syndromes[3] and heterozygote mutations in KCNQ1 (a potassium-channel gene) are thought to be responsible for Romano-Ward syndrome.[2]
  • The severity ranges from those with no apparent symptoms to others who develop tachyarrhythmias resulting in episodes of syncope, cardiac arrest and, potentially, sudden death.
  • Between such episodes, sinus bradycardia is often seen.
  • There may be a family history of recurrent syncope or sudden death.

Other causes of prolongation of the QT interval:

  • The diagnosis should be considered in all patients who present with syncope.
  • An ECG with calculation of the QT interval should be performed on all patients with a suggestive history.
  • All other family members must be fully assessed.
  • Drug treatment:[4]
  • If drug treatment is unsuccessful then selective high left stellate ganglionectomy has been shown to be effective.[5]
  • Permanent pacing in combination with betablockers may also be effective in reducing symptoms.
  • For high-risk patients, an implantable cardioverter defibrillator (ICD) reduces mortality.[6]
  • Defibrillator implantation is often a first-line therapy if there has been a cardiac arrest.

The prognosis of untreated congenital long QT syndrome is poor, with a high incidence of sudden death in childhood.

Further reading & references

  1. Watanabe A, Nakamura K, Morita H, et al; Long QT syndrome. Nippon Rinsho. 2005 Jul;63(7):1171-7.
  2. Herbert E, Trusz-Gluza M, Moric E, et al; KCNQ1 gene mutations and the respective genotype-phenotype correlations in the long QT syndrome. Med Sci Monit. 2002 Oct;8(10):RA240-8.
  3. Haack B, Kupka S, Ebauer M, et al; Analysis of candidate genes for genotypic diagnosis in the long QT syndrome. J Appl Genet. 2004;45(3):375-81.
  4. Sovari AA et al; Long QT Syndrome, Medscape, Jun 2012
  5. Moss AJ, McDonald J; Unilateral cervicothoracic sympathetic ganglionectomy for the treatment of long QT interval syndrome. N Engl J Med. 1971 Oct 14;285(16):903-4.
  6. Chiang CE; Congenital and acquired long QT syndrome. Current concepts and management. Cardiol Rev. 2004 Jul-Aug;12(4):222-34.

Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our conditions.

Original Author:
Dr Colin Tidy
Current Version:
Document ID:
1282 (v25)
Last Checked:
21/01/2011
Next Review:
20/01/2016

Did you find this health information useful?

Yes No

Thank you for your feedback!

Subcribe to the Patient newsletter for healthcare and news updates.

We would love to hear your feedback!

 
 
Patient Access app - find out more Patient facebook page - Like our page