Skip to main content

Romano-Ward syndrome

Medical Professionals

Professional Reference articles are designed for health professionals to use. They are written by UK doctors and based on research evidence, UK and European Guidelines. You may find one of our health articles more useful.

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

Continue reading below

What is Romano-Ward 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 (long QT only), while homozygous mutations cause Jervell and Lange-Nielsen syndrome (long QT and deafness).2 Heterozygous mutations in KCNE1, another voltage-gated potassium channel gene, have also been implicated in Romano-Ward syndrome, causing the long QT 5 subtype.34

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

How common is Romano-Ward syndrome? (Epidemiology)

  • 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 syndromes5 and heterozygote mutations in KCNQ1, KCNQ1 (potassium-channel genes), and other long QT-associated genes, are thought to be responsible for Romano-Ward syndrome.3

Continue reading below

Symptoms of Romano-Ward syndrome (presentation)

  • 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.

Differential diagnosis

Other causes of prolongation of the QT interval:

Continue reading below

Diagnosing Romano-Ward syndrome (investigations)

  • The diagnosis should be considered - along with other causes of cardiogenic syncope - in patients who present with syncope.

  • Syncope triggered by exertion, loud noises, or emotion are all concerning for long QT syndrome.

  • Some individuals will have a family history of sudden unexplained death.

  • 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.

  • Genetic testing can confirm the diagnosis and establish the specific genotype.

Managementof Romano-Ward syndrome6

  • Drug treatment:

    • Beta blockers are the drugs of choice.

    • Beta blockers are effective in preventing cardiac events in approximately 70% of patients. Many cardiac events that occur in people with long QT syndrome who are 'on beta blockers' occur due to non-adherence with the medication, and/or taking QT-prolonging drugs.

    • Longer-acting beta-blockers are preferred, to facilitate adherence.

  • QT-prolonging drugs must be used with extreme caution, and should only be given after careful consideration of the risk/benefit balance.

  • If drug treatment is unsuccessful then selective high left stellate ganglionectomy has been shown to be effective.7

  • Permanent pacing in combination with betablockers may also be effective in reducing symptoms.

  • For high-risk patients, an implantable cardioverter defibrillator (ICD) reduces mortality.8

  • 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 and references

  • Long QT Syndrome 1, Online Mendelian Inheritance in Man (OMIM)
  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. Faridi R, Tona R, Brofferio A, et al; Mutational and phenotypic spectra of KCNE1 deficiency in Jervell and Lange-Nielsen Syndrome and Romano-Ward Syndrome. Hum Mutat. 2019 Feb;40(2):162-176. doi: 10.1002/humu.23689. Epub 2018 Dec 12.
  4. Bohnen MS, Peng G, Robey SH, et al; Molecular Pathophysiology of Congenital Long QT Syndrome. Physiol Rev. 2017 Jan;97(1):89-134. doi: 10.1152/physrev.00008.2016.
  5. 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.
  6. Long QT Syndrome. Alders M, Bikker H, Christiaans I, GeneReviews®, February 2018.
  7. 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.
  8. Chiang CE; Congenital and acquired long QT syndrome. Current concepts and management. Cardiol Rev. 2004 Jul-Aug;12(4):222-34.

Article history

The information on this page is written and peer reviewed by qualified clinicians.

symptom checker

Feeling unwell?

Assess your symptoms online for free