Romano-Ward syndrome

Description

Congenital (autosomal dominant) long QT syndrome (LQTS).

  • Unlike Jervell-Lange-Nielsen, there is no congenital hearing loss present
  • Multiple genetic mutations identified linked to cardiac potassium and beta-adrenergic channels.
  • Mainstay of treatment is beta-blockers.
  • Risk of sudden cardiac death – if any syncope or sustained ventricular arrhythmias for ablation / ICD implantation.

History

1963-1964: Independently described in 1963 by Cesarino Romano, Italian Paediatrician, and in 1964 by Irish paediatrician Owen Conor Ward. Both reported an autosomal dominant long-term QT syndrome, later known as Romano-Ward syndrome.

1965 – Romano’s letter to the Lancet, March 20 1965 in response to JervellLange-Nielsen and Ward’s findings:

We pointed to the resemblance between our case and the syndrome of deafmutism, syncopal attacks, lengthening of the QT interval, and sudden death, but, as in Ward’s case, there was no evidence of deafmutism in our patient or in her family. The syndrome reported by us and by Ward may be a distinct entity. [Romano C, 1963] [Ward OC, 1964]

1979 – International Long-QT Syndrome Registry was initiated to collect data on any patient with LQTS.

2001 – Advances in research with paper by Schwrartz et al: Life-threatening arrhythmias in LQTS patients tend to occur under specific circumstances in a gene-specific manner.

2005 – 25th Anniversary International LQTS Registry collating clinical discoveries from the past 25 years and setting ongoing aims for future research in this challenging field: ‘Our quest for uncovering the secrets of LQTS continues‘ [PMID 15753228]


Associated Persons


Alternative names

  • Ward-Romano Syndrome
  • Congenital Long QT syndrome without deafness

References


eponymictionary CTA

eponymictionary

the names behind the name

Emergency physician MA (Oxon) MBChB (Edin) FACEM FFSEM with a passion for rugby; medical history; medical education; and informatics. Asynchronous learning #FOAMed evangelist. Co-founder and CTO of Life in the Fast lane | Eponyms | Books | vocortex |

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