Romano-Ward syndrome consists of attacks of syncope, possible sudden death, accompanied by prolonged Q-T interval on ECG. We carried out HLA antigen typing of a family of 16 members having ten cases of Romano-Ward syndrome. All the ten cases had the common HLA haplotype of A9-Bw54, while the six not showing the symptoms did not have A9-Bw54 haplotype. The genes of this syndrome, considered to be inherited through the autosomal dominant patterns, are assumed to have a close linking to the major histo compatibility complex on the short arm of chromosome number 6.
Get full access to this article
View all access options for this article.
References
1.
Jervell A., Lange-Nielsen R.: Congenital deaf-mutism, functional heart disease with prolongation of the Q-T interval and sudden death. Am Heart J54:59, 1957
2.
Romano C., Gemme G., Pongiglione R.: Aritmie cardiache rare dell 'eta' pediatrica. La Clinic Paediatr45:656, 1963
3.
Ward OC: A new familial cardiac syndrome in children. J Irish Med Assoc44:102, 1964
Ratshin, RAHunt D., Russell RO, Rackley CE: Q-T interval prolongation, paroxysmal ventricular arrthmias. and convulsive syncope. Ann Intern Med75:919, 1971
6.
Karhunen, P. , Luomäki, K., Heikkilä J.Eisalo A.: Syncope and Q-T prolongation without deafness: the Romano-Ward syndrome. Am Heart J80: 820, 1070
7.
Fraser GR, Froggat P., James 'I'N: Congenital deafness associated with electrocardiographic abnormalities, fainting attacks and sudden death. A recessive syndrome. Q j Med33:361. 1964
8.
Fraser GR, Froggatt P. Murphy J: Congenital aspects of the cardio-auditory syndrome of Jervell and Lange-Nielsen (congenital deafness and electrocardiographic abnormalities). Ann Hum Genet28: 133, 1964
9.
Mathews EC, Blount A., Townsend AI: Q-T prolongation and ventricular arrhythmias, with and without deafness, in the same family. Am JCardiol29:702, 1972
