A study of the inheritance pattern of Romano-Ward syndrome. Prolonged Q-T interval, syncope, and sudden death

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 histocompatibility complex on the short arm of chromosome number 6.