Hereditary Long Q-T Without Congenital Deafness (Romano-Ward) Syndrome

Bambang Madiyono; Alinda Rubiati Wibowo; Ismet N. Oesman; Sudigdo Sastroasmoro; Sukman Tulus Putra; Najib Advani

doi:10.14238/pi34.7-8.1994.221-30

Bambang Madiyono Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Alinda Rubiati Wibowo Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Ismet N. Oesman Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Sudigdo Sastroasmoro (Diarrheal Diseases Study Group, Medical School, Sriwidjaya University, Palembang, Indonesia
Sukman Tulus Putra Department of Biochemistry, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta
Najib Advani Department of Child Health, Universitas Indonesia Medical School/Dr. Cipto Mangunkusumo Hospital, Jakarta

DOI: https://doi.org/10.14238/pi34.7-8.1994.221-30

Keywords: hereditary long Q-T; Romano-Ward syndrome; congenital deafness

Abstract

We report a case o fhereditary long Q-T syndrome without congenital deafness (Romano-Ward syndrome). In four members of a family, a father and his daughters, the Q-T intervals on the EKG were found to be prolonged. There were no other accompanying familial anomalies such as deafness or a tendency to extracellular hypokalemia The youngest daughter which had the longest. Q-T interval had several Adams-Stokes attacks, and died in the last attack at the age of 23 months. Her two older siblings died at the age of 15 and 10 months with the same typical clinical histories. The eldest daughter, a 12-year old girl, has no clinical symptoms at all, while the fourth child, 5-yeltr old girl has several occasions of near fainting attacks. The EKG of the father showed several runs of supraventricular premature contractions that ceased spontaneously, besides evidence of the prolongation of Q-T interval. The beta-adrenergic blocking drug (propranalol) given in a relatively small maintenance dose, proved to be effective in preventing attacks of the father and the fourth child, despite the unchanged prolongation of the Q-T interval.

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