Cases reported "Romano-Ward Syndrome"

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1/2. Successful management of cesarean section in a patient with romano-ward syndrome using landiolol, a selective and short-acting beta1 receptor antagonist.

    Romano-Ward (R-W) syndrome is an autosomal dominant hereditary disorder and is characterized by a prolonged QT interval on the electrocardiogram (ECG), syncope, and sudden death. We report here a case of cesarian section in a patient with R-W syndrome whose QT prolongation was successfully managed with landiolol, a selective beta1 receptor blocker. A 25-year-old woman with R-W syndrome was scheduled for cesarean section. In the operating room, the patient's ECG showed tachycardia (102 beats x min(-1)) and marked QT prolongation (QTc = 0.56 s). After spinal anesthesia, the patient's heart rate (HR) increased to 130 beats/min accompanied by a slight decrease in arterial blood pressure to 97/57 mmHg and the QTc was prolonged to 0.57 s. Landiolol was continuously infused at a rate of 0.04 x min(-1) and the HR gradually decreased to 80-90 beats x min(-1) accompanied by the normalization of QTc to 0.48 s. We thought that the use of landiolol was more rational and was preferable to a nonselective beta receptor blocker for a term-pregnant woman because blockade of the beta2 receptor might cause uterine contraction. After the use of landiolol, intraoperative and postoperative courses in both the patient and the baby were uneventful. ( info)

2/2. The homozygous KCNQ1 gene mutation associated with recessive romano-ward syndrome.

    In a 7-year-old boy with normal hearing suffering from repeated syncope an extremely prolonged QTc interval (up to 700 ms) was found. The mother was completely asymptomatic and the father had an intermittently borderline QTc interval (maximum 470 ms) but no symptoms. In the proband a mutation analysis of KCNQ1 gene revealed a homozygous 1893insC mutation. The parents were heterozygous for this mutation. There was no consanguineous marriage in the family. The clinical relevance of these findings is that apparently normal individuals may have a latent reduction of repolarizing currents, a "reduced repolarization reserve," because they are carriers of latent ion channel genes mutations. ( info)

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