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Results for “Romano-Ward Syndrome”

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At least 19 recordsLinked to original sources
PubMed1975

QT prolongation and syncopal attacks. A case of the Romano-Ward syndrome.

The Romano-Ward syndrome is very rare as hereditary disease. Only 5 families have been reported in Japan before the present report. The patient, a 15-year-old female, had been treated for epilepsy due to syncopal attacks. Electrocardiography revealed occurrence of an augmented and labile U wave and prolongation of the Q-T interval, with recordings of frequent multifocal extrasystoles and transient ventricular fibrillation. Also noticed was sinus bradycardia. Clinical examinations presented no results coinciding with those specific ECG findings. It was inferred through surveys of her family tree that the present case had an autosomal dominant trait, heterozygously affected through the male line. Severe arrhythmias detected on her ECGs were suppressed by oral administration of 30 mg/day propranolol. Judging from the fact, propranolol may be the first to be administered among a variety of anti-arrhythmic agents in the Romano-Ward syndrome.

Adolescent↗
PubMed1977

[Familial long QT-syncope syndrome. 2 cases of Romano-Ward syndrome].

Two families with the Romano-Ward syndrome were studied; in family A there were 19 members from three generations, 11 of whom had the abnormality of a long QT interval, 2 of whom had fainting attacks, and 2 of whom died suddenly. In family B, comprising 5 members of two generations, 3 had the anomaly, one of these having fainting attacks, and also having an atrio-ventricular block. The bicycle ergometer test has an important part to play in decisions about treatment of the asymptomatic forms of the condition.

Adolescent↗
PubMedDate not supplied

The effects of stellate ganglion block on the idiopathic prolongation of the Q-T interval with cardiac arrhythmia (the Romano-Ward syndrome).

The association of a prolonged Q-T interval, congenital deafness, and syncopal attacks due to ventricular fibrillation following emotional or physical stress is known as the Jervell-Lange-Nielsen syndrome. Absence of the congenital deafness characterizes the otherwise identical Romano-Ward syndrome. Both conditions have a high mortality rate and are recognized as contributing to sudden death in children. Although the pathogenetic factors are not yet completely elucidated, the condition is well enough understood to permit effective treatment. The authors performed a left stellate ganglion block in a 12-year-old girl with a mild form of the Romano-Ward syndrome, shortening the Q-T interval and eliminating the ventricular tachyarrhythmia. The left stellete ganglion block should be considered by anesthesiologists as an emergency measure, effective in interrupting the attack.

Child↗
PubMed1977

[Romano-Ward syndrome and left stellectomy. General review apropos of a recent case].

A new familial case of the Romano-Ward syndrome in a young girl of 21 years is reported. A progressive worsening of the condition with multiple syncopal attacks, together with difficulty in controlling the patient, lead us to carry out a left stellate ganglionectomy. The operation did not lead to any shortening of the QT interval. It appeared in the end that propranolol was the most effective way of preventing the syncopal attacks.

Adult↗
PubMed1976

Electrical alternans of TU wave in Romano-Ward syndrome.

A case is presented in which an exceptional electrical alternans of the TU wave occurred in association with the Romano-Ward syndrome. This appears to be the first reported example of this association. Ventricular fibrillation was documented during the syncopal attacks. There was no evidence of abnormality of the serum electrolytes.

Aged↗
PubMed1978

[The problem of the Romano-Ward syndrome--misinterpretation as genuine epilepsy].

It is reported on a case of Romano-Ward-syndrome in a 40-year-old female patient. The problems of the most frequent false interpretation as idiopathic epilepsy as well as the differential-diagnostic considerations concerning the exclusion of symptomatic QT(U)-elongations in the ECG are entered. The pathomechanisms which perhaps are the basis of the syndrome are discussed.

Adrenergic beta-Antagonists↗
PubMed1978

Congenital QT interval prolongation. A review with a survey of three families.

The prolonged QT interval syndrome without hearing loss (Romano-Ward syndrome) is described in three families with 48 affected members. Syncope or dizziness caused by different ventricular tachyarrhythmias were the presenting symptoms in the symptomatic patients. Four of the subjects died suddenly. Right stellate ganglionectomy was performed in one patient in order to abolish the ventricular dysrhythmia. beta-blockers are considered the drug of choice in patients with hereditary prolonged QT interval; if the beta-blockers fail to abolish the syncopal attacks of severe bradycardia complicates the clinical course, a pharmacological blockade of the stellate ganglia should be performed and its results carefully evaluated in order to establish whether a stellate ganglionectomy is justified.

Adult↗
PubMed1979

Preeminence of the left stellate ganglion in the long Q-T syndrome.

In seven patients with Romano-Ward syndrome, stellate ganglion block or stimulation and pharmacologic interventions were made to assess their influence on duration of the O-T interval, electrical alteration of the T wave and ventricular tachydysrhythmias. Left stellate ganglion block and right stellate ganglion stimulation shortened Q-T interval, abolished alternans phenomena and suppressed tachydysrhythmias. Propranolol and phenytoin had a similar effect. In contrast, right stellate ganglion block, left stellate ganglion stimulation and prior administration of quinidine and procainamide had an opposite effect. These responses resemble observations in animal models which suggest that excessive or unopposed activity of the left, or subnormal activity of the right stellate ganglion, or both, account for the pathophysiologic manifestations of the long Q-T syndrome. They are also consistent with clinically correlated, cardiac neuropathologic findings in these patients. An analogous but acquired dysautonomia involving the left stellate ganglion and ischemic left ventricle may precipitate sudden coronary death.

Adult↗
PubMed1976

[Jervell and Lange-Nielsen syndrome].

A rare case of the congenital cardiological syndrome of Jervell and Lange-Nielsen in a 6-year old boy is analyzed. A literature survey of the clinical picture, treatment of and prognosis in this and related to it the Romano-Ward syndrome is given.

Child↗