Congenital Cardiac Arrhythmia

Barlow, John B.; Bosman, C; Cochrane, Josh

doi:10.1016/s0140-6736(64)90486-6

Cited by 94 publications

(5 citation statements)

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“…Both the mode of inheritance and the absence of bilateral deafness in most cases show that this disease is different from the syndrome of Jervell and Lange-Nielsen which is an autosomal recessive disease. Dominantly inherited cases of the syndrome without deafness have also been described by Barlow, Bosman, and Craig Cochrane (1964), Romano (1965), and Garza et al (1970). Ours is the first report from The Netherlands.…”

Section: Discussionmentioning

confidence: 68%

A Family with Heritable Electrocardiographic QT-prolongation

Straaten

Bruins

1973

Journal of Medical Genetics

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Summary. A report is presented concerning a family with heritable electrocardiographic QT-prolongation attacks of syncope and possible sudden death. In 23 family members investigated, nine living cases were found to have the anomaly. Of these nine patients at least two had had syncopes in early childhood. Hearing loss was found in three of the nine patients, but in one of them this could have been due to noise trauma and in another hearing loss was unilateral. The inheritance follows an autosomal dominant pattern. As far as we know this is the first report of this disease from The Netherlands.In 1967, Ward reported a family in which some members suffered from a new disease that he called familial cardiac arrhythmia. He described two sibs with unexplained attacks of loss of consciousness. On investigation they had distinct electrocardiographic abnormalities. There was prolongation of the QT-interval and the aspect of the ST segment was very abnormal. Abnormal electrocardiograms were recorded in 10 of 28 family members. The inheritance followed an autosomal dominant pattern.We describe here another family with the disease. Method of InvestigationAfter ascertainment of the propositus, his parents and three brothers were also investigated. The ResultsThe propositus, III.5, was seen at the age of 10 years in our department because of frequent syncope and because he tired easily. On admission he had a slow regular pulse and the blood pressure was normal. The heart was of normal size. The electrocardiogram showed prolongation of the QTinterval: 0-60 seconds while it should have been 0-38 seconds. The T-wave was biphasic in V1-V4 and positive in V5 and V6. Chest radiology and the phonocardiogram were normal. With audiometric testing only a slight hearing loss was recorded of ± 20 db in low frequencies until 500 Hz and a loss of ± 20 db from 1500 to 3000 Hz. The hearing loss was of the perceptive type. The values for Ca, K, Mg, SGOT, LDH, CPK, and aldolase were normal for the propositus, his parents and his three brothers. The electrocardiograms of his father (II.2) and two brothers (III.4 and III.6) were abnormal. The audiometric testing of II.2 and III.4 also demonstrated a loss of hearing. The father had a hearing loss of up to 50 db, in the high frequencies, but this could have been caused by his working in a factory.One brother (III.4) had some mild hearing loss of ± 30 db in the frequency of 4000 Hz but only in the left ear.The investigation of the five sibs of the father with their children gave the following results. In 18 family members five abnormal electrocardiograms were recorded. One sister of the father (II.3), three 158 on 1 May 2019 by guest. Protected by copyright.

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Section: Discussionmentioning

confidence: 68%

A Family with Heritable Electrocardiographic QT-prolongation

Straaten

Bruins

1973

Journal of Medical Genetics

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“…The number of reported cases is approaching fifty, but there are probably several unrecognized patients who are treated for epileptic spells. Most of the cases have been described in members of the same family (1,5,6,7,9,13,16,18) with autosomal dominent inheritance suggested for those with normal hearing (7), while auto-soma1 recessive inheritance is claimed for those with congenital deafness (5).…”

Section: Discussionmentioning

confidence: 99%

Prolonged Q‐t Interval and Cardiac Syncopes

Wennevold

Kringelbach

1971

Acta Paediatrica

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“…In 1901 Morquio described an inherited condition responsible for abnormalities in pulse rate, syncopal attacks and sudden death in children. Other inherited disorders of heart rate and myocardial conduction were described later (Romano et al 1963, Barlow et al 1964, Gamstorp et al 1964, Ward 1964, Garza et al 1970, Johansson & Jorming 1972. Arrhythmia in association with congenital deafness was also reported (Jervell & Lange-Nelson 1957, Levine & Woodworth 1958, Fraser et al 1964, Jervell et al 1966, James 1967, Sanchez Cascos et al 1969.…”

Section: Received 12 March Revised 12 May Accepted F O R Publicatiomentioning

confidence: 95%

Cardio‐cutaneous syndrome (the “LEOPARD” syndrome). Review of the literature and a new family

Seuánez

Mane-Garzon²,

Kolski

1976

Clinical Genetics

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Numerous reports of familial cardiac disorders associated with abnormalities of cutaneous pigmentation have appeared in the literature. In some of these there have been other somatic malformations including retardation of growth and anomalous development of the genitalia. In this paper we review the literature and describe another family with multiple lentigines occurring in six members out of eight examined. They were reported to occur in 10 other members not seen by us. The trait showed an autosomal dominant mode of inheritance and appeared to be transmitted by affected fathers. In previous reports transmission has usually been through an affected mother. In five of the six patients examined, only skin abnormalities were detected, but in the propositus the lentigines were associated with retardation of growth, hypertelorism, abnormal genitalia and complete atrioventricular block. The possible explanations for the lack of pleomorphy in other members of the family are discussed.

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Congenital Cardiac Arrhythmia

Cited by 94 publications

References 0 publications

A Family with Heritable Electrocardiographic QT-prolongation

A Family with Heritable Electrocardiographic QT-prolongation

Prolonged Q‐t Interval and Cardiac Syncopes

Cardio‐cutaneous syndrome (the “LEOPARD” syndrome). Review of the literature and a new family

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