Surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval present at birth, and severe anaemia and syncopal attacks in childhood

A case of the surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval in the electrocardiogram at birth, is described. The affected girl presented 3 1/2 years later with severe iron deficiency anaemia, despite apparently adequate nutrition. At the age of 4 1/2 years she had a...

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Bibliographic Details
Published in:British Heart Journal 1975-08, Vol.37 (8), p.830-832
Main Authors: Langslet, A, Sorland, S J
Format: Article
Language:English
Subjects:
1-Propanol - therapeutic use
Anemia, Hypochromic - etiology
Anemia, Hypochromic - genetics
Child
Child, Preschool
Deafness - complications
Deafness - congenital
Electrocardiography
Female
Heart Diseases - complications
Heart Diseases - physiopathology
Heart Rate - drug effects
Humans
Infant, Newborn
Iron - administration & dosage
Practolol - therapeutic use
Syncope - complications
Syncope - drug therapy
Syncope - etiology
Syndrome
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Description
Summary:A case of the surdocardiac syndrome of Jervell and Lange-Nielsen, with prolonged QT interval in the electrocardiogram at birth, is described. The affected girl presented 3 1/2 years later with severe iron deficiency anaemia, despite apparently adequate nutrition. At the age of 4 1/2 years she had a sudden attack of unconsciousness. Treatment with the beta-adrenoceptor blocker practolol was started and 3 years after initiation of this treatment she has been free from syncopal attacks. The QT interval remains prolonged. Her brother also had severe iron deficiency anaemia and had several attacks of unconsciousness before he died suddenly at the age of 3 1/2 years.
ISSN:0007-0769
1355-6037
1468-201X
2053-5864
DOI:10.1136/hrt.37.8.830