The Syndrome of Familial Defects of Heart and Upper Extremities (Holt-Oram Syndrome)

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The syndrome of familial defects of heart and upper extremities (Holt-Oram syndrome).

THE ASSOCIATION of atrial septal defect and anomalies of the upper extremities specifically involving one or both thumbs was first reported by Holt and Oram in 1960.1 In that same year, McKusick2 referred to this association in one family and used the term "atriodigital dysplasia" as well as the "HoltOram syndrome." Since that time one family has been reported by Zetterqvist,3 one family by Kuh...

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Holt-Oram syndrome vs heart-hand syndrome.

tables or figures and should relate solely to an article published in Circulation within the preceding 12 weeks. Authors of letters selected for publication will receive prepublication proofs, and authors of the article cited in the letter will be invited to reply. Replies must be signed by all authors listed in the original publication. Please submit three typewritten, double-spaced copies of ...

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A Case of Holt - Oram Syndrome

SUMMARY Holt Oram Syndrome consisting of upper limb defcts, cardiac anomalies and narrow shoulder, was first described by Holt and Oram in 1960. The inheritance pattern is autosomal dominant and most commonly encoutered cardiac anomalies being ASD, although all variaties of C.H.D are reported. Upper limb anomalies are not specific, although scaphoid bone deformity is almost characteristic in t...

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The Holt-Oram syndrome.

The classical description of this syndrome of upper limb abnormalities and congenital heart lesions was by Holt and Oram in 1960.1 They were from King's College Hospital in London and reported a four generation family with nine affected subjects. Many other families were then recognised to have the same condition, which led to a series of reports in the early 1960s. The names atriodigital dyspl...

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Holt-Oram syndrome.

A29-year-old woman presented with dizziness. Physical and radiographic examinations showed skeletal hand malformations, ie, digitalized triphalangeal thumbs and dystrophy of the carpal bones. When she was 15 years old, an atrial septal defect had been repaired. ECG recordings showed abnormalities of atrial excitation such as a wandering pacemaker, atrial ectopic activity, AV-nodal block, and si...

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ژورنال

عنوان ژورنال: Circulation

سال: 1966

ISSN: 0009-7322,1524-4539

DOI: 10.1161/01.cir.34.1.65