Natural history of trisomy 13.

Abstract:

:The poor prognosis of patients with trisomy 13 has long been accepted and has been ascribed to brain and heart malformations. It has been suggested, however, that the long term survival is better than was previously thought and that cardiac surgery may be justified. This population based study reviews the incidence, antenatal diagnosis, spectrum of survival from congenital heart disease, and mode of death for patients with trisomy 13 in the Northern Health Region from 1985 to 1992. There was an observed prevalence at birth of 0.049/1000 live births and an expected prevalence, allowing for antenatal diagnosis, of 0.077. None of the cardiac lesions found would cause early death. The median survival in this series was four days; the longest survival was 3.5 months. The principal mode of death was apnoea in 14 of 16 children, irrespective of the presence of a cranial abnormality. In the light of these findings, cardiac surgery cannot be justified in patients with trisomy 13.

journal_name

Arch Dis Child

authors

Wyllie JP,Wright MJ,Burn J,Hunter S

doi

10.1136/adc.71.4.343

subject

Has Abstract

pub_date

1994-10-01 00:00:00

pages

343-5

issue

4

eissn

0003-9888

issn

1468-2044

journal_volume

71

pub_type

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