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Life expectancy in British Marfan syndrome populations |
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| Authors: | JR Gray AB Bridges RR West L. McLeish AG Stuart JCS Dean MEM Porteous M. Boxer SJ Davies |
| Affiliation: | Institute of Medical Genetics, University Hospital of Wales, Cardiff CF4 4XW, Wales;Stirling Royal Infirmary NHS Trust, Livilands, Stirling, FK8 2AU, Scotland;Reader in Epidemiology, University of Wales College of Medicine, Cardiff;Medical Genetics, Department of Pathology, Ninewells Hospital and Medical School, Dundee;Department of Cardiology, University Hospital of Wales, Cardiff, Wales;Department of Medical Genetics, Aberdeen Royal Hospitals NHS Trust, Aberdeen Royal Infirmary, Aberdeen;Department of Medical Genetics, Western General Hospital, Edinburgh, UK |
| Abstract: | A total of 206 patients with Marfan syndrome were ascertained throughout genetic clinics in Wales and Scotland during the period 1970–1990. There were 45 deaths representing 22% of the cohort. Mean age at death was 45.3 ± 16.5 years. 50% median cumulative survival in the total cohort (n = 206) was 53 years for males and 72 years for females. Multivariate analysis confirmed severity as the best independent indicator of survival. These findings and survival curves will assist in the counselling of British families and individuals with Marfan syndrome. |
| Keywords: | life expectancy Marfan syndrome survival |