Survival, causes of death, and cardiovascular events in patients with Marfan syndrome.

ABSTRACT:

Background

To explore survival, causes of death, and the prevalence of cardiovascular events in a Norwegian Marfan syndrome (MFS) cohort. MFS is a heritable connective tissue disorder associated with reduced life expectancy-primarily due to aortic pathology.

Methods

A follow-up study of 84 MFS adults, initially investigated in 2003-2004. In 2014-2015, 16 were deceased, 47 of 68 survivors consented to new clinical investigations. Analyses of events were performed for 47 survivors and 16 deceased at follow-up. Standardized mortality ratios (SMR), using the mortality rate of the Norwegian population as reference, were calculated for all 84 and calculated for men and women separately. Causes of death and information on cardiovascular events were retrieved from death certificates

SUBMITTER: Vanem TT 

PROVIDER: S-EPMC6305663 | biostudies-literature | 2018 Nov

REPOSITORIES: biostudies-literature