Do Familial Aortic Dissections Tend to Occur at the Same Age?

Ann Thorac Surg. 2017 Feb;103(2):546-550. doi: 10.1016/j.athoracsur.2016.06.007. Epub 2016 Aug 25.

Abstract

Background: Prediction of the age at aortic dissection for family members of aortic dissection patients would enhance early detection and clinical management. We sought to determine whether these dissections tend to cluster by age in family members of the dissection patients.

Methods: Ages at dissection were examined, including 51 sporadic dissectors (one dissection in family) and 39 familial dissectors (two or more dissections in family) from 16 families. Differences between sporadic and familial dissectors and relationships between ages at dissection in family members were analyzed by linear regression and clustering analysis.

Results: Age at dissection was significantly younger in familial versus sporadic dissectors (54.1 ± 15.2 years versus 63.1±12.4 years, p = 0.002). Regression analysis of pairs of family member ages at dissection found a moderately close linear fit (R2 = 0.35). Cluster analysis indicated that age at onset of family dissectors increases as age of proband dissector increases. More than 50% of familial dissections occurred within 10 years of the median onset age for any given age decade. For familial dissectors with onset age of 30 to 49 years, 71% of other family member dissections also occurred at age 30 to 49 years, and no dissections occurred above the age of 63 years. For familial dissectors with onset age of 60 to 79 years, 80% of other family member dissections occurred beyond the age of 50 years.

Conclusions: Familial dissections occur earlier than sporadic dissections. Dissections cluster by age in families, and age at onset can predict the age of other dissectors. This finding argues for consideration of prophylactic resection of an aneurysm in family members approaching the age at onset of prior thoracic aortic dissection.

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Aged, 80 and over
  • Aortic Aneurysm, Thoracic / diagnosis
  • Aortic Aneurysm, Thoracic / epidemiology*
  • Aortic Aneurysm, Thoracic / genetics
  • Aortic Rupture / diagnosis
  • Aortic Rupture / epidemiology*
  • Cause of Death*
  • Cluster Analysis
  • Female
  • Genetic Predisposition to Disease / epidemiology*
  • Humans
  • Incidence
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Registries*
  • Retrospective Studies
  • Risk Assessment
  • Survival Analysis

Supplementary concepts

  • Aortic Aneurysm, Familial Thoracic 1