Comparing Sex Differences in Aortic Root to Somatic Growth in Children with Marfan Syndrome in Saskatchewan

Background: Marfan syndrome (MFS) is the most common heritable thoracic aortic disease and can progress to life-threatening aortic dissection without effective management. MFS males have more rapid somatic growth and are more likely to meet surgical indications based on aortic root dimension (AoRD) growth sooner. The aim of this study was to compare sex differences in AoRD to somatic growth in children with MFS in Saskatchewan.

Methods: We conducted a retrospective chart review collecting serial data on height, weight, BMI and AoRD and compared MFS males and females with normative data.

Results: Of 18 MFS patients (14 males), height in MFS tended to be taller compared with normative data for all ages, with males taller than females. Weight and BMI in MFS tended to be similar compared with normative data and with no sex differences. AoRD in MFS tended to be larger compared with normative data and with no sex differences. AoRD for height increased in both male and female MFS patients.

Conclusions: This pilot project is to be expanded within the Canadian Aortopathy and Connective Tissues Disorder (CAN-ACT) Registry, which will include ~600 MFS, to determine whether AoRD for height is a better sex-dependent determinant of AoRD growth.