Objectives: Women with Turner syndrome (TS) have an increased risk of aortic disease, reducing life-expectancy. This study aimed to systematically review the prevalence of thoracic aortic dilatation, aortic dimensions and growth, and the incidence of aortic dissection.
Methods: A systematic literature search was conducted up to July 2022. Observational studies with an adult TS population were included, and studies including children aged <15 years old or specific TS populations were excluded.
Results: In total 21 studies were included. The pooled prevalence of ascending aortic dilatation was 23% (95% CI 19-26) at a mean pooled age of 29 years (95% CI 26-32), while the incidence of aortic dissection was 164 per 100.000 patient-years (95% CI 95-284). Three reporting studies showed aortic growth over time to be limited. Risk factors for aortic dilation or dissection were older age, bicuspid aortic valve, aortic coarctation, and hypertension.
Conclusion: In adult TS women, ascending aortic dilatation is common and the hazard of aortic dissection increased compared to the general population, whereas aortic growth is limited. Conventional risk markers do not explain all aortic dissection cases; therefore, new imaging parameters and blood biomarkers are needed to improve prediction, allowing for patient-tailored follow-up and surgical decision-making.
Keywords: Aortic aneurysm; aortic coarctation; aortic dissection; bicuspid aortic valve; congenital heart disease; thoracic aorta; turner syndrome.