Turner syndrome and acute type A aortic dissection: a national inpatient analysis of risk and outcomes

Abstract Background: Although acute type A aortic dissection (ATAAD) is a recognised complication of Turner syndrome, population-level data describing its prevalence and outcomes remain limited. Methods: We queried the National Inpatient Sample (2002–2020) and generated three groups: TS + ATAAD+, TS + ATAAD−, and TS−ATAAD+ in our retrospective observational cohort study. Primary outcomes were ATAAD hospital admission rate and in-hospital mortality; secondary outcomes included length of stay. Sampling weights were applied to all analyses. Results: A total of 51,426 Turner syndrome hospitalisations were identified. Median ages were 40 years (interquartile range 31–50) for TS + ATAAD+ and 32 years (interquartile range 9–49) for TS + ATAAD− ( p < 0.001). Overall, 0.27% of Turner syndrome hospitalisations presented with ATAAD. Compared with TS + ATAAD−, those with ATAAD had higher mortality (13.1% vs 2.1%, p < 0.001) and longer length of stay (median 8 vs 3 days, p < 0.05). Rates of bicuspid aortic valve (34.3% vs 3.5%, p < 0.001), hypertension (46.0% vs 25.7%, p < 0.05), and complete heart block (14.6% vs 0.8%, p < 0.001) were also higher. Multivariable analysis showed bicuspid aortic valve (adjusted odds ratio 24.31, 95% CI 8.8–67.3) and age (adjusted odds ratio 1.02, 95% CI 1.01–1.04) predicted ATAAD. Compared with TS−ATAAD+ patients, those withTS + ATAAD+ were younger (40 vs 68 years, p < 0.001), while mortality, length of stay, hypertension, and bleeding rates were similar. Conclusion: ATAAD is highly lethal in patients with and without Turner syndrome. In Turner syndrome, ATAAD is associated with higher mortality, longer hospitalisation, and higher rates of bicuspid valve disease and hypertension.