Risk of aortic aneurysm and dissection in patients with autosomal-dominant polycystic kidney disease: A nationwide population-based cohort study

Although cardiovascular complications are the most common cause of death in patients with autosomal-dominant polycystic kidney disease (ADPKD), the incidence and risk of aortic aneurysm and dissection (AAD) in ADPKD remains unclear due to limited data and insufficient cases. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to do a population-based cohort study (1997-2008). After excluding those patients with age < 18 years old and initially concomitant diagnoses of end-stage renal disease and AAD, a total of 2076 ADPKD patients were selected from 1,000,000 of general population. Additionally, the non-ADPKD group was set up as comparison group in 1:10 ratio after matching with age, gender, income and urbanization (n=20760). The result showed that ADPKD group had higher frequency of comorbidities than non-ADPKD group. The frequency of AAD in ADPKD was significantly higher than in general population (0.92% v.s. 0.11%, p < 0.0001). Of them, 58% of AAD were acute aortic dissection. In addition, Kaplan-Meier analysis demonstrated that cumulative incidence of AAD was remarkably higher in the ADPKD than non-ADPKD group (p < 0.001). The mean time period from ADPKD diagnosis to AAD occurrence was 4.02±3.16 years. After adjusting for age, gender and comorbidities, the ADPKD patients had up to 5.49-fold greater risk for AAD occurrence as compared to non-ADPKD counterparts (95% CI 2.86-10.52, p < 0.0001). Particularly, those patients with co-existing ADPKD and hypertension had very high risk for future development of AAD. In conclusion, the risk of AAD significantly increases in patients with ADPKD as compared with those of general population.