Hupkes, A.M. (2020) Larger aortic root diameter in Osteogenesis Imperfecta type III compared to type I and IV. thesis, Medicine.
Full text available on request.Abstract
Background: Aortic root dilatation is the most common cardiovascular abnormality in osteogenesis imperfecta (OI). However, it is still unclear whether the different types of OI have an effect on aortic diameter. The aim of this study was to investigate aortic root diameters in a large cohort of adult patients with different types of OI. Methods: This retrospective, echocardiography study was conducted at the OI expert clinic for adults in The Netherlands and included 316 adults with OI divided into 3 clinical types (I, III and IV). Aortic root diameters were measured at the sinus of Valsalva by transthoracic echocardiography. Multiple variable analyses were performed to assess the potential associations between patient characteristics and aortic root diameter. Results: In 316 adult patients with different types of OI (231 type I, 30 type III and 55 type IV), aortic root diameter ranged from 22 to 44 mm (mean 31.3 mm). Patients with OI type III had significant larger aortic root diameters corrected for BSA (24.8 ± 4.7 mm/m2 ) compared to patients with OI type I (18.2 ± 2.6 mm/m2 (p < .001)) or IV (18.7 ± 3.1 mm/m2 (p < .001)). Aortic root dilatation was found in 76.7% of patients with OI type III, 12.6% of patients with OI type I and in 21.8% of patients with OI type IV. Conclusion: Patients with OI type III have larger aortic root diameters compared to patients with OI type I and IV. In addition, prevalence of aortic root dilatation is more prominent in patients with OI type III. Further research determining the incidence of valvular dysfunction and aortic dissection is necessary to conduct clinical guidelines for detection and monitoring of aortic root dilatation in patients with OI.
| Item Type: | Thesis (UNSPECIFIED) |
|---|---|
| Supervisor name: | Dr. Franken, A.A.M. |
| Faculty: | Medical Sciences |
| Date Deposited: | 30 Oct 2023 12:05 |
| Last Modified: | 30 Oct 2023 12:05 |
| URI: | https://umcg.studenttheses.ub.rug.nl/id/eprint/3716 |
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