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Aortic size predicts aortic dissection in Turner syndrome - A 25-year prospective cohort study.

Department of Clinical Genetics, Sahlgrenska University Hospital Gothenburg, Sweden; Department of Internal Medicine and Clinical Nutrition, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Electronic address: sofia.thunstrom@vgregion.se. Department of Molecular and Clinical Medicine, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden. Department of Clinical Sciences Malmö, Lund University, Lund, Sweden; Department of Endocrinology, Skåne University Hospital, Malmö, Sweden. Center of Reproductive Medicine, Skåne University Hospital, Malmo, Sweden. Department of Endocrinology, Linköping University Hospital, Department of Internal Medicine, Norrköping Hospital, Sweden; Department of Health, Medicine and Caring Sciences, University of Linköping, Linköping, Sweden. Department of Endocrinology, Faculty of Medicine and Health, Örebro University, Örebro, Sweden. Department of Clinical Science, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden; Department of Endocrinology, Metabolism & Diabetes, Karolinska University Hospital, Huddinge, Stockholm, Sweden. Department of Clinical Physiology, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Department of Internal Medicine and Clinical Nutrition, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Respiratory Medicine, Sahlgrenska University Hospital, Gothenburg, Sweden. Department of Internal Medicine and Clinical Nutrition, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Clinic of Endocrinology, Hospital of Halland, Sweden. Reproductive Medicine, Department of Obstetrics and Gynecology, Sahlgrenska University Hospital Gothenburg, Sweden; Department of Obstetrics and Gynecology, Institute of Clinical Sciences Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden. Department of Internal Medicine and Clinical Nutrition, Institution of Medicine, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden; Department of Internal Medicine, Section for Endocrinology, Sahlgrenska University Hospital Gothenburg, Sweden.

International journal of cardiology. 2023;:47-54
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Abstract

BACKGROUND Women with Turner syndrome (TS) have an increased risk of aortic dissection. The current recommended cutoff to prevent aortic dissection in TS is an aortic size index (ASI) of ≥2.5 cm/m2. This study estimated which aortic size had the best predictive value for the risk of aortic dissection, and whether adjusting for body size improved risk prediction. METHODS A prospective, observational study in Sweden, of women with TS, n = 400, all evaluated with echocardiography of the aorta and data on medical history for up to 25 years. Receiver operating characteristic (ROC) curves, sensitivity and specificity were calculated for the absolute ascending aortic diameter (AAD), ascending ASI and TS specific z-score. RESULTS There were 12 patients (3%) with aortic dissection. ROC curves demonstrated that absolute AAD and TS specific z-score were superior to ascending ASI in predicting aortic dissection. The best cutoff for absolute AAD was 3.3 cm and 2.12 for the TS specific z-score, respectively, with a sensitivity of 92% for both. The ascending ASI cutoff of 2.5 cm/m2 had a sensitivity of 17% only. Subgroup analyses in women with an aortic diameter ≥ 3.3 cm could not demonstrate any association between karyotype, aortic coarctation, bicuspid aortic valve, BMI, antihypertensive medication, previous growth hormone therapy or ongoing estrogen replacement treatment and aortic dissection. All models failed to predict a dissection in a pregnant woman. CONCLUSIONS In Turner syndrome, absolute AAD and TS-specific z-score were more reliable predictors for aortic dissection than ASI. Care should be taken before and during pregnancy.

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Publication Type : Observational Study

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