Aortic Aneurysm, Congenital Heart Defect, Hypertension, Ischemic Heart Disease, Turner Syndrome
Conditions
Keywords
Turner Syndrome, Aortic Aneurysm, Ischemic heart disease, Hypertension, Congenital Heart Defect, Pulse Wave Velocity, Augmentation Index, Central Blood Pressure, Agatston Score, Segment involvement score
Brief summary
Turner syndrome is a congenital complete or partial lack one of the female sex chromosomes affecting 1 of 2000 live born girls. The syndrome is characterized by an increased prevalence of ischemic heart disease, aortic dilation and dissection, hypertension, stroke and autoimmune diseases in general. Our study aim is: * Using MRI to further characterize and find risk factors leading to aortic dilation. * Using MRI to assess the degree of aortic distensibility. * Using Computed tomography x-ray scanning of the heart and coronary arteries to characterize the prevalence and degree of ischemic heart disease and coronary anomalies. * Using pressure sensitive ultrasound (applanation tonometry) to assess the degree of aortic stiffness when compared to controls using end points as Pulse Wave Velocity, Augmentation Index and Central Pulse Pressure.
Detailed description
Turner syndrome is a congenital complete or partial lack one of the female sex chromosomes affecting 1 of 2000 live born girls. The syndrome is characterized by an increased prevalence of ischemic heart disease, aortic dilation and dissection, congenital heart defects, hypertension, stroke, ovarian failure, infertility, decreased final height and autoimmune diseases in general Our study aim is: * Using MRI to further characterize and find risk factors leading to aortic dilation. * Using MRI to describe aortic dimensions * Using MRI to assess the degree of aortic distensibility. * Using Computed tomography x-ray scanning of the heart and coronary arteries to characterize the prevalence and degree of ischemic heart disease and coronary anomalies. * Using pressure sensitive ultrasound (applanation tonometry) to assess the degree of aortic stiffness when compared to controls using end points as Pulse Wave Velocity, Augmentation Index and Central Pulse Pressure.
Interventions
None listed
Sponsors
Study design
Eligibility
Inclusion criteria
with respect to individuals acting as controls: * Healthy age matched females
Exclusion criteria
* Contraindications for MRI * Contraindications for CT * Severe obesity * Acute og Chronic disease with known or presumed significance with concern to outcomes of the study * Previous or present malignant disease * Clinically significant liver disease * Mechanic or biological heart valves
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Aortic distensibility | Once | Aortic distensibillity as measured by MRI |
| Aortic dimensions | Once | Aortic dimensions measured by MRI |
| Agatston score | Once | Coronary CT evaluation of Agatston score |
| Segment involvement score | Once | Segment involvement score (SIS) calculated as the sum of segments with one or more coronary plaques (score 0-18). |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Central blood pressure | Once | Central blood pressure measured by applanation tonometry. |
| 24 hour blood pressure | 24 hour | 24 hour blood pressure |
| Pulse Wave Velocity | Once | Pulse Wave Velocity measured by applanation tonometry. |
| Augmentation index | Once | Augmentation index measured by applanation tonometry. |
Other
| Measure | Time frame | Description |
|---|---|---|
| echocardiography | Once | echocardiography evaluating Mitral and aortic valves, left ventricular hypertrophy, diastolic and systolic function etc.. |
Countries
Denmark