Clinical trial NCT01678274: Prospective Characterization of the Heart, Aorta and Blood Pressure in Turner Syndrome. Association With Aortic Dissection.
Official title: Prospective Characterization of the Heart, Aorta and Blood Pressure in Women With Turner Syndrome. Association With Aortic Dissection.
Study type: OBSERVATIONAL
Overall status: UNKNOWN
Conditions: Turner Syndrome
Aortic Aneurysm
Ischemic Heart Disease
Hypertension
Congenital Heart Defect
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
Lead sponsor: University of Aarhus
Sponsor class: OTHER
First posted: 2012-09-03
Enrollment: 106
Sex: FEMALE
Minimum age (years): 18
Maximum age (years): 90
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: yes
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: Females with Turner Syndrome previously participating in our study number 2001024 are recruited from out-patient clinics.

Controls are healthy volunteers recruited from the general population by advertisement.

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.

Eligibility criteria: 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

Primary outcomes:
measure: Aortic dimensions
timeFrame: Once
measure: Agatston score
timeFrame: Once
measure: Segment involvement score
timeFrame: Once
measure: Aortic distensibility
timeFrame: Once
Secondary outcomes:
measure: 24 hour blood pressure
timeFrame: 24 hour
measure: Pulse Wave Velocity
timeFrame: Once
measure: Augmentation index
timeFrame: Once
measure: Central blood pressure
timeFrame: Once
Other outcomes:
measure: echocardiography
timeFrame: Once
Locations:
city: Aarhus
country: Denmark
facility: Department of Endocrinology and Internal medicine
Study officials:
affiliation: Aarhus University Hospital
name: Claus H Gravholt, MD
role: STUDY_CHAIR
affiliation: Aarhus University Hospital
name: Christian Trolle, MD
role: PRINCIPAL_INVESTIGATOR
References:
citation: Viuff MH, Just J, Brun S, Dam TV, Hansen M, Melgaard L, Hougaard DM, Lappe M, Gravholt CH. Women With Turner Syndrome Are Both Estrogen and Androgen Deficient: The Impact of Hormone Replacement Therapy. J Clin Endocrinol Metab. 2022 Jun 16;107(7):1983-1993. doi: 10.1210/clinem/dgac167.
citation: Wen J, Trolle C, Viuff MH, Ringgaard S, Laugesen E, Gutmark EJ, Subramaniam DR, Backeljauw P, Gutmark-Little I, Andersen NH, Mortensen KH, Gravholt CH. Impaired aortic distensibility and elevated central blood pressure in Turner Syndrome: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson. 2018 Dec 13;20(1):80. doi: 10.1186/s12968-018-0497-0.
citation: Subramaniam DR, Stoddard WA, Mortensen KH, Ringgaard S, Trolle C, Gravholt CH, Gutmark EJ, Mylavarapu G, Backeljauw PF, Gutmark-Little I. Continuous measurement of aortic dimensions in Turner syndrome: a cardiovascular magnetic resonance study. J Cardiovasc Magn Reson. 2017 Feb 24;19(1):20. doi: 10.1186/s12968-017-0336-8.