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The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome

Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged...

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Autores principales: Obara-Moszynska, Monika, Rajewska-Tabor, Justyna, Rozmiarek, Szymon, Karmelita-Katulska, Katarzyna, Kociemba, Anna, Rabska-Pietrzak, Barbara, Janus, Magdalena, Siniawski, Andrzej, Mrozinski, Bartlomiej, Graczyk-Szuster, Agnieszka, Niedziela, Marek, Pyda, Malgorzata
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232706/
https://www.ncbi.nlm.nih.gov/pubmed/30459711
http://dx.doi.org/10.3389/fendo.2018.00609
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author Obara-Moszynska, Monika
Rajewska-Tabor, Justyna
Rozmiarek, Szymon
Karmelita-Katulska, Katarzyna
Kociemba, Anna
Rabska-Pietrzak, Barbara
Janus, Magdalena
Siniawski, Andrzej
Mrozinski, Bartlomiej
Graczyk-Szuster, Agnieszka
Niedziela, Marek
Pyda, Malgorzata
author_facet Obara-Moszynska, Monika
Rajewska-Tabor, Justyna
Rozmiarek, Szymon
Karmelita-Katulska, Katarzyna
Kociemba, Anna
Rabska-Pietrzak, Barbara
Janus, Magdalena
Siniawski, Andrzej
Mrozinski, Bartlomiej
Graczyk-Szuster, Agnieszka
Niedziela, Marek
Pyda, Malgorzata
author_sort Obara-Moszynska, Monika
collection PubMed
description Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged 13.9 ± 2.2 years, were studied. CMR was performed in 39 patients and angio-MR in 36. Echocardiography was performed in all patients. The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the ascending aorta (AA) and aortic arch, present in 16 patients (45.7%), a bicuspid aortic valve (BAV), present in 16 patients (41.0%), and partial anomalous pulmonary venous return (PAPVR), present in six patients (17.1%). Aortic dilatation (Z-score > 2) was mostly seen at the sinotubular junction (STJ) (15 patients; 42.8%), the AA (15 patients; 42.8%), the thoracoabdominal aorta at the level of a diaphragm (15 patients; 42.8%), and the transverse segment (14 patients; 40.0%). An aortic size index (ASI) above 2.0 cm/m(2) was present in six patients (17.1%) and above 2.5 cm/m(2) in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (Z-score < −2) in 10 (25.6%), 9 (23.1%), and 8 patients (20.5%), respectively. A webbed neck was correlated with the presence of vascular anomalies (p = 0.006). The age and body mass index (BMI) were correlated with the diameter of the aorta. Patients with BAV had a greater aortic diameter at the ascending aorta (AA) segment (p = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio (p = 0.002; r = 0.49). There was a significant correlation between the right ventricular (p = 0.002, r = 0.46) and aortic diameters at the STJ segment (p = 0.0047, r = 0.48), as measured by echocardiography and CMR. Magnetic resonance can identify cardiovascular anomalies, dilatation of the aorta, pericardial fluid, and functional impairment of the ventricles not detected by echocardiography. BMI, age, BAV, and elongation of the AA influence aortic dilatation. The ASI and AD/DD ratio are important markers of aortic dilatation. The performed diagnostics did not indicate a negative influence of GH treatment on the cardiovascular system.
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spelling pubmed-62327062018-11-20 The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome Obara-Moszynska, Monika Rajewska-Tabor, Justyna Rozmiarek, Szymon Karmelita-Katulska, Katarzyna Kociemba, Anna Rabska-Pietrzak, Barbara Janus, Magdalena Siniawski, Andrzej Mrozinski, Bartlomiej Graczyk-Szuster, Agnieszka Niedziela, Marek Pyda, Malgorzata Front Endocrinol (Lausanne) Endocrinology Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged 13.9 ± 2.2 years, were studied. CMR was performed in 39 patients and angio-MR in 36. Echocardiography was performed in all patients. The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the ascending aorta (AA) and aortic arch, present in 16 patients (45.7%), a bicuspid aortic valve (BAV), present in 16 patients (41.0%), and partial anomalous pulmonary venous return (PAPVR), present in six patients (17.1%). Aortic dilatation (Z-score > 2) was mostly seen at the sinotubular junction (STJ) (15 patients; 42.8%), the AA (15 patients; 42.8%), the thoracoabdominal aorta at the level of a diaphragm (15 patients; 42.8%), and the transverse segment (14 patients; 40.0%). An aortic size index (ASI) above 2.0 cm/m(2) was present in six patients (17.1%) and above 2.5 cm/m(2) in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (Z-score < −2) in 10 (25.6%), 9 (23.1%), and 8 patients (20.5%), respectively. A webbed neck was correlated with the presence of vascular anomalies (p = 0.006). The age and body mass index (BMI) were correlated with the diameter of the aorta. Patients with BAV had a greater aortic diameter at the ascending aorta (AA) segment (p = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio (p = 0.002; r = 0.49). There was a significant correlation between the right ventricular (p = 0.002, r = 0.46) and aortic diameters at the STJ segment (p = 0.0047, r = 0.48), as measured by echocardiography and CMR. Magnetic resonance can identify cardiovascular anomalies, dilatation of the aorta, pericardial fluid, and functional impairment of the ventricles not detected by echocardiography. BMI, age, BAV, and elongation of the AA influence aortic dilatation. The ASI and AD/DD ratio are important markers of aortic dilatation. The performed diagnostics did not indicate a negative influence of GH treatment on the cardiovascular system. Frontiers Media S.A. 2018-10-16 /pmc/articles/PMC6232706/ /pubmed/30459711 http://dx.doi.org/10.3389/fendo.2018.00609 Text en Copyright © 2018 Obara-Moszynska, Rajewska-Tabor, Rozmiarek, Karmelita-Katulska, Kociemba, Rabska-Pietrzak, Janus, Siniawski, Mrozinski, Graczyk-Szuster, Niedziela and Pyda. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Endocrinology
Obara-Moszynska, Monika
Rajewska-Tabor, Justyna
Rozmiarek, Szymon
Karmelita-Katulska, Katarzyna
Kociemba, Anna
Rabska-Pietrzak, Barbara
Janus, Magdalena
Siniawski, Andrzej
Mrozinski, Bartlomiej
Graczyk-Szuster, Agnieszka
Niedziela, Marek
Pyda, Malgorzata
The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title_full The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title_fullStr The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title_full_unstemmed The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title_short The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome
title_sort usefulness of magnetic resonance imaging of the cardiovascular system in the diagnostic work-up of patients with turner syndrome
topic Endocrinology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6232706/
https://www.ncbi.nlm.nih.gov/pubmed/30459711
http://dx.doi.org/10.3389/fendo.2018.00609
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