دورية أكاديمية
Cardiovascular Manifestations and Complications of Loeys-Dietz Syndrome: CT and MR Imaging Findings.
| العنوان: | Cardiovascular Manifestations and Complications of Loeys-Dietz Syndrome: CT and MR Imaging Findings. |
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| المؤلفون: | Loughborough WW; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Minhas KS; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Rodrigues JCL; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Lyen SM; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Burt HE; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Manghat NE; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Brooks MJ; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Stuart G; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.)., Hamilton MCK; From the Department of Clinical Radiology, University Hospitals Bristol NHS Foundation Trust, Upper Maudlin St, Bristol BS2 8HW, England (W.W.L., K.S.M., J.C.L.R., S.M.L., H.E.B., N.E.M., M.C.K.H.); Department of Vascular Surgery, North Bristol NHS Trust, Bristol, England (M.J.B.); and Department of Cardiology (Congenital Heart Disease), Bristol Royal Hospital for Children/Bristol Heart Institute, Bristol, England (G.S.). |
| المصدر: | Radiographics : a review publication of the Radiological Society of North America, Inc [Radiographics] 2018 Jan-Feb; Vol. 38 (1), pp. 275-286. |
| نوع المنشور: | Journal Article; Review |
| اللغة: | English |
| بيانات الدورية: | Publisher: Radiological Society of North America Country of Publication: United States NLM ID: 8302501 Publication Model: Print Cited Medium: Internet ISSN: 1527-1323 (Electronic) Linking ISSN: 02715333 NLM ISO Abbreviation: Radiographics Subsets: MEDLINE |
| أسماء مطبوعة: | Publication: Oak Brook, IL : Radiological Society of North America Original Publication: [Easton, Pa.] : The Society, [c1981- |
| مواضيع طبية MeSH: | Loeys-Dietz Syndrome/*complications , Loeys-Dietz Syndrome/*diagnostic imaging , Magnetic Resonance Imaging/*methods , Tomography, X-Ray Computed/*methods, Abnormalities, Multiple/diagnostic imaging ; Humans ; Phenotype |
| مستخلص: | Loeys-Dietz syndrome (LDS) is a recently described genetic connective tissue disorder with a wide spectrum of multisystem involvement. LDS is characterized by rapidly progressive aortic and peripheral arterial aneurysmal disease. LDS and the other inherited aortopathies such as Marfan syndrome have overlapping phenotypic features. However, LDS is characterized by a more aggressive vascular course; patient morbidity and mortality occur at an early age, with complications developing at relatively smaller aortic dimensions. In addition, there is more diffuse arterial involvement in LDS, with a large proportion of patients developing aneurysms of the iliac, mesenteric, and intracranial arteries. Early diagnosis and careful follow-up are essential for ensuring timely intervention in patients with arterial disease. Cross-sectional angiography has an important role in the baseline assessment, follow-up, and evaluation of acute complications of LDS, the thresholds and considerations of which differ from those of other inherited aortopathies. In this article, LDS is compared with other genetic vascular connective tissue disorders. In addition, the genetic, histopathologic, and cardiovascular manifestations of this disease process are reviewed, with a focus on computed tomographic and magnetic resonance imaging findings. Online DICOM image stacks and supplemental material are available for this article. © RSNA, 2018. |
| تواريخ الأحداث: | Date Created: 20180111 Date Completed: 20180830 Latest Revision: 20180830 |
| رمز التحديث: | 20221213 |
| DOI: | 10.1148/rg.2018170120 |
| PMID: | 29320330 |
| قاعدة البيانات: | MEDLINE |
| تدمد: | 1527-1323 |
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| DOI: | 10.1148/rg.2018170120 |