Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?

Up to 25% of patients with thoracic aortic disease have an underlying Mendelian pathogenic variant. This is a heterogeneous group of disorders known as heritable thoracic aortic diseases (HTAD). Diagnosing associated pathogenic gene variants and syndromes is critical, as the underlying genetics have...

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Main Authors: Valeria E. Duarte, Raman Yousefzai, Michael N. Singh
Format: Article
Language:English
Published: Houston Methodist DeBakey Heart & Vascular Center 2023-03-01
Series:Methodist DeBakey Cardiovascular Journal
Subjects:
Online Access:https://account.journal.houstonmethodist.org/index.php/up-j-mdbcj/article/view/1218
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author Valeria E. Duarte
Raman Yousefzai
Michael N. Singh
author_facet Valeria E. Duarte
Raman Yousefzai
Michael N. Singh
author_sort Valeria E. Duarte
collection DOAJ
description Up to 25% of patients with thoracic aortic disease have an underlying Mendelian pathogenic variant. This is a heterogeneous group of disorders known as heritable thoracic aortic diseases (HTAD). Diagnosing associated pathogenic gene variants and syndromes is critical, as the underlying genetics have an implication in medical management, surveillance, thresholds for surgical intervention, surgical risk, pregnancy risk, and risk of inheritance by the offspring. Recently released 2022 American College of Cardiology/American Heart Association guidelines for the diagnosis and management of aortic diseases provide specific recommendations to identify patients at risk for heritable conditions and who should undergo genetic testing.
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institution Kabale University
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language English
publishDate 2023-03-01
publisher Houston Methodist DeBakey Heart & Vascular Center
record_format Article
series Methodist DeBakey Cardiovascular Journal
spelling doaj-art-157163a2bdec4dda8697c11d2b50a8de2025-08-20T03:25:50ZengHouston Methodist DeBakey Heart & Vascular CenterMethodist DeBakey Cardiovascular Journal1947-61082023-03-01192242810.14797/mdcvj.12181195Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?Valeria E. Duarte0https://orcid.org/0000-0001-6616-5412Raman Yousefzai1https://orcid.org/0000-0002-3275-0178Michael N. Singh2https://orcid.org/0000-0002-8667-9872Houston Methodist DeBakey Heart & Vascular Center, Houston, Texas; Boston Children’s Hospital, Boston, MassachusettsA.T. Still University-Kirksville College of Medicine (ATSU-KCOM), Kirksville, MissouriBoston Children’s Hospital and Harvard Medical School, Boston, Massachusetts; Brigham and Women’s Hospital, Harvard Medical School, Boston, MAUp to 25% of patients with thoracic aortic disease have an underlying Mendelian pathogenic variant. This is a heterogeneous group of disorders known as heritable thoracic aortic diseases (HTAD). Diagnosing associated pathogenic gene variants and syndromes is critical, as the underlying genetics have an implication in medical management, surveillance, thresholds for surgical intervention, surgical risk, pregnancy risk, and risk of inheritance by the offspring. Recently released 2022 American College of Cardiology/American Heart Association guidelines for the diagnosis and management of aortic diseases provide specific recommendations to identify patients at risk for heritable conditions and who should undergo genetic testing.https://account.journal.houstonmethodist.org/index.php/up-j-mdbcj/article/view/1218heritable thoracic aortic diseases (htad)thoracic aortic aneurysms (tad)marfan syndrome (mfs)genetic testingnonsyndromic htadgenetic variants
spellingShingle Valeria E. Duarte
Raman Yousefzai
Michael N. Singh
Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
Methodist DeBakey Cardiovascular Journal
heritable thoracic aortic diseases (htad)
thoracic aortic aneurysms (tad)
marfan syndrome (mfs)
genetic testing
nonsyndromic htad
genetic variants
title Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
title_full Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
title_fullStr Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
title_full_unstemmed Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
title_short Genetically Triggered Thoracic Aortic Disease: Who Should be Tested?
title_sort genetically triggered thoracic aortic disease who should be tested
topic heritable thoracic aortic diseases (htad)
thoracic aortic aneurysms (tad)
marfan syndrome (mfs)
genetic testing
nonsyndromic htad
genetic variants
url https://account.journal.houstonmethodist.org/index.php/up-j-mdbcj/article/view/1218
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