Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report

We present a rare case of congenital thoracic aortic aneurysm (TAA) complicated with dissection in a nine-year-old girl with Noonan syndrome and atrial septal defect. She presented with rapid breathing and upper respiratory tract symptoms. Chest X-ray revealed a huge upper mediastinum with cardiomeg...

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Main Author: Siti Aishah Ahmad Maulana
Format: Article
Language:English
Published: Oman Medical Specialty Board 2023-11-01
Series:Oman Medical Journal
Subjects:
Online Access:https://omjournal.org/articleDetails.aspx?coType=1&aId=3584
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author Siti Aishah Ahmad Maulana
author_facet Siti Aishah Ahmad Maulana
author_sort Siti Aishah Ahmad Maulana
collection DOAJ
description We present a rare case of congenital thoracic aortic aneurysm (TAA) complicated with dissection in a nine-year-old girl with Noonan syndrome and atrial septal defect. She presented with rapid breathing and upper respiratory tract symptoms. Chest X-ray revealed a huge upper mediastinum with cardiomegaly. Echocardiogram showed possible ascending TAA. Computerized tomography angiogram of the aorta revealed huge aortic root-ascending TAA with small aortic dissection. Despite immediate treatment, the patient passed away due to severe airway compromise.
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spelling doaj.art-f7566ccbf623483abd5557d6899a1ad62024-01-24T05:29:54ZengOman Medical Specialty BoardOman Medical Journal1999-768X2070-52042023-11-01386e576e57610.5001/omj.2023.66Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case ReportSiti Aishah Ahmad Maulana0Faculty of Medicine, Universiti Sultan Zainal Abidin, Terengganu, Malaysia We present a rare case of congenital thoracic aortic aneurysm (TAA) complicated with dissection in a nine-year-old girl with Noonan syndrome and atrial septal defect. She presented with rapid breathing and upper respiratory tract symptoms. Chest X-ray revealed a huge upper mediastinum with cardiomegaly. Echocardiogram showed possible ascending TAA. Computerized tomography angiogram of the aorta revealed huge aortic root-ascending TAA with small aortic dissection. Despite immediate treatment, the patient passed away due to severe airway compromise.https://omjournal.org/articleDetails.aspx?coType=1&aId=3584atrial septal defectcomputed tomography angiographynoonan syndromemalaysia
spellingShingle Siti Aishah Ahmad Maulana
Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
Oman Medical Journal
atrial septal defect
computed tomography angiography
noonan syndrome
malaysia
title Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
title_full Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
title_fullStr Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
title_full_unstemmed Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
title_short Rare Congenital Dissecting Thoracic Aortic Aneurysm in a Child with Noonan Syndrome: A Case Report
title_sort rare congenital dissecting thoracic aortic aneurysm in a child with noonan syndrome a case report
topic atrial septal defect
computed tomography angiography
noonan syndrome
malaysia
url https://omjournal.org/articleDetails.aspx?coType=1&aId=3584
work_keys_str_mv AT sitiaishahahmadmaulana rarecongenitaldissectingthoracicaorticaneurysminachildwithnoonansyndromeacasereport