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

Siti Aishah Ahmad Maulana; Sharifah Huda Engku Alwi; Mohd Shafie Hashim; Rabiatul Adawiyah Abdul Rohim

doi:10.5001/omj.2023.66

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

Oman Med J. 2023 Nov 30;38(6):e576. doi: 10.5001/omj.2023.66. eCollection 2023 Nov.

Authors

Siti Aishah Ahmad Maulana¹, Sharifah Huda Engku Alwi², Mohd Shafie Hashim², Rabiatul Adawiyah Abdul Rohim¹

Affiliations

¹ Faculty of Medicine, Universiti Sultan Zainal Abidin, Terengganu, Malaysia.
² Department of Pediatrics, Hospital Sultanah Nur Zahirah, Terengganu, Malaysia.

Abstract

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.

Keywords: Atrial Septal Defect; Computed Tomography Angiography; Malaysia; Noonan Syndrome.

Publication types

Case Reports