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논문 기본 정보

자료유형
학술저널
저자정보
Kumar Parveen (Department of Radiodiagnosis & Imaging Fortis Escort Heart Institute New Delhi India.)
저널정보
아시아심장혈관영상의학회 Cardiovascular Imaging Asia Cardiovascular Imaging Asia Vol.5 No.1
발행연도
2021.1
수록면
9 - 12 (4page)

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Arterial tortuosity syndrome (ATS) is a rare genetic syndrome characterized by elongation, tortuosity, stenosis, and aneurysm formation of major arteries. A one year ten months old boy presented with a history of repeated chest infections. Cardiovascular examination was normal except for slightly increased heart rate (130 beats per minute). Echocardiography revealed situs solitus, levocardia, D-loop, and atrioventricular and ventricular-arterial concordance. Both right and left pulmonary arteries appeared mildly narrowed in caliber, measuring 7.1 and 9 mm, respectively. CT angiography was advised for detailed evaluation of branch pulmonary arteries. CT showed early branching of the main pulmonary trunk with narrowed caliber branch pulmonary arteries forming an inverted V sign on axial maximum intensity projection images and an V sign on coronal volume rendering technique images. All the three arch branches showed tortuous courses after their origin. The thoraco-abdominal aorta also appeared tortuous in course that extended beyond the normal expected course into adjacent anatomic areas. There was no evidence of any aortic aneurysm or dissection. In view of these typical imaging findings, the diagnosis of ATS was made. This case report highlights the role of radiological imaging to ascertain the diagnosis and detect the complications of ATS.

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