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:: Volume 1, Issue 1 (3-2015) ::
CJP 2015, 1(1): 28-30 Back to browse issues page
Arterial tortuosity syndrome–A case report from Iran
Hassan Zamani, Mohammad Goodarzi, Kazem Babazadeh *, Mohaddese Mirzapour
Non-Communicable Pediatric Diseases Research Center , kbabazadeh@hotmail.com
Abstract:   (23529 Views)

Introduction: Arterial tortuosity syndrome (ATS), an autosomal recessive rare connective tissue disorder characterized by tortuosity, elongation and stenosis in the large and medium sized arteries. It manifestations include vascular and nonvascular connective tissue related symptoms. In this literature we describe the first cases of ATS from Iran.

Case report: A six-months-old female was presented with a heart murmur in the right upper sternal edge that was followed up for diaphragmatic hernia repair. Positive sign include right axis deviation in electrocardiography (ECG), abnormal long and tortuous aortic arch with tortuosity of its branches in angiography, increased right ventricular pressure up to 60 mmHg and slight right ventricular hypertrophy with severe stenosis of pulmonary artery bifurcation.

Conclusions: ATS does not present with a unique set of clinical features and its manifestations depend on the mutation type. The most common presentation is tortuous artery and more report of this rare case can help to better diagnosis of ATS.

Keywords: Arterial Tortuosity Syndrome, Diaphragmatic Hernia, Axis Deviation
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Type of Study: case report | Subject: Special
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Zamani H, Goodarzi M, Babazadeh K, Mirzapour M. Arterial tortuosity syndrome–A case report from Iran. CJP 2015; 1 (1) :28-30
URL: http://caspianjp.ir/article-1-23-en.html

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Volume 1, Issue 1 (3-2015) Back to browse issues page
مجله کودکان کاسپین Caspian Journal of Pediatrics
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