Çocuk Sağlığı ve Hastalıkları Dergisi 2016 , Vol 59 , Num 3
Midaortic syndrome may be a cause of systemic hypertension in Turner syndrome
Nilüfer Çetiner *1 ,Elif Günay *2 ,Berna Şaylan Çevik *3 ,Figen Akalın *4
1 Adıyaman Üniversitesi Tıp Fakültesi Çocuk Kardiyoloji Uzmanı, Adıyaman
2 Marmara Üniversitesi Tıp Fakültesi Çocuk Kardiyoloji Yandal Asistanı, İstanbul
3 Marmara Üniversitesi Tıp Fakültesi Pediatri Doçenti, İstanbul
4 Marmara Üniversitesi Tıp Fakültesi Pediatri Profesörü, İstanbul
Middle aortic syndrome (MAS) is characterized by diffuse narrowing of abdominal or distal thoracic aorta and may be associated with genetic syndromes. A case of MAS accompanying Turner’s syndrome is presented in this article. A 12-year-old patient with Turner Syndrome was referred because of systemic hypertension. Physical examination revealed II-III/VI grade murmur at right sternal border. Right femoral pulse was nonpalpable, left was weak. Blood pressure at upper extremities was 120/80 mmHg. Telecardiogram showed dilated aortic root. Electrocardiogram revealed left ventricule hypertrophy. Echocardiography showed bicuspid aortic valve, mild aortic stenosis, tortuosity and incrased flow velocity in descending thoracic aorta without diastolic tail. Catheter-angiography demonstrated tortuosity of descending aorta without pressure gradient between proximal and distal parts. Aortogram showed diffuse narrowing of distal thoracic and abdominal aorta. Follow-up with antihypertensives was planned. MAS is a known entity, association with Turner syndrome is rare. Our case gives a new perspective for evaluation of hypertension in Turner’s syndrome. Anahtar Kelimeler : midaortic syndrome, coarctation of aorta, systemic hypertension, Turner’s syndrome
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