Middle Aortic Syndrome: Some Diagnostic and Therapeutic Considerations

Abstract

Introduction was 240/120mmHg measured in the right arm and 200/90 on the left. On auscultation, bronchial wheezAn isolated, segmental occlusion of the distal thoracic ing was present. Femoral pulses were absent. The or upper abdominal aorta is an unfrequent occurrence, arterial blood gas (ABG) values were as follows: pH usually named the middle aortic syndrome (MAS). In 7.34, pCO2, 4.4kPa, pO2 7.7kPa, and the arterial oxygen some cases narrowing of the visceral branches and/ saturation was 88%. The sedimentation rate was or renal arteries are associated with this entity. Its 75mm/hour, urea nitrogen 14.5mmol/l, serum creaetiology is controversial, whether congenital or autoatinine 187lmol/l, and the fibrinogen level was 4.6g/l. immune, but most often Takayasu’s arteritis is menCoagulation tests were normal. Immunoglobulins tioned as an underlying pathology. The key features (IGG, IgA, IgM) were within the normal range, anti for the diagnosis are hypertension and lower-limb ds DNA was 33.2 IU/ml, and the Le test was negative, claudication with decreased or absent femoral pulses as well as antinuclear antibodies (ANA) and antiin young females. Angiography is mandatory to demitochondrial antibodies (AMA). Chest X-ray revealed lineate the location and extent of the disease. Severe cardiomegaly, and signs of left ventricular enlargement complications such as impairment of cardiac or renal were present on electrocardiogram. The echofunction as well as cerebral haemorrhage may occur cardiography showed a left ventricular wall diameter after a prolonged course of the disease. Therefore, (LVWD) of 14mm, left atrial diameter (LAD) of 48mm surgical treatment is indicated in most cases. Aortoand the interventricular septum diameter (IVSD) of aortic bypass is the accepted procedure, followed by 15mm. Left ventricular ejection fraction (Simpson) was the visceral and renal revascularisation if necessary. 60%. CT scan revealed total occlusion of the abdominal We present the case of a 39-year-old woman with aorta between the coeliac axis and the renal arteries, the occlusion of the middle abdominal aorta with with calcification present in the aortic wall within the impairment of the renal and cardiac function, and affected region. Stenoses of the proximal portion of progressive lower-limb ischaemia, whose surgical the left subclavian artery and the distal thoracic aorta treatment is discussed. were obvious on transaxillary aortography. The descending thoracic aorta showed ectasia. The visceral and parietal collaterals were well developed around the aortic occlusion with a predominant Riolan arch. Case Report The infrarenal aorta was hypoplastic and free of atherosclerotic plaques as shown on the transfermoral anA 39-year-old female, with a previous history of smokgiogram (Fig. 1). ing, hypertension and claudication was admitted beThe patient was treated with bronchodilatators, stercause of heavy breathing, headache, dizziness and oids and antihypertensive drugs. Her respiratory connon-critical lower-limb ischaemia. Her blood pressure dition improved, but the hypertension remained resistant to conservative treatment, and the signs of ∗Please address all correspondence to: M. Petrunic, Department of the lower-limb ischaemia showed progression. She Surgery, University Hospital Rebro, Kispaticeva 12, 10000 Zagreb, Croatia. was therefore scheduled for surgical treatment. The