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 Table of Contents  
Year : 2016  |  Volume : 28  |  Issue : 3  |  Page : 128-130

Bicuspid aortic valve associated with aortic thrombotic occlusion

Department of Cardiology, Siyami Ersek Thoracic and Cardiovascular Surgery Center, Training and Research Hospital, Istanbul, Turkey

Date of Submission20-Nov-2015
Date of Acceptance01-Dec-2015
Date of Web Publication27-Feb-2017

Correspondence Address:
Altuğ Ösken
Dr. Siyami Ersek Thoracic and Cardiovascular Surgery Center, Cardiology Clinic, Tibbiye Cad. 13, 34846 Kadikoy, Istanbul
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-7782.200969

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The bicuspid aortic valve is associated with disorders of the aortic wall, including coarctation of the aorta and aortic dissection. The histologic abnormality underlying aortic complications in bicuspid aortic valve is cystic medial necrosis. The elastic properties of the aorta are abnormal in bicuspid aortic valve. Here, we present a case of aortic thrombotic occlusion in a patient with a bicuspid aortic valve.

Keywords: aortic occlusion, bicuspid aortic valve, Leriche syndrome

How to cite this article:
Zehir R, Ösken A, Öz TK, Dayı &&. Bicuspid aortic valve associated with aortic thrombotic occlusion. Egypt J Intern Med 2016;28:128-30

How to cite this URL:
Zehir R, Ösken A, Öz TK, Dayı &&. Bicuspid aortic valve associated with aortic thrombotic occlusion. Egypt J Intern Med [serial online] 2016 [cited 2020 Oct 29];28:128-30. Available from: http://www.esim.eg.net/text.asp?2016/28/3/128/200969

  Introduction Top

The bicuspid aortic valve (BAV) affects 1–2% of the population and may be complicated by aortic stenosis or aortic insufficiency and associated with abnormalities of the aortic wall, such as coarctation of the aorta, aortic dissection, and aortic aneurysm [1]. The increased risk for aortic disease in patients with BAV, including those that function normally, is mediated by coexisting defects in the aortic media, including fragmentation of elastin, loss of smooth muscle cells, and an increase in collagen. Thrombotic occlusion of the abdominal aorta just above the site of its bifurcation, known as Leriche syndrome, is related to severe atherosclerotic disease, hypercoagulable states, and low flow states secondary to cardiac dysfunction or severe volume depletion [2]. Herein, we present a 35-year-old man with BAV-developed Leriche syndrome.

  Case Report Top

A 35-year-old man was admitted to our institution with a history of severe hip and buttock pain of 10 days duration. The pain was aching in nature, associated with weakness of the thigh and hip on walking and relieved on rest. He was a heavy smoker and under investigation for erectile dysfunction. He had no hypertension or hyperlipidemia. Pulsation was present in both brachial arteries, but bilateral femoral and popliteal pulses were absent on examination. Blood pressure was the same in both arms. Diastolic decrescendo murmur 3/6 in severity, best heard at the left sternal border, and systolic ejection click were found on cardiac auscultation. On both sides from the groin to the feet, duplex ultrasonography examination revealed monophasic doppler signals. Transthoracic echocardiography showed no segmental wall motion abnormality, mild degree of aortic regurgitation, and BAV characterized by systolic doming and an eccentric valve closure in the parasternal long-axis view [Figure 1]a and single commissural line in diastole with two cusps and two commissures in the parasternal short-axis view [Figure 1]b. In doppler echocardiographic analyses, significant gradient was not observed above the aortic valve and left ventricular outflow tract. Screening for thrombophilia including AT III, proteins C and S, plasminogen, fibrinogen, factor V Leiden, prothrombin 20210A polymorphism, MTHFR mutation and antiphospholipid antibodies-lupus anticoagulant, and β2-glycoprotein I was negative. Because of the patient's progressive clinical complaints, which were supported with duplex ultrasonographic evidence, we decided to perform a diagnostic angiography.
Fiugre 1: (a) Parasternal long-axis view showing systolic doming of the aortic valve. (b) Parasternal short-axis view showing two cusps.

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An aortogram was performed through the brachial artery route revealing no false lumen or intimal flap but complete thrombotic occlusion of the aorta just below the renal arteries (Leriche syndrome) [Figure 2]. An aorta-bifemoral bypass operation was planned.
Figure 2: Thrombotic occlusion of the abdominal aorta just below the renal arteries.

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  Discussion Top

BAV being the most common congenital cardiovascular anomaly affects 1–2% of the population. The exact mechanism triggering the development of BAV remains unclear. Some researchers have implicated anomalous behavior of cells derived from the neural crest as a possible etiology [1], whereas others suggest that a molecular abnormality in the extracellular matrix may lead to abnormal valvulogenesis given that matrix proteins help to direct cell differentiation and cusp formation during valvulogenesis [3].

Although the BAV is usually an isolated defect, 20% and possibly up to 50% of subjects with BAV have additional congenital cardiovascular malformations such as coarctation of the aorta, Turner's syndrome, patent ductus arteriosus, supravalvular aortic stenosis, congenital coronary anatomic variants, and ventricular septal defect. The presence of a BAV can account for a significant degree of the morbidity associated with these associated syndromes. The most common complication is aortic stenosis. Moreover, BAV is related to aortic medial abnormalities including aortic dissection, ascending aortic dilation, and aneurysm formation. Loss of smooth muscle cells due to apoptosis and severe degeneration of the medial elastic fibers – so-called 'cystic medial necrosis' – are histological hallmarks of these abnormalities [4]. In contrast, these abnormalities may be mediated at least in part by increased release of matrix metalloproteinase-2 from microfibrils due to deficient expression of fibrillin-1, the protein that is abnormal in Marfan syndrome. The fibrillin-1 abnormality is also found in the bicuspid valve [5].

Aortoiliac occlusive disease, also referred to as Leriche syndrome, is the triad of claudication, impotence, and absent femoral pulses due to thrombotic occlusion of the abdominal aorta just above the site of its bifurcation [2]. The absence of bilateral femoral pulses associated with ischemic signs/symptoms of both lower extremities is a key finding in aortic occlusion. Bilateral aortoiliac disease that is severe enough to cause symptoms almost always causes impotence in men; another diagnosis should therefore be entertained if impotence is absent. Risk factors include hyperlipidemia, hypertension, diabetes mellitus, and smoking. Osteoarthritis of the hip and neurogenic claudication due to lumbar neurospinal canal compression are two differential diagnoses of bilateral aortoiliac occlusive disease. Aortic thrombosis is related to underlying atherosclerotic disease, hypercoagulable states, and low flow states secondary to cardiac dysfunction or severe volume depletion [6].

In our case, the only risk factor for thrombotic occlusion of the abdominal aorta is smoking. The patient also had BAV accepted as an aortopathy. Although aortic medial changes in BAV are confined to the aortic root and paracoarctation area if present, thrombotic changes leading to total occlusion of the abdominal aorta may be accelerated by cytic medial necrosis due to BAV.

To our knowledge, in the literature no coexistence of BAV with Leriche syndrome has been reported. Although they are two different clinical entities, one's disease process may be an aggrevating factor for the other.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Mancuso D, Basso C, Cardaioli P, Thiene G Clefted bicuspid aortic valve. Cardiovasc Pathol 2002; 11:217–220.  Back to cited text no. 1
Leriche R, Morel A. The syndrome of thrombotic obliteration of the aortic bifurcation. Ann Surg 1943; 127:193–206.  Back to cited text no. 2
Eisenberg LM, Markwald RR. Molecular regulation of atrioventricular valvuloseptal morphogenesis. Circ Res 1995; 77:1–6.  Back to cited text no. 3
Bonderman D, Gharehbaghi-Schnell E, Wollenek G, Maurer G, Baumgartner H, Lang IM. Mechanisms underlying aortic dilatation in congenital aortic valve malformation. Circulation 1999; 99: 2138–2143.  Back to cited text no. 4
Fedak PW, de Sa MP, Verma S, Nili N, Kazemian P, Butany J, et al. Vascular matrix remodeling in patients with bicuspid aortic valve malformations: implications for aortic dilatation. J Thorac Cardiovasc Surg 2003; 126:797–806.  Back to cited text no. 5
Surowiec SM, Isiklar H, Sreeram S, Weiss VJ, Lumsden AB. Acute occlusion of the abdominal aorta. Am J Surg 1998; 176:193–197.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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