Author + information
- Yoshito Kadoya1,
- Kan Zen2,
- Noriyuki Wakana2,
- Kenji Yanishi2,
- Naohiko Nakanishi2,
- Takeshi Nakamura2 and
- Satoaki Matoba2
Patient initials or identifier number
Relevant clinical history and physical exam
A 60-year-old Japanese man visited our hospital in May 2016 because he developed bilateral intermittent claudication and a small ulcer on his right first toe. He had a history of smoking (Brinkman index score, 400). His medical history included hypertension, dyslipidemia, and fatty liver. He was on dual antiplatelet therapy (aspirin [100 mg/day] and clopidogrel [75 mg/day]) for Raynaud syndrome.
Relevant test results prior to catheterization
Connective-tissue disease screening revealed a positive lupus anticoagulant test, whereas tests for other markers, including antinuclear antibody, were negative. The ankle-brachial index was 0.77 and 0.75 for the right and left leg, respectively. Computed tomography (CT) angiography revealed severe infrarenal aortic stenosis; no atherosclerotic lesions observed in the other areas, such as the thoracic aorta, iliac arteries, or femoropopliteal arteries.
Relevant catheterization findings
The initial angiography showed severe infrarenal aortic stenosis with no calcified lesions (Video 2). Intravascular ultrasound showed a non-calcified plaque with an irregular surface, suggestive of a thrombotic lesion. The diameter of the aorta was >16 mm, and the lesion was approximately 55 mm in length. The patient was diagnosed with infrarenal aortic stenosis, and primary antiphospholipid syndrome (APS) was suspected to be the underlying cause.
Since anticoagulant therapy alone was considered insufficient, endovascular treatment (EVT) was performed using balloon-expandable stents (Palmaz XL stent). First, a 12-French (Fr) sheath was inserted via the right femoral artery as the device access route; a 9-Fr temporary occlusion balloon guiding catheter (OPTIMO®) was inserted via the left femoral artery to prevent distal embolization, and a 4-Fr sheath was inserted via the left radial artery to inject the contrast media. Next, we performed direct stenting using two Palmaz XL stents (40 mm) on a 15-mm balloon because self-expandable stents are associated with a risk of malapposition due to under-sizing. Thereafter, angiography showed plaque protrusion in the proximal site of the stent and plaque shifting in the terminal aorta (Figure 1). Finally, we implanted an additional Palmaz XL stent in the proximal site (Figures 2 and 3). The plaque shifting in the terminal aorta was considered acceptable. The final angiography showed no complications, including aortic dissection or distal embolization (Video 4). After the EVT, the ulcer resolved quickly. Three months later, CT angiography showed good expansion of the stents and disappearance of the shifted plaque in the terminal aorta. A retest for lupus anticoagulant was positive, and therefore a final diagnosis of primary APS was made. We concluded that the infrarenal aortic stenosis was due to thrombotic complications of primary APS.
Arterial thrombosis is a well-known complication of APS, occurring in 66% of Japanese patients. However, infrarenal aortic stenosis due to thrombotic APS is extremely rare. In this case study, we describe the successful use of EVT for infrarenal aortic stenosis due to primary APS. EVT has good long-term patency and a reduced perioperative mortality rate as compared to surgical treatment options, such as aortobifemoral bypass or aortic endarterectomy. The appropriate stenting strategy depending on the lesion characteristics and vessel diameter should be considered to avoid stent malapposition, aortic dissection or distal embolization. In the patients with infrarenal aortic stenosis with less atherosclerotic burden, screening tests for APS should be conducted.