Case reports
A young woman with Takayasu arteritis: surgical and anesthetic management of Bentall procedure in a patient with abdominal aortic aneurysm and renal artery stenosis

Introduction

Takayasu’s arteritis (TA) is a rare chronic vasculitis that is associated with inflammatory changes in large arteries [1, 2]. Clinical and pathological studies on TA resulted in the classification of four different types of arterial lesions: 1) type I: the involvement is limited to the aortic arch and its branches; this affects 8.4% of patients; 2) type II: the lesions are localized in the descending and abdominal aorta; it concerns 11.2% of cases; 3) type III: the lesions share features of types I and II; it affects 65.4% of patients; 4) type IV: involvement of the pulmonary arteries in addition to the other lesions, which is observed in 15% of patients [3].

In this presentation, we discuss the clinical outcome of a young adolescent female patient with a diagnosis of TA who was scheduled for an elective Bentall procedure after being under appropriate medical treatment for twelve months duration.

Case report

We present a 19-year-old female patient with TA who was on medical treatment for a year prior to admission for an elective Bentall procedure. She was started on a calcium channel blocker agent and on prednisolone treatment one year prior to admission. During her last six-month follow-up visit, she was found to have enlargement of the aorta up to 4 cm in the angiographic study which was not present in the previous studies. Although she was on steroid treatment, dipyridamole and immunosuppressive cyclophosphamide therapies were added to her regimen. Six months after this, she was admitted because of a moderate degree of aortic regurgitation and enlargement of the aorta up to 6 cm. On admission, the clinical findings during the physical examination included elevated superior limb blood pressure (148/92 mm Hg), decreased ratio of inferior to superior limb blood pressures (0.66), and detection of vascular murmurs at the bilateral regions of the neck and the abdomen. The results of hematologic examinations (C-reactive protein: 0.3 mg/dL; ESR: 16 mm/60 min) demonstrated no inflammatory findings. There were no abnormal findings in any other examinations. She was found to have normal biochemical laboratory data and normal chest X-ray without signs of cardiac dilatation or calcification. Occlusive disease and aneurysms were visualized in the ascending, descending and abdominal aorta in the angiographic study (Figs. 1A-B). Left sided renal artery stenosis was observed in this study as well. The visualization of the dilated ascending aorta and aortic insufficiency during dissection of the cardiac structures prior to the operative procedure is shown in Fig. 2. The surgical procedure involving aortic valve replacement (St Jude 21) and placement of a graft on the ascending aorta (Bentall procedure) was performed. Blood pressure, hemodynamic and renal function were adequately regulated throughout the surgery by appropriate anesthetic medical therapies including inhaled anesthetic agents (sevoflurane), an intravenous anesthetic agent (propofol), and intravenous doses of nitroglycerine, dopamine and furosemide. She required intravenous dobutamine at the dose of 5 µ/kg/hour at the end of cardiopulmonary bypass and continued for a total of 6 hours postoperatively. The patient was extubated on the first postoperative day and stayed three days in the intensive care unit. The patient was discharged home on the eighth postoperative day with continuance to her usual medical treatment. At her follow-up visit six months after surgery, her clinical and angiographic studies revealed that she remained stable.

Discussion

Surgery for TA should be deferred in the active phase of the disease as surgical interventions are associated with more bleeding, friable tissue and a high risk of thrombosis [1, 3, 4]. In our present case, the surgical procedure was successful without complications as the patient was well maintained on appropriate medical treatment before and during surgery. As the evaluation of the angiographic studies showed involvement of abdominal and renal arteries, TA can be classified as type III. For this reason, she was followed for deterioration in gastrointestinal and kidney functions after surgery for a week in hospital and during the next six months of the follow-up period. Surgical treatment is reported to be challenging due to the diffuse nature of the disease and involvement of adjacent aortic walls. A high incidence of anastomotic aneurysm formation and graft failure was reported [5, 6]. In a report of 26 patients after a mean follow-up period of five years, it was suggested that surgical treatment of aortic lesions of the abdominal aorta in TA must be reserved for patients whose arterial hypertension is not under control despite medical treatment due to frequently occurring late degeneration of repairs [7]. However, another group of surgeons reported their findings on 10 patients after a 20-year follow-up and their opinion is that children with Takayasu’s arteritis could benefit from reconstructive surgery with successful long-term results for mortality and morbidity [8].

In patients with TA, percutaneous angioplasty or stenting should be considered during long follow-up periods of symptomatic stenotic or occlusive disease. Percutaneous transluminal angioplasty is assuming a valuable role in treating involvement of the renal arteries [9, 10]. Endovascular therapy (usually balloon angioplasty) is safe to control hypertension with success rates ranging between 80 and 96%. The cumulative 5-year patency rate for the management of renal artery stenosis is 67% and the overall complication rates are low. Angioplasty has been reported to show less successful results in relieving obstruction in the mesenteric arteries [4, 11].

In patients with abdominal aortic aneurysm secondary to TA, a consensus on whether these patients should be treated with abdominal aortic resection has not been established. The incidence of abdominal aneurysm rupture has been reported to be low; however, as the disease occurs in young patients, a yearly routine follow-up is required to decide whether an abdominal aortic reconstruction is needed [4, 12].

The diagnosis of Takayasu’s disease was made by clinical presentation, arteriography, and laboratory findings. In our clinic, patients with a diagnosis of TA are routinely assessed by routine physical examination, arteriography every 6 to 12 months and sedimentation rate. The use of steroid treatment is considered in the active state of TA and immunosuppressive drug treatment is added to the therapeutic regimen if steroid treatment is not successful to control the active disease state, which involves the presence of two or more of the following features: 1) fever or arthralgia, 2) raised erythrocyte sedimentation rate (ESR) (> 20 mm/h), 3) new claudication, bruit, or vascular pain, 4) new typical angiographic features. Arteriography is the best modality for the diagnosis of Takayasu’s disease along with the use of magnetic resonance angiography (MRA) and computed tomography (CT) scans that are commonly used during the process of diagnosis [7, 11, 12]. Surgery consists always of a bypass operation to vessels normal on angiography and distal to the occlusive or stenotic lesion. However, it has been reported that patients with normal angiographic studies showed microscopic involvement of these arteries up to 44% in the biopsies of arterial anastomatic sites [1].

Conclusions

In young patients with a diagnosis of TA, appropriate medical treatment before surgery is required to establish a successful operation without complications. Recently it has been reported in the literature that adequate control of the disease prior to surgical intervention is related to successful long-term outcomes.

Disclosure

The authors do not have a conflict of interest. The authors have no financial or other interest in the manufacture or distribution of any materials that were used.

References

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