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Anesthetic Management of Patients with Takayasu’s Arteritis: A Case Series and Review

Kathirvel, Subramaniam MD; Chavan, Shivanand MD; Arya, Virender K. MD; Rehman, Iqbal MD; Babu, Venkatesh MD; Malhotra, Navin MD; Bhukal, Ishwar MD; Chari, Pramila MD, MNAMS

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doi: 10.1097/00000539-200107000-00014
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Case 1

A 30-yr-old male was referred to our institute with sudden onset of pain and swelling in the right groin that appeared after failed angioplasty for renal artery stenosis secondary to Takayasu’s arteritis (TA). The swelling was pulsatile and progressively increasing. The diagnosis of right femoral artery aneurysm was made by angiography and the patient was scheduled for emergency exploration. He had a history of hypertensive disease, which was treated with amlodipine, atenolol, and prazosin. Preoperative arterial blood pressure recorded on the left upper limb was 150/90 mm Hg by the auscultatory method and correlated well with lower limb recordings. Angiography showed partial obstruction of the right subclavian and left common carotid artery along with complete obstruction of left renal artery. The patient also had features of chronic renal insufficiency (hemoglobin 5.7 g/dL, blood urea nitrogen 50 mg/dL, and serum creatinine 4.5 mg/dL). Bleeding time, platelet count, activated partial thromboplastin time, and prothrombin time were within normal limits. Chest radiography and standard 12-lead electrocardiography (ECG) showed cardiomegaly and left ventricular hypertrophy, respectively. Echocardiography revealed left ventricular diastolic dysfunction. She was given “graded epidural anesthesia” with 12 mL of 0.5% bupivacaine. The level of the block was maintained at T12 by repeated boluses of 0.5% bupivacaine. Noninvasive blood pressure (NIBP) was monitored every 10 min in the left upper limb. Because aneurysmectomy lasted for 6 h, the patient required a propofol infusion, which was titrated to sedation and to the maximum dose of 1.5 mg · kg1 · 1. He remained hemodynamically stable in the intraoperative period and the postoperative hospital stay was uncomplicated.

Case 2

A 51-yr-old woman with TA was admitted to hospital with right femoral artery pseudoaneurysm with impending rupture for aneurysmectomy. The patient developed femoral artery pseudoaneurysm after renal angioplasty, which was performed for renal artery stenosis 1 wk earlier. The patient was taking antihypertensive medication, prednisone and azathioprine. Her past history revealed treatment for pulmonary and abdominal tuberculosis and a hospital admission for hypertensive encephalopathy. Her arterial blood pressure on admission was 180/80 mm Hg on the right arm and unrecordable on the left arm by the auscultatory method. ECG showed left anterior hemiblock. Left ventricular ejection fraction (LVEF) was 67% as determined by transthoracic echocardiography. Angiography showed bilateral renal and subclavian artery block. The patient was given epidural anesthesia with 15 mL of 0.5% bupivacaine. The level of block was maintained at T12 by repeated boluses of 0.5% bupivacaine. Surgery lasted for 3.5 h. Monitoring of NIBP was performed in the right arm using the oscillometric method. The patient received hydrocortisone 100 mg before surgery. Her intraoperative and postoperative courses were uneventful.

Case 3

A 40-yr-old woman known to have had TA was hospitalized with a diagnosis of uncontrolled renovascular hypertension. Her blood pressure was not controlled despite treatment with nifedipine 10 mg thrice daily and atenolol 100 mg once daily. A renogram showed a nonfunctioning left kidney; hence, a left nephrectomy was planned. The patient was also receiving prednisolone and showed features of Cushing’s disease (central obesity, muscle wasting, purple striae, thin skin, and easy bruisability) on preoperative examination. She gave a history of pulmonary tuberculosis 20 yr before admission for which she had received a full course of antitubercular treatment. Her left radial, brachial and axillary arterial pulsations were feebly (1+) palpable (3+ is normal). Blood pressure recording in the right upper limb was 160/80 mm Hg; in the left upper limb 90/50 mm Hg; and in the right lower limb 170/130 mm Hg. The left carotid pulsation was weakly palpable and bruit could be heard over both the carotid arteries. An ECG showed left ventricular hypertrophy and echocardiography revealed left ventricular diastolic dysfunction. Renal and liver function tests were within normal limits. Angiography revealed bilateral subclavian and renal artery stenosis along with diffuse narrowing of the left common carotid artery. The patient received combined general and epidural anesthesia. NIBP was monitored in the right upper and lower limbs. Blood pressure was maintained at preoperative basal levels. Any increase in blood pressure was treated with isoflurane after ensuring adequate analgesia with epidural 0.25% bupivacaine. Postoperative pain relief was provided with 3 mg of epidural morphine in 10 mL of 0.125% bupivacaine every 12 h for the first 48 h. The postoperative course was uneventful.

Case 4

A 31-yr-old female was admitted to the hospital with renal artery stenosis secondary to TA. She was known to be hypertensive and was treated with amlodipine, atenolol, and prazosin. Preoperatively, blood pressure was 160/90 mm Hg in the upper limb and 180/130 mm Hg in the lower limbs. Renal and liver function tests were normal. ECG showed left ventricular hypertrophy, and LVEF was 65% by echocardiography. Angiography revealed bilateral renal and subclavian artery stenosis. The surgical plan was renal arterioplasty with reversed saphenous vein graft. General anesthesia was induced with morphine 6 mg and thiopental 250 mg IV. The trachea was intubated after paralysis was achieved with atracurium and anesthesia was with isoflurane. Anesthesia was maintained with 66% N2O and 1–2 minimum alveolar anesthetic concentration isoflurane in oxygen. Blood pressure was monitored in the upper limb by direct radial artery cannulation and in the lower limb by NIBP. Epidural bupivacaine 0.25% with 3 mg of morphine was used for intraoperative and postoperative analgesia. Blood pressure was maintained at preoperative levels throughout the intraoperative period. The surgical procedure lasted for 7 h. The postoperative course was uncomplicated and the patient was discharged and advised to continue antihypertensives.

Case 5

A 14-yr-old girl with the diagnosis of TA was admitted with severe uncontrolled hypertension secondary to renal artery stenosis on treatment with enalapril and amlodipine. She gave a history of exertional dyspnea and was classified as New York Heart Association class II. Her blood pressure in the right upper limb was 160/80 mm Hg; it was not recordable in the left upper limb. Blood pressure in the lower limb was 160/100 mm Hg. She was also receiving prednisolone and azathioprine. Chest radiography and ECG showed massive cardiomegaly and left ventricular hypertrophy, respectively. Global hypokinesia with LVEF of 29% was detected on echocardiography. Angiography showed stenosis of the left subclavian and renal arteries. Renal function tests were within normal limits. A renogram showed a nonfunctioning left kidney, and therefore a left nephrectomy was planned. General anesthesia was induced with thiopental 5 mg/kg and the trachea was intubated after neuromuscular blockade with atracurium. Lidocaine 1.5 mg/kg was given 2 min before intubation. Intraoperatively, nitroglycerine IV infusion 0.5–2 μg · kg1 · min1 was titrated to control the blood pressure after ensuring adequate analgesia and depth of anesthesia with morphine IV and isoflurane, respectively. The patient was extubated at the end of the procedure. The postoperative course was uneventful.

Case 6

A 24-yr-old, full-term pregnant woman was scheduled for emergency cesarean delivery for nonprogression of labor and fetal distress as indicated by meconium stained liquor. She was diagnosed to have TA during her previous pregnancy when she developed hypertension, absent and asymmetric pulses, and fetal loss. Hypertension persisted, even after the end of pregnancy, for which she was given amlodipine 5 mg once daily. Blood pressure recorded in her left arm was 130/80 mm Hg. Her right brachial and radial pulsations were not palpable. She had eaten solid food 1 h before surgery. After administration of 1000 mL lactated Ringer’s solution, a subarachnoid block was performed with 2.0 mL 0.5% hyperbaric bupivacaine. The systolic blood pressure decreased to 90 mm Hg and was treated with IV fluids. Apgar scores of the neonate were 8 and 10 at 1 and 5 min, respectively. On the second postoperative day, the patient had multiple hypertensive episodes for which atenolol was added to her treatment regime. She was discharged on the fourth postoperative day.

Case 7

A 40-yr-old female with a history of TA was admitted with dysfunctional uterine bleeding for total abdominal hysterectomy. She gave a history of numbness in both upper limbs. Angiography revealed bilateral subclavian artery stenosis. Combined spinal and epidural blockade was given for the surgical procedure. Spinal anesthesia was given with 2.5 mL of 0.5% bupivacaine. The level of block was T10 at 10 min. The height of block was increased to T4 by 3-mL increments of 0.25% bupivacaine given through an epidural catheter. She remained hemodynamically stable throughout the procedure. The pulse oximetry trace was clear and blood pressure was recordable using NIBP in both the upper limbs despite poor palpable pulses and correlated well with lower limb blood pressure. Postoperative analgesia was given with epidural morphine 2 mg every 12 h until the second postoperative day.

Case 8

A 40-yr-old female with a history of TA was admitted with dysfunctional uterine bleeding for fractional curettage. Her right brachial and radial pulsations could not be felt on preoperative examination. A history of chest pain and syncope on exertion were present for which she was receiving aspirin 325 mg/day. Angiography revealed stenosis of both the subclavian arteries. There was no involvement of coronary arteries, and carotid Doppler was normal. She had a history of pulmonary tuberculosis for which she had received a complete course of antitubercular treatment. General anesthesia was induced with thiopental 5 mg/kg and maintained with 66% N2O and 1–2% of halothane in oxygen via face mask through a Magill circuit. Analgesia was provided with morphine 6 mg IV NIBP was monitored in the left upper limb. The entire perioperative course was uneventful.


TA is characterized by a focal stenotic process involving the aorta and the proximal segments of its main branches (1). The affected vessel appears very thick-walled, shortened, and rigid, with marked perivascular sclerosis and adhesion to surrounding tissues (2). The disease is prominent in women, has an onset before 40 years of age, with a preocclusive phase of rheumatic or systemic symptoms, a previous history of tuberculosis or streptococcal infection and segmental arterial involvement often in several sites (2). In our series of 8 patients, there were 7 females and the mean age of the patients was 33.5 years. Tuberculosis has been implicated in the pathogenesis of TA (3). In this series, five patients completed their antitubercular treatment for active pulmonary or nonpulmonary tuberculosis. The diagnosis is rarely suspected in the preocclusive phase and these symptoms are retrospectively related to arterial disease when the arteriopathy is obvious (2). Hence, all patients will be in the occlusive phase when they present for surgery and anesthesia.

Four types of patients with TA can be distinguished (4). Type I disease involves the aortic arch and its main branches, type II lesions are restricted to descending thoracic and abdominal aorta, type III patients show features of both type I and type II, type IV patients additionally have involvement of the pulmonary artery. Angiography in seven patients revealed that five belonged to type III and that two had only an involvement of subclavian arteries (type I). Pulmonary angiography was not performed because clinical evidence of pulmonary hypertension or impaired gas exchange was not seen in any of these patients. In most series of TA, patients were asymptomatic with respect to pulmonary involvement (2). Severe pulmonary hypertension with cyanosis and right heart failure is very rare in these patients (5).

Ishikawa (6) graded TA depending on the presence of four major complications i.e., hypertension, retinopathy, aneurysm formation, and aortic regurgitation. In stage I, none of these complications were present, stage IIa patients had only one of these (in a milder form), stage IIb had only one of these complications but a rather severe one, and in stage III more than one complication was present. Two patients had none of these complications (stage I). Five patients had only hypertension. Two of these were graded as IIa; the other three had severe uncontrolled hypertension despite multidrug antihypertensive therapy (stage IIb). One patient with femoral pseudoaneurysm had hypertension along with retinal involvement and belonged to stage III. Aneurysm formation and aortic regurgitation were not seen in any of these patients.

Hypertension, the major complication affecting anesthetic management in patients with TA, is commonly renovascular, and TA is the most common cause of renovascular hypertension in India (7). Hypertension in TA could also result from reduced elasticity and marked narrowing of aorta and major arteries and abnormal function of carotid and aortic sinus baroreceptors (7). Three patients with renovascular hypertension (stage IIb) were referred for surgical treatment i.e., nephrectomy in two patients with nonfunctioning unilateral kidney and arterioplasty with saphenous venous graft in one patient because the kidney was partially functioning. These patients were referred for surgical management when their hypertension was not controlled by multidrug treatment such as with calcium channel blockers, β-adrenergic blockers, and α-adrenergic blockers. An angiotensin converting enzyme inhibitor, enalapril, was used in only one patient with left-sided renal artery stenosis because bilateral renal artery involvement makes the use of converting enzyme inhibitors risky (8). Because these patients are referred for surgery for their uncontrolled hypertension, preoperative optimization to normotension may be difficult. All drugs were continued until the morning of surgery when the patients had surgery.

The choice of anesthetic technique should take into consideration the maintenance of blood pressure in the intraoperative and postoperative periods. Regional anesthesia is associated with sympathetic block and a subsequent decrease in blood pressure that may be hazardous in a patient with compromised regional circulation because of stenosed arteries (9,10). Epidural blockade is associated with gradual onset of sympathetic block and a decrease in blood pressure. Adequate administration of crystalloids (20 mL/kg), slow administration of local anesthetics, and titration of the local anesthetic to the desired level of block will avoid a precipitous decrease in blood pressure and allow enough time to adjust for the development of sympathetic block. A further decrease in blood pressure can be treated with a further increase in the infusion of fluids, Trendelenburg position, and IV ephedrine. Vasopressors are best avoided in these patients with preexisting compromised organ perfusion (11). An increase in blood pressure associated with intubation, extubation, and inadequate depth during general anesthesia can be avoided by the use of epidural anesthesia. Epidural block with local anesthetics ensures complete pain relief, which helps in the smooth control of blood pressure in the intraoperative and postoperative periods. An awake patient under regional anesthesia provides a simple and reliable monitor of adequate cerebral perfusion. “Graded epidural anesthesia” was used in two patients with femoral pseudoaneurysm for aneurysmectomy. Because the level of block required was only up to T12, the decrease in blood pressure was not clinically significant in either case. One of these patients required sedation with propofol. Hypotension may be exaggerated with drugs such as propofol or midazolam, so they must be given to these patients with caution for sedation under regional anesthesia.

Epidural anesthesia has been used to provide stable hemodynamics and pain relief during labor and delivery in patients with TA (12–14). There are two reports of successful epidural anesthesia for cesarean delivery (15,16). Pregnancy does not change the evolution of TA but increases the risk of decompensation of hypertension (9). Peripheral vascular resistance normally decreases during pregnancy. Because the patients with TA have stenotic vessels and a decrease in afterload, blood pressure increases secondary to the increase in the blood volume that accompanies pregnancy (17). One patient in our series had undergone cesarean delivery. The indication was obstetric, i.e., fetal distress indicated by meconium stained liquor and nonprogression of labor. This patient gave a history of fetal loss and had mild hypertension controlled with amlodipine. Because the patient came for emergency cesarean delivery, angiography was not performed to assess the involvement of various arteries. General anesthesia was avoided because of a full stomach and unassessed carotid circulation. Epidural block was considered but was not used because of fetal distress. Spinal anesthesia was given after administration of 1000 mL of lactated Ringer’s solution. This is the first report of spinal anesthesia in a patient with TA for cesarean delivery, although Hampl et al. (18) reported successful spinal anesthesia in a patient with TA for therapeutic abortion. Though the level of block required for cesarean delivery (T4) was higher than for therapeutic abortion, our patient tolerated this block very well. Beilin and Bernstein (15) suggested that spinal anesthesia, which is associated with a profound decrease in preload, should be avoided in patients with TA. Van Bogaert (19) in an observational study of 24 women with pregnancy-induced hypertension, demonstrated that small-dose spinal block with 7.5 mg of 0.5% hyperbaric bupivacaine is safe as regards fetal and maternal outcome. We feel that spinal anesthesia can be used in selected cases of controlled hypertension when epidural and general anesthesia are relatively contraindicated.

Choi et al. (20) used combined spinal-epidural anesthesia in pregnant patients for cesarean delivery. They administered 1.5 mL of 0.5% spinal bupivacaine followed by 10 mL of 0.25% epidural bupivacaine 10 minutes later and compared this “combined spinal and epidural technique” with “standard epidural block” using 20–25 mL of 2% epidural lidocaine. They found that the hemodynamic variables were comparable. Onset of block, intraoperative analgesia, muscle relaxation, and recovery were better and side effects were fewer in patients who received combined spinal-epidural anesthesia. We used this technique for a nonhypertensive patient with dysfunctional uterine bleeding scheduled for hysterectomy. However, considering the advantages, this technique can be used in hypertensive women for procedures such as cesarean delivery.

General anesthesia is required in some patients because of the site and prolonged nature of the surgery. Gozal et al. (21) and Thorburn and James (22) used epidural anesthesia and analgesia as an adjuvant to general anesthesia for hysterectomy and kidney transplantation, respectively. We used epidural block in two patients who received general anesthesia for nephrectomy and renal arterioplasty. Epidural local anesthetics and narcotics were used for analgesia, thereby producing smooth control of blood pressure in the intraoperative and postoperative periods.

General anesthesia may be associated with hypertensive episodes that can lead to cerebral hemorrhage and infarction or cardiac dysfunction in a patient with TA. Preoperatively, clinical features suggestive of carotid involvement, such as dizziness, syncope on extension of head and carotid bruit should be evaluated. During anesthesia, procedures that diminish carotid blood flow should be avoided. Two patients had involvement of the carotid arteries. One was given an epidural block for pseudoaneurysmectomy of the femoral artery. The other required general anesthesia for nephrectomy. No specialized neurological monitoring was used in this patient because it was not available and he recovered without any neurological deficits. Warner et al. (23) used electroencephalography monitoring in a patient who had undergone suprarenal aortic thrombectomy and bypass surgery and found it to be useful for detecting cortical ischemia. Gozal et al. (21) and Clark and al-Qatari (24) used electroencephalography for their patients with TA for cesarean delivery and hysterectomy, respectively. Kawaguchi et al. (25) used transcranial Doppler to monitor cerebral blood flow. There are several case reports of uncomplicated neurological outcomes in which general anesthesia was used without specialized neurological monitoring (22,26,27). There is only one report of postoperative cerebral infarction in a patient with TA (28). The patient underwent uneventful general anesthesia for pectoralis musculocutaneous flap to provide cover for the defect of neck dissection. They did not use any specialized neurological monitoring. The patient developed right hemiparesis after three hours of recovery from anesthesia and the authors suggested that cerebral vasodilators such as sodium nitroprusside might be useful in these cases to prevent cerebral ischemia. We used isoflurane to maintain the blood pressure at basal preoperative levels, which decreases the cerebral metabolic rate of oxygen and produces cerebral vasodilation.

Cardiac dysfunction in TA is usually secondary to hypertension although obstructive changes can also involve coronary arteries (2). Left ventricular hypertrophy on ECG was seen in all patients with hypertension. None had ischemic changes suggestive of coronary artery involvement. Echocardiography detected two patients with diastolic dysfunction and one with systolic dysfunction and global hypokinesia. Two of these patients had left ventricular failure (New York Heart Association class II) preoperatively and none developed perioperative cardiac failure or ischemia. IV nitroglycerin infusion was required to treat an intraoperative hypertensive crisis in one patient with severe cardiac involvement (global hypokinesia with ejection fraction 29%). An epidural block with local anesthetic was avoided in this patient because of his low ejection fraction.

Patients with TA may be receiving chronic steroid treatment and may present with Cushingoid features preoperatively. Perioperative steroid supplementation is needed to prevent the occurrence of Addisonian hypotensive crises. Patients with renal artery involvement may have features of renal failure. Only one patient in our series had increased urea and creatinine. This patient was evaluated for coagulation and platelet dysfunction before receiving epidural block for aneurysmectomy.

Blood pressure monitoring was possible noninvasively using the oscillometric method in all cases. The upper limb blood pressure was correlated with recordings from the lower limbs preoperatively because the disease remains clinically uncommon beyond the bifurcation of the aorta. If there was a discrepancy of more than 20 mm Hg, NIBP was monitored throughout the surgical procedure in both the upper and lower limbs. Meikle and Milne (29) reported a case of TA with extreme arterial pressure differentials of 200 mm Hg between upper and lower limbs and concluded that monitoring of both upper and lower limb blood pressures should be considered. They cautioned against the use of regional anesthesia, which may provide a steal-like phenomenon to affect regional blood flow. Such a discrepancy was noted in one of our patients with renal vascular hypertension who underwent nephrectomy. We used combined general and epidural anesthesia, monitored both upper and lower limb blood pressures and maintained them at basal preoperative levels throughout the procedure.

In patients with weak or absent pulses in the extremities, pulse oximetry, automatic NIBP, and Doppler flow signals can be used to record blood pressure. In case 7, both upper limb pulses were not palpable, but blood pressure could be recorded by NIBP cuff and pulse oximetry. Ramanathan et al. (26) observed pulsatile flow in clinically nonpalpable arteries in patients with TA using Doppler blood flow signals. Chawla et al. (30) used pulse oximeter signals to monitor upper limb systolic blood pressure in two patients with pulseless disease. Determination of systolic arterial pressure was made at the disappearance of visual display on blood pressure cuff inflation and reappearance of visual display on cuff deflation and by averaging the two. Warner et al. (23) recorded central aortic pressures and used a pulmonary artery catheter in a patient with pulseless disease for major aortic vascular surgery. Pulmonary artery catheterization was not performed for any patient in this series and noninvasive methods were able to record reliable blood pressure in all patients. We avoided invasive monitoring as far as possible because of the possibility of inflicting trauma to already diseased vessels. Two patients developed pseudoaneurysm of the femoral artery after renal angioplasty, indicating the susceptibility of the arterial tree for injury and subsequent formation of aneurysms. However, indication for invasive arterial pressure monitoring remains similar to that in the other surgical patients. We placed radial artery cannula in a patient who had undergone renal arterioplasty with reversed saphenous vein graft. Prolonged vascular surgery (six hours) and anticipated fluctuation in blood pressure in uncontrolled renovascular hypertension were the indications for invasive monitoring in this patient.

Patients with TA should be assessed for the anatomical pattern of the disease and evaluated for hypertension and its complications. In our case series, the anesthetic technique performed varied from patient to patient based on the patient’s stage and severity of disease, site of surgery, and the preference of the anesthesiologist. Maintenance of blood pressure at a preoperative level was the common thread in all cases irrespective of the anesthetic technique used. Regional anesthesia is preferred in patients with carotid artery involvement because consciousness provides a simple and reliable monitor of neurological function. NIBP monitoring using the oscillometric method and pulse oximetry can provide simple and reliable blood pressure readings, even in patients with pulseless extremities. However, upper limb blood pressure should be correlated with lower limb blood pressure because the lower extremity blood vessels are spared clinically in these patients.


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