ANESTH ANALG 58:247-249. 1979
Anesthetic Considerations in Takayasu Arteritis Sham Ramanathan, MD,* Usha Gupta, MD,J. Jack Chalon, MD,$ and Herman Turndorf, MDtj Takayasu arteritis is a chronic, idiopathic occlusive inflammation of the aorta and its major branches, affecting females in over 85% of the cases.' Although the disease was first described in the Oriental population, there have been several recent reports of the syndrome in other races.', The major clinical finding is loss of palpable pulses in the upper limbs and neck. The unsuspected ischemia of vital regional vascular beds may render these patients poor anesthetic risks. This report discusses the anesthetic management of a patient suffering from this condition with special reference to intraoperative monitoring techniques.
Case Report A 31-year-old Caucasian female was scheduled for laparoscopic tuba1 ligation and extraction of impacted molar teeth. The medical history included a subarachnoid hemorrhage in 1975 when roentgenographic studies revealed extensive narrowing of the branches of the aortic arch and intracranial arteries. Takayasu arteritis was diagnosed and the patient was discharged on a 3-month course of steroids. Her only symptom was occasional upper limb weakness. She had been taking aspirin and phenobarbital during the 6 months that preceded admission. Preoperative physical examination revealed a slightly obese female. Carotid pulsations were very weak on both sides. Right upper limb pulses were impalpable and those of the left were feeble. Normal pulsations were present in the lower limbs. Korotkoff sounds were inaudible in arteries of the upper extremities but clearly audible in the popliteal fossae where blood pressure was 100/60 torr on both sides. The remainder of the physical examination was normal, including occular fundoscopy. Hematocrit, hemoglobin, and * Assistant Professor.
t Resident.
$ Professor. 5 Professor and Chairman. Received from the Department of Anesthesiology, New York University Medical Center, New York, New York 10016. Accepted for publication January 16, 1979. Reprint requests to: Dr Ramanathan, 25 Eisenhower Road, Closter, New Jersey 07624.
serum chemistries were within normal limits, as were EKG, EEG, brain scan, chest roentgenogram, C-reactive protein, and erythrocyte sedimentation rate. Biopsy of the temporal artery showed no giant cell arteritis. The latex fixation test for rheumatoid arthritis was positive and IgM globulin level reached 282 mg/100 ml (normal range 70-280 mg/100 ml). Aortography showed narrowing of the descending thoracic aorta with stenosis of the internal carotid, subclavian, and axillary arteries on the right side and of the common carotid and subclavian arteries on the left. Multiple stenoses were present in both carotid and basivertebral systems. The remainder of the aortic tree was normal. The patient received 0.4mg of atropine, 50 mg of meperidine, and 100 mg of pentobarbitol intramuscularly 45 minutes before surgery. A Doppler blood flow probe (P-84, 5.4 MHz, Medsonics, Mountainview, CA) attached to a Versatone unit (Medsonics) was used to record blood flow signals from peripheral arteries (Fig). Flow signals were continuously displayed on an oscilloscope screen and inscribed on a multichannel recorder (Fig). Blood flow was demonstrable even in clinically nonpulsatile arteries. The right radial artery generated a low output signal, abnormal in configuration. The dorsalis pedis artery was selected to monitor blood pressure because it had the strongest pulse, had satisfactory flow, and was easily accessible in the lithotomy position during the sterilization procedure. A lower extremity sphygmomanometer cuff was applied around the middle third of the thigh. Systolic pressure was obtained by intermittent inflation and deflation of the cuff, noting the pressures at which the trace disappeared and reappeared (Fig). Systolic pressure was taken as the mean of these two values, and recorded at 40 torr in the right upper extremity, 60 torr in the left upper extremity, and 100 torr in both lower limbs. Cardiac stroke volume was continuously monitored by an impedance cardiograph (Instrumentation for Medicine, Inc., Greenwich, CT), using Mylar strip electrodes. The (dz/dt),.. wave (Figure, bottom panel) was displayed and printed simultaneously with the blood flow signals. Change in (dz/dt)max was used as an index of alteration in stroke output. EKG and esophageal temperature were also monitored. After 5 minutes of preoxygenation, anesthesia was induced with a "sleep" dose of thiopental and the trachea was intubated without hyperextension of the neck following succinylcholine. Nitrous oxide in oxygen was used for maintenance of anesthesia with pancuronium and fentanyl as needed. The intraoperative period was uneventful except for a brief period of reduced stroke volume (from 60 to 44 ml) after abdominal distension by carbon dioxide during laparoscopy and except for a short period of hypertension, from 110 to 160 torr, lasting for 15 minutes during dental extraction. Pancuronium was reversed with neostigmine prior to extubation. The recovery period and postoperative course were unremarkable. ANESTHESIA AND ANALGESIA Vol58. No 3. May-June 1979
247
CLINICAL REPORTS TABLE Summary of Abnormal Pathophydologic Features In Idiopathic Takayasu Arteritis Svstem
Central ous
LEFT CAROTID
da/d
LLfT
TRACIWB FROU
THf
CAWD~OSRACH
FIGURE. Recordings of Doppler flow signals from various peripheral arteries (top two panels). Note the abnormal low output signal from the right radial artery. The third panel shows a flow tracing from the dorsalis pedis artery before, during, and after occlusion by the sphygmomanometer cuff during blood pressure measurement. The bottom panel shows the first derivative [(dz/ dt),] of the impedance cardiogram. The decrease in peak height seen at the right represents a 25% reduction in cardiac stroke volume during pneumoperitoneum.
Shortened, stenosed carotid arteries Cerebrovascular accidents Syncopal attacks
Cardiovascular
Multiple arterial occlusions Pulseless disease Hypertensive heart disease Coronary heart disease Endocardial and valvular involvement' Conduction defects, hypertrophy, ischemia, and right ventricular strain5
Pulmonary
Pulmonary hypertension Pulmonary artery narrowing Tuberculosis
HematoIog y
Increased erythrocyte sedimentation rate C-reactive protein. IgG, IgM, and IgA Positive rheumatoid factors
Musculoskeletal
Ankylosing spondylitis Rheumatoid arthritis
Renal
Renal artery stenosis Renal hypertension Decreased renal function
Endocrine
Adrenal suppression due to chronic steroid therapy
L f r i RADIAL
WlCnlAL
UlCfOIYCf
nerv-
Pathophysiolcgy
Discussion The Takayasu syndrome is a collective name given to a group of diseases in which there is no palpable pulse in the upper half of the body. Although etiologic factors such as syphilis, atherosclerosis, and trauma have been described,' the majority of cases are idiopathic. The patient, usually a female, loses arterial pulsations in the upper limb and neck; these symptoms are responsible for its name: "pulseless disease of the young female." Roentgenographic and pathologic studies'. show that all systemic arteries including pulmonary vessels may be involved. The symptoms are protean and depend on the regional vasculature involved. Treatment consists mainly of steroids and anticoagulants. Life-threatening or incapacitating arterial occlusions are sometimes amenable to surgical intervention. Pathologic changes consist primarily of a panendarteritis affecting the large and medium caliber arteries. The veins are usually spared. Involved vessels are characteristically thickened and shortened and are subject to thrombus formation and luminal obstruct i ~ nHyperextension .~ of the neck may reduce carotid blood flow by stretching the shortened arteries. Patients often hold their heads in the drooping position to prevent ~ y n c o p eNakao .~ et all have classified the
248
ANESTHESIA AND ANALGESIA VoI 58. No 3, May-June 1979
aortic involvement by aortography into three major patterns which can occur singly or in combination: (1)arch-type involving the aortic arch and its major branches; (2) atypical coarctation, which affects the descending thoracic and abdominal aorta; and (3) extensive involvement of the coronary, hepatic renal, pulmonary, and mesenteric arteries. Pulmonary arterial disease more freuently involves the right pulmonary artery with stenosis of both the artery and its lobar branches5 Anesthesiologists may encounter these patients during obstetrical anesthesia,', incidental surgery, or corrective vascular procedures.6 A summary of the major pathophysiologic features is shown in the Table. Reduced cerebral pulmonary and coronary blood flows, systemic and pulmonary hypertension, and adrenal suppression due to chronic steroid therapy may lead to cardiorespiratory instability during and after anesthesia. Prolonged anticoagulant therapy and ankylosing spondylitis' may contraindicate regional anesthesia. Upper extremity blood pressures may be difficult to measure by classic methods. However, the Doppler blood flow probe, which detects flow velocity rather than arterial wall movement, can be used.' We were
CLINICAL REPORTS
able to elicit flow signals, even in unpulsatile arteries, with this device. Flow signals displayed continuously on an oscilloscope can serve as an index of peripheral perfusion and can be used to obtain systolic blood pressure. An easily accessible artery with a strong flow signal (such as the dorsalis pedis artery) may be used. When measurements of lower extremity blood pressures are impractical or unreliable because of atypical coarctation of the descending aorta and/or femoral artery narrowing,' impedance cardiography may prove useful. This technique measures cardiac stroke volume noninvasively and can monitor hemodynamic stability." The first derivative [(dz/dt),,,] of the impedance cardiogram (ZCG)may be displayed continuously on the oscilloscope (Figure) and changes in peak height may be taken as an index of alteration of stroke output. Other important considerations include avoidance of hypotension because of the abnormal regional blood flows, hyperextension or improper positioning of the head during laryngoscopy, or surgical procedures which may decrease carotid artery blood flow. Pulmonary artery occlusion is likely to create interferences in the pulmonary ventilation-perfusion relationship" and with the placement of balloon-tipped flotation catheters. In summary, a Doppler blood flow probe and
impedance cardiograph are useful in monitoring patients with Takayasu arteritis. REFERENCES 1. Nakao K, Ikeda M, Kimata 5, et al: Takayasu's arteritis: clinical
report of eighty-four cases and immunological studies of seven cases. Circulation 35:1141-1155,1967 2. Hauth JC, Cunningham FG, Young BK: Takayasu's syndrome in pregnancy. Obstet Gynecol 50:373-375,1977 3. Sicuranza BJ, Maiorino W, Tidsall LH: Takayasu's disease (pulseless disease) in pregnancy. Am J Obstet Gynecol 83: 1516-1518,1962 4. Nasu T: Pathology of pulseless disease: a systematic study and critical review of twenty-one cases reported in Japan. Angiology 16:225-242,1963. 5. Lupi HE, Sanchez G, Horowitz 5, et al: Pulmonary artery involvement in Takayasu's arteritis. Chest 6769-74, 1975 6. Crawford ES, DeBakey ME, Norris GC, et al: Thrombo-obliterative disease of the great vessels arising from the aortic arch. J Thorac Cardiovasc Surg 43338-53. 1962 7. Chhetri MK, Pal NC, Neelakantan, et al: Endocardia1 lesion in a case of Takayasu's arteriopathy. Br Heart J 32:859-862,1970 8. Paloheimo JA, Julkunen H, Siltanen P, et al: Takayasu's arteritis and ankylosing spondylitis: report of four cases. Acta Med %and 179:77-85,1966 9. Strandness DE Jr, Schultz RD, Sumner DS, et al: Ultrasonic flow detection: a useful technique in the evaluation of peripheral vascular disease. Am J Surg 113:311-320,1967 10. Secher NJ, Thomsen A, Amsbo P: Measurements of rapid changes in cardiac stroke volume: an evaluation of the impedance cardiography method. Acta Anaesthesiol %and 21:353358,1977 11. Kawai C, Ishikawa K, Kato M, et al: "Pulmonary pulseless disease": pulmonary involvement in so called Takayasu's disease. Chest 73:651-657,1978
ANESTHESIA AND ANALGESIA Vol58. No 3. May-June 1979
249
Anesthetic Considerations in Takayasu Arteritis Sham Ramanathan, MD,* Usha Gupta, MD,J. Jack Chalon, MD,$ and Herman Turndorf, MDtj Takayasu arteritis is a chronic, idiopathic occlusive inflammation of the aorta and its major branches, affecting females in over 85% of the cases.' Although the disease was first described in the Oriental population, there have been several recent reports of the syndrome in other races.', The major clinical finding is loss of palpable pulses in the upper limbs and neck. The unsuspected ischemia of vital regional vascular beds may render these patients poor anesthetic risks. This report discusses the anesthetic management of a patient suffering from this condition with special reference to intraoperative monitoring techniques.
Case Report A 31-year-old Caucasian female was scheduled for laparoscopic tuba1 ligation and extraction of impacted molar teeth. The medical history included a subarachnoid hemorrhage in 1975 when roentgenographic studies revealed extensive narrowing of the branches of the aortic arch and intracranial arteries. Takayasu arteritis was diagnosed and the patient was discharged on a 3-month course of steroids. Her only symptom was occasional upper limb weakness. She had been taking aspirin and phenobarbital during the 6 months that preceded admission. Preoperative physical examination revealed a slightly obese female. Carotid pulsations were very weak on both sides. Right upper limb pulses were impalpable and those of the left were feeble. Normal pulsations were present in the lower limbs. Korotkoff sounds were inaudible in arteries of the upper extremities but clearly audible in the popliteal fossae where blood pressure was 100/60 torr on both sides. The remainder of the physical examination was normal, including occular fundoscopy. Hematocrit, hemoglobin, and * Assistant Professor.
t Resident.
$ Professor. 5 Professor and Chairman. Received from the Department of Anesthesiology, New York University Medical Center, New York, New York 10016. Accepted for publication January 16, 1979. Reprint requests to: Dr Ramanathan, 25 Eisenhower Road, Closter, New Jersey 07624.
serum chemistries were within normal limits, as were EKG, EEG, brain scan, chest roentgenogram, C-reactive protein, and erythrocyte sedimentation rate. Biopsy of the temporal artery showed no giant cell arteritis. The latex fixation test for rheumatoid arthritis was positive and IgM globulin level reached 282 mg/100 ml (normal range 70-280 mg/100 ml). Aortography showed narrowing of the descending thoracic aorta with stenosis of the internal carotid, subclavian, and axillary arteries on the right side and of the common carotid and subclavian arteries on the left. Multiple stenoses were present in both carotid and basivertebral systems. The remainder of the aortic tree was normal. The patient received 0.4mg of atropine, 50 mg of meperidine, and 100 mg of pentobarbitol intramuscularly 45 minutes before surgery. A Doppler blood flow probe (P-84, 5.4 MHz, Medsonics, Mountainview, CA) attached to a Versatone unit (Medsonics) was used to record blood flow signals from peripheral arteries (Fig). Flow signals were continuously displayed on an oscilloscope screen and inscribed on a multichannel recorder (Fig). Blood flow was demonstrable even in clinically nonpulsatile arteries. The right radial artery generated a low output signal, abnormal in configuration. The dorsalis pedis artery was selected to monitor blood pressure because it had the strongest pulse, had satisfactory flow, and was easily accessible in the lithotomy position during the sterilization procedure. A lower extremity sphygmomanometer cuff was applied around the middle third of the thigh. Systolic pressure was obtained by intermittent inflation and deflation of the cuff, noting the pressures at which the trace disappeared and reappeared (Fig). Systolic pressure was taken as the mean of these two values, and recorded at 40 torr in the right upper extremity, 60 torr in the left upper extremity, and 100 torr in both lower limbs. Cardiac stroke volume was continuously monitored by an impedance cardiograph (Instrumentation for Medicine, Inc., Greenwich, CT), using Mylar strip electrodes. The (dz/dt),.. wave (Figure, bottom panel) was displayed and printed simultaneously with the blood flow signals. Change in (dz/dt)max was used as an index of alteration in stroke output. EKG and esophageal temperature were also monitored. After 5 minutes of preoxygenation, anesthesia was induced with a "sleep" dose of thiopental and the trachea was intubated without hyperextension of the neck following succinylcholine. Nitrous oxide in oxygen was used for maintenance of anesthesia with pancuronium and fentanyl as needed. The intraoperative period was uneventful except for a brief period of reduced stroke volume (from 60 to 44 ml) after abdominal distension by carbon dioxide during laparoscopy and except for a short period of hypertension, from 110 to 160 torr, lasting for 15 minutes during dental extraction. Pancuronium was reversed with neostigmine prior to extubation. The recovery period and postoperative course were unremarkable. ANESTHESIA AND ANALGESIA Vol58. No 3. May-June 1979
247
CLINICAL REPORTS TABLE Summary of Abnormal Pathophydologic Features In Idiopathic Takayasu Arteritis Svstem
Central ous
LEFT CAROTID
da/d
LLfT
TRACIWB FROU
THf
CAWD~OSRACH
FIGURE. Recordings of Doppler flow signals from various peripheral arteries (top two panels). Note the abnormal low output signal from the right radial artery. The third panel shows a flow tracing from the dorsalis pedis artery before, during, and after occlusion by the sphygmomanometer cuff during blood pressure measurement. The bottom panel shows the first derivative [(dz/ dt),] of the impedance cardiogram. The decrease in peak height seen at the right represents a 25% reduction in cardiac stroke volume during pneumoperitoneum.
Shortened, stenosed carotid arteries Cerebrovascular accidents Syncopal attacks
Cardiovascular
Multiple arterial occlusions Pulseless disease Hypertensive heart disease Coronary heart disease Endocardial and valvular involvement' Conduction defects, hypertrophy, ischemia, and right ventricular strain5
Pulmonary
Pulmonary hypertension Pulmonary artery narrowing Tuberculosis
HematoIog y
Increased erythrocyte sedimentation rate C-reactive protein. IgG, IgM, and IgA Positive rheumatoid factors
Musculoskeletal
Ankylosing spondylitis Rheumatoid arthritis
Renal
Renal artery stenosis Renal hypertension Decreased renal function
Endocrine
Adrenal suppression due to chronic steroid therapy
L f r i RADIAL
WlCnlAL
UlCfOIYCf
nerv-
Pathophysiolcgy
Discussion The Takayasu syndrome is a collective name given to a group of diseases in which there is no palpable pulse in the upper half of the body. Although etiologic factors such as syphilis, atherosclerosis, and trauma have been described,' the majority of cases are idiopathic. The patient, usually a female, loses arterial pulsations in the upper limb and neck; these symptoms are responsible for its name: "pulseless disease of the young female." Roentgenographic and pathologic studies'. show that all systemic arteries including pulmonary vessels may be involved. The symptoms are protean and depend on the regional vasculature involved. Treatment consists mainly of steroids and anticoagulants. Life-threatening or incapacitating arterial occlusions are sometimes amenable to surgical intervention. Pathologic changes consist primarily of a panendarteritis affecting the large and medium caliber arteries. The veins are usually spared. Involved vessels are characteristically thickened and shortened and are subject to thrombus formation and luminal obstruct i ~ nHyperextension .~ of the neck may reduce carotid blood flow by stretching the shortened arteries. Patients often hold their heads in the drooping position to prevent ~ y n c o p eNakao .~ et all have classified the
248
ANESTHESIA AND ANALGESIA VoI 58. No 3, May-June 1979
aortic involvement by aortography into three major patterns which can occur singly or in combination: (1)arch-type involving the aortic arch and its major branches; (2) atypical coarctation, which affects the descending thoracic and abdominal aorta; and (3) extensive involvement of the coronary, hepatic renal, pulmonary, and mesenteric arteries. Pulmonary arterial disease more freuently involves the right pulmonary artery with stenosis of both the artery and its lobar branches5 Anesthesiologists may encounter these patients during obstetrical anesthesia,', incidental surgery, or corrective vascular procedures.6 A summary of the major pathophysiologic features is shown in the Table. Reduced cerebral pulmonary and coronary blood flows, systemic and pulmonary hypertension, and adrenal suppression due to chronic steroid therapy may lead to cardiorespiratory instability during and after anesthesia. Prolonged anticoagulant therapy and ankylosing spondylitis' may contraindicate regional anesthesia. Upper extremity blood pressures may be difficult to measure by classic methods. However, the Doppler blood flow probe, which detects flow velocity rather than arterial wall movement, can be used.' We were
CLINICAL REPORTS
able to elicit flow signals, even in unpulsatile arteries, with this device. Flow signals displayed continuously on an oscilloscope can serve as an index of peripheral perfusion and can be used to obtain systolic blood pressure. An easily accessible artery with a strong flow signal (such as the dorsalis pedis artery) may be used. When measurements of lower extremity blood pressures are impractical or unreliable because of atypical coarctation of the descending aorta and/or femoral artery narrowing,' impedance cardiography may prove useful. This technique measures cardiac stroke volume noninvasively and can monitor hemodynamic stability." The first derivative [(dz/dt),,,] of the impedance cardiogram (ZCG)may be displayed continuously on the oscilloscope (Figure) and changes in peak height may be taken as an index of alteration of stroke output. Other important considerations include avoidance of hypotension because of the abnormal regional blood flows, hyperextension or improper positioning of the head during laryngoscopy, or surgical procedures which may decrease carotid artery blood flow. Pulmonary artery occlusion is likely to create interferences in the pulmonary ventilation-perfusion relationship" and with the placement of balloon-tipped flotation catheters. In summary, a Doppler blood flow probe and
impedance cardiograph are useful in monitoring patients with Takayasu arteritis. REFERENCES 1. Nakao K, Ikeda M, Kimata 5, et al: Takayasu's arteritis: clinical
report of eighty-four cases and immunological studies of seven cases. Circulation 35:1141-1155,1967 2. Hauth JC, Cunningham FG, Young BK: Takayasu's syndrome in pregnancy. Obstet Gynecol 50:373-375,1977 3. Sicuranza BJ, Maiorino W, Tidsall LH: Takayasu's disease (pulseless disease) in pregnancy. Am J Obstet Gynecol 83: 1516-1518,1962 4. Nasu T: Pathology of pulseless disease: a systematic study and critical review of twenty-one cases reported in Japan. Angiology 16:225-242,1963. 5. Lupi HE, Sanchez G, Horowitz 5, et al: Pulmonary artery involvement in Takayasu's arteritis. Chest 6769-74, 1975 6. Crawford ES, DeBakey ME, Norris GC, et al: Thrombo-obliterative disease of the great vessels arising from the aortic arch. J Thorac Cardiovasc Surg 43338-53. 1962 7. Chhetri MK, Pal NC, Neelakantan, et al: Endocardia1 lesion in a case of Takayasu's arteriopathy. Br Heart J 32:859-862,1970 8. Paloheimo JA, Julkunen H, Siltanen P, et al: Takayasu's arteritis and ankylosing spondylitis: report of four cases. Acta Med %and 179:77-85,1966 9. Strandness DE Jr, Schultz RD, Sumner DS, et al: Ultrasonic flow detection: a useful technique in the evaluation of peripheral vascular disease. Am J Surg 113:311-320,1967 10. Secher NJ, Thomsen A, Amsbo P: Measurements of rapid changes in cardiac stroke volume: an evaluation of the impedance cardiography method. Acta Anaesthesiol %and 21:353358,1977 11. Kawai C, Ishikawa K, Kato M, et al: "Pulmonary pulseless disease": pulmonary involvement in so called Takayasu's disease. Chest 73:651-657,1978
ANESTHESIA AND ANALGESIA Vol58. No 3. May-June 1979
249
