Heart Vessels Suppl. (1992) 7:32-36
Heart andVessel S © Springer-Verlag1992
Takayasu arteritis in China: A report of 530 cases Zheng Deyu, Fan Dijun, and Liu Lisheng Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences, Beijing 10037, China
Summary. A total of 530 patients with Takayasu arteritis were studied. Among 346 patients who were diagnosed by aortography, the female to male ratio was 2.9 to 1, and the age of onset ranged from 5 to 45 years. Three hundred and eighteen (60%) patients with secondary hypertension, including renovascular hypertension in 281, and 197 (37.2%) with pulseless extremities were found in the series. All the patients were treated with medical or surgical procedures. Surgical treatment is preferred if clinical status of the patient permits. The patients were followed for 1-29 years (average 7.8 years). There were 55 deaths (10.4%) in this series. Cerebral hemorrhage was found as a common cause of death. Five-year and ten-year survival rates were 93.1% and 91.1%, respectively.
Key words: Takayasu arteritis - Renovascular hypertension - Cerebral hemorrhage - Cerebral thrombosis
Introduction Since the early 1950s, we have observed a number of patients suffering from partial or complete obstruction of the aorta and its main branches. Becauses of their divergent clinical pictures these cases were diagnosed as "pulseless disease . . . . atypical congenital coactation of aorta", "hypertension due to renal arterial disease", and others with the help of retrograde arterial catheterization and selective aortography. Hwang and Liu found that the different clinical features were all manifestations of a common pathologic process, namely aortoarteritis [1, 2]. In 1962 they put forward the concept that aortoarteritis is a disease entity and this term should be substituted for the timehonored "pulseless disease . . . . atypical coactation of aorta". Since than a large number of cases have been Address correspondence to: Z. Deyu
diagnosed and treated in our hospital [3-7]. The present series comprises 530 patients with aortoarteritis admitted to Fu Wai Hospital between the years 1958 and 1990.
Material and methods A retrospective study was carried out in 530 patients with aortoarteritis during a 32-year period up to 1990. Among them, 346 patients with Takayasu arteritis were diagnosed by aortic angiography and 184 patients by their age, history, pulselessness or neck or abdominal bruit, as well as by the exclusion of artherosclerosis, congenital aortic constriction, and other findings. In addition, arterial biopsy or autopsy confirmation was obtained in 49 cases. The patients were separated into 4 categories, according to the classification of Ueno and associates and modified by Lupi HE. et al. [8]. Pulmonary angiography was carried out in 67 cases. The criteria used to evaluate the effects of surgical treatment were as follows: recovery of blood pressure and blood flow to the normal range after operation was considered as excellent, decrease in systolic and diastolic blood pressure by more than 20 mmHg as good, and no change in blood pressure and blood flow as poor.
Results Females comprised 395 cases (74.5%) and 135 (25.5%) were males. Their age ranged from 7 to 59 years (average 25.8 years) and the age of onset a n g e d from 5 to 45 years (average 22.3 years) (Table 1). History of onset of illness varied from 2 weeks to 38 years. Past history of tubereulosis was found in 102 cases (19.2%) and active lesion was found in 157 cases (29.6%). The clinical features of the patients are shown in Table 2. Of the 318 patients with secondary hypertension, including renovascular hypertension in 281, hypertension due to constriction of the descending thoracic aorta in 37 were confirmed by angiography and clinical findings. Among them, mild hypertension in 79
Z. Deyu et al.: Takayasu arteritis in China
33
Table 1. Sex and age of the patients studied Years
Left
Left > Right
Total
Right > Left
Left > Right
Total
65 25 26 22 15 42 195
38 17 7 3 5 14 84
103 42 33 25 20 56 279
73 11
58 15 2 1 1 20 97
131 26 2 2 3 33 197
10-30 31-50 51-70 71-90 91-110 111Total
1 2 13 100
Table 6. Topography of the arterial lesions in 105 patients with Takayasu arteritis (from DSA) Artery Left Subclavian Right Left Carotid Right Left Vertebral Right Innominate Thoracic aorta Abdominal Left Renal Right Superior Mesenteric Inferior Celiac Left Iliac Right Hepatic Pulmonary
Stenosis
Obstruction
Dilatation or aneurysm
Irregular
Total
29 21 13 10 6 2 11 15 47 28 28 5 1
47 21 13 13 2 2 2
1 3
5 3 1
82 48 27 24 9 4 17 26 58 34 36 25 3 4 8 10 4 33
5 6 1 33/73
1 6 8 20 2 4 3 2 3
1 1 2 4 5
2
2 7 5
DSA, Digital subtraction angiography
80/153) were type IV. Involvement of the pulmonary artery was found by lung perfusion scanning and chest X-ray or pulmonary angiography in 153 patients with aortoarteritis. Pulmonary arterial involvements were found by pulmonary angiography in 40 (53.3%) out of 75 cases. The right pulmonary artery in the upper lung field was most often involved, while the left pulmonary artery in the lower or middle-lower lung field was next in involvement. Five patients with pulmonary hypertension were confirmed by right heart catheterization. Pulmonary artery systolic pressure was as high as l l 2 m m H g and diastolic pressure was 32mmHg in one patient. Medical treatment included hormones, vasodilator, antituberculosis drugs antihypertensive drugs, and traditional Chinese medicine. Seventy cases in the active stage were treated with prednisone (Dexamethasone in 3 cases). The initial does was 20 to 40mg per day for 4 - 5 weeks and the dose was reduced gradually to a minimum maintenance dose of 5mg per day for 6 months-1 year (18-24 years in 2 cases). Long-term administration
of 5mg prednisone was an effective therapy in the present series. Eighty cases were treated with antituberculosis drugs with no effect found in the patients with Takayasu arteritis. Thirteen patients with renovascular hypertension were treated by percutaneous transluminal renal angioplasty (PTRA), 10 by unilateral P T R A (autorenal transplantation in one case), and 3 by bilateral PTRA. The success rate of P T R A was 84.6%. There were 8 cases followed-up for 2 - 8 years. The levels of blood pressure were still normal, except in one case (restenosis). One hundred and thirty-seven patients underwent 147 procedures in the present series, including type I in 3 cases, type II in 84 cases, and type III in 50 cases. One hundred and four cases were followd-up for 6 months to 23 years (average 8.7 years). The long-term results of r e p h r e c t o m y (excellent or good in 55/57, poor in 2/57) and revascularization (excellent or good in 40/47, poor in 7/47) were observed. All the patients were followed-up for 1-29 years (average 7.8 years). The age at death ran~ed
Z. Deyu et al.: Takayasu arteritis in China
35
Table 7. Causes of death in 530 patients with Takayasu
arteritis Cause Cerebral hemorrhage Postoperative complication Renal failure Cardiac failure Acute myocardial infarction Cerebral thrombosis Rupture of dissecting aneurysm Rupture of false aneurysm Tuberculosis Cancer of stomach Unknown Total
Total
%
13 4 4 3 3 2 1 1 1 1 22 55
23.6 7.3 7.3 5.5 5.5 3.6 1.8 1.8 1.8 1.8 38.2 100.0
from 14 to 56 years. There were 55 cases (10.4%) of death (Table 7). Six cases were autopsied. Thirteen cases died of cerebral hemorrhage (Table 7). Five-year and ten-year survival rates were 93.1% and 91.1% respectively.
Discussion
A total of 1200 patients with Takayasu arteritis (including our series) were reported in China. Of the patients, the female to male ratio was 3.3 to 1 and their age ranged from 5 to 64 years. The most important sign was a systolic or systolic-diastolic bruit heard over the epigastrium or umbilical region or neck. Systolic bruits over the epigastrium and umbilical region radiating the back or down the iliac arteries orten indicated the extensive involvement of the descending aorta. In comparison with renal arterial angiography, 81 of 104 (77.9%) unilateral renal artery stenosis and 91 of 99 (91.9%) bilateral stenotic cases had bruits, but more than 50% showed only I - I I degree soft bruits and the degree of the murmur was not related to the degree of stenosis. Arterial hypertension was present in 58.4% of our patients, mainly resulting from involvement of the renal arteries which occured in 51.2% of cases demonstrated by aortography. About 37% of the cases had diastolic blood pressure over 125 mmHg and 31% had systolic blood pressure over 200 mmHg. Abrupt onset of hypertension was observed in 53 cases (16.6%), manifested as accelerated or malignant hypertension or left heart failure. Renal artery lesion due to aortoarteritis is one of the most important causative processes of renovascular hypertension in China, particularly in female adolescents and young adults. About 37% of the cases had pulseless extremities as the first clinical sign. Ocular manifestation of "Pulseless Disease" was not found frequently in the present series (14%). Diastolic murmur was heard in the aortic valves in 11 of the patients with aortic regurgitation;
X-ray findings showed dilatation of the ascending aorta in all the patients with aortic regurgitation. Subclavian artery and carotid artery were involved in these cases [9]. Takayasu arteritis is a nonspecific inflammatoryfibrotic process involving the aorta and its main branches as well as the pulmonary artery. The end results of the marked fibrosis and thickening of the arterial wall was usually a constriction or obstruction (95%) and occasionally a saccular dilatation or aneurysm (5%) in the present series. The number of vessels involved with aortoarteritis varied from 1 to 13 branches (average 3.5). According to DSA findings, subclavian artery, especially the left subclavian artery, abdominal aorta, renal artery, thoracic aorta, carotid artery, superior mesenteric artery, and pulmonary artery were more frequently involved. The involvement of the abdominal aorta with the renal artery was found in 77% of the patients. There was no significant difference between the involvement of the left or right renal artery. Associated lesions involved the renal arter is bilaterally in about 2/3 of the cases, orten being more severe on one side [10]. The involvement of the pulmonary artery in patients with aortoarteritis is not uncommon. About one-half of the patients in this series had pulmonary artery involved, especially the right pulmonary artery in the upper lung field, a finding similar to that of others [11]. Clinically, the diagnosis should be differentiated from pulmonary artery disease of other etiology such as pulmonary thromboembolism or primary pulmonary hypertension. Unilateral popliteal artery pressure could not be measured in 41 cases in the present series. Diseases such as Bueger's disease or atherosclerosis should also be differentiated. As to the treatment of Takayasu arteritis, no specific remedy is as yet avalable. Corticosteroids administered to 67 cases in the present series in the active or acute stage induced remarkable clinical remission. We belive that long-term administration of a small dosage of corticosteroids is effective in treating this disoder. The response to treatment, if heart failure due to systemic arterial hypertension has occurred, is usually good. However, the effect of treatment of antituberculosis in the patients is still unknown. PTRA should be performed first when indicated. Surgical treatment should be preferable during the chronic phase and be carefully evaluated in each case. Because it is an important factor affecting prognosis of the disease, the renal artery lesion and associated lesions of the aorta and its main branches is of vital importance in surgical intervention as well as in judging the prognosis. With the development of surgical techniques, revascularization should be adopted first when indicated. Prognosis is good in most patients with aortoarteritis. The patients are able to engage in light work because of the development of collateral circulation of the
36 involed artery in the present series. The oldest patient in China was 64 years old. The prognosis of aortoarteritis depends mainly on the level of elevated blood pressure and the status of cerebral circulation. About 70% of the patients developed complications within 5 years of onset of this disease. Cerebral hemmorrhage was more orten found in patients with renovascular hypertension and ischemic complications. However, cerebral thrombosis was more frequently found in patients with normotension. We found that 18 patients with congestive heart failure due to severe renovascular hypertension had no aortic regurgitation. Two of them had elevation of central arterial pressure with enlargement of the heart while the blood pressure was unmeasurable in the both extremities, thus necessitating a differential diagnosis. As to the mechanism of aortic regurgitation, dilatation of the involved ascending aorta, separation of the aortic commissures and thickening of the valve cusps should be considered. Five patients with myocardial infarction, and 6 with angina pectoris were found in this series. Coronary angiography was normal in two patients with angina pectoris due to aortic regurgitation. In one patient with angina pectoris, the occlusions in both cephalicbrachial arteries and the branches of the coronary artery (left arterial branch) supplied blood to the branchialcephalic artery to form collateral circulation, known as coronary steal syndrome. There were changes of pulsation of the radial artery during the 1-10 year follow-up period, but no further changes of pulsation after 10 years of medical treatment. About 40% of the pulsation of the involved arteries in both upper arms after treatment was improved due to the development of collateral circulation or thrombolysis. Mortality rate of aortoarteritis is 10.4%
Z. Deyu et al.: Takayasu arteritis in China in the present series. About one third of the patients died of original complications. The mortality rate of cerebral hemorrhage is higer than that of congestive heart failure in present series. The five-year and ten-year survival rates were 90.3% and 91.3% respectively. This might be because most of that patients had been subjected to long-term drug treatment.
References 1. Hwang W, Liu LS (1962) Constrictive arteritis of the aorta and its main branches. Chin Med J 81:526 2. Liu LS, Hwang W (1963) Constrictive arteritis report of cases. Chin J Int Med 4:293 3. Zheng DY, Wu X, Liu LS, Liu QH (1973) Aortoarteritis, clinical study of 200 cases. Chin J Cardiovas Dis 1:43 4. Zheng DY, Liu LS, Liu QH (1985) Clinical study in 372 patients with aortoarteritis. Beijing Med J 7:200 5. Zheng DY, Liu LS, Liu QH (1981) Clinical study of pulmonary artery involvement in aortoarteritis. Chin J Cardiovas Dis 9:22 6. Liu LS, Zheng DY (1990) Aortoarteritis: A report of 480 cases. J Human Hypertension 4:135 7. Zheng DY, Liu LS, Fan DJ (1990) Clinical studies in 500 patients with aortoarteritis. Chin Med J 103:536 8. Lupi HE, Sanchez TG, Horwitz S, Gutierrez FE (1975) Pulmonary artery involvement in Takayasu's arteritis. Chest 67:69 9. Zheng DY, Wang J, Xin JX, Liu LS (1989) Clinical study of aortic regurgitation in aortoarteritis. Chin Cardiovasc Dis 17:21 10. Liu YQ, Du JH (1982) Aorto-arteritis Further angiography study of 231 cases. Chin Med J 95:15 11. Liu YQ, Jin BIù, Ling J (1990) Pulmonary artery involvement in Aortoarteritis an angiographic study. Chin Circulation J 5:433
Heart andVessel S © Springer-Verlag1992
Takayasu arteritis in China: A report of 530 cases Zheng Deyu, Fan Dijun, and Liu Lisheng Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences, Beijing 10037, China
Summary. A total of 530 patients with Takayasu arteritis were studied. Among 346 patients who were diagnosed by aortography, the female to male ratio was 2.9 to 1, and the age of onset ranged from 5 to 45 years. Three hundred and eighteen (60%) patients with secondary hypertension, including renovascular hypertension in 281, and 197 (37.2%) with pulseless extremities were found in the series. All the patients were treated with medical or surgical procedures. Surgical treatment is preferred if clinical status of the patient permits. The patients were followed for 1-29 years (average 7.8 years). There were 55 deaths (10.4%) in this series. Cerebral hemorrhage was found as a common cause of death. Five-year and ten-year survival rates were 93.1% and 91.1%, respectively.
Key words: Takayasu arteritis - Renovascular hypertension - Cerebral hemorrhage - Cerebral thrombosis
Introduction Since the early 1950s, we have observed a number of patients suffering from partial or complete obstruction of the aorta and its main branches. Becauses of their divergent clinical pictures these cases were diagnosed as "pulseless disease . . . . atypical congenital coactation of aorta", "hypertension due to renal arterial disease", and others with the help of retrograde arterial catheterization and selective aortography. Hwang and Liu found that the different clinical features were all manifestations of a common pathologic process, namely aortoarteritis [1, 2]. In 1962 they put forward the concept that aortoarteritis is a disease entity and this term should be substituted for the timehonored "pulseless disease . . . . atypical coactation of aorta". Since than a large number of cases have been Address correspondence to: Z. Deyu
diagnosed and treated in our hospital [3-7]. The present series comprises 530 patients with aortoarteritis admitted to Fu Wai Hospital between the years 1958 and 1990.
Material and methods A retrospective study was carried out in 530 patients with aortoarteritis during a 32-year period up to 1990. Among them, 346 patients with Takayasu arteritis were diagnosed by aortic angiography and 184 patients by their age, history, pulselessness or neck or abdominal bruit, as well as by the exclusion of artherosclerosis, congenital aortic constriction, and other findings. In addition, arterial biopsy or autopsy confirmation was obtained in 49 cases. The patients were separated into 4 categories, according to the classification of Ueno and associates and modified by Lupi HE. et al. [8]. Pulmonary angiography was carried out in 67 cases. The criteria used to evaluate the effects of surgical treatment were as follows: recovery of blood pressure and blood flow to the normal range after operation was considered as excellent, decrease in systolic and diastolic blood pressure by more than 20 mmHg as good, and no change in blood pressure and blood flow as poor.
Results Females comprised 395 cases (74.5%) and 135 (25.5%) were males. Their age ranged from 7 to 59 years (average 25.8 years) and the age of onset a n g e d from 5 to 45 years (average 22.3 years) (Table 1). History of onset of illness varied from 2 weeks to 38 years. Past history of tubereulosis was found in 102 cases (19.2%) and active lesion was found in 157 cases (29.6%). The clinical features of the patients are shown in Table 2. Of the 318 patients with secondary hypertension, including renovascular hypertension in 281, hypertension due to constriction of the descending thoracic aorta in 37 were confirmed by angiography and clinical findings. Among them, mild hypertension in 79
Z. Deyu et al.: Takayasu arteritis in China
33
Table 1. Sex and age of the patients studied Years
Left
Left > Right
Total
Right > Left
Left > Right
Total
65 25 26 22 15 42 195
38 17 7 3 5 14 84
103 42 33 25 20 56 279
73 11
58 15 2 1 1 20 97
131 26 2 2 3 33 197
10-30 31-50 51-70 71-90 91-110 111Total
1 2 13 100
Table 6. Topography of the arterial lesions in 105 patients with Takayasu arteritis (from DSA) Artery Left Subclavian Right Left Carotid Right Left Vertebral Right Innominate Thoracic aorta Abdominal Left Renal Right Superior Mesenteric Inferior Celiac Left Iliac Right Hepatic Pulmonary
Stenosis
Obstruction
Dilatation or aneurysm
Irregular
Total
29 21 13 10 6 2 11 15 47 28 28 5 1
47 21 13 13 2 2 2
1 3
5 3 1
82 48 27 24 9 4 17 26 58 34 36 25 3 4 8 10 4 33
5 6 1 33/73
1 6 8 20 2 4 3 2 3
1 1 2 4 5
2
2 7 5
DSA, Digital subtraction angiography
80/153) were type IV. Involvement of the pulmonary artery was found by lung perfusion scanning and chest X-ray or pulmonary angiography in 153 patients with aortoarteritis. Pulmonary arterial involvements were found by pulmonary angiography in 40 (53.3%) out of 75 cases. The right pulmonary artery in the upper lung field was most often involved, while the left pulmonary artery in the lower or middle-lower lung field was next in involvement. Five patients with pulmonary hypertension were confirmed by right heart catheterization. Pulmonary artery systolic pressure was as high as l l 2 m m H g and diastolic pressure was 32mmHg in one patient. Medical treatment included hormones, vasodilator, antituberculosis drugs antihypertensive drugs, and traditional Chinese medicine. Seventy cases in the active stage were treated with prednisone (Dexamethasone in 3 cases). The initial does was 20 to 40mg per day for 4 - 5 weeks and the dose was reduced gradually to a minimum maintenance dose of 5mg per day for 6 months-1 year (18-24 years in 2 cases). Long-term administration
of 5mg prednisone was an effective therapy in the present series. Eighty cases were treated with antituberculosis drugs with no effect found in the patients with Takayasu arteritis. Thirteen patients with renovascular hypertension were treated by percutaneous transluminal renal angioplasty (PTRA), 10 by unilateral P T R A (autorenal transplantation in one case), and 3 by bilateral PTRA. The success rate of P T R A was 84.6%. There were 8 cases followed-up for 2 - 8 years. The levels of blood pressure were still normal, except in one case (restenosis). One hundred and thirty-seven patients underwent 147 procedures in the present series, including type I in 3 cases, type II in 84 cases, and type III in 50 cases. One hundred and four cases were followd-up for 6 months to 23 years (average 8.7 years). The long-term results of r e p h r e c t o m y (excellent or good in 55/57, poor in 2/57) and revascularization (excellent or good in 40/47, poor in 7/47) were observed. All the patients were followed-up for 1-29 years (average 7.8 years). The age at death ran~ed
Z. Deyu et al.: Takayasu arteritis in China
35
Table 7. Causes of death in 530 patients with Takayasu
arteritis Cause Cerebral hemorrhage Postoperative complication Renal failure Cardiac failure Acute myocardial infarction Cerebral thrombosis Rupture of dissecting aneurysm Rupture of false aneurysm Tuberculosis Cancer of stomach Unknown Total
Total
%
13 4 4 3 3 2 1 1 1 1 22 55
23.6 7.3 7.3 5.5 5.5 3.6 1.8 1.8 1.8 1.8 38.2 100.0
from 14 to 56 years. There were 55 cases (10.4%) of death (Table 7). Six cases were autopsied. Thirteen cases died of cerebral hemorrhage (Table 7). Five-year and ten-year survival rates were 93.1% and 91.1% respectively.
Discussion
A total of 1200 patients with Takayasu arteritis (including our series) were reported in China. Of the patients, the female to male ratio was 3.3 to 1 and their age ranged from 5 to 64 years. The most important sign was a systolic or systolic-diastolic bruit heard over the epigastrium or umbilical region or neck. Systolic bruits over the epigastrium and umbilical region radiating the back or down the iliac arteries orten indicated the extensive involvement of the descending aorta. In comparison with renal arterial angiography, 81 of 104 (77.9%) unilateral renal artery stenosis and 91 of 99 (91.9%) bilateral stenotic cases had bruits, but more than 50% showed only I - I I degree soft bruits and the degree of the murmur was not related to the degree of stenosis. Arterial hypertension was present in 58.4% of our patients, mainly resulting from involvement of the renal arteries which occured in 51.2% of cases demonstrated by aortography. About 37% of the cases had diastolic blood pressure over 125 mmHg and 31% had systolic blood pressure over 200 mmHg. Abrupt onset of hypertension was observed in 53 cases (16.6%), manifested as accelerated or malignant hypertension or left heart failure. Renal artery lesion due to aortoarteritis is one of the most important causative processes of renovascular hypertension in China, particularly in female adolescents and young adults. About 37% of the cases had pulseless extremities as the first clinical sign. Ocular manifestation of "Pulseless Disease" was not found frequently in the present series (14%). Diastolic murmur was heard in the aortic valves in 11 of the patients with aortic regurgitation;
X-ray findings showed dilatation of the ascending aorta in all the patients with aortic regurgitation. Subclavian artery and carotid artery were involved in these cases [9]. Takayasu arteritis is a nonspecific inflammatoryfibrotic process involving the aorta and its main branches as well as the pulmonary artery. The end results of the marked fibrosis and thickening of the arterial wall was usually a constriction or obstruction (95%) and occasionally a saccular dilatation or aneurysm (5%) in the present series. The number of vessels involved with aortoarteritis varied from 1 to 13 branches (average 3.5). According to DSA findings, subclavian artery, especially the left subclavian artery, abdominal aorta, renal artery, thoracic aorta, carotid artery, superior mesenteric artery, and pulmonary artery were more frequently involved. The involvement of the abdominal aorta with the renal artery was found in 77% of the patients. There was no significant difference between the involvement of the left or right renal artery. Associated lesions involved the renal arter is bilaterally in about 2/3 of the cases, orten being more severe on one side [10]. The involvement of the pulmonary artery in patients with aortoarteritis is not uncommon. About one-half of the patients in this series had pulmonary artery involved, especially the right pulmonary artery in the upper lung field, a finding similar to that of others [11]. Clinically, the diagnosis should be differentiated from pulmonary artery disease of other etiology such as pulmonary thromboembolism or primary pulmonary hypertension. Unilateral popliteal artery pressure could not be measured in 41 cases in the present series. Diseases such as Bueger's disease or atherosclerosis should also be differentiated. As to the treatment of Takayasu arteritis, no specific remedy is as yet avalable. Corticosteroids administered to 67 cases in the present series in the active or acute stage induced remarkable clinical remission. We belive that long-term administration of a small dosage of corticosteroids is effective in treating this disoder. The response to treatment, if heart failure due to systemic arterial hypertension has occurred, is usually good. However, the effect of treatment of antituberculosis in the patients is still unknown. PTRA should be performed first when indicated. Surgical treatment should be preferable during the chronic phase and be carefully evaluated in each case. Because it is an important factor affecting prognosis of the disease, the renal artery lesion and associated lesions of the aorta and its main branches is of vital importance in surgical intervention as well as in judging the prognosis. With the development of surgical techniques, revascularization should be adopted first when indicated. Prognosis is good in most patients with aortoarteritis. The patients are able to engage in light work because of the development of collateral circulation of the
36 involed artery in the present series. The oldest patient in China was 64 years old. The prognosis of aortoarteritis depends mainly on the level of elevated blood pressure and the status of cerebral circulation. About 70% of the patients developed complications within 5 years of onset of this disease. Cerebral hemmorrhage was more orten found in patients with renovascular hypertension and ischemic complications. However, cerebral thrombosis was more frequently found in patients with normotension. We found that 18 patients with congestive heart failure due to severe renovascular hypertension had no aortic regurgitation. Two of them had elevation of central arterial pressure with enlargement of the heart while the blood pressure was unmeasurable in the both extremities, thus necessitating a differential diagnosis. As to the mechanism of aortic regurgitation, dilatation of the involved ascending aorta, separation of the aortic commissures and thickening of the valve cusps should be considered. Five patients with myocardial infarction, and 6 with angina pectoris were found in this series. Coronary angiography was normal in two patients with angina pectoris due to aortic regurgitation. In one patient with angina pectoris, the occlusions in both cephalicbrachial arteries and the branches of the coronary artery (left arterial branch) supplied blood to the branchialcephalic artery to form collateral circulation, known as coronary steal syndrome. There were changes of pulsation of the radial artery during the 1-10 year follow-up period, but no further changes of pulsation after 10 years of medical treatment. About 40% of the pulsation of the involved arteries in both upper arms after treatment was improved due to the development of collateral circulation or thrombolysis. Mortality rate of aortoarteritis is 10.4%
Z. Deyu et al.: Takayasu arteritis in China in the present series. About one third of the patients died of original complications. The mortality rate of cerebral hemorrhage is higer than that of congestive heart failure in present series. The five-year and ten-year survival rates were 90.3% and 91.3% respectively. This might be because most of that patients had been subjected to long-term drug treatment.
References 1. Hwang W, Liu LS (1962) Constrictive arteritis of the aorta and its main branches. Chin Med J 81:526 2. Liu LS, Hwang W (1963) Constrictive arteritis report of cases. Chin J Int Med 4:293 3. Zheng DY, Wu X, Liu LS, Liu QH (1973) Aortoarteritis, clinical study of 200 cases. Chin J Cardiovas Dis 1:43 4. Zheng DY, Liu LS, Liu QH (1985) Clinical study in 372 patients with aortoarteritis. Beijing Med J 7:200 5. Zheng DY, Liu LS, Liu QH (1981) Clinical study of pulmonary artery involvement in aortoarteritis. Chin J Cardiovas Dis 9:22 6. Liu LS, Zheng DY (1990) Aortoarteritis: A report of 480 cases. J Human Hypertension 4:135 7. Zheng DY, Liu LS, Fan DJ (1990) Clinical studies in 500 patients with aortoarteritis. Chin Med J 103:536 8. Lupi HE, Sanchez TG, Horwitz S, Gutierrez FE (1975) Pulmonary artery involvement in Takayasu's arteritis. Chest 67:69 9. Zheng DY, Wang J, Xin JX, Liu LS (1989) Clinical study of aortic regurgitation in aortoarteritis. Chin Cardiovasc Dis 17:21 10. Liu YQ, Du JH (1982) Aorto-arteritis Further angiography study of 231 cases. Chin Med J 95:15 11. Liu YQ, Jin BIù, Ling J (1990) Pulmonary artery involvement in Aortoarteritis an angiographic study. Chin Circulation J 5:433
