Cardiovasc Intervent Radiol (1994) 17:147-151 CardioVascular and Intervenfional Radiology �9 Springer-Verlag New York Inc. 1994

Influence of Angiographic Morphology on the Acute and Longer-Term Outcome of Percutaneous Transluminal Angioplasty in Patients with Aortic Stenosis Due to Nonspecific Aortitis

Sanjiv Sharma, Savitri Shrivastava, Shyam Sunder Kothari, Upendra Kaul, Mira Rajani Department of Cardiovascular Radiology, Department of Cardiology, C.N. Centre, All India Institute of Medical Sciences, New Delhi 110029, India

Abstract Purpose: We studied the relationship o f initial angio- graphic morphology in patients with aortic stenosis due to nonspecific aortitis and its relationship to immediate and later outcome following percutaneous transluminal angioplasty (PTA). Methods: Correlation was performed in 10 successive patients by retrospective analysis. All had clinically in- active nonspecific aortitis and hemodynamical ly sig- nificant aortic stenosis resulting in hypertension or lower limb claudication. Five patients had discrete con- centric stenosis; the other five had eccentric stenosis with diseased aortic segments adjacent to the stenosis. Results: The five patients with concentric stenosis (Group I) had primarily successful and uncomplicated PTA with sustained improvement. The five patients (Group II) with eccentric stenosis had initial success in three patients and two initial treatment failures with one patient showing late improvement. Four o f these pa- tients developed large intimal flaps. One of these had an aneurysm during follow-up. Follow-up angiograms in five patients showed remodelling with further angi- ographic and clinical improvement. Conclusion: Eccentricity of the stenosis and diffuse aortic disease correlate unfavorably with immediate outcome of PTA but late improvement may still be seen.

Key words: Aortic arch syndrome--Ar te r ies , translu- minal a n g i o p l a s t y - - A o r t a - - H y p e r t e n s i o n - - Aorti- t i s - - T a k a y a s u ' s arteritis

Nonspecific aortitis, also known as Takayasu 's disease, is a rare form of primary aortitis of unknown cause that

Correspondence to: S. Sharma, M.D.

commonly involves the aorta, its major branches and the pulmonary arteries [1 -5] . It results in stenosis, oc- clusion, dilatation, or formation of aneurysms in the involved blood vessels [2, 3]. Of these, stenosing le- sions are the most common, frequently involve the aorta, and can result in systemic hypertension or lower limb claudication [3]. Some form of revascularization is necessary to relieve symptoms resulting from isch- emia distal to the stenosis. Reconstructive vascular sur- gery is technically difficult due to the widespread nature and complexity of pathological changes in the wall of the aorta [6]. Percutaneous transluminal angio- plasty (PTA) provides an alternative mode of treat- ment. The acute results of this treatment method have been reported by several authors [7 -15] .

In our patients, we observed that angiographic fea- tures of the stenosis appeared to influence the acute result of PTA [10, 11]. There is a paucity of data in the literature addressing this issue. In this study, we have analyzed the influence of angiographic features o f the stenosis on the acute and long-term results o f aortic PTA in a group of patients.

Materials and Methods

During a 54-month period, nine patients with systelnic hypertension, and one patient with lower limb claudication secondary to stenosis of the aorta by nonspecific aortitis were treated with PTA, The group included six females and four males (mean age, 18.5 years; range, 6-28 years) (Table I). Each patient's diagnosis was based on the criteria established by the Aortitis Syndrome Research Committee of Japan [16]. The patients were divided into two groups based on the angiographic appearance of the stenosis and the adjacent aortic seg- ments. Group I included five patients with a discrete concentric ste- nosis of the aorta and normal adjacent segments (Fig. 1). The lesion was located in the thoracic aorta in three and in the abdominal aorta in two patients. Group II included five patients with an eccentric stenosis of the descending thoracic aorta with diffuse disease in the adjacent segments (Fig. 2).

148 S. Sharma et al.: Aortic Angioplasty in Takayasu's Arteritis

Table 1. Salient features of patients who underwent angioplasty of the aorta

S. Age Sex Location and Clinical Pressure Luminal Blood pressure Drugs Complication Follow-up No (years) angiographic features indication gradient diameter response period

of aortic stenosis (mm Hg) stenosis (%) (mm Hg)

B A FU B A FU B A FU* B A

1. 23 F Concentficshort Intermittent 32 6 0 90 30 0 130/80 130/80 120/80 segment infrarenal clandication (6 mo)

2. 12 M Concentric short Uncontrolled 40 10 12 80 20 10 180/120 130/90 120/80 4 segment perirenal hypertension (24 mo)

3. 10 F Eccentric long segment Uncontrolled 70 50 - - 80 30 - - 160/110 150/110 120/80 4 descending thoracic hypertension

4. 28 M Eccentric long segment Uncontrolled 90 0 - - 75 10 0 200/120 160/100 120/80 4 descending thoracic hypertension (18 mo)

5. 6 F Eccentric longsegment Uncontrolled 100 80 20 descending thoracic hypertension

6. 14 M Concentric short Uncontrolled 70 20 - - segment descending hypertension thoracic

7. 27 F Concentric long Uncontrolled 70 12 - - segment descending hypertension thoracic

8. 28 F Concentric short Uncontrolled 60 12 - - segment descending hypertension thoracic

9. 14 M Eccentric long segment Uncontrolled 90 40 - - descending thoracic hypertension

10. 10 F Eccentric longsegment Uncontrolled 90 48 26 descending thoracic hypertension

90 30 0 160/110 130/80 120/80 4 (3 too)

80 10 - - 170/120 140/90 120/80 4

None

1 None

4 Large flap

2 Extensive dissection; aneurysm on follow up

l Large flap

1 None

80 0 - - 170/110 130/90 130/80 3 1 None

70 0 - - 190/110 130/80 120/80 4 1 None

36 months

53 months

I month

24 months

12 months

12 months

12 months

14 months

70 10 - - 220/106 130/80 120/80 3 1 None 7months

90 0 0 180/110 120/70 120/70 4 1 Large flap 7 months (7 mo)

B = before PTA; A = after PTA; FU = follow up; * = at the end of follow up Drugs: 1 = single dose; 2 = double dose; 3 = triple dose; 4 = quadruple dose

Patients eligible for angioplasty had angiographic evidence of >70% aortic stenosis with a peak systolic pressure gradient of >20 mm Hg; and a normal sedimentation rate (<20 nun in first h). Pa- tients were treated with oral aspirin (175 mg) and dipyridamole (75- 150 mg) daily for 3 days before angioplasty, and this regimen was

continued daily for 6 months after treatment. Heparin (100 IU/kg body weight) was given IV during the procedure, and for the follow- ing 24 h in order to avoid acute thrombotic complications. Four pa- tients with large intimal flaps did not receive beparin after the procedure. The blood pressure was kept below 140/90 mm Hg during the 24 h after the procedure.

All procedures were performed by the percutaneous transfemoral route. The stenosis was crossed with a multipurpose catheter (Cordis, Miami, FL, USA) over a 0.035-inch steerable guidewire (Terumo, Tokyo, Japan) and a transstenotic pressure gradient was measured. After performing a diagnostic arteriogram, the angiographic catheter was replaced by an appropriately sized balloon catheter (Medi-Tech Inc., Watertown, MA, USA). The diameter of the balloon was chosen as three times the diameter of the stenosed segment of the aorta, or equal to the diameter of the "normal" segment adjacent to the ste- nosis, whichever was smaller [17]. The balloon diameters ranged from 5 to 12 mm. The balloon was inflated by hand three to five times using a 20-ml syringe, for up to 45 s each, until the balloon "wais t" disappeared or decreased substantially. Gradually increasing balloon sizes were used in most patients, beginning with a smaller balloon diameter and gradually increasing to the calculated balloon size for the patient. Immediately after PTA the transstenotic pressure gradient was remeasured, and an aortogram was performed. PTA was considered technically successful if 1) the aortic lumen after angio-

plasty had <30% residual stenosis, with at least 50% increase in its pretreatment diameter, and 2) the pressure gradient was <20 mm Hg.

In the patients with hypertension, the clinical results of PTA were judged as follows: 1) "cured" (normal blood pressure after the pro- cedure without antihypertensive drug therapy) 2) " improved" (at least 15% reduction in diastolic blood pressure or a diastolic pressure less than 90 mm Hg with the patient taking less antihypertensive medication than before the procedure), and 3) "fai led" (no change in blood pressure after the procedure). All patients "cured" or " im- proved" were considered to have benefited from PTA. The clinical result of F r A in the patient with lower limb claudication was assessed by relief of symptoms, and improvement in the peripheral pulses. Follow-up examination included blood pressure and medication eval- uation at 1 week, 4 - 6 weeks, and at 6-month intervals thereafter. Angiography was performed during follow-up in five patients.

Results

A l l 10 p a t i e n t s e x p e r i e n c e d i n t e n s e , t r a n s i e n t b a c k a c h e

d u r i n g b a l l o o n i n f l a t i o n , w h i c h s u b s i d e d s o o n a f t e r t h e

b a l l o o n w a s d e f l a t e d . F o r r e d u c t i o n in s t e n o s i s a n d

b l o o d p r e s s u r e r e s u l t s s e e T a b l e 1 a n d F i g u r e 3.

A l l f ive p a t i e n t s in G r o u p I u n d e r w e n t t e c h n i c a l l y

a n d c l i n i c a l l y s u c c e s s f u l a n g i o p l a s t y , w i t h o u t c o m p l i -

S. Sharma et al.: Aortic Angioplasty in Takayasu's Arteritis 149

Fig. 1. A. 28-year-old woman with uncontrolled hypertension. Aortogram in the anteroposterior and lateral views shows a smooth, discrete con- centric stenosis in the descending thoracic aorta. B. Post-angioplasty aortogram in same views shows improvement in aortic caliber with < 10% residual stenosis.

cations. The procedure resulted in clinical cure in the patient with lower limb claudication, and clinical im- provement in all four patients with hypertension.

In Group II, PTA was technically and clinically successful in three patients. The remaining two patients showed minimal reduction in the transstenotic pressure gradient after PTA, despite >50% increase in the aortic caliber. The drug requirement for hypertension did not decrease. These two cases were, therefore, considered technical and clinical failures. During follow-up, one patient showed marked clinical improvement, and re- peat catheterization at 3 months showed a 20-mm Hg residual gradient and an almost normal aortic caliber. The second patient was lost to follow-up. Large intimal flaps were seen at the angioplasty site in four of five patients in Group II, including the two patients consid- ered procedural failures. One of them developed an ex- tensive dissection. In this patient, the balloon waist was suddenly relieved during a fourth inflation. The patient complained of transient severe back pain but remained hemodynamically stable. The transstenotic pressure gradient disappeared. Post-PTA aortography showed a normal caliber but extensive dissection beginning at the PTA site and extending to the aortic bifurcation. The blood pressure did not fall immediately but returned to

normal over the next 48 h. The patient remained asymptomatic during this period. He developed mild hypertension after 1 week and was well-controlled on two drugs. Intravenous digital subtraction angiography at 18 months showed an aneurysm at the angioplasty site. The patient refused surgery. At 2-year follow-up, his blood pressure was well-controlled and he had no chest symptoms.

The follow-up period for all patients ranged be- tween 1 and 53 months (mean, 17.8 months). No clin- ical evidence of restenosis was seen. Remodelling at the angioplasty site with further angiographic and clin- ical improvement was seen in all five patients who un- derwent follow-up angiograms (see Table 1). During follow-up, no clinical or laboratory evidence of disease activity was found.

D i s c u s s i o n

Most reports of PTA in nonspecific aortoarteritis relate to the renal arteries [ 18-23]. Transluminal angioplasty in the treatment of stenosis of the aorta has been re- ported infrequently [7-15]. Tyagi et al. [13] recently reported that patients with a short segment ( < 4 cm)

150 S. Sharma et al.: Aortic Angioplasty in Takayasu's Arteritis

Fig. 2. A. 6-year-old girl with uncontrolled hypertension. Aortogram shows a tight steno- sis in the descending thoracic aorta with dif- fuse disease in the adjacent segments. B. Aor- togram in the anteroposterior and lateral views after angioplasty shows good opening of the lesion with a large intimal flap (arrow). C. Follow-up aortogram in the same views 3 months later shows sustained improvement with remodelling at the angioplasty site.

stenosis experienced more relief than patients with long segment (>4 cm) stenosis.

In this study, we observed that angiographic fea- tures of the stenosis influenced the initial result of angioplasty. Those with discrete concentric stenosis (Group I) all had successful and uncomplicated PTA, 0Ol

181.1

150 1~6

~12.9

100

50

| |

SBP DBP

m PRE PTA ~ POST PTA PRE PTA m POST PTA

3.8

L |

DRUGS

END FU

END FU = END OF FOLLOW-UP

Fig. 3. Graph showing the changes in systemic blood pressure and drug requirement during follow-up.

whereas four of five patients with long, eccentric ste- nosis (Group II) developed large intimal flaps. One of them had a downward extension of the dissection which was attributed to angiographic features of the stenotic segment, sudden disappearance of the "waist ," and continued presence of hypertension in the post-PTA period [11]. Incidently, this was the first pa- tient who underwent angioplasty in this study. Follow- ing this complication, we resorted to using gradually increasing balloon sizes, and carefully controlled the blood pressure in the post-PTA period. These precau- tions probably helped to contain the flap to the angio- plasty site.

The exact mechanism of successful angioplasty in nonspecific aortoarteritis remains unclear. This disease is a panarteritis involving all layers of the vessel wall [2]. Underlying chronic inflammation, panarteritis, ex- tensive periarterial fibrosis, thickening, and adhesions combine to produce tough, noncompliant and rigid ves- sel walls. The resultant stenosis resists prolonged, re- peated mechanical distension before responding to balloon dilatation [23]. The sustained pressure required to increase the arterial caliber can inflict significant trauma at the angioplasty site. The presence of an ec-

s. Sharma et al.: Aortic Angioplasty in Takayasu's Arteritis 151

centric, long segment stenosis, and diffuse disease in the adjacent segments may contribute to the severity of the int imal injury and its extension.

Block et al. [24] have suggested that the presence of disease in the adjacent segments helps to limit the extent of intimal split. One patient in their study de- veloped an extensive dissection, despite the presence of diffuse disease in adjacent segments. It is probable that the main reason for the extension of the intimal split in these patients is sustained hypertension in the immediate post-PTA period. This hypothesis is sup- ported by the fact that hypertension persisting after an- gioplasty was not immediately treated in one patient, and he developed an extensive dissection. We did not allow hypertension to develop after angioplasty in the remaining patients by using aggressive medical treat- ment, if necessary. The intimal injury remained local- ized to the angioplasty site in these patients. However, it is difficult to draw definite conclusions because this study involves a very small number of patients and does not compare the angiographic results in patients with hypertension versus those without hypertension post- PTA.

The pressure gradient did not fall despite > 5 0 % increase in the aortic diameter in two patients. Such a response can be produced by an obstructing intimal flap, with or without associated spasm. Although no immediate response to vasodilator drugs was seen, the femoral pulses which were not palpable before the pro- cedure were well felt 24 h after the procedure in both patients, with no pressure difference between the upper and the lower limb. One of them showed hemody- namic, angiographic, and clinical improvement dur- ing follow-up, probably due to remodell ing at the angioplasty site. Remodel l ing with further improve- ment was also seen in four other patients who under- went follow-up angiograms.

We conclude that PTA can be effective in treating stenosis in the aorta due to nonspecific aortitis. Re- modell ing may occur at angioplasty sites in nonspecific aortitis and can be responsible for late clinical improve- ment, despite initial technical and clinical failure. Eccentric stenosis with adjacent disease may be asso- ciated with dissection flaps, although the numbers in this series are too small to achieve statistical signifi- cance, but clinical success may still be achieved. Residual or reactive hypertension persisting after an- gioplasty should be aggressively managed.

Acknowledgment. We acknowledge the help of Drs. Kewal K. Tal- war, Vinay K. Bahl, Anita Saxena, and Vishwa Dev from the De- partment of Cardiology in the management of these patients.

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