Pulseless diseaseOcclusive thromboaortopathy

Martorell syndromeAortic arch syndrome

Dr Frijo Jose A

Takayasu’s Arteritis

• 1830- Yamamoto • Kitsuo idan, meaning “Medical records of my private

hospital with the big orange tree”

• 1905- Takayasu, proff oph, presented the case of a 21 year old woman with characteristic fundal arteriovenous anastamoses

• Chronic inflammatory arteritis • Large vessels, esp, Aorta & its main branches

(brachiocephalic, carotid, SCL, vertebral, RA) • as well as Coronary & PA

• Chronic vasculitis • Affects Intima, Media, and Adventitia of larger

vessels • Wall thickening, Fibrosis, Stenosis, & Thrombus

formation →end organ ischaemia• More acute inflammation → destroys arterial

media → Aneurysm (fibrosis inadequate)• Stenotic lesions predominate & tend to be B/L• Nearly all pts with aneurysms also have stenoses

Two stage process

• 1. “Pre-pulseless” phase– Non-specific inflammatory features- fever, myalgias,

weight loss, & arthralgias• 2. Late occlusive phase– Vasc insufficiency, Diminished/absent pulses (84–

96%), Bruits (80–94%), Hypertension (33–83% ), RAS(28–75%) & CCF

• ± Intermittent flares

CHARACTERISTIC FEATURES

• ↓/− pulses (84–96%) -claudication & BP Diff• Bruits (80–94%) -carotids, subcl & abd vess• HTN (33–83%) -RAS (28–75%)• Retinopathy (37%)• AR (20–24%) -Dilated asc ao, valve thickening• CCF -HTN,AR,DCM• Neurological (HTN ±isch) -postural dizziness,

seizures, and amaurosis

d/d Coartation of aorta

• Symmetrical clinical involvement-co a(+rt ul)• Palpable collaterals-co a• Palpable abdominal ao pulsations- ta• Myocarditis- ta• Extensive bruits- ta• Fundus• Ar-both

Tuberculosis

• Erosion of vessel wall • True/False aneurysms• Esp- Desc thoracic & abdominal Ao• Dissection & Rupture rather than Stenoses

Syphilis

• Older age group• Calcification• Spares Desc thoracic Aorta• Stenoses are not a feature

1990 ACR criteria

A diagnosis of Takayasu arteritis requires that at least 3 of the 6 criteria are met

Moriwaki et al Indian JapaneseFemales 63% 96%

Presentation Headache, HTN, and LVH

Dizziness, Vertigo,Pulselessness

Abd aorta & RALess AR

Ao arch & branchesMore AR

Diffuse disease Diffuse disease

Ishikawa clinical classification of Takayasu arteritis

4 Complications Retinopathy, Secondary HTN, AR, & Aneurysm

Severity of Retinopathy (Uyama & Asayama‘s Classification)• stage 1- Dilatation of small vessels• stage 2- Microaneurysm formation • stage 3- Arterio-venous anastomoses• stage 4- Ocular complications

Mild -stage 1Moderate -stage 2Severe -stages 3 & 4

BP values were graded according to: Mild • brachial – SBP -140 to 159 mm Hg and/or 90 to 94 mm

Hg DBP, • popliteal – SBP- 160 to 179 mm Hg and/or 90 to 94

mm Hg DBP; Severe • brachial – SBP - ≥200 mm Hg and/or ≥110 mm Hg

DBP• popliteal – SBP- ≥230 mm Hg and/or ≥110 mm Hg DBPModerate • Between mild and severe forms.

Severity of aneurysm of Ao & branches (Angio)• Severe – D >twice that of normal vesselsSeverity of AR• estimated angiographically or clinically

Lupi-Herrera Classification

4 types: 1.Aortic arch variety • ≥1 of the 3 arch vessels 2.Thoracoabdomimal variety • Descending thoracic and/or abdo aorta and their

branches3.Combined variety• Both Arch vessels & thoracoabdo aorta4.Pulmonary variety • PA in combination with any of the preceding 3 types

4 types Type I (Shimizu- Sano variety) • Aortic arch & Brachiocephalic VesselsType II (Kimoto variety) • Thoracic descending & abdominal AortaType III• Both types togetherType IV (Lupi-Herrera variety)• Features of types I, II, & III in any combination

with PA involvement

Cumulative survival • 5years -91% (event free survival -74.9%)• 10 years -84% (event free survival -64%)Single mild complication or no complication • 5 year event free survival 97% Single severe or multiple complications• 5 year event free survival 59.7%No deaths in groups I and IIA19.6% mortality in groups IIB and III (CVA,CCF)

New angiographic classification ofTakayasu arteritis, Takayasu conference 1994

Treatment of TA

　・　　

Steroids

immunosuppressants：Cyclosporin,Cyclophosphamide,Methotrexate,Mycophenolate mofetil

Anti-platelet therapy（ low-dose Aspirin）

angioplasty/surgery

If uncontrolled

Control of vasculitis

Symptomatic occlusion

thrombosis

Medical treatment

• Steroids → 50% response• Methotrexate →further 50% respond• 25% with active disease will not respond to

current treatments

Indications and continual of steroids

Surgical treatment

• HTN with critical RAS• Extremity claudication limiting daily activities• Cerebrovascular ischaemia or critical stenoses of ≥3

cerebral vessels• Moderate AR• Cardiac ischaemia with confirmed coronary involvement

Recommended at quiescent state (restenosis, anastamotic failure, thrombosis,

haemorrhage, & infection)

Renal artery involvement

• Best treated by PTA• Stent placement following PTA– Ostial lesions– Long segment lesions– Incomplete relief of stenoses – Dissection

Sharma S et al, AIIMS Am J Roentgenol. 1992 Feb;158(2):417-22

• Renal PTA - 33 stenoses (20 pts) • Tech success -28 lesions (85%) • Failures - Coexistent abd Ao disease & tight, prox RAS• Tech diffi - tough, noncompliant stenoses, difficult to

cross & resisted repeated, prolonged balloon inflations - backache & ↓SBP during balloon inflation

• Follow-up 1-18 /12 (~8/12) -restenosis in 6(21%)• Renal PTA in TA -tech difficulties; Short-term results -

good, Complication rate-acceptable

aortoarteritic lesions

Balloon dilation diff from atherosclerotic lesions

• Minimal intimal involvement –permits easy wiring and balloon crossing

• Resistance to dilation – high fibrotic element in the stenotic lesion

Rao SA et al, SCT Radiology. 1993 Oct;189(1):173-9

• PTA -desc thoracic and/or abd Ao (TA) stenosis

• 16 pts (12+4)- HTN/severe B/L LL claudication• Aortography – stenosis→ desc

thoracic Ao-5, abd Ao-10, Both -1• Initial tech & clinical success -100% • Follow-up (mean 21/12,2/52)- Restenosis -3 • PTA has a definite role in TA management

Joseph S et al, SCTJ Vasc Interv Radiol 1994;5:573–580

• PTA- Scl A in TA• 24 pts (15 +9) →26 Scl A

VB insufficiency, UL claudication, or both

• Aortography → 19 steno ,7 occlu (focal-14 ,< 3 cm) • Initial tech & clinical success – 81%

(17 /19 steno,4/7occlu) • Follow-up → ~26/12 (max 82/12) → ISR -6 ( all ext)• Long-term results -excellent in focal lesions ,less

durable extensive disease

Tyagi S et al, GB Pant Cardiovasc Intervent Radiol. 1998 May-Jun;21(3):219-24

• To compare PTA- Scl A in TA & athero• 61 Scl A PTA (TA = 32 & athero = 29) • 3-120/12 (~43.3 +/- 28.9/12) follow-up of 40 pts• TA -Higher balloon inflation P• TA -more residual stenosis • TA –restenosis more• These lesions could be effectively redilated • TA -Subclavian PTA - Safe, can be performed as

effectively as in athero, good long-term results

Surgical techniques• Carry high morbidity & mortality• Steno /aneurysm -anastomotic points • Progressive nature of TA• Diffuse nature of TA

Balloon dilation • safe & reasonably effective• Can be performed repeatedly without any

added risks

However

• Post-PTA restenosis more frequently in TA than in atherosclerotic diseases, esp,in diffuse and long stenotic lesions

Takahashi et alAm J Neuroradiol 23:790–793, May 2002

• 1 TA pt –multiple supra-aortic lesions• B/L CCA, innominate, Lt Scl A• 2-staged stent implantation (to avoid drastic

changes in cerebral hemodynamics)• No restenosis -2 years despite rec inflamm

(2relapses) -may be effects of predonisolone

Stage-1

• 30-mm Palmaz stent mounted on a 40 mm PTA balloon-deployed at Lt Scl A stenosis

• Prox Rt CCA -dilated with 40 mm PTA balloon • Because long-term outcome of carotid PTA, with

or without stent , was not known for inflammatory arteritis-did not use stent support for CCA lesion during 1st treatment

• Ticlopidine (100 mg/day), cilostazol (100 mg/ day), prednisolone (5 mg/day)

Stage-2

• After 1/12 • 50 mm self-expanding Easy Wallstent -placed

from Rt CCA to innominate A• 50 mm Easy Wallstent -deployed in Lt CCA• Dilated -40 mm PTA balloon

Coronary involvement in TA

• Occurs in 10～ 30%• Often fatal• Classified into 3 pathorogic types

Type1:stenosis or occlusion of coronary ostiaType2:diffuse or focal coronary arteritisType3:coronary aneurism

Treatment for cor A occulusion in TA

Surgery (CABG,MIDCAB)- often not indicated・ IMA can’t be used often– occlu of Innomi A / Scl A– calcification of aorta

High incidence of restenosis:36％Angioplasty(PTCA)・ alternative to surgery Very high incidence of restenosis:78％DES?

Arterial access in ta-all 4 limbs involved

• Venous- trans-septal• Trans-seeptal problem– No aortic land mark– May be tackled by– His bundle catheter– Atrio-ventricular groove fat visualisation on flouro

PREGNANCY

• Pregnancy per se does not appear to exacerbate TA- management of HTN essential

• Meas of BP in UL –impossible/unreliable → oft more accurate in legs

• HTN in 2nd stage Labour –risk for ICH; shortening stage by low forceps /vacuum –reasonable

• pre-eclampsia, CCF, progressive RF,CVA

Take home messages

• Angiography remains gold standard for diagnosis• 4 most imp complications - Retinopathy,

secondary HTN, AR & aneurysm• ~½ on steroids will respond, and ~½ remaining

respond to methotrexate, mycophenolate mofetil• Pregnancy does not appear to exacerbate the

disease, although management of hypertension is essential

Thank You..