1. PRESENTING AS COARCTATION OF AORTA
AND RENOVASCULAR HYPERTENSION
WITH DISSECTION OF AORTA.
DR. ABHAY MANGE
TAKAYASU‟S ARTERITIS

2. A 16 yrs old female patient was admitted with c/o
 Breathlessness since 2 months
 Swelling over lower limbs since 15
day
 Decreased urine output since 8
days
 h/o fever since 1 month +
 No h/o cough/chest
pain/hemoptysis
 No h/o joint pain
 No h/o intermittent claudication
 No h/o DM/PTB /RHD in past

3. General examination
 Conscious and oriented
 febrile
 Pulse -104/min in rt radial,regular,bounding
lt radial weak ,no r-r delay. femorals and popliteals
dorsalis pedis palpable, no radio-femoral delay.
 Blood pressure(mmHg) :- RT. UL =210/110 LT. UL
=180/100
 RT. And LT. LL =240/130
 JVP –raised
 Edema feet +
 pof +
 Pallor +
 No icterus/cynosis/clubbing

4. Systemic examination
 CVS:- PSM + III/VI AT APEX
 RS:-Basal crepitations present
 PA :-liver palpable 4cm /tender
Abdominal bruit +
 CNS :- NAD

5. Investigations
 Hb=8.8 gms%
 TLC=7000/cumm P=69%,L=27%,E=2%,M=2%
 PS:-RBC‟S were normocytic normochromic with mild
anisopoikilocytosis,platelets –adequate,no parasite
 ESR =36 mm in 1st hr.
 URINE :Protein ++,sugar-nil,5-6 pus cells ,10-12 rbcs
 KFT: –BUL=265mg%,Sr.Cr.=3.5mg%
 Na =122 ,K=3.8 meq/l
 LFT:-TP =6.2, TB=2.7,ALP=325,OT =66,PT=268
 ANA :-0.29 OD RATIO

6. Investigations
 ECG:- S/O LVH
 X-RAY CHEST PA s/o cardiomegaly
 2-D ECHOCARDIOGRAPHY s/o LVH, LV
hypokinesia,moderateMR,severe TR ,severe PH, LVEF
=20%.
 RENAL DOPPLER S/O obscured rt. Renal artey origin
 AV DOPPLER :Both upper and lower limb AV Doppler
was wnl except low psv biphasic waveform lt. upper
limb system.

7. ECG

8. X- Ray chest

9. Provisional diagnosis
 AORTOARTERITIS WITH RENAL ARTERY STENOSIS
WITH RENOVASCULAR HYPERTENSION WITH
SEVERE LV DYSFUNCTION.

10. MR Renal angiography
 Study revels Type B standford aortic dissection at
level of origin of renal arteries ,2 cm in diameter and 2.1
cm in length
 Lt. renal artery arising from true lumen
 Rt. Renal artery arising from false lumen with significant
reduction in caliber.
 Lt. kidney measure 9.6/4.7/5.2 cms and artery measure
6mm at ostia,3mm at hilum.
 Rt . Kidney measure 7.2/3.6/3.3cms and artery measure
2.5mm at ostia,1.8mm at hilum

11. MR Renal angiography

12. MR Renal angiography

13. MR Aortography
 Narrowing of mid 1/3 descending aorta for length 21 mm
and 7 mm diameter.
 No e/o collaterals
 f/s/o POST DUCTAL COARCTATION OF AORTA.

14. MR Aortography

15. Final diagnosis
 TAKAYASU‟S ARTERITIS
 COARCTATION OF AORTA
 RENAL ARTERY STENOSIS
 DISSECTION OF AORTA
 RENOVASCULAR HYPERTENSION
 RENAL INSUFFICENCY
 LV DYSFUNCTION

16. Treatment
 ANTIHYPERTENSIVE :-Tab Nicardepine 20 mg BD
Tab Metoprolol 50 mg OD
Tab Prazocin 5 mg BD
 DIURETICS :- Inj. Lasix 40 mg BD
 CVTS OPINION :-Advised endovascular stenting.

17. DISCUSSION

18. Introduction
 Takayasu‟s arteritis (TA) is an inflammatory vascular
disease of the young involving the large elastic arteries
resulting in occlusive or ecstatic changes mainly in the
aorta and its major branches as well as the pulmonary
artery and its branches.
 Also known by “pulseless disease”, “occlusive
thromboaortopathy”, and “Martorell syndrome”,
 predilection for females with wide geographical
variations
 M : F ratio in 1 : 6.4 in india
 TA may be diagnosed from childhood to late adult life,
 Rarely occurs over 40 years of age

19. History
 1827 by R. Adams who was the first physician to note
absence of pulse in all four extremities
 In1856 Savory reported a case of young female with
absence of pulses.
 In 1908, Mikito Takayasu, a Japanese opthalmologist
described a peculiar wreath-like appearance of retinal
blood vessels with absence of radial pulse.

20. Pathophysiology
 All the three layers causing extensive intimal
proliferation, inflammation of media and adventitia
followed by marked fibrous scarring.
 In older cases, hyalinization of deeper layers of intima
and dystrophic calcification can be seen.
 Skipped areas of aortic involvement are quite
characteristic of aortoarteritis.
 Histopathologically, adventitial mononuclear infiltrate
with perivascular cuffing of the vasa vasorum,
mononuclear inflammation, and rarely granulomatous
modifications.
 The subclavian artery, common carotid artery, vertebral
artery, and renal artery are more often affected.

21. Clinical presentation
 Early phase or pre pulseless phase described by
nonspecific systemic features (malaise, arthralgia mild
synovitis, weakness, myalgias weight loss, and low-grade
fever),
 Pulseless phase (vascular inflammatory phase) is
characterized by claudication, headaches, dizziness, and
amaurosis or diplopia, difficulty in looking upwards,
renovascular hypertension, chest pain or palpitation,
pulmonary (dyspneea,hemoptysis and pleurisy),
gastrointestinal (anorexia, nausea), and skin (rare, ulcers).
 Occlusive phase: usually involves common carotid artery
(visual defects, strokes, TIA), vertebral artery (dizziness,
visual defects), subclavian artery (arm claudication), aorta
(aortic regurgitation,cardiac heart failure), pulmonary artery,
cardiac and celiac axis, renal artery (renovascular
hypertension), iliac artery (claudication).

22. Diagnosis criteria
 The diagnosis of TA is largely based on the combination
of
1) clinical manifestations,
2) laboratory evaluation, and
3)diagnostic imaging.
 When the diagnosis is presumed, it is typically confirmed
by a radiographic technique.

23. American College OF Rheumatology
Classification
Criteria to be diagnosed with Takayasu’s
arteritis
must meet 3 out of 6 criteria:
 Age under 40 at disease onset;
 Claudication of extremities;
 Decreased branchial artery pulse;
 Blood pressure difference>10 mmHg between arms;
 Bruit over subclavian arteries or aorta;
 Arteriogram abnormality: occlusion or narrowing in aorta
or main branches.

24. Diagnostic criteria by Ishikawa
(1988).
 These consist of one
 obligatory criterion (age <40 years at diagnosis or onset of
disease),
 two major criteria (left and right mid subclavian artery
lesions) and
 nine minor criteria
 (high ESR, common carotid artery tenderness, hypertension,
aortic regurgitation or annulo-aortic ecatisa, lesions of
pulmonary artery, left mid common carotid artery, distal
branchio-cephalic trunk, thoracic aorta and abdominal aorta).
 In addition to the
 obligatory criterion, the presence of two major criteria , or
one major plus two or more minor criteria; or four or
more minor criteria suggests a high probability of the
presence of aortoarteritis.

25. The Ishikawa diagnostic criteria modified
by Sharma et al 1995
 Type I -only the branches of the aortic arch.n
 Type IIa - aorta only at its ascendingportion and/or at the aortic arch. The
branches ofthe aortic arch may be involved as well. The rest ofthe aorta is
not affected.
 Type IIb - the descending thoracic aorta withor without involvement of the
ascending aorta orthe aortic arch with its branches. The abdominalaorta is
not involved.
 Type III is concomitent involvement of thedescending thoracic aorta, the
abdominal aorta,and/or the renal arteries. The ascending aorta, theaortic
arch, and its branches are not involved.
 Type IV involves only the abdominal aorta and/orthe renal arteries.
 Type V is a generalized type, with combined

27. Sharma modified criteria for clinical diagnosis
of TA
Major criteria
 1. Left midsubclavian artery lesion: stenosis or occlusion 1 cm proximal to the left
vertebral artery orifice up to 3 cm distal
 2. Right midsubclavian artery lesion: stenosis or occlusion from the right vertebral
artery orifice to 3 cm beyond
 3. Characteristic signs and symptoms (> 1-month duration)
 A. Limb claudication
 B. Pulselessness or blood pressure differential > 10 mmHg in arms
 C. Exercise ischaemia
 D. Neck pain
 E. Fever
 F. Amaurosis fugax
 G. Syncope
 H. Dyspnoea
 I. Palpitations
 J. Blurred vision

28. Minor criteria
 1. High ESR: Westergren ESR > 20 mm/h
 2. Carotodynia
 3. Hypertension: brachial blood pressure > 140/90 mmHg o popliteal blood
pressure > 160/90 mmHg
 4. Aortic regurgitation or annuloaortic ectasia: determined by auscultation,
arteriography or echocardiography
 5. Pulmonary artery lesion: lobar or segmental artery occlusion, or stenosis or
aneurysm of pulmonary trunk
 6. Left middle common carotid artery lesion: stenosis or occlusion of middle 5
cm portion starting 2 cm from its orifice
 7. Distal innominate artery lesion: stenosis or occlusion in the distal third
 8. Descending thoracic aorta lesion: narrowing, aneurysm, or luminal
irregularity
 9. Abdominal aortic lesion: narrowing, aneurysm, or luminal irregularity
 10. Coronary artery lesion: documented by arteriography in patients < 30 years
of age and without risk factors for atherosclerosis
 Two major, or one major and two minor, or four minor
criteria indicate a high probability of Takayasu‟s arteritis.

29. Clinical Classification 2004
 Ueno et 1967 classified it into three types.
 Type-I, localized to the arch of aorta and its branches.
 Type-II,involve the thoracoabdominal aorta and its branches
without involvement of the arch.
 Type-III is combined features of both Type-I and Type-II.
 Type-IV in which there is pulmonary involvement in addition to
features of Type-I, II or III.( Lupi Harrera et al in 1975)
 Type- V proposed by Panja et al (2004) and it indicates
involvement of coronary arteries.

30. Incidence
The distribution of various types is not uniform globally.
As far as India is concerned, Agarwal et al reported among North
Indian patients the
 Incidence of various types as
 Type-I - 22%,
 Type-II - 25%,
 Type-III - 53% and
 Type-IV - 26%.
Panja et al involving 650 patients belonging to Eastern India and
Bangladesh (largest series from India) revealed
 Type- I - 16%;
 Type-II - 8%;
 Type-III - 76%;
 Type-IV - 36% and
 Type-V - 10%.

31. Imaging
 Angiography is investigation of choice for the diagnostic
evaluation of TA
 Echocardiography and Duplex Doppler
ultrasonography are the first investigations and the
characteristic findings of TA consist of wall
thickening,stenosis, aneurysms, calcifications,
occlusions, and pulsatility.
 Computed tomography (CT) and magnetic resonance
imaging (MRI) are the best diagnostic techniques for
assessing pulmonary vascular lesions
 PET technique or FDG-PET ((18) F-fluorodeoxyglucose
positron emission tomography permits an early diagnosis
of TA during the inflammatory or „„pre-pulseless‟‟ phase

32. Treatment
 High-dose corticosteroids are the mainstay of TA
therapy if caught early ; consists of prednisone 1
mg/kg/day given for the first 1–3 months, then reduction
after an alternate-day
 Cytotoxic therapy is mainly used in cases with ongoing
disease activity, regardless of glucocorticoid therapy.
 cyclophosphamide (1-2 mg/kg/day), azathioprine (1-2
mg/kg/day), or methotrexate (0.3 mg/kg/week)
 Recently, mycophenolate mofetil, and anti-tumour
necrosis factor alpha biological agents have been
proposed in patients unresponsive to other
immunosuppressor agents

33. Surgical care
Indications for surgical repair or angioplasty are as
follows:
1.Renovascular stenosis causing hypertension
2.Coronary artery stenosis leading to myocardial ischemia
3.Extremity claudication induced by routine activity
4.Cerebral ischemia and/or critical stenosis of 3 or more
cerebral vessels
5.Aortic regurgitation
6.Thoracic or abdominal aneurysms larger than 5 cm in
diameter
7.Severe coarctation of the aorta
8.Percutaneous transluminal coronary angioplasty is followed
by restenosis at the angioplasty site within 1-2 years in a
substantial number of patients.

34. References:
 TAKAYASU’S ARTERITIS – AN UPDATE- Daniela
Bartoº1,Ecaterina Bontaº1,S. Ghiorghe1 Cercetãri
Experimentale & Medico-Chirurgicale Anul XIII l Nr3-
4/2006 l Pag. 149-152
 Takayasu’s arteritis: a review of the literature -Silvia
Maffei, Michela Di Renzo, (Intern Emerg Med 2006; 1
(2): 105-112
 Current Status of Aortoarteritis in India Manotosh
Panja*, PC Mondal**
 Aortoarteritis presenting as coarctation of aorta.-
Bahuleyan CG, Bhuvaneswaran KP

35. Thanks !