Kilic SS. Takayasu arteritis. Recent Advances in Pediatrics,  Jaypee Brothers Medical Publishers, New Delhi, ISBN 81-8061-297-X , pp 338-354, 2006.


Takayasu's arteritis (TA) has become increasingly recognized as a worldwide entity with a variable spectrum of disease expression (1). It was first reported in 1908 by a Japanese ophthalmologist Takayasu, who described retinal arteriovenous shunts which, appeared in a wreath-like distribution around the optic disc and microaneurysms of the retinal vessels in a 19-year-old Japanese woman (2). TA, also known as pulseless disease, occlusive thromboaortopathy, is a chronic inflammatory arteritis affecting large vessels, predominantly the aorta, its main branches, and the coronary and pulmonary arteries. In juvenile onset disease, the thoracic and abdominal aorta are the commonly affected sites. Vessel inflammation leads to wall thickening, fibrosis, stenosis, and thrombus formation. The clinical features usually reflect limb or organ ischemia resulting from gradual stenosis of involved arteries. More acute inflammation can destroy the arterial media and lead to aneurysm formation (3). TA is rare, but most commonly seen in Japan, South East Asia, India, and Mexico. Early reports suggested that the disease was confined to females from Eastern Asia, but it has now been recognised worldwide in both sexes, although disease manifestations vary between populations. The disease commonly presents in the 2nd or 3rd decade of life, often with a delay in diagnosis from the onset of first symptoms of months to years. The onset of illness may be earlier, including in childhood but rarely in infancy . Twenty percent of all patients with TA are younger than 19 years of age at disease onset. The female:male ratio has varied from 9: 1 in reports from Japan to 1.3: 1 in India (4). The estimated incidence rate is 2.6 cases per million persons per year (5). The incidence rate of TA appears to have wide variations in different geographical areas: it was calculated to be 0.12 cases/100,000/ year in Sweden , 0.22 in Kuwait , 0.26 in USA , and probably higher in Japan (6). Genetic susceptibility to Takayasu’s arteritis has been extensively studied. There are heterogeneous population data regarding HLA associations in TA. HLA B-52 and DR-2 are associated with Takayasu’s arteritis in Japan, which also confers a worse prognosis (7). Yoshida et al (8) reported the increased frequency also of B39.2, a subtype of HLA-B39, which shares the peptide binding region with HLA-B52.  HLAB-52 and B-5 association is also reported from Korea and India, whereas HLA B-39 is frequently found in Mexican Takayasu’s arteritis patients (9-11). In Italy, it is recently reported the association of TA with HLA-DRB1*0405, an allelic variant of DR4, and with A32, which are also associated with a younger age of disease onset (12).


Etiology and Pathogenesis
Etiopathogenesis of TA is poorly understood, but is thought to be a cell-mediated process. Infections such as tuberculosis and an autoimmune component have been accused of the development of TA. Evidence is mounting that heat shock protein (HSP)-65 may be one of the important antigens. HSP-65 is a major antigen of mycobacterium tuberculosis. Cross reactivity and sequence homology between HSP-65 and HLA class II molecules has been described (13,14). Moreover, expression of HSP-65, HLA class I and II antigens are also markedly increased in affected aortic tissue (15). Thus, genetically linked immune responses to unidentified antigen may incite autoimmune damage by cell mediated pathways, and may result in the disease and relapses.

The inflammatory lesions in TA originate in the vasa vasorum and are followed by cellular infiltration, mainly composed of T cells, but also of natural killer cells, dendritic cells, monocytes and granulocytes, invading the outer layer of the media and/or its neighbouring adventitia. T cells and natural killer cells infiltrate and incite the damage by liberating perforin on to arterial tissue. In patients with aorta arteritis, immunological investigations have shown a decrease in the titre of complement and phagocytic activity of neutrophil granulocytes, deep depression of T cell immunity, and hypergammaglobulinaemia (16-18). Low CD4+ lymphopenia was reported in a patient with TA (19). Chemokines and proinflammatory cytokines also seem to play a role in amplifying the inflammatory response that follows the primary immune reaction. Interleukin-6, interleukin-1 and RANTES are released in large amounts by infiltrating inflammatory cells within damaged tissue, as well as by circulating inflammatory cells, and very likely help maintain the aberrant immune activation, by promoting endothelial cells activation and by inducing lymphocyte infiltration. Although the nature of the antigen that triggers the auto-immune process is still unknown, the infiltrating T lymphocytes may recognize one or a few self-antigens processed and presented in association with HLA. It remains to be demonstrated that pentraxin PTX3 represents a novel active phase reactant produced at the sites of active vasculitis also in TA patients (20-22). 
Histopathologically the inflammatory process is observed at the media of the arteries and extends to the adventitia and intima . The most characteristic lesion is destruction of the elastic fibers of the media, and is associated with the atrophy, disappearance and replacement with fibrosis of the smooth muscle of the media. In the initial acute stage of the disease, exudative and granulomatous inflammation occur, whereas fibrosis predominates later, but the two stages may co-exist. The cellular infiltrate characterising TA is primarily composed of lymphocytes, plasma cells and histiocytes, with a variable number of giant cells (23). Later in the disease process, nodular fibrosis in all layers of the artery is seen and the intima may become several times thicker than media obliterating the lumen. Rapid or more severe inflammation leads to vessel dilatation and aneurysm formation, but stenosis and occlusions are more common. Thromboses in stenosed arteries are sometimes seen. The corresponding organ shows ischaemic changes, and this ischaemia largely determines the clinical features of the disease (24).
 Signs and symptoms

Systemic symptoms of fatigue, loss of weight, night sweats, arthralgia, myalgia and low-grade fever are common in early phase but the correct diagnosis of TA is seldom made in the early phase. Patients often look chronically ill. The active phase of the illness lasts for weeks to months, and may have a remitting and relapsing course. Clinical presentation may be insidious and diagnosis is often delayed. Physical examination typically reveals markedly diminished or absent pulses and low or unobtainable blood pressure in the involved arteries arising from the aortic arch. Unless there is an acquired coarctation, these contrast sharply with the generally brisk pulses in the legs, where the true blood pressure must be determined and followed. Bruits may be heard over partially narrowed arteries. Central blood pressure determinations should always be obtained in patients with extremity vessel stenosis to assess the reliability of conventionally obtained blood pressure measurements (1, 25, 26).

The clinical presentation is widely heterogeneous and involves two stages: (1) an initial inflammatory process or ‘prepulseless phase’ with variable systemic manifestations occurring, followed by (2) a later ‘pulseless phase’ with multiple arterial occlusions producing symptoms of cerebral, visceral, or extremity ischemia. Acute symptoms are usually of short duration, rarely lasting more than a few weeks, but may recur after intervals of weeks to years. This phase gradually subsides and is followed by a more chronic stage characterized by inflammatory and obliterative changes in the aorta and its branches.  Narrowing, occlusion, and dilatation of the affected arteries cause a variety of vascular symptoms. Symptoms include intermittent claudication of the arms, legs and mesentery, decreased artery pulses, carotodynia, visual loss, stroke, aortic regurgitation, hypertension, and congestive heart failure. Reduced blood pressure in one or both arms is common with a differential of >10 mmHg between the arms. Diminished and asymmetrical arterial pulses and bruits over the affected arteries are usually heard. Stenotic lesions predominate and tend to be bilateral. In the late stage, weakness of the arterial walls may give rise to localized aneurysms. Nearly all patients with aneurysms also have stenoses and most have extensive vascular lesions. More acute progression causes destruction of the media of the arterial wall, leading to the formation of aneurysms or rupture of the vessels (25-30).


Cardiac involvement 

Hypertension in TA results from renal artery stenosis or aortic narrowing and aortic fibrosis. Arterial baroreceptor abnormalities can be hypothesized considering the high frequency of carotid arteries and aortic arch involvement. Hypertension is often severe and may cause hypertensive encephalopathy or heart failure. Heart failure from TA is common in children  and is an important cause of mortality. Myocarditis, coronary arterial involvement, organic valvar involvement, or pulmonary artery involvement may cause or contribute to the heart failure (31). Coronary arterial involvement in TA is usually ostial and proximal, but diffuse lesions or arteritis and aneurysm rarely occur. Coronary arterial involvement may cause myocardial infarction, angina or heart failure and may necessitate angioplasty, or surgical treatment even in children. Occlusive changes in the descending thoracic aorta sometimes produce a form of acquired coarctation. The diagnosis of hypertension may be entirely missed if all the peripheral pulses are not carefully examined.  Familiarity with the disease may suggest the correct diagnosis, even when no obvious pointers are present. Aortic regurgitation from a dilated aorta is rarely seen in children. Treatment of hypertension or aortic obstruction ameliorates the heart failure in the majority of children. Pulmonary arterial involvement is usually mild. A history of haemoptysis, chest pain, disproportionate pulmonary arterial hypertension, or abnormal ventilation-perfusion scan53 may suggest pulmonary involvement (32).

Renal involvement

The ostia of the renal arteries are commonly involved, but the intrarenal vasculature and small vessels are generally normal. Non specific ischemic glomerular lesions resulting from arterial narrowing or long-standing reno-vascular hypertension are the most common renal manifestations of TA. Primary glomerular diseases have been described rarely. Renal arterial stenosis may be bilateral and usually coexists with aortic involvement (33). Surgical correction of renal artery stenosis is usually an effective means of decreasing or eliminating hypertension (34).

Neurologic Findings

Neurological symptoms such as headache, visual disturbances and amaurosis fugax are common in TA. Visual disturbances are most often bilateral. Inflammation in the carotid arteries may cause visual problems or neurological problems such as dizziness or stroke. In children, cerebrovascular accidents are often secondary to severe hypertension and its complications. Hypertensive retinopathy is commoner than ischemic retinopathy in TA (25, 35).

Cutaneous manifestations

Erythema nodosum, erythema induratum and pyoderma gangrenosum have been described most frequently. It appears to be an autoimmune condition, where immune cells are wrongly targeted against the body's own tissues, and may involve other systems (36).

Diagnosis of TA is often challenging because the clinical features are similar to those of other systemic inflammatory diseases. TA is relatively rare and may have a subtle and insidious presentation. Nevertheless, a prompt diagnosis is very important because failure to treat vascular inflammation in a timely manner may have serious consequences for the patient. Systemic symptoms are seen in a high proportion of children with TA. The usual presenting symptoms are due to hypertension, heart failure or a neurological event. A high index of suspicion, proper evaluation of the patient’s history, carrefully examination in search of arterial bruit, evaluation of all peripheral pulses, and recording of blood pressure in all 4 limbs would aid in early diagnosis of TA. The criteria for the diagnosis of TA as suggested by Ishikawa (37) are included in Table 1. The criteria adopted by the American College of Rheumatology are shown in Table 2 (38). Children with Takayasu’s arteritis have higher morbidity and mortality than adults. A familiarity with the radiographic, computed tomographic, magnetic resonance imaging, and angiographic features of Takayasu arteritis may permit and earlier diagnosis and treatment.















Table 1 : Ishikawa’s criteria (37) for the clinical diagnosis of Takayasu’s disease



Obligatory criteria


Age <40 year 

Age < 40 year at diagnosis or at onset of "characteristic signs and symptoms" of one month duration in patient history. 

Two major criteria


Left mid subclavian artery 

The most severe stenosis or occlusion present in the mid portion from the point one cm proximal to the left vertebral artery orifice to that three cm distal to the orifice determined by angiography.

Right mid subclavian artery lesion

The most severe stenosis or occlusion present in the mid portion from the right verterbral artery orifice to the point 3 cm distal to the orifice determined by angiography. 

Nine minor criteria


High ESR

Unexplained persistent high ESR>20 mm/hat diagnosis or presence of evidence in patient history.

Carotid artery tenderness

Unilateral or bilateral tenderness of common carotid arteries by physician palpation; neck muscle tenderness is unacceptable.


Persistent blood pressure 140/90mmHg brachial or > 160/90mmHg popliteal at age <40 year. Or presence of the history at age <40 year. 

Aortic regurgitation or annuloaortic ectasia

By auscultation or Doppler echocardiography or angiography

Pulmonary artery lesions

By angiography or two dimensional echocardiography. Lobar or segmental arterial occlusion or equivalent determined by angiography or perfusion scintigraphy; or presence of stenosis, aneurysm, luminal irregularity or any combination in pulmonary trunk or in unilateral or bilateral pulmonary arteries determined by angiography.

Left mid common carotid lesion

Presence of the most severe stenosis or occlusion in the mid portion of 5cm in length from the point 2 cm distal to its orifice determined by angiography.

Distal brachiocephalic trunk lesion

Presence of the most severe stenosis or occlusion in the distal third lesion determined by angiography.

Descending thoracic aorta lesion

Narrowing, dilatation or aneurysm, luminal irregularity or any lesion combination determined by angiography; tortuosity alone is unacceptable.

Abdominal aorta lesion

Narrowing, dilation or aneurysm, luminal irregularity or any combination and absence of lesion in aorto-iliac region consisting of 2cm of terminal aorta and bilateral common iliac arteries determined by angiography; tortuosity alone is unacceptable.



The proposed criteria consist of one obligatory criterion, two major criteria and nine minor criteria. In addition to the obligatory criterion, the presence of two major criteria, or one major and two or more minor criteria or four more minor criteria suggests a high probability of the presence of Takayasu’s disease. 


Table 2: 1990 criteria of American College of Rheumatology for the classification of Takayasu arteritis (38)



Age at disease onset in year 

Development of symptoms or findings related to Takayasu arteritis at age <40 years. 

Claudication of extremities 

Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities. 

Decreased brachial artery pulse

Decreased pulsation of one or both brachial arteries 

BP difference >10mmHg

Difference of >10mmHg in systolic blood pressure between arms 

Bruit over subclavian arteries or aorta

Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta

Arteriogram abnormality 

Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal uppper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes usually focal or segmental 

Takayasu arteritis is diagnosed if at least three of six criteria are present






Anemia of chronic disease and marker of nonspecific inflammatory process, including an elevated erythrocyte sedimentation rate (ESR), increased serum C-reactive protein and alpha-2 globulin, and hypoalbuminemia are often present, but usually there is no increase in the white cell count (39). ESR is a good indicator of disease activity . Besides, an increased in serum soluble E-selectin and thrombomodulin have been reported, but their value in routine diagnosis or follow-up is uncertain (40,41).

To date, other surrogate markers of active disease (endothelins, von Willebrand factor antigen, factor VIII), have either not been adequately studied or have not been found to be superior to the erythrocyte sedimentation rate in monitoring patients (42).  Raised interleukin-6 and RANTES have been reported to correlate with the disease activity, and may prove useful in the monitoring of therapy (44). Angiography is considered the gold standard in delineating abnormal vessels in patients with Takayasu arteritis (39).



Chest radiography

In several patients, chest radiography may be the first examination to suggest the diagnosis of Takayasu's arteritis. Chest radiographic features include an irregular outline and linear calcifications of the aorta, decreased pulmonary vessels, dilatation of ascending aorta, cardiomegaly and rib notching (1).


CT and MRI techniques have been used in the last several years with increasing frequency in patients suspected of having TA. Both modalities are effective in the detection of vascular wall thickening of large arteries such as the aorta and pulmonary artery. CT is an excellent method for demonstrating pathologic changes of the aorta and large deep vessels, such as aneurysms, dissections, ruptures, thrombus formation and calcifications. The main indication for CT scanning in Takayasu disease is the morphologic evaluation of wall changes in the aorta and pulmonary artery; dynamic information about the mural inflammatory process are provided by spiral CT (44).
Major advantages of MRI are the low invasivity (lack of ionizing radiation and iodinated contrast material) and the ability to obtain high quality images in the different spatial planes. This is especially useful in the follow-up of chronic, late phase Takayasu's arteritis, when careful depiction of aortic lumen in its longitudinal plane and exact determination of the degree and extent of inflammatory mural changes are needed (44,45).

CT angiography is useful in depicting the aorta and brachiocephalic vessels, but its use is potentially limited by long reconstruction time and ionizing radiation. MR angiography is a non-invasive technique, which does not expose patients to ionizating radiation, and does not require the use of iodinate tracers. It provides high resolution anatomic information in a moltitude of obliquities, including lumen configuration and vascular wall thickness. CT and MR angiography are both very useful for evaluating the disease involvement of thoracic aorta and pulmonary arteries (44, 46-51).


Angiography is still the gold standard technique for the diagnosis of vascular diseases conditioning luminal abnormalities. Because the clinical features of Takayasu's arteritis are determined by the extent of the specific artery involved in the occlusive phase of the disease, a pan-angiography is required (). The indications for intrarterial DSA are: 1) to show the extension and the degree of the vasculitic process by demonstrating the segmental narrowing, collaterals, and the distal intact portion of the involved arteries, 2) to show the subclavian steal phenomenon, 3) to confirm an extensive pulmonary artery involvement, 4) to perform follow-up studies for radiological or surgical intervention, 5) to guide and evaluate interventional procedures (52, 53).

Aortography is the traditional imaging technique used in the diagnosis of the occlusive phase of Takayasu's arteritis, demonstrating luminal irregularity, vessel stenosis, occlusion, dilatation or aneurysms in the aorta or its primary branches. Conventional or digital subtraction angiography has been considered the gold standard for the diagnosis of Takayasu’s arteritis. In type I, the disease is localized to the aortic arch and its branches; in type II, the disease involves the descending thoracic and abdominal aorta and its branches; type III is the combination of type I and II; type IV is defined when the pulmonary artery is involved in any of the above groups . Type III is the most common, accounting for about 70 % of cases. Thoracoabdominal aortic involvement is commoner (type II/III) in children.  A percutaneous transluminal angioplasty (PTA) has emerged as a viable alternative to surgery in recanalization of stenotic lesions.  Simultaneuos evaluation of luminal and vascular wall changes may now allow a simpler diagnosis of this condition also in its early phase and the effective therapy monitoring (54-64).

Sonographic techniques

Ultrasonography is very sensitive method in detecting early vascular wall involvement or mild stenosis of the common carotid artery. Color Doppler sonography is actually the non invasive method of choice for evaluation of the neck vessels;  moreover, color Doppler sonography can easily detect the hemodynamic changes occurring in common carotid artery occlusion, and is very helpful in distinguishing between high grade carotid stenosis and occlusion (65).  High-resolution B-mode ultrasonography may provide useful information on the grade of the stenosis, and on thickening of the vessel wall, especially of carotid arteries. It is a non-invasive and not expensive test, which can be repeated routinely. In expert hands, it may represent an invaluable monitoring instrument.


Treatment of Takayasu arteritis is difficult, but results with appropriate therapy are encouraging. Early detection is important. Steroids and immunosuppressive agents form the cornerstone of treatment, with surgery reserved for complications caused by narrowed arteries (66).

Medical Treatment

Glucocorticoids are still the mainstay of active disease treatment and effective in suppressing the systemic symptoms, arresting the progression of disease, and reverting the anemia and elevated acute phase reactants. A large proportion of patients with active disease respond to treatment with glucocorticoids, but a significant proportion will either require additional immunosuppression to prevent steroid toxicity or will remain unresponsive to treatment (67-69). Various cytotoxic drugs like cyclophosphamide or azathioprine have been used with variable success (70). Methotrexate is an alternative immunosuppressive agent to cyclophosphamide and may induce remission if other treatments have failed (71). The relapsing nature of the disease often required repeated courses of therapy. The duration of treatment varies empirically on clinical assessment of activity.

Surgical Treatment

Surgery continues to play a critical role in the long-term management of Takayasu's arteritis and is recommended when appropriate during inactive disease. The relevant role of surgery in TA is to ensure correct blood flow, by preventing or reducing the ischaemic damage caused by artery obstruction, and by preventing aneurysm rupture. Surgery is preferred when the disease is quiescent, and areas of arterial tree that are uneffected by disease are the preferred anastomotic sites (72). Previous studies have shown the increased risk for failure of surgical procedures during active disease.

Main indications for surgical interventions are renal vascular hypertension, cerebral hypoperfusion, and limb claudication. Cumulative incidence of undergoing a surgical procedure increased very quickly during the first phase of the disease. Indeed, renal arterial stenosis, often present at disease onset, is not eligible for medical treatment and requires a prompt surgical approach. On the contrary, severe hypoperfusion secondary to multiple vascular involvements occurs later and accounts for delayed procedures (73,74).

Percutaneous transluminal angioplasty (PTA), eventually with stent placement, may represent the treatment of first choice for patients with renal or subclavian artery stenosis (75). Balloon dilatation of aortic narrowing is highly effective even in diffuse, long segment stenoses . Close to 90% success rates have been reported for aortic angioplasty in children. However, the rate of re-stenosis are fairly high, probably because of the inflammatory characteristic of the disease. Because of the diffuse, inflammatory and possibly progressive nature of the disease, surgical treatment is not preferred for Takayasu’s arteritis except for undilatable symptomatic stenotic lesions and for large aneurysms (76). Renal autograft transplantation has been offered as a new technique in the management of hypertension in Takayasu's arteritis (77).

Assessment of disease activity
Criteria for active disease in patients with TA are  fever, musculoskeletal pain, elevated erythrocyte sedimentation rate, features of vascular ischemia or inflammation, such as claudication, diminished or absent pulse, bruit, asymmetric blood pressure in either upper or lower limbs (or both), and typical angiographic features. Besides, computed tomography and magnetic resonance imaging can be conveniently used to during follow-up.


The major morbidity and mortality of Takayasu’s arteritis results from stenosis and occlusion of the aorta, renal and carotid arteries. Widely varying mortality rates are documented, but obviously depend on the severity of the disease and therapeutic strategies. Five-year and 10-year survival rates of 80% to 90% have been reported from large series of patients (25, 31). Hypertension, cardiac involvement, aortic or arterial aneurysms (or both), and severe functional disability predict greater morbidity and mortality.

A mortality of 10-30% has been reported on followup of TA in children. The prognosis has significantly improved due to interventional procedures for the treatment of renal and aortic stenosis (25). The presence of severe Takayasu’s arteritis (defined as the presence of severe grades of hypertension, aortic regurgitation, retinopathy, or aneurysms), poor functional class or cardiac involvement, predicted a poorer outcome.


Takayasu’s arteritis is a chronic inflammatory disease that involves the aorta, its branches and the pulmonary arteries. The etiology remains obscure, with an autoimmune pathogenesis postulated. The inflammation results in varying degree of stenosis, occlusion or dilatation of the involved vessels. Early diagnosis and therapy, possibly aided by the newer imging techniques, is essential for a good prognosis. The chronicity and relapsing nature of the disease require treatment regimens of low toxicity. A combination of these modalities and surgical intervention may improve the outcome.


Minor abnormalities affecting aortic and pulmonary artery walls must be stressed; an early diagnosis and the institution of appropriate therapy may lead to a significant improvement of clinical and imaging findings, which might also prevent future occlusive changes.
According to the ACR 1990 criteria, a panangiography must be considered the first choice technique for confirming a suspicion of Takayasu's arteritis, by demonstrating the luminal abnormalities, which are typical of the late-phase, pulseless disease. In experienced hands, the color Doppler technique gives an accurate global evaluation of the arterial tree, by disclosing most of the inflammatory localizations in sonographically accessible arteries, thus being particularly useful in the long term evaluation of disease activity.
Early diagnosis followed by an aggressive treatment, with glucocorticoids and cytotoxic agents, may avoid rapid progression of the vascular lesions. Clinical parameters, acute phase reactants, and periodical vascular imaging techniques (e.g. duplex ultrasound examination) may more accurately detect disease activity.  Furthermore, the radiological approach has proved to be very useful in the treatment of arterial stenosis in several districts through percutaneous transluminal angioplasty (PTA) and stenting.









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Vital Learning  Points


  • An initial inflammatory process or ‘prepulseless phase’ with variable systemic manifestations occurring, followed by  a later ‘pulseless phase’ with multiple arterial occlusions producing symptoms of cerebral, visceral, or extremity ischemia.
  • A young patient with hypertension should be further evaluated for extremity claudication, cerebral ischemia, and renal artery stenosis.
  • The presence of anemia, elevated ESR, and symptoms such as anorexia, fatigue, and fever should alert the physician to the possibility that the respiratory symptoms are the consequence of disease exacerbation.
  • Angiography is sensitive in detecting abnormal anatomy in patients with Takayasu arteritis.
  • New therapeutic modalities aimed at abating the inflammatory and ensuing fibrotic responses would decrease the statistically significant morbidity experienced by most patients.

















Figure 1  Magnetic resonance imaging represents  dilatation and irregular contour of  the descending aorta and narrowing of the abdominal aorta.