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Pediatric Cardiology ISSN 0172-0643 Pediatr CardiolDOI 10.1007/s00246-011-0025-6

Takayasu’s Arteritis Mimicking UnilateralPulmonary Artery Agenesis in a ChildWith Severe Pulmonary Hypertensionand Right Heart Failure: A DiagnosticDilemmaSudeep Kumar, Nagaraja Moorthy, AdityaKapoor & Sunil Kumar

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CASE REPORT

Takayasu’s Arteritis Mimicking Unilateral Pulmonary ArteryAgenesis in a Child With Severe Pulmonary Hypertensionand Right Heart Failure: A Diagnostic Dilemma

Sudeep Kumar • Nagaraja Moorthy •

Aditya Kapoor • Sunil Kumar

Received: 18 March 2011 / Accepted: 19 May 2011

� Springer Science+Business Media, LLC 2011

Abstract Affliction of the pulmonary arteries in Taka-

yasu’s arteritis is uncommon. Moreover the incidence of

pulmonary artery involvement in this condition is often

underestimated because of asymptomatic nature in most

patients. Severe involvement may however present with

pulmonary artery hypertension and hemoptysis, which

may prove to be fatal. This case report describes a 9-year-

old girl with severe pulmonary hypertension and right

heart failure secondary to total occlusion of the right

pulmonary artery. Detailed clinical examination and

computed tomography (CT) angiography confirmed this

diagnosis.

Keywords Pulmonary artery agenesis �Pulmonary hypertension � Takayasu’s arteritis

Pulmonary artery (PA) involvement in Takayasu’s arteritis

is not only uncommon but often remains asymptomatic,

leading to significant underestimation of its overall fre-

quency [7]. Presentation with PA hypertension, hemopty-

sis, and right heart failure as a manifestation of PA

involvement in Takayasu’s arteritis is very rare. We report

a young girl with Takayasu’s arteritis who had total ostial

occlusion of the right PA.

A high index of suspicion is needed to make the correct

diagnosis. The disease is associated with a high incidence

of morbidity and a significant risk of premature death.

Takayasu’s arteritis most often affects the aorta and its

branches, and the involvement of pulmonary arteries is

underestimated [7].

Case Report

A 9-year-old girl presented with recent onset progressive

distension of the abdomen, pedal edema, and recurrent

hemoptysis during the preceding 6 months. Before the

onset of symptoms, she had experienced an episode of

febrile illness accompanied by arthralgia and mild short-

ness of breath, which had subsided within a week. An

echocardiography showed a dilated left ventricle (LV) with

severely reduced LV function (LV ejection fraction, 25%).

The girl’s disorder had been labeled as postmyocarditis

dilated cardiomyopathy with severe LV dysfunction, and

she subsequently was referred to our Institute.

The girl also reported gradually worsening claudication

in both lower limbs for the preceding 3 months. Clinical

examination showed sinus tachycardia (heart rate,

100 bpm), mild pallor, bilateral pitting pedal edema, and

elevated jugular venous pressures, with prominent a and v

waves. The left upper limb, the left carotid, both lower

limbs, and abdominal aortic pulsations were absent. The

blood pressure in her right upper limb was 100/70 mmHg,

whereas it was unrecordable in both lower limbs and the

left upper limb.

Auscultation exhibited a loud pulmonary component of

the second heart sound, a prominent pan systolic murmur at

Electronic supplementary material The online version of thisarticle (doi:10.1007/s00246-011-0025-6) contains supplementarymaterial, which is available to authorized users.

S. Kumar (&) � N. Moorthy � A. Kapoor

Department of Cardiology, Sanjay Gandhi Postgraduate Institute

of Medical Sciences, Lucknow 226014, India

e-mail: sudeep@sgpgi.ac.in

S. Kumar

Department of Radiology, Sanjay Gandhi Postgraduate Institute

of Medical Sciences, Lucknow 226014, India

123

Pediatr Cardiol

DOI 10.1007/s00246-011-0025-6

Author's personal copy

the left lower sternal border suggestive of tricuspid regur-

gitation (TR), and a continuous bruit over the D10-L1

vertebrae. Abdominal examination showed ascites with

gross hepatomegaly.

An electrocardiogram showed sinus tachycardia, right

axis deviation, and evidence of right ventricular (RV)

hypertrophy. The chest radiograph showed absence of right

PA shadow with relative hyperlucency on the right side,

with no significant volume loss noted (Fig. 1a).

Routine blood investigations exhibited a hemoglobin

level of 9 g/dl, an erythrocyte sedimentation rate of 20 mm

at 1 h, and elevated serum C-reactive protein levels (4 mg/

dl). The serology results for connective tissue disorders

were negative.

Transthoracic echocardiography confirmed severe LV

dysfunction, dilated right atrium and right ventricle, RV

systolic dysfunction, moderate TR, mild pulmonary

regurgitation, and a predicted PA pressure of 110 mmHg.

The main pulmonary and left pulmonary arteries were

dilated, whereas the right PA was not visualized (Fig. 2,

Video 1).

A lung perfusion imaging study was performed, which

showed a total absence of right lung enhancement.

(Fig. 1b). A computed tomographic (CT) angiography was

performed, which showed total ostial occlusion of the right

PA, and the lobar, segmental, and subsegmental arteries

were not traceable (Figs. 3a, b, 4b). The perfusion to the

right lung was via extensive collaterals from the bronchial

and intercostal arteries. The left descending PA showed

only irregular thickening of the intima and media without

any significant stenosis.

There was total ostial occlusion of the left subclavian,

the left common carotid, and the left vertebral arteries, as

well as severe long-segment stenosis of the lower thoracic

aorta at the level of the diaphragm distal to the renal

arteries, with typical rattail tapering (Fig. 4a). The renal

arteries were not involved and showed only mild disease.

Based on the CT angiography findings (involvement of

both the aorta and its branches and the pulmonary vascu-

lature), a diagnosis of Takayasu’s arteritis was made, and

the patient was started on intravenous methyl prednisolone

together with weekly doses of methotrexate and diuretics.

The patient improved symptomatically and was doing well

at the last 3 month follow-up visit.

Discussion

Takayasu’s arteritis is a chronic inflammatory disease that

involves the aorta, its branches, and less commonly the

Fig. 1 a Chest radiograph showing cardiomegaly with hyperlucency

in the right lung field with no right pulmonary artery shadow. Note that

the main pulmonary artery segment at the left upper border of the

cardiac silhouette is prominent, indicating main pulmonary artery

dilation secondary to severe pulmonary artery hypertension. b Lung

perfusion combined computed tomography with single photon emis-

sion tomography (CT-SPECT) imaging showing total absence of right

lung enhancement features mimicking pulmonary artery embolism

Fig. 2 Transthoracic echocardiogram in basal short-axis view show-

ing dilated main and left pulmonary arteries with no right pulmonary

artery shadow

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pulmonary arteries [3, 5]. Although PA involvement in

Takayasu’s arteritis is known, shown for nearly 70% of

patients on angiographic studies [9, 12], it often is mild,

and most patients with PA involvement in Takayasu’s

arteritis are asymptomatic [11].

Pulmonary artery involvement usually correlates with

extensive aortic involvement, but an isolated PA lesion

presenting as a primary manifestation of Takayasu’s

arteritis also has been described [4]. Generally, PA

involvement is limited to segmental and subsegmental

branches, especially in the upper lobes, although larger

branches also may be involved [5]. The reported patient

had simultaneous involvement of the aorta and its branches

as well as PA in the form of total ostial occlusion of the

right PA and a thickened wall of the left pulmonary vas-

culature, which is extremely rare in Takayasu’s arteritis.

Pulmonary artery involvement in Takayasu’s is an

expression of the systemic nature of the disease, supporting

Fig. 3 a Computed tomographic (CT) pulmonary angiogram showing total occlusion of the right pulmonary artery at the ostium. b Coronal CT

pulmonary angiogram showing decreased pulmonary vasculature on the right side and dilated pulmonary vasculature on the left side

Fig. 4 a Volume-rendered computed tomographic (CT) aortogram

showing ostial occlusion of the left subclavian and left common

carotid artery with severe disease involving the thoracic and

abdominal aorta. b Volume-rendered CT pulmonary angiogram

showing total occlusion of the right pulmonary artery at the ostium

(arrow)

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the hypothesis of an immunologic etiology [5]. The lesions

in the pulmonary arteries are inflammatory or granuloma-

tous, involving the elastic, medial, and adventitial layers,

often culminating in thickness, stenosis, and even short-

ening of the artery in some cases [5, 8]. Although occlusion

of unilateral pulmonary arteries can occur in advanced

cases [6, 12], it is extremely rare.

Because most patients remain asymptomatic despite

extensive involvement of the PA vasculature, the incidence

of PA involvement in Takayasu’s arteritis often is under-

estimated. Hence, it is important to evaluate the pulmonary

arteries in all cases of Takayasu’s arteritis, even in the

absence of any significant respiratory symptoms. In fact, it

is important to consider and rule out late-phase Takayasu’s

arteritis in cases of chronic PA obstruction of unknown

cause, especially in endemic areas, where Takayasu’s

arteritis is common [2].

Pulmonary hypertension generally is moderate and

found to be severe in only a few cases. Although pul-

monary hypertension increases the probability of pulmon-

ary vascular involvement, normal pressures do not exclude

lesions in the pulmonary vasculature.

Diffuse wall thickening and stenosis or dilations are the

most frequent PA changes detected by CT in patients with

Takayasu’s arteritis. These signs are suggestive of PA

involvement and often are associated with ascending aorta

involvement, as seen in the reported patient. Visualization

of a systemic PA shunt on a thoracic aortogram or a

selective bronchial arteriogram in Takayasu’s arteritis has

already been described [12]. In the reported patient, these

shunts led to an anterograde opacification of the distal

pulmonary vasculature through extensive bronchial and

intercostal collaterals. Such systemic PA shunts appear to

be indicative of extensive PA involvement in Takayasu’s

arteritis [12].

Heart failure from Takayasu’s arteritis is common in

children and an important cause of mortality. Hypertension

is the most common reason for heart failure, but it may

occur in the absence of severe hypertension [10]. Myo-

carditis, coronary arterial involvement, organic valvular

involvement, or PA involvement may cause or contribute

to the heart failure. Frequently, children with Takayasu’s

arteritis who experience heart failure are misdiagnosed as

having dilated cardiomyopathy because the echocardio-

gram shows systolic ventricular dysfunction, and hyper-

tension may be missed. Heart failure in the absence of

hypertension suggests myocarditis as a cause for ventric-

ular dysfunction.

Radiologically, PA involvement in Takayasu’s arteritis

with a total cutoff of the PAs, as happened in the reported

case, may mimic congenital unilateral PA agenesis [1].

However, the absence of other signs suggestive of congenital

agenesis (ipsilateral contracted lung with a mediastinal

shift and hyperinflation of the contralateral lung) and sub-

sequent CT findings confirmed the reported case to be severe

ostial stenosis of the right pulmonary artery secondary to

Takayasu’s arteritis. Although similar x-ray findings may

occur in cases of pulmonary embolism, the latter more often

involves larger pulmonary branches such as the main pul-

monary, lobar, and segmental branches, especially the lower

lobe branches. Moreover, the presence of associated abnor-

malities in the aorta and its branches further support the

diagnosis of Takayasu’s arteritis.

The reported case is unique in several aspects. The

initial presentation simulated dilated cardiomyopathy,

probably secondary to myocarditis. However, at this stage,

Takayasu’s arteritis was either not suspected or missed

clinically due to the absence or clinically undetectable

involvement of the aorta and its branches. During the fol-

low-up period, the LV dysfunction responded to medica-

tions, but there was ongoing progression of the disease,

resulting in severe involvement of the aorta and its bran-

ches as well as the pulmonary vasculature, resulting in

severe pulmonary hypertension with congestive heart

failure.

Conclusion

Pulmonary artery involvement in Takayasu’s arteritis is not

so rare and may range from mild asymptomatic thickening

of the intima to total occlusion of the main or branch

pulmonary arteries. Pulmonary circulation should be rou-

tinely studied for all patients with suspected Takayasu’s

arteritis. The PA involvement may be responsible for

unexplained pulmonary hypertension with heart failure in

children and may be a diagnostic challenge.

Acknowledgment The authors thank Dr. Amithab Arya from

Department of Nuclear Medicine, SGPGIMS, for providing SPECT

CT lung perfusion images.

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