Takayasu Arteritis Leading to Dilated Cardiomyopathy

N D Vaswani; Geeta Gathwala

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N D Vaswani, Geeta Gathwala.

Department of Pediatrics, PGIMS, Rohtak, India.

ADDRESS FOR CORRESPONDENCE
Dr N D Vaswani, 9J-55 Medical Campus PGIMS Rohtak.
Email: dr_vaswani@yahoo.co.in

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Abstract

Takayasu arteritis (TA) is a rare condition in which inflammatory process involves aorta and its major branches. Vessel inflammation leads to wall thickening, fibrosis, stenosis, thrombosis, end organ ischemia and aneurysm formation. As a result weak or absent pulses occurs in peripheral part of extremities, hence the term "Pulseless disease." The cause is largely unknown. Although TA may present in many ways, its presentation as dilated cardiomyopathy (DCM) is rare.

Keywords

Takayasu arteritis (TA), Dilated cardiomyopathy (DCM).

Introduction

TA, or Pulseless Disease, is a chronic vasculitis of large vessels. TA is rare, but most commonly seen in Japan, South East Asia, India, and Mexico. In 1905 Takayasu, professor of ophthalmology at Kanazawa University Japan presented the case of a 21 year old woman with characteristic fundal arteriovenous anastamoses. Due to narrowing of large and medium size vessels claudication, hypertension, dizziness, headaches and visual problems can occur. Using a stethoscope, one may also hear "bruits" (harsh, "whooshing" sounds made by the flow of blood through abnormally narrowed vessels). Smaller vessels are spared. It is infrequently reported in children. After Henoch-Schonlein purpura & Kawasaki Disease, TA may be the third most common form of childhood vasculitis in the world (1). We hereby report a rare case of DCM with renovascular hypertension secondary to TA.

Case Report

A 12 year old girl presented with history of difficulty in respiration for 2 months, swelling all over the body for 1 1/2 months and pain while walking for 1 month. There was no history of contact with tuberculosis, oliguria, joint pain or jaundice though mild fever with cough was complained of before the start of illness 2 months back. She did not receive any treatment prior to admission. Examination revealed a child who was thin built, with swelling over face & mild edema over lower limbs. She was febrile and was dyspnoeic grade 3 (NYHA). Pulse was 140/min in right upper limb and was absent in left upper limb and both lower limbs. Blood pressure recorded in right upper limb was 140/110 mm Hg and was not recordable in rest of all three limbs. Common carotid pulsations were equal on both sides. Bruit over left suprasternal region was heard. Bruits were also audible over renal angles bilaterally. The girl developed claudication after walking few steps. Examination of chest revealed fine basal crepitations. Jugular venous pulsations were raised. On auscultation heart sounds were normal and there was no murmur. There was tender hepatomegaly. Fundus was normal. Based on clinical findings diagnosis of vasculitis with hypertension and congestive heart failure was made. Coarctation of aorta was considered as a second possibility. On Investigation hemoglobin was 9 gm%, blood sugar was 78 mg%, urea was 46 mg %, serum creatinine was 0.9 mg% and ESR was 8 mm in 1st hr. Mantoux was negative. ASO titer was normal. Rheumatoid factor was negative. Skiagram chest showed enlarged cardiac shadow with pulmonary edema. ECG showed sinus tachycardia with left ventricular hypertrophy. Ultrasonography abdomen revealed free fluid with hepatomegaly. Echocardiography showed global hypokinesia with dilatation of left ventricle and an ejection fraction of less than 30% consistent with DCM. Mild mitral regurgitation was present. MRI angiography revealed narrowing of proximal descending thoracic aorta over a distance of 4.8 cm. Narrowing started 4.5 cm away from the origin of common subclavian artery. Also there was narrowing of bilateral renal arteries. A diagnosis of TA was made. The child was treated initially for CHF with furosemide and digoxin and received antihypertensives for hypertension. Steroids (prednisolone) were started after the diagnosis of TA was confirmed. Once failure was controlled the child was put on nonselective beta blocker (carvedilol). Surgery as well as other interventions was not done as attendants were not willing to give consent.

Discussion

TA is the commonest cause of renovascular hypertension in India (2). The etiology of TA is unknown, but an association with tuberculosis has been reported (3). Pathogenesis of TA starts in genetically susceptible individual, with perhaps a specific humoral milieu, followed by exposure to a yet unidentified antigen, leading to immune response that targets the large vessels (4). TA affects patients in 2nd and 3rd decade of life. Females are affected more commonly than males [ratio being 2.5:1] (5). Absent pulses along with hypertension & vascular bruits could be the commonest mode of presentation (6). The index case was also a female child in the second decade of life and presented similarly. DCM as was present in the index case is however reported to be seen in only 5-6% of cases of TA (7).

TA is classified according to the site of involvement (6). Type I- Aortic Arch. Type II- Descending thoracic aorta. Type III -Descending thoracic aorta and abdominal aorta. Type IV- Abdominal aorta only. Type V - Aortic arch, descending Thoracic aorta & abdominal aorta. In a study by Hata et al., Japanese patients showed a higher frequency of involvement of the ascending aorta, aortic arch & its branches than did the Indian in whom the frequency of involvement of the abdominal aorta & / or renal arteries was higher(8,9). In the present case the proximal descending aorta and renal arteries were involved.

Therapeutic modalities include steroids, immunosuppressive agents and antihypertensive drug therapy. 20%-100% success rate of steroids has been reported in different studies (9). cyclophosphamide and methotrexate are often needed to control intense inflammatory response. In addition balloon dilatation or stenting is often necessary (10). Angioplasty may be needed to prevent development of chronic hypertension & decreased perfusion. More than 50% cases achieve remission, but 28% of cases never achieve remission. The 5 years mortality rate is as high as 35%. There is no basis for genetic counseling (1). The best outcome appears to be associated with early diagnosis & institution of medical & surgical therapy.

The aim of this case reporting was to sensitize all practicing physicians in the field of medicine as well as pediatrics so that diagnosis of TA should not get delayed or missed.

Compliance with Ethical Standards

Funding None

Conflict of Interest None

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