CASE REPORT

TAKAYASU'S ARTERITIS AND RHEUMATIC HEART DISEASE

Ahmed Almotarreb; Mohammed Alabsi 
Cardiac Center, Al-Thawra Hospital, Sana'a -Yemen

Introduction
Case Presentation
Discussion
References

Introduction

    Since Takayasu, a Japanese ophthalmologist, reported the first case of the disease in 1908, there have been many clinical reports of the disease and its various associations. The majority of cases have been reported from Asia and Africa. The association between Takayasu's arteritis and rheumatic heart disease has not been described clearly in the literature. We report the first case of Takayasu's arteritis in combination with rheumatic heart disease from Yemen .

Case Presentation

    A 29-year-old male patient presented with dizziness, blurring of vision and headache. The attacks became more frequent and troublesome in the month prior to presentation but there was no history of syncope. He gave no history of dyspnea, orthopnea, paroxysmal nocturnal dyspnea, chest pain, or intermittent claudication. The patient gave a history of migrating arthritis involving the large joints 15 years previously. There were no other pertinent past medical history. 

    On physical examination the patient was conscious, oriented and not in distress. BP was not detectable in the upper limbs but was 150/100 mmHg in the lower limbs. The right and left brachial and radial pulses were not palpable. Cardiac auscultation revealed a loud first heart sound with a grade 3/6 apical pansystolic murmur radiating to the axilla and a mid-diastolic rumbling murmur at the apex. Chest and the abdominal examinations were normal. Fundoscopy showed retinal venous congestion.

    The ECG and chest X-ray were normal. Echocardiography confirmed rheumatic mitral valve disease with grade 2/4 mitral regurgitation and an estimated mitral valve area of 2.8 cm2 by planimetry and pressure half time. Routine investigation of stool, urine, CBC, ESR, urea and createnine and liver function tests were within normal limits. 

    The thoracic aortography demonstrated complete occlusion of the left common carotid and subclavian arteries, gradual narrowing of the brachiocephalic trunk ending with a severely stenotic segment, with post-stenotic dilatation. There was near total occlusion of the proximal portion of both the right common carotid and right subclavian arteries (Figure 1).

Figure 1.  Arch angiography reveals the whole arch of the aorta with the total occlusion 
of the left subclavian (thin black arrow) and left common carotid artery (thick black arrow).  
A tight narrowing noted in the distal brachiocephalic trunk (arrowhead), with post stenotic 
dilatation of the subclavian and filling up of the right common carotid artery (white arrow).

    Good contrast opacification between the right common carotid artery and the lateral and medial jugular veins suggested possible fistulous communication between the diseased artery and the adjacent vein (Figure 2).

Figure 2.  Angiography of the brachiocephalic artery reveals visualization of the normal right 
carotid artery (white arrow).  The distal right subclavian shows faint filling 

    There was complete occlusion of the right external carotid artery with patent internal carotid artery. The proximal part of the right vertebral artery was visualized but its origin was not clearly demonstrated and was possibly occluded. There was rich collateral circulation on the left side of the aortic arch suggesting good communication of the systemic and pulmonary circulation via enlarged brachial 
arteries.

Discussion

    Takayasu's arteritis is a worldwide disease, more common in Asia and Africa. The age of onset is usually between 10 - 40 years1,2. The majority of cases occur in women. Park et al studied the clinical and angiographic features of 129 patients in Korea and found a female to male distribution of 6.6:13. In India, over a period of 18 years (1972-1990), 83 cases of Takayasu's arteritis were reported with a female to male ratio of 1.6:1 Sixteen per cent of the patients presented with fever and arthralgia4. A large series of Takayasu's arteritis from China reported a female to male ratio of 2.9:15. The only report published of Takayasu in the Arab world was by Sattar et al involving four Arab females6.

    Takayasu arteritis primarily affects the aorta and its primary branches7. The inflammation may be localized to a portion of the thoracic or abdominal aorta and branches, or may involve the entire vessel. Although there is considerable variability in disease expression (due perhaps to geographic differences8. The initial vascular lesions frequently occur in the left middle or proximal subclavian artery. As the disease progresses, the left common carotid, vertebral, brachiocephalic, right middle or proximal subclavian artery, right carotid, and vertebral arteries, and aorta are also affected. The abdominal aorta and pulmonary arteries are involved in approximately 50 percent of patients.

    Takayasu's arteritis has been linked to rheumatic fever and other streptococcal infections, rheumatoid arthritis, and other collagen vascular diseases9. In 1988, Doi et al reported a patient with Takayasu's disease and non-rheumatic mitral stenosis10. Nikolic et al reported a case of Takayasu's arteritis associated with aortic regurgitation and mitral stenosis11. That patient had suffered erythema nodosum-like lesions eight years before presentation with classical aortic branch involvement.

    This is the first case of Takayasu' arteritis and rheumatic heart disease to be reported in Yemen.¨

    Heart Views. 2004; 5(1):24-25 
    © 2004 Gulf Heart Association

References 

1.  Lupi-Herrera, E, Sanchez-Torres, G, Marcushamer, J, et al. Takayasu's arteritis. Clinical study      of 107 cases. Am Heart J 1977; 93:94.

2.  Arend, WP, Michel, BA, Block, DA, et al. The American College of Rheumatology 1990      criteria for the classification of Takayasu arteritis. Arthritis Rheum 1990; 33:1129.

3.  Park YB, Hong SK, Choi KJ et al: Takayasu's arteritis in Korea: Clinical and angiographic      features. Heart Vessel - Suppl. 1992:7:55-59.

4.  Sharma BK, Sagar S, Singh AP and Suri S: Takayasu's arteritis in India. Heart Vessel -      Suppl. 1992:7:37-43.

5.  Zheng D, Fan D, Liu L: Takayasu's arteritis in China: A report of 530 cases. Heart-Vessel -      Suppl. 1992:7:32-36.

6.  Sattar MA, White AG, Eklof B and Feneeh FF: Takayasu's arteritis in Arabs. Postgraduate      Med J. 1985 May: 61(715): 387-390.

7.  Hata, A, Noda, M, Moriwaki, R, Numano, F. Angiographic findings of Takayasu arteritis: New      classification. Int J Cardiol 1997; 54(Suppl):155.

8.  Cid, MC, Font, C, Coll-Vinent, B, Grau, JM. Large vessel vasculitides. Curr Opin Rheumatol      1998; 10:18.

9.  Braunwald E. Heart Disease. 4th ed. 1992:1544.

10. Doi Yl, Seo H, Hamashige M, Jin-nouchi Y and Ozawa T: Takayasu's arteritis and mitral      stenosis. Clin Cardiol. 1988 Feb; 11(2): 123-125.

11. Nikolic J, Peclard M, Deleamont P, Chavaz P, Schifferli J and Bounameaux H: Takaysu's      arteritis preceded by cardiac and cutaneous lesions. A case report. Vasa 1993; 22 (4):347-      351.