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Year : 2022  |  Volume : 12  |  Issue : 1  |  Page : 28-30

A case of middle aortic syndrome in takayasu arteritis with left ventricle dysfunction and heart failure

Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Science and Research, Bengaluru, Karnataka, India

Date of Submission05-Jan-2021
Date of Decision06-Feb-2021
Date of Acceptance16-Feb-2021
Date of Web Publication08-Feb-2022

Correspondence Address:
Dr. Darshan P Thakkar
Department of Cardiology, Sri Jayadeva Institute of Cardiovascular Science and Research, Bengaluru - 560 078, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jicc.jicc_4_21

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Left ventricular dysfunction is an uncommon complication of Takayasu arteritis (TA) with a prevalence of about 10%–15%. We report a case of a 27-year-old girl who presented with dyspnea, bipedal edema, loss of weight, and easy fatigability for 3 months. Patient also developed right hemiparesis 3 months ago. Computed tomography of the brain was suggestive of the left temporal infarct, on evaluation she was diagnosed as left ventricle (LV) dysfunction and she was treated for CVA and heart failure. Clinical examination revealed the absence of the left upper limb and bilateral lower limb pulses with right upper limb hypertension. Echocardiography revealed severe LV dysfunction (ejection function ~20%). Computed tomography angiogram and other inflammatory markers confirmed the diagnosis of TA and revealed the presence of coaraction of the thoracic and abdominal aorta. Coaractoplasty was done, and immunosuppressant therapy with oral prednisolone and weekly oral methotrexate was started. On follow-up, patient is asymptomatic with improved LV function. Any patient with LV dysfunction or dilated cardiomyopathy, reversible causes have to be ruled out and TA has to be thought of as a differential diagnosis particularly in young females. Checking of all four limbs pulses and blood pressure is strictly recommended. TA is a systemic vasculopathy that can progress to cause vital organ ischemia. Therefore, early diagnosis and management as well as long-term follow-up is recommended.

Keywords: Left ventricle dysfunction, middle aortic syndrome, Takayasu arteritis

How to cite this article:
Thakkar DP, Shankar S, Soudri R, Babu Reddy T S, Nagesh C M, Srinivas B C, Manjunath C N. A case of middle aortic syndrome in takayasu arteritis with left ventricle dysfunction and heart failure. J Indian coll cardiol 2022;12:28-30

How to cite this URL:
Thakkar DP, Shankar S, Soudri R, Babu Reddy T S, Nagesh C M, Srinivas B C, Manjunath C N. A case of middle aortic syndrome in takayasu arteritis with left ventricle dysfunction and heart failure. J Indian coll cardiol [serial online] 2022 [cited 2022 May 27];12:28-30. Available from: https://www.joicc.org/text.asp?2022/12/1/28/337350

  Introduction Top

Takayasu arteritis (TA) is a chronic inflammatory disease of unknown etiology that primarily involves the aorta, its major branches. TA is more common in Asians, and females are more frequently being affected. The diagnosis of TA is often delayed because many patients manifest nonspecific symptoms. Left ventricle (LV) dysfunction is an uncommon complication of TA and seen in about 10%–15% of patients affected with this disorder. We are presenting a case of a 27-year-old girl who had LV dysfunction, right side hemiparesis cerebrovascular accident (CVA), and coaractation of the aorta (middle aortic syndrome).

  Case Report Top

A 27-year-old girl was admitted with dyspnea, pedal edema, loss of weight, and anorexia (NYHA IV). She was diagnosed with LV dysfunction for the past 3 months when she developed right hemiparesis before 3 months ago, computed tomography of the brain was suggestive of left temporal lobe infarct. On examination, absent pulse was observed in the left upper limb and both lower extremities while right upper limb blood pressure (BP) was 160/90 mm Hg. General physical examination revealed engorged jugular venous pressure, tachycardia, severe wasting, and pedal edema. Cardiovascular examination revealed the presence of the third heart sound. The complete blood count showed hemoglobin 10.2 g%, leukocyte count 11,400/mm3, and platelet count 275,000/mm3. The C-reactive protein (CRP) level was 1.9 mg/dL, and the erythrocyte sedimentation rate (ESR) was 30 mm/h. She had both liver and renal dysfunction due to congestive heart failure. Antinuclear antibody (ANA) test was found to be negative. Chest X-ray showed cardiomegaly.

Echocardiography revealed dilated LV with moderate mitral regurgitation [Figure 1]a and [Figure 1]b. Moreover, decreased LV ejection function (LVEF 20%). A 64-slice computed tomography aortogram showed proximal right coronary artery: 60%–70% stenosis. Ostio proximal left common carotid artery: 80%–90% stenosis. Ostio proximal left subclavian artery is occluded. Coaractation of descending and abdominal aorta with maximum severity of 90%, ostio proximal celiac artery is occluded for a length of 3.6 mm. Ostio proximal superior mesenteric artery (SMA) shows 80%–90% stenosis. Bilateral renal, common iliac, external and internal iliac artery are patent [Figure 1]c.
Figure 1: (a and b) Showing dilated left ventricle with moderate mitral regurgitation. (c) A 64-slice computed tomography aortogram showing coaractation of descending and abdominal aorta. (d-f) Showing coaracted aortic segment with normal coronary arteries

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After confirming the diagnosis of TA-associated CVA, Coarctation of the aorta (MIDDLE AORTIC SYNDROME) with LV dysfunction, she was treated with methylprednisolone pulse therapy, oral prednisolone (1 mg/kg/d), and weekly methotrexate (15 mg/m2). For congestive heart failure, she was given furosemide diuretic, spironolactone, and carvedilol.

After improvement in renal parameters, aortogram done through using pigtail catheter through right femoral artery approach revealed long segment narrowing of descending thoracic and abdominal aorta with maximal severity of 90% above mesenteric and renal arteries with gradient of 50 mmHg [Figure 1]e and [Figure 1]f. Coronary angiogram shows normal epicardial coronaries. Hence, it was decided to do coarctoplasty with stent implantation [Figure 1]d.

It was done through the right femoral artery approach. A 6F arterial sheath was inserted into the right femoral artery. With multipurpose catheter and straight Terumo wire lesion is crossed. Pre dilatation is done with 10 mm×40 mm balloon. After that 14 mm×120 mm Bard e luminous stent is deployed across the lesion, post dilatation is done with 12 mm×40 mm atlas balloon. After post dilatation check shoot shows good results with no residual gradient across the lesion [Figure 2]a and [Figure 2]b.
Figure 2: (a and b) Showing final result post coaractoplasty

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At 6 months follow-up, patient is symptomatically better with NYHA I symptoms with LV function had improved slightly with LVEF ~40%, lower limb pulses are now palpable and with BP is under control (BP 110/70), she was put on treatment with furosemide, ramipril, carvedilol, and spironolactone for LV dysfunction with daily oral prednisolone and weekly methotrexate therapy.

  Discussion Top

TA also known as pulseless disease, is a granulomatous systemic idiopathic disease involving the large arteries including the aorta and its primary branches. It leads to segmental stenosis, occlusion, fibrosis, dilatation, infarction, or aneurysm formation.[1] It is predominantly a disease of young females in their second and third decades. Although this disorder is more frequent in Asians, it occurs worldwide and no race seems to be immune. Manifestations range from asymptomatic disease, found as a result of impalpable pulses or bruits, nonspecific symptoms such as fatigue, malaise, weight loss, fever, arthralgia, myalgia, or anemia to catastrophic neurological impairment. As the inflammation progresses and stenosis develops, the more characteristic features become apparent, influenced by the development of collateral circulation. These patients often have arterial hypertension, which is frequently associated with coaraction of the aorta, renal artery stenosis.[2] The laboratory data usually includes findings that are indicative of generalized inflammation, with mild leukocytosis, anemia, thrombocytosis, and elevation of the CRP, ESR. Other laboratory data for connective tissue disorder (ANA, rheumatoid factor, etc.,) are very often within the normal limits. The disease can affect the cardiovascular system and manifests as cardiomyopathy, valvular disease, aortic root disease, the involvement of the aorta and its branches (subclavian, carotids, renal, etc.), coronary artery disease, or constrictive pericarditis.[3] Dilated cardiomyopathy (DCM), LV dysfunction is seen less often in TA and it is either due to secondary hypertension from coaraction of the aorta, renal artery stenosis, primary myocarditis, or due to coronary artery involvement. Sometimes, coaraction of the aorta with resultant hypertension may cause LV dysfunction or DCM in the late stage.[4]

In our case, she had presented with congestive cardiac failure, and clinical examinations revealed left upper limb and bilateral lower limb pulselessness and hypertension in the right upper limb with right-sided hemiparesis with positive markers for inflammation. Echocardiography revealed severe LV dysfunction. Computed tomography aortogram showed features suggestive of TA with normal coronary arteries and involvement of carotids, subclavian, superior messenteric artery (sma), thoracic andabdominal aorta hence it fits into Type V according to new angiographic classification of TA.[5] In our case, LV dysfunction could be due to either secondary hypertension due to coarctation of the aorta or myocarditis. Sometimes, it may take several months to observe improvement in the LV function after starting the immunosuppressant therapy. In some cases, there may be no improvement in LV function.

Treatment should aim to control disease activity and preserve vascular competence, with minimal long-term side effects; those with disease that carries a good prognosis should not be put at risk by treatment that is more harmful than the disease itself corticosteroids in high doses are the primary treatment recommended for TA. Cytotoxic therapy, such as methotrexate, cyclophosphamide, and mycophenolate mofetil, is recommended for those patients that are resistant to glucocorticoid treatment.[6] Antihypertensive and decongestive therapies are given to patients with heart failure and systemic hypertension, usually with good response. Surgical or PTA therapy is important in the revascularization of stenosis due to occluded vessels, which procedure significant ischemia.[6]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Johnston SL, Lock RJ, Gompels MM. Takayasu arteritis: A review. J clin pathol 2002;55:481-6.  Back to cited text no. 1
Yoshida M, Watanabe R, Ishii T, Machiyama T, Akita K, Fujita Y, et al. Retrospective analysis of 95 patients with large vessel vasculitis: A single center experience. Int J Rheum Dis 2016;19:87-94.  Back to cited text no. 2
Panja M, Kar AK, Dutta AL, Chhetri M, Kumar S, Panja S. Cardiac involvement in non-specific aorto-arteritis. Int J Cardiol 1992;34:289-95.  Back to cited text no. 3
Horáková L, Pudil R, Hrncír Z, Vizd'a J. Cardiomyopathy as one of the less frequent manifestations of Takayasu's arteritis. Acta Medica (Hradec Kralove) 2011;54:167-9.  Back to cited text no. 4
Nakagomi D, Jayne D. Outcome assessment in Takayasu arteritis. Rheumatology (Oxford) 2016;55:1159-71.  Back to cited text no. 5
Schmidt J, Kermani TA, Bacani AK, Crowson CS, Cooper LT, Matteson EL, et al. Diagnostic features, treatment, and outcomes of Takayasu arteritis in a US cohort of 126 patients. Mayo Clin Proc 2013;88:822-30.  Back to cited text no. 6


  [Figure 1], [Figure 2]


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