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

Abstract

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