Complete recovery of severe left ventricular systolic dysfunction and mitral regurgitation after stenting of the descending thoracic and infra-renal aorta in a patient with Takayasu arteritis

A 16-year-old girl was referred for evaluation of worsening breathlessness, limb claudication (both legs and left arm) and weight loss of three years duration. Physical examination revealed discrepancy in arterial pulses, palpable pulsations in the supra-sternal region and intercostal spaces carotid, intercostal and abdominal aortic bruit S3 gallop and pansystolic murmur of mitral regurgitation. Inflammatory markers were markedly elevated. Echocardiography showed severe left ventricular (LV) systolic dysfunction (LV ejection fraction 25 ) with moderately severe mitral regurgitation. On angiography, long segment tubular stenosis of descending thoracic aorta and infrarenal aorta was seen with significant narrowing of multiple branches of the aorta. Central aortic pressure was 20078 (124) mm-Hg with peak gradients of 48 mm-Hg across the descending thoracic aorta and 40 mm-Hg across the infra-renal abdominal aorta. A diagnosis of type-V Takayasu arteritis was made using the angiographic classification. Percutaneous angioplasty and deployment of self-expanding Wallstents was performed across both stenotic segments after initial medical stabilization. Since then she has been free of lower limb claudication, and at 6 months follow-up her LV systolic function had normalized (LV ejection fraction 52) with regression of mitral regurgitation