Severe aortic regurgitation revealing Takayasu arteritis: a case report

Takayasu arteritis (TA) is a rare chronic autoimmune vasculitis affecting large and medium-sized arteries, mainly in young women, and is more common in Asia. It may cause stenosis, occlusions, aneurysms, aortic dilation, and aortic valve disease. We are reporting a rare case of severe aortic valve insufficiency and aortic dilation in the context of TA.

We report the case of a 39-year-old patient presenting with progressively worsening dyspnoea, associated with fatigue, weight loss, and febrile sensations. Physical examination revealed signs of heart failure (orthopnoea, tachycardia, pulmonary crackles) with a diastolic murmur at the left sternal border and bounding peripheral pulses. The transthoracic echocardiography revealed a severe aortic valve regurgitation with a dilated aortic annulus, slightly thickened cusps, and left coronary cusp prolapse in addition to dilation of the initial aorta confirmed by aortic computed tomography (CT) angiography. Doppler ultrasound of the supra-aortic trunks revealed a regular circumferential hypoechogenic halo-like thickening of the walls of the common carotid arteries and the left subclavian artery suggestive of TA. Positron emission tomography (PET)-CT confirmed active inflammation of the aortic arch. Laboratory tests showed an inflammatory pattern with anaemia, elevated sedimentation rate and C-reactive protein. Immunological workup was negative. Initial management included heart failure management and immunosuppressive therapy with corticosteroids followed by a Bentall procedure. Histopathological examination of the excised aorta confirmed chronic arteritis with aneurysmal changes. The patient demonstrated significant clinical and inflammatory improvement during follow-up.

This case underscores the importance of considering TA in young women with aortic regurgitation and the value of multimodal imaging and multidisciplinary care.