[Pulmonary hypertension in Takayasu arteritis]

Area of Science:

• Cardiology
• Rheumatology
• Vascular Medicine

Background:

• Takayasu arteritis (TA) is a rare chronic vasculitis affecting large arteries, predominantly the aorta and its branches.
• TA predominantly affects young women (8:1 ratio) with onset typically before age 40.
• While aortic regurgitation and pulmonary artery stenosis are rare TA manifestations, they can occur.

Observation:

• A 49-year-old female patient with TA presented with severe left pulmonary trunk stenosis, leading to pulmonary hypertension.
• The patient also exhibited severe aortic regurgitation (due to ascending aorta dilatation), mitral insufficiency, critical left renal artery stenosis, and possible left main coronary artery stenosis.
• No significant stenosis or occlusion was noted in the mid-portion of the subclavian arteries.

Findings:

• The patient's presentation included severe congestive heart failure secondary to the complex vascular involvement.
• Surgical intervention involved mitral valvuloplasty and aortic valve replacement.
• Despite treatment, the patient succumbed to refractory pulmonary hypertension in the early postoperative period.

Implications:

• This case underscores the potential for severe and multi-systemic complications in Takayasu arteritis, even in older patients.
• It highlights the critical role of pulmonary hypertension as a cause of mortality in TA patients with significant cardiovascular compromise.
• Aggressive management strategies for pulmonary hypertension may be crucial for improving outcomes in such complex cases.