My 16 year old daughter has a rare auto-immune disease called "Takayasu Arteritis". Her medicines consist of Prednisone 10 mg daily (down from 40 mg), Azathirophrine 150 mg daily, Methotrexate 1cc 25mg injection weekly, Humira 40mg 1cc injection weekly, & Levothyroxine .5mcg daily. Despite these medications, her Sed-rate ranges 13 - 40 and its checked every 2 weeks along with CRP which ranges 3.2 - 12. Her Liver Panel, Kidney Panel, & CBC are normal except for WBC ranges 10 - 13. She has pulseless left arm, left subclavian occlusion at 100% with collateral vessels suppling blood to arm. Partial right subclavian occlusion with bruits and reduced pulse. Current & chronic symptoms are: Dizziness daily, Headaches daily, eye pain occasionally, memory/concentration issues. No vascular surgery is recommended at this time. She's been referred to a neurologist even though CT last week shows no brain atrophy and major vessels & circle of wills shows good blood supply. NEW ECHO-CARDIOGRAM from last week, shows 3 valve leaks. TRICUSPID REGURGITATION, AORTIC REGURGITATION, & PULMONIC REGURGITATION. These are all considered small and should be watched. 6 months ago she only had a small Aortic Valve leak. Could these 3 valve leaks be causing Pulmonary Hypertension? I have read that many of my daughters symptoms could be explained by Pulmonary Hypertension, but most doctors seem to think she needs a neurologist? CT showed brain had good blood circulation. I know TA is an extremely difficult disease to manage, but I want to be proactive rather than waiting for an life-threatening emergency to happen. Whats your thoughts? Should we pursue a visit to the Pulmonary Specialist? Also do all three of these valve leaks show up together often? Could the fact that she has all three at the same time be a tell-tale sign of something not yet discovered? Your help is greatly appreciated.
As you mentioned, Takayasu arteritis is an autoimmune disease that causes progressive inflammation and obstruction of blood vessels throughout the body. The echocardiographic findings that you mention, tricuspid, aortic, and pulmonic valve regurgitation, are difficult for me to evaluate without seeing your daughter’s echocardiogram. Typically, we characterize these leaks as trivial, mild, moderate, and severe. You say that these are “small”, which suggests that, at most, they are mild. All of the valves of the heart can have at least trivial regurgitation, which is a normal finding and is not associated with any disease. It is unclear to me whether your daughter’s findings are any worse than this. I will say that a valve leak does not cause pulmonary hypertension; it is actually the other way around. Pulmonary hypertension is elevated blood pressure across the lungs. This pressure can push back to the right side of the heart and lead to pulmonic and tricuspid valve regurgitation. One of the helpful things that echocardiography has given us is the ability to noninvasively measure the pressures in certain chambers of the heart. Specifically, with tricuspid valve regurgitation, we can estimate the peak pressure across the lungs. Therefore, what would be more important here is not how much leak there is at the tricuspid valve, but what is the estimated right ventricular pressure. If this is normal, there is no evidence of pulmonary hypertension. Your cardiologist should be able to give you this information. Sadly, pulmonary hypertension has been reported in conjunction with Takayasu’s.
Of note, a pulmonary specialist will not be able to assist with pulmonary hypertension, if she does, in fact, have it. It would be better treated by a cardiologist who specializes in the management of pulmonary hypertension, who may use medications to help to lower the pulmonary pressure. As well, as you likely know, catheter interventions with balloon angioplasty and stent placement for the arterial obstruction in Takayasu arteritis have also been intermittently successful in improving blood flow to the lungs.
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