pulmonary hypertension

[Glasscrafter]

In 2003  a routine physical indicated a possible MI.Testing revealed a soft systolic ejection murmur.The EKG was written off as a athletic heart after a stress test.In 2004, I experienced swelling in my legs (+1 to 2). An echo showed mild regurgitation in the mitral and aortic valves, moderate tricuspid, LVEF 55-65%, moderate pulmonary hypertension 41mm, mild leaflet thickening of the mitral valve.Hypercontractile ventricle. TEE at the time estimated hypertension at 34mm. My 2007 annual showed PACs.The ankle edema was noted again. Echo revealed LVEF>55%,all valves showed regurgitation. Tricuspid is moderate. Diastolic dysfunction and moderate pulmonary hypertension of 40-50mm. The right atrium mildly dilated in size. The Holter monitor showed QRS 107530, with 57 Tachycardias, the longest lasting 36 minutes, 14 Brachycardias and 3316 PACs, 2 of which were couplets. I am also diagnosed with ankylosing spondylitis and ulcerative colitis. I am experiencing some SOB, excessive sweating while exercising, swelling in my ankles, occasional nausea, and sometimes my hands and feet get extremely cold when exercising. I am currently on 10mg/kg remicade every 4 weeks.What is my prognosis? I've read that survival with pulmonary hypertension is in the range of 2-15 years. What procedures should I expect my new cardiologist to recommend? Could the asthma I thought I had and was taking Advair for, actually be pulmonary hypertension? What medications, if any, are effective with my condition? My BP is usually less than 110/65. My PFT and CT lung scan both came back essentially normal. Do I have PH?

[Forum-M.D.-bkj]

Glasscrafter,

thanks for the post.

What is my prognosis?


This is an impossible question for me to answer. There are different prognoses for pulmonary hypertension from different conditions, so I wouldn't focus on those numbers.

The most important thing to figure out is what the underlying cause of the pulmonary hypertension is (Connective tissue disease, HTN, idiopathic, etc)  There are treatments available for different types of pulmonary hypertension so understanding the cause is a must.  I would recommend seeing a specialist in pulmonary hypoertension.

Generally pulmonary hypertension presents as shortness of breath. This may overlap with a diagnosis of asthma.

Depending on the underlying cause of the pulmonary hypertension, there are multiple different medications. Most likely your physician would start with vasodilators or calcium channel blockers depending on their evaluation.

If you've had equivocal findings, your physician may perform a heart catheterization to evaluate the actual pulmonary pressures.

[tickertock]

I have been diagnosed with ankylosing spondylitis, I do experience excessive sweating, but according to my last echo I have no leaks of any of the valves, or no swelling of the ankles. may I ask how long have you been diagnosed with AS, I"m 42 male and had the diagnosis since 2000. It cause me alot a pain & stiffness and a slight loss of mobility and flexibility, can I ask How about you? Thanks in advance , I think it has just been coming to light what a debilitating disease this can be.

[tickertock]

Oh forgot to mention with a connective tissue disease, I ' m made to understand slight PH is a common finding and except for only a few of the CTDs it rarely leads to life threatening problems from my understanding and AS is generally not considered life threatening.

[Glasscrafter]

I've probably had AS for 30 years, but was diagnosed about 1 1/2 years ago. As far as dibilitating, it is quite. I'm starting to fuse in the SI joints, have total stiffness and no flexibility in the lumbar , and prior to starting remicade (10mg/kg every 4 weeks), I could barely turn my neck. I've got an incredible amount of damage in my back from the SI joints right up to the cervical. I've had plantar fascitis, costochondritis, uveitis, ulcerative colitis, carpal tunnel, and tendonitis/bursitis in my elbow , knee and hip as a result of it. I can't stand for more than about 30 minutes nor sit for more than about 1 hour without pain. Walking any significant distance is a problem. I also have an implanted spinal cord stimulator to relieve some of the pain and am on morphine 30mg, 3x a day. I used to bike 150-200 miles a week and do 8 hours of aerobics. I haven't biked in 5 months and am down to 5 hours of aerobics a week. If you're lucky, your case won't progress. Are you currently being treated by a rheumatologist?

[tickertock]

Thanks for responding, i'm very sorry hear that your AS appears to be very severe, I would classify mine as being moderate though it does appear to be getting worse as I can barely move my neck now without turning my whole body.  I was offered immuniosuppressants & steroidal treatment a few years back when first diagnosed but I refused. I know understand AS has been reclassified as an autoimmune/CTD disease rather than just a CTD. I suffer from chronic costochondritis myself and have a problem with fatigue, but I try to keep going no matter what.

It's my understanding that the damage it does cause to lungs and heart are somewhat mild and limited when it does happen so that in itself is a comfort, unless amyloidosis develops(which is a very rare complication) I understand also it is a rarely a threat to life just very punishing. I consulted with a rheumatologist when first diagnosed but since then has been mainly treated by an internist/surgeon who has been my doctor for the last 25 years.I only take aspirin every day and co-dyrammol when the pain is severe, also diazepam(valium) 5 mg on as needed basis mainly as a muscle relaxant and not an anti anxiety agent which I probably only take twice a week.

I hpe you feel better and continue to improve. Thanks very much for sharing. Wishing you all the best.

[Flycaster305]

I'm also a AS patient.  I'm in my late 60's, have serious heart problems but they aren't related to AS as far as my doctors are concerned.  My spine and ribs froze 35 years ago.  However, when I was in my 50's the pain left.  It's unusual to be on aspirin therapy as asprin isn't usually very effective as a pain killer with AS patients. I had  a genetic test for AS.  It is usually traced to a problem with people with roots in the Alsace-Loraine area in Europe, and the marker was there. I think heart-related problems with AS are pretty rare.  Good luck, AS is miserable stuff.  

[Glasscrafter]

I found out this week that I have sclerosis of the aortic valve which from what I've read on the internet, is related to connective tissue diseases. Could this be the cause of my left ventricular diastolic dysfunction, or is it the hypertrophy? Seems like everything is interconnected----the AS causes the aortic valve sclerosis----the diastolic dysfunction can cause the pulmonary hypertension----the right heart dilation is caused by the pulmonary hypertension. It just seems to go on and on.....