right heart enlargement and possible Pulmonary hypertension

[helix]

I am a 32 years old male (5-7, 140 lbs) with good health habits. For the last six years, I had a history of light-headedness and fatigue. Since last May, I have been experiencing chest pain, irregular heart beat, and reduced exercise capability. An Echo done in last June showed no abnormality in my heart, with a pulmonary artery pressure (PAP) of 32. A blood test showed high TSH level (6.02 MIU/L), but it came down to normal in two other tests months later. Since then,I have developed further symptoms such as short of breath, tight throat, back pain, blue/purple discoloration (after shower) and varicose veins in hands and feet. A stress Echo done in last Oct. showed a mildly dilated right heart ventricle, moderate TR and PI, and PAP of 46 at rest and 55 at peak exercise. A right heart Cath in last Nov. measured a normal mean PAP of 17 mmHg (25/12) at rest and 14 (22/7) after exercise. However, an abnormal increase in oxygen saturation (85.7%) was discovered in my IVC blood, compared to 70% in SVC. The cardiac output was 5.79 liters/min. So an arteriovenous shunt was suspected and MRI tests were done for the chest, abdomen, and pelvis regions with normal results.

My doctors seem to be out of ideas and my conditions keep getting worse. What is your take on this case?
(1). How accurate and reliable is Cath in terms of measuring the pulmonary pressure?
(2). How abnormal is O2 saturation 85.7% in IVC blood? Is it a definite indication of AVF?
(3). Without MRA, is MRI enough in detecting AVF?

Thank you very much for your time and any advice will be high appreciated.

[Cleveland Clinic]

helix,

thanks for the post.  


(1). How accurate and reliable is Cath in terms of measuring the pulmonary pressure?

It would be the gold standard.


(2). How abnormal is O2 saturation 85.7% in IVC blood? Is it a definite indication of AVF?

The IVC blood will have a higher saturation then the SVC blood because the brain extracts more oxygen.  85% seems a bit high.  Depending on how the blood was drawn it may not be an accurate readin if the rest of your workup is completely negative.  The only way to tell if it was the case would be to repeat the data point.

(3). Without MRA, is MRI enough in detecting AVF?

For the most part pretty good if the study is a good study.  

Even if you had a AV fistula, it really wouldnt explain your specific constellation of symptoms.  I would want to examine you, repeat your blue discoloration phenomenon in the office and see what rhythm your heart is having during your symptoms.

Perhaps a second opinion might help tie things together.

good luck

[helix]

Thank you very much for your reply, Dr. bkj.

"Depending on how the blood was drawn it may not be an accurate readin if the rest of your workup is completely negative. The only way to tell if it was the case would be to repeat the data point."

Actually, the doctor whom did the Cath was surprised at the reading and repeated it several time, both at rest and after exercise. The data are mid IVC 85.7, low IVC 81.0, iliac vein 83.2, femoral vein 83.8.

"Even if you had a AV fistula, it really wouldnt explain your specific constellation of symptoms"

If the AV fistula causes right-heart failure, as suggested by the enlargement, Would it explain my symptoms? I mean the bule discoloration in my hands/feet may due to pulmonary insufficiency and low O2 saturation.

[Cleveland Clinic]

Your step up isnt that marked through your IVC and femoral vein, certainly within a reasonable amount of laboratory error.  The echo didnt show findings consistant with right heart failure and your pumonary pressures are not that elevated. I would want to do a carful physical to try to make sure there isnt some other systemic cause for your symptoms. Hope this is a start.

good luck