Cardiac question related to Thal Intermedia

Hello Everyone,

I have Thal Intermedia..missing a beta and I have an extra alpha.  Hbg run 7.5....starting more regular transfusions because the tiredness and shortness of breath have been so bad. 
I have had my spleen and gallbladder removed.  Also have had a lot of trouble with blood clots.  The main one is in the portal vein, which causes Portal vein hypertension and large esphogeal varicies.  I have also had clots in the messentery and cecum in which a right hemicoloectomy was done.  Random venous clots in the lower legs....

I recently went to a cardiologist for baseline testing...had an echo and this is what it showed

Rhythm: Sinus
BP: 114/58

SUMMARY:

1. Normal LV size and systolic function.
2. The quantitative left ventricular ejection fraction by biplane
Simpson's method is 68%.
3. The left atrium is severely dilated.
4. Mildly elevated right atrial pressure with mild pulmonary
hypertension.
5. The diastolic filling pattern is normal with normal left atrial
pressure.
6. There is mild to moderate (1-2+/4+) mitral regurgitation present.
7. There is posterior mitral annular calcification.

I also see a pulmonologist this week.  No calls from any doctors related to the Echo, just wondering what you all think.

Thanks,,,blessings to all
Linda

Hi Linda,

Have you been diagnosed with pulmonary hypertension or been treated for it? I am no expert in diagnosing PHT, but what's going on with your heart does seem to have to be related to PHT in the context of thalassemia. Please make sure your pulmonolgist is looking at this in the context of thalassemia, as the changes in the heart are often reversible with proper iron chelation and treatment for PHT.

And this is a classic example of why I don't recommend that patients submit to routine splenectomy.

Also, have you had a cardiac MRI to measure iron loading?

Some important information about this type of beta and alpha thal: The combination of beta trait and a triplicated alpha gene can really wreak havoc on patients due to the great excess of unmatched alpha globin, and most often, this is not properly appreciated by doctors. It can manifest as transfusion dependent thalassemia and should be taken seriously.

Haven't seen a pulmonary doc yet.... I go next week. I had a cardiac MRI a few years ago but nothing routine.  My docs are good.....but I feel as though they don't always look at the big thalassemia picture. This is the first echo since 2013 and first time anything odd has come up. Thanks. Just wondering what questions to have ready when I see the pulmonary doc. This SOB is a a bummer

The question that needs to be asked is if PHT is developing and what can be done to prevent it. PHT in thals is mostly related to the depletion of nitric oxide, NO. This causes the pulmonary artery to lose flexibility, causing the heart to work harder to pump blood through the artery. Make sure the doctor understands the NO depletion that takes place in thalassemics. Supplements like carnitine and arginine help add NO, as do drugs like sildenafil are often used.