Cardiac monitoring and treatment in thalassemia major

Hi everyone I want how to diagnosis early cardiac problem in thalassemia major as alone LVEF is not enough enough ..??
What's the treatment for cardaic complications as many cardaiclogist as not train to detect cardaic problem in thal and how to treat it
Thanks to all in advance

If cardiac problems beyond those that are caused by reversible iron overload are suspected, which can be detected by MRI, I believe that the patient should be investigated for any signs of pulmonary hypertension.

Andy, I've always had increased heart beats since several years now (I can't ever remember them being 70-80 anyway since I was child lol). Does that mean I have a chance for PHT? I don't have any other symptoms of it except increased heartbeats (90-105).

I however notice that they get better in the first week after I transfuse (stays around 90 or so mostly). So I guess all Thal majors mostly has this especially on the last few days before transfusion due to slightly lower hb?

Thanks,

-P

All transfusing patients are at risk of PHT. And as you observe, the lower the Hb, the harder the heart has to work. Antioxidants and nitric oxide fixers like carnitine and arginine can help prevent this, as will keeping the Hb higher.

Hi Andy,you mentioned "the lower the Hb, the harder the heart has to work." Are there any medical journal articles published supporting this. I need to take them to my PHT Dr who has somehow been reluctant to take this into account when considering my course of treatment and giving her input into frequency of BTs.

Thanks.

It's baffling that any doctor treating thalassemics with transfusion would not understand this. Please see the article at http://www.thalassemiapatientsandfriends.com/index.php/topic,1451.0.html

Cardiac structure and function in TM are mainly affected by two competing factors: iron load and increased cardiac output (CO). The cardiac iron deposition results in a decrease of left ventricular function. The anaemia together with marrow expansion leads to volume overload and increased CO that then demands increased contractility adding additional stress to the heart. (Starling’s Law).

The article goes into depth about the factors affecting the heart in thalassemia, so there is much in this article to support maintaining a higher Hb level.

As CO may be close to normal when Hb level is ≥10 g/dL, patients’ Hb concentration should be kept above 10 g/dL by regular blood transfusions. The patients should only be transfused with one packed red cell unit no greater than 250 mL with diuretic treatment as well.

Thank you very much. I will be sure to take it to my next appointments.

Khwahish