ASD with Thalasemia major

[prelude]

My son Sugam Singh age 7 yrs male has been diagnosed Thalesemia Major and has Congenital heart disease. Following were the conclusions of his latest ECHO

Report (dated 10-12-07)
Estimated PA Pressure 65 mm hg (Systolic )
Size of ASD 2.5 cm
Impressions
1 Ostium secondum type of ASD of large size
2 Pulmonary hypertension
3 Grade 1 + tricuspid regurgitation

Toaday my son suffered from a syncope after moderate exersion yesterday...Local Doctor Suspected Acute Heart failure .

Last year we opted for surgery for the closure of ASD but the surgery was cancelled at the last moment since the surgeon found his serum ferritin level by CILA to be 2209 ng/ml …The surgen said he doesn’t know if surgery is indicated under such elevated ferritin levels ..since then my son is under chilleting agents but there hasn’t been much improvements of serum ferritin level .

Here are my questions
1 can my son undergo surgery under high ferritin levels …if not what should be the target ferritin level ?

2 Considering the Size of the ASD of my son …which procedure is the best
Surgical closure or some interventional devices like “umbrella “ etc

3 Is there any cure for thalasemia major …if so what ?

[John Kenyon, CNA]

Hello -

To try and answer your questions, out of order, first a surgeon would best know what the desirable and safe ferritin level should be in order to perform surgery. I don't know the answer to that, but do know that it probably could be somewhat above normal. I am assuming that right now the doctors taking care of your son are performing chelation therapy in order to reduce the level of ferritin and I do know this iron overload condition is caused by the frequent transfusions involved in treating thalassemia major. Chelation is the most efficient route toward getting ferritin levels normalized (you probably already know this -- I am thinking out loud here).

Given the size of the ASD, I would think an umbrella or else a good-sized teflon patch, placed via catheter, would be the two most likely approaches rather than open surgery.

The syncopal episode was most likely caused by pulmonary hypertension, and once the other problems are addressed, management of this will be the over-riding concern unless, of course, it is being caused because of the large-sized ASD, in which case closure may resolve everything but the TM, which is manageable and compatable with a fairly normal life (and life span).

My thoughts will be with you and your son. Please keep us updated as to how this all goes. My best to you.