Is a cardiac catheterization a good idea?

[mshanson]

I've posted on here severeal times about vmy daughter's issues with cyanosis. At first it was just periperal: hands and feet. Now, it's going clear up her arms and legs. Our family doctor called our daughter's cardiologist, and he said (without seeing her), that no, it's just normal acryocyanosis. However, NOW, her lips are starting to turn blue when she gets these spells. She gets these spells for no reason, warm or cold, eating or sleeping, and they last from a few minutes to 10-15 minutes. several times a day, hands and feet turn dark purple, including nail beds, and the discoloration continues up her arms and legs, with her lips turning darker, and she gets that "moustache" of purplish color in the space between her lips and nose. What concerns me, is because she NEVER has had this spells until the last couple weeks. Not even as a newborn.

Now, she was born with a moderate VSD that is as of yet unrepaired. On her echo a month ago, the VSD was restrictive due to her TV tissue, with a max PG of 68 mmhg. However, they've never checked the actual shunt, to see how much blood is flowing across. She is having issues with her liver swelling, and they have had to increase her Lasix.

I know that normally VSDs are an acyanotic disease, however, is it possible that she is developing Pulmonary Hypertension? Her last chest xray a month ago, did state that she showed slightly prominent central pulmonary vasulature with mild increased interstitial marking bilaterally. From her echo, She had both left and right ventricular hypertrophy (mild), her PA max PG was only 7.9 mmHg however. But, could that have changed over the last month for some reason?

My last question (I promise), is, would a cardiac catheterization be potentially a good idea at this point, to get a better idea of what's going on in her lungs and heart? I asked them awhile ago and they said no, they didn't think it was worth the risk... but my mothers instinct is telling me something's wrong here.

[mshanson]

Thank you for your reply. I forgot to mention her age. She is now 6 months old. They've never checked her sats, hardly ever. The other issue is now in the last week or so, she's no longer peeing nearly as much, even with her fluid medications. She is only having 4-5 "good" pees, and the other day woke up completely dry after 12 hours.  They keep telling me she's doing fine since she has lot of energy and is alert.

[Jeffrey R Boris, M.D.]

Dear Mshanson,

It would be instructive to know what her oxygen saturations are when she has these cyanotic episodes, and what is actually happening here.  It is difficult to tell what is happening without evaluating her myself, and I forget her age (which would be helpful to put her disease in context), but what I'm hearing is somewhat more concerning.  Certainly pulmonary hypertension is a concern, although if there is a gradient of 68 mmHg across the defect with LEFT to RIGHT flow, the likelihood that there is not pulmonary hypertension.  If it is RIGHT to LEFT flow, then it is certainly pulmonary hypertension.  However, the other entity to think about is the development of a double-chambered right ventricle, which can sometimes be missed by echo or by cath unless it's specifically thought about.

If she is truly desaturated and the echocardiogram is not giving a definitive answer, then either catheterization or MRI in the right hands should be able to better help to understand the hemodynamics.