14 y.o. with low DLCO and clear CT of Lungs

[LisaSD]

I noticed my 14 year old daughter getting short of breath about 6 months ago and thought she just needed more exercise although her BMI is 18% and she does go out and play a lot.  However, the breathing kept getting worse so I took her to the doctor.  They ordered a PFT which was done January 9th.  The results from that are:

1.  There is a proportionate decrease in FVC and FEV1 with a preserved FEV1/FVC ratio consistent with a Restrictive Lung Defect.      
2.  Reduced total lung capacity measured by Plethysmography suggesting a mild restrictive pattern.    
3.  Elevated residual volume suggests Air Trapping.    
4.  DLCO is Moderately Reduced.

Her adjusted DLCO value was 54% of the predicted value, her TLC was 76%, the FVC was 79%,  and the FEV1 was 81%.  She had a CT scan of her lungs on Tuesday night and the results are normal.  She feels very tight in her chest, almost like someone is sitting on her, but she does not complain of arm or finger pain/tingling and asthma has been completely ruled out. Her Pulmonologist has diagnosed her with a Restrictive Lung Defect, but I don't think that would cause her DLCO to be so low.  Could this be a heart problem?

[grendslori]

My first question would be, has anyone thought to sent your daughter to see a Pediatric Cardiologist? My next question would be: is she taking any kind of drugs? Amiodarone (a serious heart medication) causes Restrictive Lung Disease (my daughter had a problem with this when she was taking the drug, before her heart transplant) I would take her to see a cardiologist. As far as not having chest pain, arm pain/tingling things like that, children normally have none of those symptoms unless they have a very severe form of heart disease which you couldn't miss if you tried. Those symptoms are usually found only in adults with Coronary Heart Disease or Hypertrophic Cardiomyopathies due to the fact there is a decrease in blood flow to the heart muscle walls. CHD and HCM are EXTREMELY RARE in children.  

[LisaSD]

Thank you so much for your post.  We see a genetic specialist this Wednesday and I am going to ask them to refer her to a Cardiologist for a work up.  She has been on chronic antibiotics for bladder and kidney infections and wellbutrin for add issues.  Other than that she takes calcium pills for mild osteopenia.  I am not sure if either of the two meds would cause such problems.  I certainly hope not.  How is your daughter doing?

[grendslori]

My daughter is not doing too bad. She has had 3 bouts of congestive heart failure since her transplant and so I sometimes wonder how long this heart will last. But we keep plugging along. She just got married 4 months ago and her and her husband are living with my husband and I. They have the walkout level of our home and my husband and I have the upper levels. This is a trip!