Pulmonary Function insights

[whatsnext0408]

Hi all,
I am a pacemaker patient experiencing shortness of breath.  I have a connective tissue disorder Ehlers Danlos, and have been trying to figure out why my lung tests seem to not match up.  Lung volumes seem to be amazing but diffusion has always been low.  Thought once I got the pacemaker that breathing would be improved but still having issues.  If anyone can interpret with some true insight I would be very greatful.  Thanks

My recent PFT is as follows:

_________Raw score _______% Predicted
FEV 1 ______3.80 ___________126.9% flagged Red
FVC _______4.26 ____________119.7%
FEV1/F _____89.1
FEV1/I _____100.41 __________119.8% flagged Red
25-75 _______5.34 ___________160.2% flagged Red
PEF ________8.09____________ 124.6%

MVV 132.84

SVC _______4.26_______________ 119.7%
ERV _______1.81
ITGV _______3.05 _______________104.3%
IC _________2.45
RV ________1.24 ________________77.3%
RV/TLC ____22.5 ______________75.6%
TLC _______5.50 _____________104.2%
VBEex _____0.16
VBe/FV _____3.75
R0.5IN _____1.98 _______________64.6%
sR05IN _____6.78


DLCOSB _____20.42 ___________67.6% Flagged Red
DLCOc blank
VA __________4.98
DL/VA _______4.10 ____________69.4% Flagged Red
DLc/VA blank
VIN _________3.84
BH _________9.10
Hb _________0.0


The report reads lung volumes within normal limits but diffusing capacity is reduced.

Thanks again

[whatsnext0408]

Thank you for your response.  I have also read that many people with EDS have enlarged lungs and low diffusion capacity.  I have spoken with many on the EDS sites that also have shortness of breath.  We all experience drops in blood pressure upon standing along with a myriad of other symptoms.  Could they all work together to cause the shortness of breath?

My echos look good for now, I have had high arterial pressure after my open heart but that seems to be going down and my RV and LV are enlarged but within normal limits.  Otherwise all looks good within the heart with the exception of mildly leaky valves.  I do hold alot of fluid but take lasix daily to try and keep it down.  The shortness of breath occurs alot when its humid, could the low diffusion and humidity be an issue when combined?

As for the mitral valve I work with a team of doctors at Baylor and they are keeping a very close eye on all of it.  I just cant seem to get a clear answer on the shortness of breath or ways to make it any better.  Thank you again for your reply.

[David Tinkelman, M.D.]

With the exception of the diffusing capacity, which I would characterize as low normal, your pulmonary function measurements are within normal limits.  As the only abnormal PFT value, it is unlikely to be of clinical significance, especially in the absence of other signs or X-ray imaging abnormalities.  In other words, this one borderline value is not likely to provide the explanation for your shortness of breath.  One possibility that is not specifically a lung disease but would compromise lung function would be recurrent “silent” pulmonary emboli (blood clots to the lungs).

Other than that, the most likely cause of your shortness of breath would be some type of heart disease including disorders of heart rhythm (not responsive to the pacemaker) or heart failure.

The following should also be kept in mind:  Patients with most forms of Ehlers Danlos Syndrome EDS are prone to develop mitral valve prolapse, and progression to mitral regurgitation occurs more often than in the common form of mitral valve prolapse.  I suggest that further diagnostic studies, including an echocardiogram, be directed to your heart.

Good luck