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Pulmonary Hypertension

Raynaud`s, Scleroderma

02/05/2008

Question:

I have had Raynauds (15yrs) and Limited Sclerderma (CREST) (10yrs). Resent ECHO`s: systolic pressures @ 33 & most resent 35mmHG. Aortic Value/Structure-Abnormal,Function-Normal,Mitral Value/Structure-Abnormal, Function-Normal, all other indicators- Normal. Plethysmograph Report:FEV1: 65%,Mildly Deceased, FEVI/FVC: Moderaterly Deceased, FEF 25/75: 27%, Severely Deceased, TLC,RV,FRC-Normal, DLCO: 61%,Midly Deceased,DLCO/VA: 61%, Mildly Deceased. Impression: Spirometry showing moderately obstructive airflow with severe air flow obstruction in small airways. Mildly deceased diffusion may be to interstial lung disease or pulmonary vascular disease. Suggest excercise oximetry. What is your opinion on these readings? Thank you for your time.

Answer:

Scleroderma causes several lung complication most commonly pulmonary hypertension or lung fibrosis.  You do not say what your lung symptoms are; the pulmonary function tests are very worrisome for lung fibrosis. The usual evaluation would include a CT scan of the chest. The systolic pressures are not very high on the echo but again that has to be taken in the context of your symptoms.

If you have symptoms and there is no lung disease on the CT, then a right heart catheterization would be needed to assess the pulmonary artery pressures.

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Response by:

Namita   Sood, MD, FCCP Namita Sood, MD, FCCP
Associate Professor of Pulmonary, Critical Care & Sleep
College of Medicine
The Ohio State University