Retrictive Lung Disease

[Sunshine_2267]

My husband is a 49 wm, non-smoker, healthy weight and very active. He recently experienced chest pressure with shortness of breath. He is unable to do common, daily activities without giving out of breath as if he has just completed a 50 yard dash sprint. He has been hospitalized twice in the past 3 weeks.  A heart attack and embolism were ruled out. He has had the following which have all came back normal: chest x’ray, both regular and high resolution CT scan of his lungs and brain, total body MRI, neuro-muscular diseases, EKG, blood gas, and a heart cauterization. A heart echocardiogram revealed “minor” right heart thickening (doctor said it was normal with hypertension) and a lung function with revealed minor restrictive lung disease. His blood pressure is controlled with medication. My husband is literally not able to walk more than 15-20 feet without giving out of breath, needing to sit down. Up until 3 weeks ago, he was extremely active walking/running daily. He is not able to eat without taking several breaks to just catch his breath. He describes his shortness of breath as the same when he runs sprints on a cold day.  His symptoms include shortness of breath, chest pressure, excessive belching (with no gastric juices), dry cough, neck/throat tightening and fatigue.  The doctors have stated his lung function results should not give him the extent of “sob” he is experiencing. They have admitted his is a hard case and are very cooperative in assisting with getting to the bottom. In the meantime, we want him to get a diagnosis as soon as possible. He was instructed not to drive nor over extort himself. Please shed some light on this and thank you  for the taking the time.

[David Tinkelman, M.D.]

The rapid decline in your husband’s health is puzzling and very worrisome, especially the rapid worsening of symptoms, suggestive of an acute progressive disorder of the heart or lungs; the severity of his symptoms is of great concern.  I phrase the situation this way to convey a sense of urgency in establishing a diagnosis and initiating therapy, without delay. The test result information you provide, if valid, effectively rules out the common causes of such acute symptoms, such as classical pulmonary embolic disease and coronary artery disease with acute heart failure and raises the possibility of uncommon disorders.

One caveat is that in a circumstance such as this, tests that may ultimately provide a diagnosis (for example, heart catheterization, CT scanning of the lungs, echocardiograms) may be normal or marginally abnormal at the outset.  In this category I would place conditions such as pulmonary hypertension (of whatever cause) interstitial lung disease (including acute bronchiolitis), inflammatory pulmonary vascular disease, cardiomyopathy, and constrictive pericarditis,   The “right heart thickening” you mention could be an important early sign of pulmonary hypertension, not grossly elevated on the initial testing but rapidly progressive.

One specific form of pulmonary arterial hypertension (PAH) is pulmonary veno-occlusive disease (PVOD), characterized by widely disseminated clot formation in the pulmonary veins.  With this relatively rare condition, narrowing or obliteration of the pulmonary arteries is found in approximately half the cases of PVOD.

It is clear that his doctors have been diligent in seeking to establish a diagnosis.  They ave ordered all the conventionally appropriate tests.  What is also clear to me is that your husband’s condition is rapidly progressive and there is an urgent need for diagnosis and treatment.  There is no time for further speculation.  I suggest that you and other members of your family meet with his physicians and request immediate consultation with the nearest University Hospital specialists in unusual cardiac diseases, pulmonary hypertension and interstitial lung disease.  Do not delay.  Feel free to share this message with his physicians.

Good luck

[Sunshine_2267]

Thank you for your response. My husband has been referred to the Mayo Clinic. He is currently undergoing several tests. The possibilities you mentioned are being considered. I feel very confident the medical team at the Mayo Clinic will provide a diagnosis and treatment.

Again, thank you for taking the time to answer my post.