>>Understanding pulmonary hypertension
http://www.phassociation.org/page2.htm
What is Pulmonary Hypertension?
Pulmonary hypertension is a rare blood vessel disorder of the lung in which the
pressure in the pulmonary artery (the blood vessel that leads from the heart to
the lungs) rises above normal levels and may become life threatening.
Symptoms of pulmonary hypertension include shortness of breath with minimal
exertion, fatigue, chest pain, dizzy spells and fainting. When pulmonary
hypertension occurs in the absence of a known cause, it is referred to as
primary pulmonary hypertension (PPH). This term should not be construed to mean
that because it has a single name it is a single disease. There are likely many
unknown causes of PPH. PPH is extremely rare, occurring in about two persons
per million population per year.
Secondary pulmonary hypertension (SPH) means the cause is known. A common cause
of SPH are the breathing disorders emphysema and bronchitis. Other less
frequent causes are the inflammatory or collagen vascular diseases such as
scleroderma, CREST syndrome or systemic lupus erythematosus (SLE). Congenital
heart diseases that cause shunting of extra blood through the lungs like
ventricular and atrial septal defects, chronic pulmonary thromboembolism (old
blood clots in the pulmonary artery), HIV infection, liver disease and diet
drugs like fenfluramine and dexfenfluramine are also causes of pulmonary
hypertension.
Pulmonary hypertension is frequently misdiagnosed and has often progressed to
late stage by the time it is accurately diagnosed. Pulmonary hypertension has
been historically chronic and incurable with a poor survival rate. However, new
treatments are available which have significantly improved prognosis.
Recent data indicate that the length of survival is continuing to improve, with
some patients able to manage the disorder for 15 to 20 years or longer.
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Excerpted with Permission from
Pulmonary Involvement with Scleroderma
by Barbara White, MD, Professor of Medicine
The fourth problem in the lung is called pulmonary artery hypertension. in
persons with Scleroderma. This condition may occur because the blood vessels
are abnormal. Normally, the lung blood vessels have a nice large, open
diameter.
However, as the blood vessel walls thicken, there is not much room for blood to
go through. In pulmonary artery hypertension, structural narrowing of the blood
vessels occurs, which increases the work of the heart. The heart has to pump
harder to push blood through those narrow blood vessels instead of the normal
blood vessels with wider openings. Pulmonary artery hypertension may happen as
a consequence of interstitial lung disease if it is severe and the blood oxygen
level is low. Or, it can happen in patients who do not have a lot of
interstitial disease. In this case, the structural changes in the blood vessels
happens for reasons we do not understand. In either case, the heart has to pump
harder.
What are the symptoms of pulmonary artery hypertension?
Symptoms associated with pulmonary artery hypertension may be shortness of
breath, and ankle and/or abdominal swelling. I often explain pulmonary
hypertension to my patients as a simple plumbing problem. The right side of the
heart normally pumps blood to the lungs where it picks up oxygen. This is a low
pressure system. The vessels open up easily to let the blood in so that they
heart doesn't have to pump hard to get the blood to the lungs. However, if
those
blood vessels in the lungs are abnormal, the heart has to pump harder to
generate more pressure to get the blood through the narrower vessels. Sometimes
this generates high pressure and fluid backs up resulting in swelling of the
abdomen or feet.
How do you test for pulmonary artery hypertension?
There may be clues in the pulmonary function tests. If there is a particularly
low diffusing capacity on PFTs, the physician would consider pulmonary artery
hypertension a possibility. The test most frequently used to determine
pulmonary
artery hypertension is called an echocardiogram. With an echocardiogram, sound
waves are bounced off the heart to estimate pulmonary artery hypertension.
Cardiologists are getting better at estimating pulmonary artery pressure with
the echocardiogram. If it is really necessary to actually measure blood
pressure
in the pulmonary arteries, a catheter can be put into the heart to more
accurately measure the pressures in the pulmonary arteries.
How is pulmonary artery hypertension treated?
Oxygen may be prescribed if your blood oxygen level is low. Physicians may also
prescribe anticoagulants to reduce the risk of blood clots, a complication that
can make the condition worse. If fluid build-up is present because of heart
failure from the pulmonary artery hypertension, diuretics may be given. Other
medications can dilate the blood vessels of the lungs such as calcium channel
blocker, and Prostycycline. If the pulmonary artery hypertension is due to
interstitial lung disease with active inflammation of the lung. The lung
inflammation can be treated to try to prevent the condition from getting worse.
Finally, lung transplantation can be used to treat pulmonary artery
hypertension.
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Helpful resources
Mayo Pulmonary Hypertension Clinic Mayo Clinic
http://www.mayo.edu/cv/wwwpg_cv/pul_cln/pted~1.htm
Primary Pulmonary Hypertension Net Medicine
http://www.netmedicine.com/pt/PTINFO/Prim-ph.htm
Primary Pulmonary Hypertension NIH
http://www.nhlbi.nih.gov/nhlbi/lung/other/gp/pph.htm
What is Pulmonary Hypertension? On this page, you can submit your questions
about PH for a doctor to answer. WebMD
http://my.webmd.com/experts_article/DMK_ARTICLE_3961283
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Flolan (Prostacyclin) is given continuously through an catheter.
See the Scleroderma Clinical Trials Consortium for doctors who may be
participating in Flolan, or other drug trials, for Pulmonary Hypertension.
http://pilot.msu.edu/user/martinr3/sctc_13.htm
A Randomized Controlled Trial of Epoprostenol Therapy for Severe Congestive
Heart Failure:
http://www.medscape.com/mosby/AmHeartJ/1997/v134.n01/ahj1341.califf/ahj134
1.califf.html
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A NEWSLETTER FOR THE PULMONARY HYPERTENSION ASSOCIATION (PHA)
Editor: Sue Kelly
http://www.phassociation.org/PersistentVoices.htm
The purpose of Persistent Voices (PV) is to allow patients and their families,
care-givers, significant others, and friends to voice their personal
experiences
and coping strategies with one another.
Submission Schedule:
Submissions for PV are accepted all year long and published according to date
received.
Story Submissions:
Please limit submissions to between one to one and one-half single-spaced
pages.
I also accept legible handwritten material. Title your stories, this makes them
more your own. I am especially interested in hearing how our members are
"living
each day of their lives." Feel free to include humor, your honest emotions, and
photographs, newspaper clippings, etc.
Note: Persistent Voices Needs Roving Reporters! Be the one on your "block" to
report the stories and events happening in your PHA "neighborhood." It's not a
tough job, you just need to be alert and aware. Contact editor Sue Kelly for
the details.
Send Submissions, Comments, and Questions to:
Sue Kelly, Ed.
P.O. Box 1931
Tucson, AZ 85702
gsk@u.arizona.edu
520-888-3608
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List of support groups in your area for pulmonary hypertension and contact
information if you would like to begin one yourself.
http://www.phassociation.org/SGroup2.htm
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A great resource for a lot of issues pertaining to pulmonary hypertension
http://www.phcentral.org/
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What is PH? Information on support and treatment for PH
http://www.pstweb.com/schmidt/pph.html
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pulmonary hypertension. U. Indiana - ALL-NET Pediatric Critical Care Textbook
Source: University of Indiana at Indianapolis, Department of Radiology. On-line
tutorials in radiography provide in-depth training in interpretation of
pediatric chest x-rays. pulmonary hypertension Just...
http://www.med.ub.es/All-Net/spanish/cardpage/operate/vsd
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Pulmonary Hypertension - Internet Medical Education Discussion Forum for
Pulmonary Hypertension
http://www.einthoven.com/HyperNews/patient/get/congenital
<<>>
Sherry Messick
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