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 idiopathic pulmonary arterial hypertension (IPAH). 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 IPAH. IPAH is extremely rare, occurring in about two persons
per million population per year.
Secondary pulmonary hypertension means the cause
is known. A common cause of secondary PH 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.
Check-out this informative PH
brochure.
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it.
The
information provided on the PHA website is provided for general information
only. It is not intended as legal, medical or other professional
advice, and should not be relied upon as a substitute for consultations
with qualified professionals who are familiar with your individual
needs.