What is PH?
By
Pulmonary Hypertension Association
Pulmonary hypertension (PH) 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.
The information provided on
the PHA Web site 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.
Pulmonary Hypertension
Association, 850 Sligo Ave, Suite 800, Silver Spring, MD 20910,
please contact PHA’s offices at 301-565-3004.
