English Information
2
- Clinical picture
- Diagnosis
- Therapy
Clinical picture :
Severe pulmonary hypertension (PH) is a life-threatening
disease, characterised by sustained elevation of pulmonary
artery pressure and plexogeneic arteriopathy of the
small pulmonary vessels due to different diseases. Some
of these diseases are classified as pulmonary arterial
hypertension (PAH) which is either idiopathic (IPAH,
previously called as primary pulmonary hypertension,
PPH) or related to the use of anorexigen drugs, portal
hypertension, systemic connective disease, congenital
heart disease, and HIV infection.
PH can also occur with chronic thromboembolic lung disease,
diseases with hypoxemia or rare diseases. PH due to
left heart failure or left heart valve disease is classified
as pulmonary venous disease (PVH).
Who is affected by IPAH?
The disease can occur at any age, but most commonly
in the third decade of life in woman and the fourth
decade in men. Women are affected about twice as often
as men.
Course:
Mostly progressive course, rarely spontaneous remission.
The most frequent cause of death is chronic right ventricular
failure.
Symptoms:
| • |
shortness
of breath |
| • |
chest pain |
| • |
collapse |
| • |
leg oedema |
| • |
blue lips |
Development of the symptoms:
Due to the constricted pulmonary vessels a very high
pressure is needed for a sufficient blood flow. The
right ventricle can only build up a limited pressure.
The cardiac output is reduced and consequently the blood
flow and oxygen supply of the organs.
Diagnosis:
| • |
Right heart catheterization with
selective pulmonary vasodilator testing is indicated
to confirm suspected PAH. |
| • |
Echocardiography is the
best non-invasive test for screening and follow-up
of PAH by providing assessment of the right ventricular
function, cardiac output and systolic pulmonary
artery pressure. |
| • |
Chest X-ray to exclude lung diseases
and progression control of heart size. |
| • |
Lung function tests to exclude lung
disorders. |
| • |
Blood gas analysis to check the indication
for oxygen therapy. |
| • |
Electrocardiography commonly reveals
right axis deviation and prominent P-waves. |
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Therapy:
| • |
Several medical treatments are now available for severe pulmonary hypertension. |
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Cause of the disease
| • |
Special immunological features can
often be identified, but their overall significance
is still unclear. |
| • |
Appetite suppressants increase the
risk of PPH ten-fold. |
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Problems with the research in Germany
| • |
PH is a rare disease.
There is little information about the
frequency and prognosis of the disease in Germany. |
| • |
At Heidelberg University the identification
of genetical causes and possible early forms of
the disease are currently investigated. |
| • |
At Heidelberg, a family screening
has been done for the first time. The Universities
of Heidelberg and Gießen initiated a European
Multicentric Study for the early diagnosis of PAH.
The study is supported by the Commission of the
EU. |
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