English Information | pulmonale hypertonie e.V.

Welcome!

on the Website of pulmonale hypertonie e.V.
Nonprofit self-help association

What is PH?

Pulmonary hypertension can be primary or associated with many conditions affecting lung and heart. The narrowing of the pulmonary blood vessels causes an increase of pulmonary artery pressure. This leads to a disturbance of the pulmonary circulation, a reduced gas exchange and an increasing strain on the right ventricle, culminating in right ventricular failure.

People with this condition experience chronic shortness of breath and limited exercise capacity. The disease can take a progressive course and lead to premature death. Pharmacological therapy is difficult and should better be performed in specialized centres for PH.

Several medical treatments are now available for severe pulmonary hypertension. At end stage the double lung transplantation will be a further option.

The first subtle symptoms of less advanced disease, like dyspnoea and fatigue, are often of a non-specific nature and therefore not even recognised by the patient himself.

In many cases, medical examination is not very conclusive as well, which leads to makeshift diagnoses like „lack of training“ or „psycho-vegetative exhaustion“. The causes of the disease are still unknown. It can be of hereditary nature and affect subsequent generations of a family. Therefore special attention has to be focused on first degree relatives of the patients.

Who are we, and what are our aims?
We are a group of PH-patients their families and friends who have founded the pulmonale hypertonie (ph) e.v., a foundation dedicated to help patients and their families to cope with their personal fate.

The foundation established an information service for patients and other interested people and an international co-operation with different institutions dealing with PH.
We want to provide informations on symptoms, diagnosis and therapy options and arrange contacts to specialised physicians. For this purpose, the association compiles literature on PH and gets it translated if it is needed.
Furthermore, the self-help group wants to contribute to medical research in this disease, together with physicians and supported by friends, members and sponsors. With the help of grants we would like to support promising scientific projects which otherwise could not be handled.

Excerpt of the statutes:

Who was René Baumgart?

René Baumgart was a young man who at the age of 19 years was diagnosed to suffer from „primary pulmonary hypertension“ and died of this insidious disease at 23 years of age.

René Baumgart was born in Aalen (Baden-Württemberg, Germany) in 1971. His mother died of primary pulmonary hypertension when he was 10 years old. He had learned the profession of a printer and was just about to pass his profession examination before he died.

His uncle, Bruno Kopp, affected by the disease as well, is the initiator of the foundation pulmonale hypertonie (ph) e.v., with the aim to improve self-help for patients and their families and to intensify scientific research in this field.

With the „René Baumgart – Stiftung“ the premature death of René Baumgart shall be given a significance.

The René Baumgart-Stiftung
was founded on March 31st 2001 by the association
pulmonale hypertonie (ph) e.v.

It is the aim of the foundation to support research
in the field of the disease Pulmonary Hypertension in
order to help children and adults .

Legal background of the self-help group
ph e.v. was founded on October 11th 1996. It is headquartered in Rheinstetten, district Karlsruhe, Baden-Württemberg, Germany.

The group is registered at the court district in Karlsruhe.

The tax and revenue office Ettlingen has acknowledged the non-profit character of the association. Therefore contributions and donations are tax privileged.

The self-help group acts on a selfless and non-profit-minded basis. The management committee functions are honorary.

Donations are requested on

Bank account:
Account No. 8024596
Spar- und Kreditbank Rheinstetten eG
Bank code 660 614 07
BIC: GENODE61RH2
IBAN: DE09 6606 1407 0008 0245 96

Account holder:

pulmonale hypertonie (ph) e.v.

Patronage:
Social Minister a. D.
Dr. Erwin Vetter

Registered office:
Rheinaustr. 94
76287 Rheinstetten
GERMANY

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.

Therapy:

Several medical treatments are now available for severe pulmonary hypertension.

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.

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.