What is PAH?

In patients with Pulmonary Arterial Hypertension (the most rare form
of PH)* characteristic changes occur within the pulmonary circulation, which include thickening of the linings and obstruction of the small pulmonary blood vessels. They are both structurally and functionally abnormal. In severe cases up to 80% of these very small blood vessels are rendered non-functional. As a result the pressure in the pulmonary circulation rises well above normal and this places strain on the right side of the heart. This strain can cause the heart to enlarge and the patient may develop heart failure. This is a disease that can affect all ages and is more commonly seen in females. Pulmonary Arterial Hypertension has an estimated prevalence of about 30-50 per million population.

* Class 1 WHO diagnostic classification

(a) Cross-section of normal
pulmonary artery

(b) Reduced opening of pulmo-
nary vessels due to cell
proliferation and remodelling
in advanced pulmonary

(c) Longitudinal section with
pathological changes within
the vessels (proliferation,
deposition of blood clots,
thickenig) causing difficul
ties for the heart to pump
blood through the lungs



The future for Pulmonary Arterial Hypertension

While a cure for this aggressive and life threatening disease is still some way off, there is much to be optimistic about. There are an ever increasing number of therapies available for the effective treatment
for Pulmonary Arterial Hypertension, which improve the quality and length of life. The aim for the present should be to ensure that all
patients with Pulmonary Arterial Hypertension have access to centres
of excellence in the diagnosis, management and ongoing treatment
of this disease.

Last updated: March 14, 2012


Giuliana, Italy
I wish for the tulips I planted yesterday to flower and that I’ll be so busy planning my wedding that I’ll forget about PH!

Giuseppe, Switzerland
Healthy people have a lot of dreams. Sick people have only one.

Radu, Romania
I dream to still be around to see my 18 year old son become an architect.

Nina, Switzerland
I want to be cured and have a pony.

Tuulia, Finland
I hope to live a good life in spite of illness and that one day there will be a cure.

Jennifer, USA
I hope to feel better and to train to be a nurse.

Jana, Slovakia
I am on a waiting list for lung transplantation and hope that the operation will take place in the next 6 months.

Marion, France
I dream to be able to dance and party ! Walk in the mountains… and swim !

Patrik, Sweden
My dream is that a cure will be found for my son and for everybody else who suffers from PH.