• Pulmonary Hypertension Association


What is pulmonary hypertension?

What is pulmonary hypertension?

Pulmonary hypertension is increased blood pressure in the pulmonary arteries – blood vessels that convey venous blood from the right heart ventricle to the lungs. The higher the pressure of the pulmonary arteries, the harder it is for the heart to distribute blood to the body. Inadequate flow of oxygenated blood to the body causes lung blood vessels to narrow, that can also lead to thrombosis.

Pulmonary hypertension classification


Classification is based on pathogenetic mechanism and divides pulmonary hypertension into five categories:


Group 1: This group includes disorders in which the pulmonary hypertension is associated with abnormalities in the small branches of the pulmonary artery (PAH). It is the rarest form and the one of the fastest progression. Every fourth PH patient in Latvia is diagnosed with this form. Mortality in patients with PH can be even higher than in patients with some forms of cancer, including breast and colorectal cancer.

Group 2: This group includes pulmonary hypertension caused by left-sided heart disease.

Group 3: This group includes pulmonary hypertension due to lung problems.

Group 4: This group includes pulmonary hypertension due to pulmonary embolus

Group 5: This group includes pulmonary hypertension due to other miscellaneous causes, which do not fit into the other four categories.


Pulmonary arterial hypertension

Pulmonary arterial hypertension is characterized by an increased pulmonary vascular resistance. As a result, right heart overload and failure occur. Although PAH is the rarest type of pulmonary hypertension, there is worldwide approved and available specific pathogenetical therapy only for this group. It is estimated that the prevalence of PAH in the developed countries is 50 cases per million. In 2010 the prevalence of PAH in Latvia was 13.9 cases per million. To improve the quality of life and lifespan it is essential to carry out early, precise and advanced PAH patient diagnostics.

PAH symptoms

Symptoms of the disease are nonspecific and, initially, are often confused with other cardiac, pulmonary or haematological conditions. The most common symptoms are shortness of breath, chronic fatigue, chest pain, palpitations, fainting, lips cyanosis (bluish tinge). Due to nonspecific symptoms the diagnostic is complicated and often delayed.

 Who suffers from PAH?


People who suffer from congenital heart diseases, systemic scleroderma or other systemic diseases, portal hypertension, HIV infection are at higher risk of PAH. The disease can also be genetically inherited or passed on in the family. Sometimes the disease can develop due to long-term use of certain medication or because of appetite suppressant medication. PAH can be contracted at any age – in both children and adults, but more often occurs in young women of childbearing age (20 – 40 years). Due to late detection of the disease in Latvia, the mean age of patients is about 50 years.

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