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Lung diseases

Pulmonary arterial hypertension (PAH)

A disease in which blood pressure rises in the blood vessels of the lungs. The right side of the heart is put under strain. Shortness of breath on exertion and fainting are the most common symptoms.

Not part of the panel of Türkiye's National Newborn Screening Programme (heel-prick test). Source

Orphanet has a guideline for emergency doctors for this disease (language: French). See on Orphanet

Prepare an emergency card

This page is translated from the Turkish original.

This disease is followed through the “Lung diseases” group.

Understanding the disease

This section has not yet been reviewed by a physician. It summarises patient information from sources such as MedlinePlus, Orphanet and the NHS in plain language. The disease can differ from person to person; talk to your doctor about diagnosis and treatment.

What happens in this disease?

The right side of the heart pumps blood to the lungs. In this disease, the small arteries in the lungs become narrow and stiff. It becomes harder for blood to pass, and the pressure inside the vessels rises. The right side of the heart works harder and can become tired over time.

Which symptoms can occur?

Symptoms usually begin slowly. They get worse with exertion.

  • Shortness of breath on exertion (common)
  • Fainting or dizziness (common)
  • Tiredness
  • Chest pain
  • A fast or pounding heartbeat
  • Swelling in the ankles, legs or abdomen

How is it diagnosed?

The symptoms are similar to those of other heart and lung diseases. Because of this, the diagnosis can take time. An ultrasound of the heart is done first. The diagnosis is confirmed by measuring the pressure with a right heart catheterization. Lung tests, a lung scan and blood tests help to rule out other causes.

Treatment and care

The disease does not go away completely, but treatment reduces the symptoms. There are approved medicines specific to this disease that relax the blood vessels of the lungs. These medicines can be taken by mouth, into a vein or by inhaling. Medicines that prevent blood clots, water tablets and oxygen at home may also be used. In severe cases, a lung transplant may be considered. Follow-up is done at specialist centers. The doctor who follows the disease decides which treatment is suitable.

For the family: inheritance and genetic counselling

Most people have no other affected family members. In some families, the disease is inherited. The BMPR2 gene is most often responsible. Inheritance is mostly dominant. This means that the chance of passing it from an affected parent to a child is 1 in 2 in each pregnancy. But not everyone who carries the gene becomes ill. If there are other affected family members, genetic counselling is recommended.

Source: MedlinePlus Genetics: Pulmonary arterial hypertension · NHS: Pulmonary hypertension – Diagnosis · NHS: Pulmonary hypertension – Treatment · Orphanet: Pulmonary arterial hypertension (ORPHA:182090)

Key facts

How common is it?
Prevalence: 1-9 per 100 000 (Europe)
When does it usually start?
Any age

These facts are taken from Orphanet and MedlinePlus records (last checked: October 7, 2026). Frequency values are approximate ranges.

You are not alone: patient associations in Türkiye

Non-profit patient and family organizations in Türkiye working on this disease or disease group. Links are for information; Doktorclub is not affiliated with these organizations and does not endorse them.

For all rare diseases: Nadir Hastalıklar Federasyonu

Links checked on October 8, 2026.

Guides related to this disease

  • Home care and medical devices · Home health service, oxygen and respiratory device reports, wheelchairs and orthoses, power outage notification, emergency plan.

Support and help

Doktorclub does not diagnose, recommend treatments or refer you to hospitals or doctors. These pages are meant to help you talk with your doctor.

News about PAH

Clinical trials (ClinicalTrials.gov)

A clinical trial is a regulated study that tests whether a new treatment is safe and whether it works, in patients who volunteer. Most trials look for a certain age, genetic change and health condition. Taking part is voluntary.

This list is not a treatment recommendation. Treatments tested in phase 1–3 trials are investigational (experimental): their efficacy and safety have not yet been proven and they are not approved treatments. Taking part depends on each trial’s criteria and a physician’s assessment; the first step is to talk to the doctor who manages your care.

Questions you can ask your doctor

  • Could this trial be suitable for us?
  • If we take part, how would current treatment and check-ups be affected?
  • Where does the trial take place, and how are travel and accommodation handled?
  • What happens if we want to leave the trial?

ClinicalTrials.gov lists 96 active interventional studies for this disease. 52 are recruiting now; 4 have sites in Türkiye. Last checked: October 7, 2026.

Selected records (studies with a site in Türkiye first):

See all studies on ClinicalTrials.gov · Joining clinical trials

Lung diseases: other news

Medical codes (for talking with your doctor and finding sources)

These codes identify the disease in international classifications and databases. They can help when you talk with your doctor or look for reliable sources.

Orphanet code: ORPHA:182090 · ICD-11 BB01.0

Codes checked against WHO ICD-11 and Orphanet on October 7, 2026.

This page provides general information and does not replace diagnosis or treatment. Talk to your doctor about symptoms, diagnosis and treatment.

The information on these pages is for general information only and does not replace medical advice. Talk to your doctor about diagnosis and treatment decisions. In an emergency in Türkiye, call 112.

Every update is based on an open source and labeled with its level of evidence. No advertising and no drug brand names. Our scientific principles

This page was translated from Turkish by the Doktorclub editorial team.