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Lung diseases

Chronic thromboembolic pulmonary hypertension (CTEPH)

A disease in which old blood clots in the lung vessels cause permanent narrowing. Blood pressure in the lung vessels rises. The right side of the heart is put under strain.

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

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?

A clot that travels to a blood vessel in the lungs is called a pulmonary embolism. Sometimes this clot does not fully dissolve. The hard tissue left behind narrows or blocks the vessel. The pressure in the lung vessels rises. The right side of the heart works harder and can become tired over time.

Which symptoms can occur?

Symptoms begin slowly and get worse with exertion.

  • Shortness of breath on exertion (common)
  • Tiredness
  • Dizziness or feeling faint
  • Chest pain
  • Palpitations
  • Swelling in the legs, ankles or abdomen

How is it diagnosed?

An ultrasound of the heart is done first. A lung scintigraphy, which is a scan that shows air and blood flow, looks for clots. The diagnosis is confirmed by measuring the pressure with a right heart catheterization.

Treatment and care

A blood-thinning medicine is used to prevent new clots. In suitable people, the old clots are removed with surgery. If surgery is not suitable, the vessels can be opened with a balloon from inside. There are also approved medicines for this disease that relax the blood vessels. If needed, oxygen and water tablets are added. Care is provided at specialist centers. The doctor who follows the disease decides which treatment is suitable.

For the family: inheritance and genetic counselling

This disease is not inherited. It develops after a clot that has traveled to the lungs. Sometimes the clot may have gone unnoticed. If there is a tendency to blood clots in the family, it is helpful to talk about this with a doctor.

Source: NHS: Pulmonary hypertension – Causes · NHS: Pulmonary hypertension – Diagnosis · NHS: Pulmonary hypertension – Treatment · Orphanet: Chronic thromboembolic pulmonary hypertension (ORPHA:70591)

Key facts

How common is it?
New cases per year: 1-9 per 100 000 (Europe)
How is it inherited?
Not inherited
When does it usually start?
Adolescence, Adulthood, Older age
Reliable sources
Orphanet

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 CTEPH

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 9 active interventional studies for this disease. 6 are recruiting now; none has a site 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:70591 · ICD-11 BB01.3

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.