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Chronic Thromboembolic Pulmonary Hypertension

Chronic pulmonary hypertension that develops after recurrent pulmonary embolism

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Hematoloji department. Book Appointment →

What is Chronic Thromboembolic Pulmonary Hypertension?

Chronic thromboembolic pulmonary hypertension (CTEPH) develops when thrombi from acute pulmonary embolism fail to fully resolve and become organized into fibrotic tissue, raising pulmonary vascular resistance over time. Roughly 2 to 4 percent of patients who survive an acute pulmonary embolism go on to develop CTEPH.

Diagnosis begins with ventilation-perfusion (V/Q) scintigraphy as the screening test; mismatched perfusion defects are characteristic. Pulmonary angiography and right heart catheterization confirm the diagnosis and assess surgical candidacy.

Pulmonary endarterectomy (PEA) is the only potentially curative treatment for CTEPH and should be performed in experienced centers. For patients who are not surgical candidates, riociguat (a soluble guanylate cyclase stimulator) and balloon pulmonary angioplasty (BPA) are alternatives. Lifelong anticoagulation is required for all patients.

Symptoms

Exertional dyspnea
Reduced exercise tolerance
Chest pain
Syncope (fainting)
Lower extremity edema
Hemoptysis (rare)

Risk Factors

Prior pulmonary embolism
Antiphospholipid syndrome
Post-splenectomy state
Ventriculoatrial shunt
Chronic inflammatory disease

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent dyspnea after a pulmonary embolism
  • Progressive decline in exercise capacity
  • Leg edema and signs of right heart failure

Treatment Methods

01
Pulmonary endarterectomy (PEA) as curative surgery
02
Riociguat (sGC stimulator) for inoperable disease
03
Balloon pulmonary angioplasty (BPA)
04
Lifelong anticoagulation
05
Oxygen therapy and diuretics
06
Pulmonary rehabilitation

Which Department to Visit?

You can visit our Hematoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Hematoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.