The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Chronic Thromboembolic Pulmonary Hypertension (CTEPH)

A potentially curable cause of pulmonary hypertension, classified as Group 4.

Written by: Saygı Hospital Health Guide Editorial Board
Published:

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

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

What is Chronic Thromboembolic Pulmonary Hypertension (CTEPH)?

Chronic thromboembolic pulmonary hypertension develops when residual thromboemboli organize into fibrotic vascular lesions and unaffected vascular beds undergo small-vessel arteriopathy similar to PAH. Approximately 2–4% of patients with acute pulmonary embolism develop CTEPH within 2 years.

CTEPH is classified as Group 4 pulmonary hypertension. Diagnostic criteria require mean pulmonary arterial pressure >20 mmHg and pulmonary capillary wedge pressure ≤15 mmHg, plus mismatched perfusion defects on V/Q scan and confirmation by CT pulmonary angiography or conventional pulmonary angiography after at least 3 months of effective anticoagulation.

It is the only form of pulmonary hypertension potentially curable by surgery (pulmonary endarterectomy, PEA), with balloon pulmonary angioplasty (BPA) and medical therapy as alternatives for inoperable disease.

Symptoms

Progressive exertional dyspnea (most common)
Reduced exercise tolerance, fatigue
Exertional chest pain or syncope
Hemoptysis (occasionally)
Symptoms and signs of right heart failure: peripheral edema, ascites, jugular venous distension
Persistent dyspnea after a documented pulmonary embolism (red flag)

Risk Factors

Prior acute pulmonary embolism (especially recurrent or unprovoked)
Antiphospholipid syndrome and lupus anticoagulant
Splenectomy
Ventriculoatrial shunt or chronic infected venous catheter
Inflammatory bowel disease, osteomyelitis, malignancy
Thyroid replacement therapy, oral contraceptives, hormone replacement
Inherited thrombophilia (factor V Leiden, prothrombin gene mutation, protein C/S deficiency)

When to See a Doctor?

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

  • Persistent dyspnea or impaired exercise tolerance more than 3 months after acute pulmonary embolism
  • Unexplained progressive dyspnea with abnormal echocardiogram suggesting right heart strain
  • Patients with high CTEPH risk (recurrent VTE, antiphospholipid syndrome) developing new respiratory symptoms
  • Confirmed pulmonary hypertension after pulmonary embolism awaiting CTEPH workup

Treatment Methods

01
Lifelong therapeutic anticoagulation (vitamin K antagonists or DOACs)
02
Pulmonary endarterectomy (PEA) — gold standard, potentially curative for operable proximal disease
03
Balloon pulmonary angioplasty (BPA) for inoperable distal disease, persistent PH after PEA, or as adjunct
04
Medical therapy for inoperable or residual CTEPH: riociguat (sGC stimulator) is first-line; macitentan and treprostinil have evidence
05
Diuretics, supplemental oxygen, exercise rehabilitation
06
Multidisciplinary CTEPH center evaluation for treatment planning
07
Treatment of underlying conditions (antiphospholipid syndrome, thrombophilia)
08
Right heart catheterization follow-up to assess hemodynamic response

Which Department to Visit?

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

Learn About Göğüs Hastalıkları Department

Let us help you

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

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Asthma

Göğüs Hastalıkları

Asthma is characterized by wheezing, coughing and shortness of breath attacks; with proper treatment it can be kept under control.

COPD (Chronic Obstructive Pulmonary Disease)

Göğüs Hastalıkları

COPD is an irreversible lung disease characterized by shortness of breath and chronic cough; quitting smoking slows its progression.

Pneumonia

Göğüs Hastalıkları

Pneumonia presents with high fever, cough and shortness of breath; the vast majority recover with appropriate antibiotic treatment.

Tuberculosis (TB)

Göğüs Hastalıkları

Tuberculosis presents with weeks-to-months of cough, fever, and night sweats; early diagnosis and treatment lead to full recovery.

Pleural Effusion

Göğüs Hastalıkları

Pleural effusion is the accumulation of excess fluid in the pleural space, resulting from imbalances in fluid production and removal, and represents a manifestation of diverse cardiopulmonary, infectious, and malignant disorders.

Pneumothorax

Göğüs Hastalıkları

Pneumothorax is the presence of air in the pleural space resulting in partial or complete lung collapse, classified as spontaneous (primary/secondary), traumatic, or iatrogenic, with tension pneumothorax representing a life-threatening emergency.

Bronchitis (Acute and Chronic)

Göğüs Hastalıkları

Acute bronchitis is mostly viral and resolves spontaneously, while chronic bronchitis is a smoking-related component of COPD.

Bronchiectasis

Göğüs Hastalıkları

Bronchiectasis is a chronic respiratory disease characterized by permanent, abnormal dilation of bronchi with associated destruction of muscular and elastic components of airway walls, resulting in impaired mucociliary clearance and recurrent infection.

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.