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Thromboembolic Pulmonary Hypertension

CTEPH, or chronic thromboembolic pulmonary hypertension, is a rare form of pulmonary hypertension that may result from an unresolved pulmonary embolism.

Hypertension: elevated pressure in arteries
Pulmonary: related to the lungs
Thromboembolism: formation of a blood clot that can migrate and cause an embolism
Chronic: said of a disease that has been present for some time

CTEPH can develop in one out of 25 people who have had a pulmonary embolism (even in those treated for at least three months with an anticoagulant).

Cause

Acute pulmonary embolism (PE; blood clot in the lungs) is the main factor that can cause chronic thromboembolic pulmonary hypertension (CTEPH). The most common form of pulmonary embolism is caused by a clot that travels to the lungs from the lower extremities, also called deep vein thrombus.  This clot forces the heart to work harder to pump blood, which can lead to an enlargement of the heart.

People at risk

Even if pulmonary embolism (PE) is considered the main cause of chronic thromboembolic pulmonary hypertension (CTEPH), many people with CTEPH do not have a medical history of PE or do not know that they have suffered from a PE in the past. Here are some other factors that increase the risk of CTEPH:

  • Pulmonary embolism (PE) or several episodes of PE (main risk factor)
  • Cancer
  • Splenectomy
  • Ventriculoatrial shunt
  • Chronic inflammatory disease
  • Hyperthyroidism
  • Hypercoagulable disorders
  • High blood clot risk
  • Genetic factors

Signs and symptoms

Symptoms of CTEPH can occur six months to two years after an acute pulmonary embolism. CTEPH can cause the following:

  • Exercise-induced shortness of breath
  • Chest pain
  • Swelling of the ankles, arms and abdomen
  • Loss of consciousness
  • Coughing up blood
  • Fatigue

A deterioration or a change in usual symptoms should always be reported to your treating doctor.

Diagnosis

Chronic thromboembolic pulmonary hypertension (CTEPH) can be difficult to diagnose, because its symptoms are similar to those of other disorders, such as asthma, chronic obstructive pulmonary disease (COPD), heart failure and acute PE. Therefore, when CTEPH or another form of pulmonary hypertension is suspected, the physician performs various tests to guide and clarify the diagnosis, including the following:

  • Screening echocardiogram
  • Right heart catheterization (RHC)
  • Ventilation-perfusion scintigraphy (V/Q) (standard screening method for CTEPH)
  • Pulmonary angiography (to confirm the diagnosis of chronic thromboembolic disease and assess operability)

To confirm the diagnosis of HPTEC, the patient will have to be referred to one of the two pulmonary hypertension clinics in the province of Quebec.

Treatment

Chronic thromboembolic pulmonary hypertension (CTEPH) can be cured with a surgical intervention called pulmonary endarterectomy (PEA). If you receive a diagnosis of CTEPH, the members of your healthcare team will determine if you are a candidate for PEA. Although PEA is the recommended treatment for CTEPH, it is not suitable for everyone.

This surgical intervention does not always cure CTEPH. In fact, CTEPH can recur in about one-third of patients who have undergone PEA. This is called recurrent CTEPH. Other treatments may be offered to such persons as well as to those who are not eligible for a PEA. In addition to treatments that specifically target CTEPH, patients with CTEPH also often receive anticoagulants.

Regular follow-ups are recommended with your pulmonary hypertension clinic.

Certains symptômes d’HPTEC peuvent être présents même après une endartériectomie pulmonaire (EAP). De plus, l’EAP ne guérit pas toujours l’HPTEC. En effet, au cours de plusieurs études, l’HPTEC est de nouveau survenue chez environ un patient sur trois ayant subi une EAP. C’est ce qu’on appelle l’HPTEC récurrente.

En plus des traitements qui ciblent spécifiquement l’HPTEC, les patients atteints d’HPTEC sont aussi souvent traités par des anticoagulants. Ceux-ci sont prescrits pour prévenir la formation de caillots dans la circulation sanguine.

Did you know that

The Quebec Lung Association offers direct services to the population. For more information, visit our Patient Resources section.