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Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Symptoms & Risk Factors

Chronic thromboembolic pulmonary hypertension (CTEPH) is high blood pressure in the lungs. This happens when the blood vessels in the lungs are blocked by a clot that prevents normal blood flow and causes pressure in the vessels to build. Normally, CTEPH occurs after a pulmonary embolism (PE) — a sudden blockage in one of the pulmonary arteries in the lung.


Many people who develop CTEPH do not experience symptoms in the early stages of the disease. When symptoms start to develop, they can include:
  • Shortness of breath during exercise
  • Chest pain/discomfort after exertion
  • Fatigue
Later stage symptoms include:
  • Feeling faint or fainting after exertion
  • Swelling of the legs or abdomen
  • Bluish tint to extremities (fingers and toes)
Severe Cases include:
  • Fainting
  • Breathlessness with moderate activity
  • Coughing up blood
  • Right heart ventricle failure

Risk Factors

The following conditions can lead to a pulmonary embolism:
  • Being sedentary for long periods of time (i.e. a prolonged hospitalization/illness or air/car travel)
  • Taking birth control pills or hormone replacement therapy
  • Pregnancy
  • Smoking
  • Obesity
  • Cancer
  • Minority of people who take blood thinners are able to clear PEs, but could still develop CTEPH for unclear reasons
Other risk factors:
  • Infected surgical cardiac shunts, pacemakers or defibrillator leads
  • Chronic inflammatory disorders
  • Atrial fibrillation or other conditions that cause irregular heartbeat
  • Diabetes
  • Thyroid replacement therapy
  • Having your spleen removed
  • High risk of blood clots (i.e. deep vein thrombosis or peripheral artery disease)
  • Dehydration
  • Organ transplantation or implanted devices
  • Genetic factors