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Pulmonary hypertension

Chronic thromboembolic pulmonary hypertension (CTEPH)

Over half of patients suffering from chronic thromboembolic pulmonary hypertension (CTEPH) are known to have suffered a previous pulmonary embolism (spread of a blood clot into the pulmonary artery) after deep vein thrombosis.

After an acute pulmonary embolism, the blood clot normally dissolves within four weeks, either spontaneously or with conservative treatment. With CTEPH patients, the blood clot does not completely dissolve. Scarred changes occur in the innermost layer of the vessel wall, which narrow the vessel lumen and thus increase blood pressure in the pulmonary vessels. It is assumed that at least 4% of all patients who suffer an acute pulmonary embolism later develop CTEPH. With the other CTEPH patients, there is no evidence of prior pulmonary embolism in the previous history. Here we assume that the pulmonary vessels themselves are diseased and as a result numerous small blood clots form in the pulmonary arteries. The increase in pulmonary artery blood pressure leads to increasing strain on the right side of the heart. The limited lung perfusion reduces the supply of oxygen in the blood. Both aspects lead to organ damage as the disease progresses. The key symptom for affected patients is increasing dyspnoea during exercise.

Patients are frequently examined for structural lung diseases and diseases of the left side of the heart. The diagnosis of CTEPH is then usually not made until the disease has progressed. CTEPH is the only disease where it is possible to operate and clean out the pulmonary arteries by performing a pulmonary endarterectomy. In many cases, this cures the disease. About 4000 of these operations have so far been performed around the world. In Germany, about 60 to 70 of these cases are operated on per year, and our department treats about 50 of these patients annually. To diagnose CTEPH and assess the operability, a series of tests is essential:

• Ventilation/perfusion scintigraphy (indicates vascular occlusion
• Echocardiography (shows strain on the right side of the heart and conclusive evidence of pulmonary hypertension)
• Right heart catheterisation (for precise measurement of the blood pressure in the pulmonary vessels and determining the pulmonary vascular resistance and cardiac output)
• Pulmonary angiography (the gold standard; shows the changes to the pulmonary vessels and gives the best overall view for the planned operation
• CT/MRI of the chest (shows the changes within the pulmonary vessels and the right side of the heart)
• 6-minute walking test, spiroergometry (show the patient’s preserved performance capacity)
• Possibly coronary angiography (for patients over 45 years of age, to assess the coronary vessels

If CTEPH is diagnosed, the operability of the patient should be thoroughly investigated. The results of the examinations are assessed by an experienced team of experts consisting of surgeons, pneumologists, radiologists and cardiologists. The operation is on principle performed under general anaesthetic using a heart-lung machine. The endarterectomy itself is performed under circulatory arrest, so the patient's body is cooled to 18 °C.

For the sake of safety, pacemaker wires and drainage are inserted and usually removed on the fifth day after the operation. After the operation, the patient is taken to the intensive care ward and is ventilated there until the next day. After the patient wakes up, he usually remains on the intensive care ward for a few more days while physiotherapy is commenced. After the pacemaker cable and the drainage are removed, the patient is finally moved to the normal ward. Lifelong marcumarisation (‘blood thinning’) is commenced with a target INR of 2.5-3.5 (Quick’s value 20-25%).

After a total of about two weeks, the patient is discharged home again.

The average mortality rate for the operation described is about 6% at specialist centres. At our hospital the overall mortality risk is less than 5%, although the risk for the individual patient can vary. Every patient gets a risk assessment before the operation.

If the operation is successful and marcumarisation is consistent, the PEA is a curative therapy. By contrast, medical treatment of the CTEPH can only delay the progress of the disease and can neither stop it nor cure it.

We recommend and are happy to organise for each patient subsequent rehabilitation measures in specialised clinics.

We admit our patients again after a year for a follow-up check. Follow-up care is currently undertaken by an internist specialising in pulmonary hypertension.

We recommend the involvement of a specialist outpatients department.