Severe CTEPH leads to an impaired physical capacity and a restricted quality of life and poor prognosis.

Pulmonary endarterectomy represents the best choice as therapy, when the thrombi are located in the central pulmonary vessels and therefore can be operated. By this operation the pulmonary artery pressure can be normalised and the patients' survival improved. Up to now, after successful endarterectomy patients only receive anticoagulation.

Despite operation many patients remain symptomatic and are restricted in their physical capacity. Therefore a hypothesis of this project is that most of the patients, even after successful operation, show peripheral vascular remodelling with a ventilation-perfusion mismatch and elevated pulmonary pressure during exercise.

In this study we aim to analyse how many patients with CTEPH after endarterectomy show elevated pulmonary artery pressures at rest or during exercise and are limited in their physical capacity, hemodynamics, oxygen uptake and quality of life and need further therapy.

Another aim is to examine whether exercise and respiratory therapy may improve the patients postoperatively.

Therefore 30 patients with CTEPH > six months after endarterectomy, with ongoing restricted exercise capacity shall be included. After baseline examination in the University hospital Heidelberg the patients receive exercise and respiratory therapy for three weeks. The patients will receive further examinations at the end of rehabilitation after 3 weeks and after 15 weeks. All examinations include medical history, family history, physical examination, ECG and echocardiography at rest and during exercise, cardiopulmonary exercise testing, assessment of the respiratory muscle strength, the SF-36 questionnaire for quality of life, laboratory testing and MRI.

Rehabilitation will be conducted in the clinic for rehabilitation Koenigstuhl, Heidelberg. Participants will be randomised into two groups, a control group receiving a conventional therapy for three weeks, in which physical exertion is to be avoided and a training group with additional exercise and respiratory therapy.

Inclusion Criteria:

• A Screening

  1. Informed consent
  2. Men and women 18 - 75 years
  3. Patients ≥ 6 months after endarterectomy because of chronic thromboembolic pulmonary hypertension (CTEPH)

• B Training

See A + all patients who showed a restricted physical capacity in the screening:

• Latent pulmonary hypertension
• Restricted physical capacity

Exclusion Criteria:

1. Pregnancy or lactation
2. Change in medication during the last 2 months
3. Patients with signs of right heart decompensation
4. Disease which affects the gait
5. Unclear diagnosis
6. Acute illness, infection, fever
7. Severe lung diseases with FEV1 <50% and TLC< 70% of reference