Chronisch thromboembolische pulmonale Erkrankung
摘要
Chronic thromboembolic pulmonary disease (CTEPD) occurs with (CTEPH) and without pulmonary hypertension and is a rare complication of acute or recurrent pulmonary embolism. Inadequate thrombolysis and contributing factors such as inflammation, infection or disorders of fibrinolysis lead to persistent obstruction of the pulmonary arteries. This subsequently leads to increased resistance in the pulmonary circulation and the development of pulmonary hypertension with right heart load, which can lead to right heart failure. The main symptom is progressive exertional dyspnea. The initial symptoms are often unspecific, making the diagnosis difficult. On average 14 months pass from the onset of symptoms to presentation of the patient at a specialized center. In addition to therapeutic anticoagulation three specific treatment modalities are currently available: pulmonary endarterectomy (PEA) is the treatment of choice, in addition, balloon pulmonary angioplasty (BPA) as an interventional approach as well as pharmacotherapy are available. The decision on which mechanical procedure is most suitable depends on the location of the vascular obstructions. Severe comorbidities require individual assessment but do not constitute absolute exclusion criteria. Approximately two thirds of CTEPH patients are suitable for surgery. The treatment decision is made by a multidisciplinary team, where individual multimodal treatment concepts are also discussed. All CTEPH patients should undergo long-term follow-up controls at a specialized PH center.