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P2267CTEPH patients long term follow-up: results from a single center


 
 
 Chronic thromboembolic pulmonary hypertension (CTEPH) is a thrombotic pulmonary disease associated with pulmonary vasculopathy. Pulmonary endarterectomy (opus, PEA) is the first treatment choice in CTEPH, and specific PAH medication when there is a contraindication for surgery or residual pulmonary arterial hypertension (rPAH) occurs. In the presence of PAH balloon pulmonary angioplasty (BPA) might be also recommended if available.
 
 
 
 We investigated the long term outcome of our CTEPH patients.
 
 
 
 CTEPH from our institution retrospectively analyzed (data between 2003 and 2018). Baseline, treatment and outcome data were documented. We compared the outcome, together with mortality in those with and without surgery (PEA vs. non PEA group). NYHA class, 6 minutes walking distance (6MWD) and NT-proBNP were also reported during follow-up.
 
 
 
 Of 29 CTEPH patients (mean age was 62±19 years, 52% male) 16 (55%) were accepted for PEA, and further 12 of them had a long term follow-up post surgery (n=3 periop exit, n=1 waiting for surgery). Half of the PEA patients were cured (n=6) and the other half (n=6) required specific PAH treatment (n=1, in combination with BPA) for rPAH. All patients from the non-PEA group (n=13) were started on specific PAH treatment (n=1 in combination with BPA). Patients with or without PEA did not differ hemodynamically. At the late follow-up there was a significant improvement in PEA group for NYHA class and NT-proBNP (p<0,001, and p=0,046), and in non PEA group for NYHA class and the 6MWD (p=0,012, and 0,006). We found significant difference in mortality at 1,3,5 year (Kaplan-Meier survival analysis) follow-up, for PEA group 100%-100%-100% and non PEA group 100%-85%-78% (p=0,013), respectively.
 
 
 
 
 55% of CTEPH patients were suitable for PEA, and those who survived the surgery 50% were cured. Non PEA patients improved functionally on the long term, but had worse survival.
