Patients with chronic thromboembolic pulmonary hypertension: a long term follow-up study
█ Original article
Mladoniczky Sára1, Balla Patrícia1, Szegedi Margit1, Takács Edit1, Piróth Zsolt1, Németh József1, Ablonczy László2, Temesvári András1, Andréka Péter1, Bálint Olga Hajnalka1
Gottsegen György Országos Kardiológiai Intézet,
1Felnőtt Kardiológiai Osztály,
2Gyermekszív Központ, Budapest
Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a thrombotic pulmonary disease associated with pulmonary vasculopathy. Pulmonary endarterectomy (surgery, PEA) is the first choice of treatment in CTEPH. Specific PAH medication is required when there is a contraindication for surgery or residual pulmonary hypertension (rPH) occurs. In the presence of PAH balloon pulmonary angioplasty (BPA) may also be recommended.
Objective: We investigated the long term outcome of our CTEPH patients.
Methods: CTEPH patients from our institution were retrospectively analyzed (data between 2003 and 2018). Baseline (clinical and right heart catheterization values), treatment and outcome data were documented. We compared outcome and mortality data in patients with and without surgery (PEA vs. non-PEA group). The clinical outcome was compared based on the following data analysis: NYHA class, 6 minutes walking distance (6MWD) and NT-proBNP.
Results: Of 29 CTEPH patients (mean age: 62±19 years, 52% male) 16 (55%) were proved to be eligible for PEA, but only 13 of them had a long term follow-up post-surgery (n=3 perioperative death). 54% of the PEA patients fully recovered (n=7) and 46% (n=6) required specific PAH treatment in the postoperative period (n=1, in combination with BPA) due to rPH. In all patients from the non-PEA group (n=13) specific PAH treatment (n=2 in combination with BPA) was initiated. Patients from PEA and non-PEA group did not differ hemodynamically. In PEA group NYHA class and NT-proBNP (p<0,001, and p=0,046), and in non-PEA group NYHA class and 6MWD (p=0,012, and 0,006) showed significant improvement at the late follow-up. We found significant difference in survival at 1, 3 and 5 year follow-up, for PEA group 100-100-100% and non-PEA group 100-85-78% (p=0,013), respectively.
Conclusions: 55% of CTEPH patients were suitable for PEA. 54% of patients who survived the surgery fully recovered, and the remaining required PAH specific therapy for rPH and had a good long term outcome. Non-PEA patients improved functionally on the long term, but had worse survival rate.