Background and aim: To assess the safety and efficacy of a refined balloon pulmonary angioplasty (BPA) strategy in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: There were 157 BPA sessions performed in 56 CTEPH patients (47 non-operable, nine after pulmonary endarterectomy; aged 58.6 ± 17.9 years; 28 females) with severely impaired pulmonary haemodynamics (mean pulmonary artery pressure [mPAP]: 51.3 ± 12.2 mm Hg, pulmonary vascular resistance [PVR]: 10.1 ± 3.9 Wood Units). The first 50 sessions aimed to recanalise chronic occlusions and prevent reocclusion with aggressive anticoagulation. The next 107 sessions aimed to relieve “web” and “ring” lesions using reduced tip load guidewires and less intensive anticoagulation. Results: There was significant reduction in haemoptysis (22% vs. 7%, p = 0.01), vessel injury (30% vs. 13%, p = 0.01), and reperfusion pulmonary injuries (22% vs. 4%, p = 0.01) after changing the BPA strategy. Mortality at 14 days was also reduced (6% vs. 0%; p = 0.05). The cumulative survival rate was 94.6% at 24 months after the first BPA, which was more favourable than medically treated historic controls. In the 31 patients with > 3 BPA sessions, there was significant reduction of PVR (10.3 ± 3.7 vs. 5.9 ± 2.8 Wood Units; p = 0.01), mPAP (50.7 ± 10.8 vs. 35.6 ± 9.3 mm Hg; p = 0.01) and improvement in World Health Organisation functional class (3.19 ± 0.48 vs. 1.97 ± 0.80; p < 0.001). Conclusions: Balloon pulmonary angioplasty improves haemodynamics and outcome but requires refined strategy to limit early complication rate. Background and aim: To assess the safety and efficacy of a refined balloon pulmonary angioplasty (BPA) strategy in patients with chronic thromboembolic pulmonary hypertension (CTEPH). Methods: There were 157 BPA sessions performed in 56 CTEPH patients (47 non-operable, nine after pulmonary endarterectomy; aged 58.6 ± 17.9 years; 28 females) with severely impaired pulmonary haemodynamics (mean pulmonary artery pressure [mPAP]: 51.3 ± 12.2 mm Hg, pulmonary vascular resistance [PVR]: 10.1 ± 3.9 Wood Units). The first 50 sessions aimed to recanalise chronic occlusions and prevent reocclusion with aggressive anticoagulation. The next 107 sessions aimed to relieve “web” and “ring” lesions using reduced tip load guidewires and less intensive anticoagulation. Results: There was significant reduction in haemoptysis (22% vs. 7%, p = 0.01), vessel injury (30% vs. 13%, p = 0.01), and reperfusion pulmonary injuries (22% vs. 4%, p = 0.01) after changing the BPA strategy. Mortality at 14 days was also reduced (6% vs. 0%; p = 0.05). The cumulative survival rate was 94.6% at 24 months after the first BPA, which was more favourable than medically treated historic controls. In the 31 patients with > 3 BPA sessions, there was significant reduction of PVR (10.3 ± 3.7 vs. 5.9 ± 2.8 Wood Units; p = 0.01), mPAP (50.7 ± 10.8 vs. 35.6 ± 9.3 mm Hg; p = 0.01) and improvement in World Health Organisation functional class (3.19 ± 0.48 vs. 1.97 ± 0.80; p < 0.001). Conclusions: Balloon pulmonary angioplasty improves haemodynamics and outcome but requires refined strategy to limit early complication rate.

Chronic thromboembolic pulmonary hypertension (CTEPH) is an ominous disease leading to progressive right heart failure. Selected patients can be treated by pulmonary endarterectomy (PEA). We assessed long-term clinical outcome of patients with CTEPH who underwent PEA and patients who remained on medical treatment alone. A total of 112 consecutive patients with CTEPH referred between 1998 and 2008 to one center were followed for a mean of 35 (range 0-128) months after diagnosis. All the patients had advanced pulmonary hypertension at baseline. The operated group had higher World Health Organization functional class compared to the nonoperated group. No other differences in hemodynamic, echocardiographic, or biochemical parameters were observed at baseline. Despite the perioperative mortality rate of 9.1%, patients who underwent PEA had significantly lower long-term mortality compared to nonoperated patients (12.7% vs 34.8%; P = .003), and PEA survivors showed sustained clinical improvement. All efforts should be undertaken to perform PEA in all patients with operable CTEPH.

The incidence of COVID-19 infection in patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) is similar to that in the general population, but the mortality rate is much higher. COVID-19 vaccination is strongly recommended for PAH/CTEPH patients. The aim of our cross-sectional study was to identify reasons why PAH/CTEPH patients refused vaccination against COVID-19. Moreover, we assessed the safety profile of approved COVID-19 vaccines in PAH/CTEPH patients. We examined 261 patients (164 PAH patients and 97CTEPH patients) with a median age of 60 (18–92) years, 62% of which were female. Sixty-one patients (23%) refused to be vaccinated. The main reason for unwillingness to be vaccinated was anxiety about adverse events (AEs, 61%). Age and fear of COVID-19 in the univariate analysis and age ≥60 years in the multivariate regression analysis were factors that impacted willingness to be vaccinated (OR = 2.5; p = 0.005). AEs were reported in 61% of vaccinated patients after the first dose and in 40.5% after the second dose (p = 0.01). The most common reported AEs were pain at the injection site (54.5%), fever (22%), fatigue (21%), myalgia (10.5%), and headache (10%). A lower percentage of AEs was reported in older patients (OR = 0.3; p = 0.001). The COVID-19 vaccines are safe for PAH/CTEPH patients. The results obtained in this study may encourage patients of these rare but severe cardio-pulmonary diseases to get vaccinated against COVID-19.