The impact of exercise training and physiotherapy on heart function and pulmonary circulation parameters in heart failure with preserved ejection fraction (HFpEF) patients is uncertain. Hence, we performed a systematic review of published trials studying physical training in HFpEF population, with a focus on exercise and physiotherapy effect on left ventricular (LV), right ventricular (RV) morphological, functional, and pulmonary circulation parameters. We searched Cochrane Library and MEDLINE/PubMed for trials that evaluated the effect of exercise training and/or physiotherapy in adult HFpEF patients (defined as LVEF ≥ 45%), including publications until March 2021. Our systematic review identified eighteen articles (n = 418 trained subjects, 4 to 52 weeks of training) and covered heterogeneous trials with various populations, designs, methodologies, and interventions. Five of twelve trials revealed a significant reduction of mitral E/e’ ratio after the training (− 1.2 to − 4.9). Seven studies examined left atrial volume index; three of them showed its decrease (− 3.7 to − 8 ml/m²). Findings were inconsistent regarding improvement of cardiac output, E/A ratio, and E wave DecT and uncertain for RV function and pulmonary hypertension parameters. For now, no reliable evidence about rehabilitation effect on HFpEF cardiac mechanisms is available. There are some hypotheses generating findings on potential positive effects to parameters of LV filling pressure (E/e’), left atrium size, cardiac output, and RV function. This encourages a broader and more complex assessment of parameters reflecting cardiac function in future HFpEF exercise training studies.

Background and Objectives: Pregnancy and delivery in patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH) carry a very high risk for maternal and foetal complications and are contraindicated according to the guidelines. In the last decades, when an available modern PAH-targeted medication therapy and a new management concept improved patients’ well-being and survival, some PAH-CHD females decided to conceive. Of note, despite advanced treatment and modern healthcare system possibilities, dealing with pregnancy in a diverse PAH-CHD population is still challenging. The study aimed to share our experience with PAH-CHD pregnancies and discuss the risk assessment and current management of these patients with the combination of two rare diseases. Materials and Methods: The retrospective search of pulmonary hypertension and adult CHD registries in our hospital was performed, selecting all patients with CHD and PAH who conceived pregnancy from 2013 to 2021. Baseline demographic, clinical, and functional characteristics and clinical outcomes were collected. Results: Thirteen pregnancies in eight patients with PAH-CHD resulted in seven live births, three miscarriages, and three terminations. Five women were diagnosed with Eisenmenger syndrome (ES) and three with residual PAH after CHD repair. Before pregnancy, half of them were in WHO functional class III. Seven (87.5%) patients received targeted PAH treatment with sildenafil during pregnancy. In addition, the two most severe cases were administered with iloprost during peripartum. Three ES patients delivered preterm by Caesarean section under general anaesthesia. No neonatal mortality was reported. Maternal complications were observed in half of our cases. One patient died 12 days after the delivery in another hospital due to deterioration of heart failure. Conclusions: On the basis of our clinical experience, we conclude that pregnancy and delivery carry a high risk for maternal complications and should be avoided in women with PAH-CHD. The individualised approach of multidisciplinary care and appropriate monitoring are mandatory in reducing the risk of adverse outcomes.

Background: Left heart failure (HF) is characterized by an elevation in left-sided filling pressures, causing symptoms of dyspnea, impairing exercise capacity, and leading to pulmonary venous congestion and secondary pulmonary hypertension (PH). There is an increased incidence of PH associated with left heart disease, particularly with heart failure with preserved ejection fraction (HFpEF-PH). Treatment possibilities in HFpEF-PH are non-specific and very limited, thus additional pharmacological and non-pharmacological therapeutic strategies are needed. Various types of exercise-based rehabilitation programs have been shown to improve exercise capacity and quality of life (QoL) of HF and PH patients. However, no study focused on exercise training in the population of HFpEF-PH. This study is designed to investigate whether a standardized low-intensity exercise and respiratory training program is safe and may improve exercise capacity, QoL, hemodynamics, diastolic function, and biomarkers in patients with HFpEF-PH. Methods: A total of 90 stable patients with HFpEF-PH (World Health Organization functional class II–IV) will be randomized (1:1) to receive a 15-week specialized low-intensity rehabilitation program, including exercise and respiratory therapy and mental gait training, with an in-hospital start, or standard care alone. The primary endpoint of the study is a change in 6-min walk test distance; secondary endpoints are changes in peak exercise oxygen uptake, QoL, echocardiographic parameters, prognostic biomarkers, and safety parameters. Discussion: To date, no study has investigated the safety and efficacy of exercising specifically in the HFpEF-PH population. We believe that a randomized controlled multicenter trial, which protocol we are sharing in this article, will add important knowledge about the potential utility of a specialized low-intensity exercise and respiratory training program for HFpEF-PH and will be valuable in finding optimal treatment strategies for these patients. Trial registration: ClinicalTrials.gov NCT05464238. July 19, 2022.

Background and Objectives: The aim of this study was to evaluate the effectiveness and safety of balloon pulmonary angioplasty (BPA) in patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) in the Vilnius Pulmonary Hypertension (PH) Referral Centre and to provide a comparative analysis with other centres. Materials and Methods: This study included all BPA procedures performed between 2019 and 2024 in a single tertiary centre. Invasive haemodynamic parameters and clinical variables were assessed at baseline; at the end of invasive treatment; and at the conclusion of follow-up, an average of 8.6 months after the last BPA. A literature review was also performed. Results: Twenty-six patients with inoperable CTEPH were enrolled. The mean age of the patients was 61.6 (40–80) years. Each patient underwent a mean of 3.84 (1–9) procedures. Follow-up data were available for 12 patients with an average of 6.08 (3–9) procedures. Mean pulmonary arterial pressure decreased by 32% (p < 0.001) and pulmonary vascular resistance by 41% (p = 0.001) at follow-up compared with the baseline measurements. There was also a significant 80% (p < 0.001) reduction in brain natriuretic peptide levels and a 30% (p = 0.04) increase in 6-min walk distance. The BPA procedures were generally safe in this low-volume centre setting, with only 17% of procedures having non-severe and non-fatal procedure-related complications. The most common complications included vessel dissection (10%), pulmonary vascular injury with haemoptysis (3%), and hyperperfusion pulmonary oedema (1%), which was successfully treated in all patients. Conclusions: The results of the present study demonstrate that the BPA procedure is an effective and safe treatment for individuals with inoperable CTEPH, being associated with significant improvements in hemodynamic parameters and functional capacity and a low risk of major complications in the low-volume tertiary PH centre setting.

Background and Objectives. COVID-19 infection has a significant burden on global morbidity and mortality, especially in elderly people and in patients with chronic respiratory and cardiovascular diseases, such as pulmonary hypertension (PH). We aimed to evaluate the impact of COVID-19 infection on patients diagnosed with severe chronic PH. Materials and Methods. A single-center prospective cohort study was performed. Patients were enrolled from 1 November 2020 to 31 December 2022. Follow-up was until 31 December 2023. Data were collected on PH diagnosis, clinical presentation, outcomes, brain natriuretic peptide (BNP) levels, pulmonary function test with lung diffusion capacity for carbon monoxide (DLCO), and computed tomography pulmonary angiography (CTPA) analysis. Results. During the 26 months of our study, 51 PH patients were diagnosed with COVID-19 infection. The majority, 44 (86.3%) of all COVID-19 infected patients, were treated on an outpatient basis, and 7 (13.7%) required hospitalization. During the follow-up period, 8 (15.7%) patients died: 4 (7.8%) due to complications of COVID-19 infection, and the other 4 (7.8%) died in the later stages of the follow-up period after recovery from acute COVID-19 infection. Therefore, the in-hospital mortality in our study was 43% (n = 3). As mentioned above, the overall mortality was 7.8% (n = 4). Higher BNP levels in the third month after COVID-19 were associated with higher mortality rates (p = 0.028). Lung function, including DLCO, did not significantly worsen with COVID-19. In our study, 24 patients (47.1%) were referred for a follow-up CTPA scan and one of them developed typical fibrotic lung changes after COVID-19. Conclusions. The incidence of COVID-19 infection in patients diagnosed with PH was 34%. In our patients with severe chronic PH, the overall mortality rate due to COVID-19 infection was low. Pulmonary fibrosis was a rare complication in our cohort. COVID-19 infection in severe PH may increase the risk of worsening chronic heart failure.

Background/Objectives: Since 2015, pulmonary arterial hypertension (PAH)-specific medications have been fully reimbursed in Lithuania. To describe the current situation of PAH treatment in the country and to determine survival during different PAH treatment regimens. Methods: The data from the Institute of Hygiene and the State Data Agency of Lithuania cases with administrative codes I27.0 and I27.8 have been evaluated. Results: In 2025, 225 confirmed cases of PAH were treated with PAH-specific medications in two PH centers. At least one PAH-specific medication was prescribed to 163 (72.4%) female and 62 (27.6%) male patients. Among these, 96 (42.7%) received sildenafil monotherapy, 82 (36.4%) received a combination of sildenafil and an ERA, 36 (16.0%) were on triple PAH-specific therapy (including selexipag or treprostinil), and 11 (4.9%) received other regimens due to specific medical considerations. The age of adults treated with sildenafil monotherapy vs. other therapies was 63.9 ± 14.8 (n = 117) and 51.5 ± 17.3 (n = 116) years, respectively (p < 0.05). A total of 191 PAH patients who received targeted therapy died during the observational period 2017–2025. Of these, 105 received monotherapy, 57 sildenafil and endothelin receptor antagonist and 29 triple therapies (treprostinil [n = 19], selexipag [n = 6], or inhaled iloprost [n = 4] were prescribed as the third drug). Patients who died and received triple therapy were younger than those on mono- and dual therapy (age at diagnosis 45.0 ± 21.6, 67.2 ± 14.7 and 61.6 ± 16.3 years, respectively, p < 0.01). Survival was longer in patients on dual therapy compared with monotherapy (43.1 ± 28.1 vs. 31.7 ± 25.0 months, p = 0.04), and the longest was in those receiving triple therapy (59.9 ± 29.4 months; p < 0.05). Conclusions: The availability of reimbursed medications dramatically increased the number of treated PAH cases in Lithuania. In 2025, most of the PAH patients received sildenafil monotherapy. Patients treated with sildenafil only were significantly older than the rest of cohort. In the survival analysis, combination PAH therapies were more often prescribed to younger patients and were associated with longer duration of life than monotherapy.