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Background Coronavirus disease 2019 (COVID-19) is still an ongoing entity and every day we face new sequalae of the disease. We hereby present surgical results of patients who are treated for post-COVID chronic thromboembolic pulmonary hypertension. Methods Data were collected among patients who underwent pulmonary endarterectomy and had a diagnosis of post-COVID chronic thromboembolic pulmonary hypertension. All data were retrospectively reviewed from a prospectively conducted database. Operative mortality was described as death in hospital or within 30 days of surgery. Results Eleven patients (seven males, four females; median age, 52 [22–63] years) were identified. Pulmonary vascular resistance improved significantly from 572 dyn/s/cm−5 (240–1,192) to 240 (195–377) dyn/s/cm−5 (p < 0.005). Significant difference was also detected in median mPAP, as it decreased from 40 mm Hg (24–54) to 24 mm Hg (15–36) following surgery (p < 0.005). Mortality was observed in one patient due to sepsis on the fifth postoperative day. Median time from COVID-19 disease to surgery was 12 months (6–24). Median length of hospital stay of the survivors was 10 days (8–14). Conclusion In the new era of chronic thromboembolic pulmonary hypertension, hybrid approach including surgery, balloon pulmonary angioplasty, and medical treatment has been recommended. pulmonary endarterectomy is still the only curative treatment when the disease is surgically accessible. We hereby report the first publication of post-COVID chronic thromboembolic pulmonary hypertension patients who were surgically treated. As we see a lot of long-term symptoms and clinical manifestations in patients who had COVID-19, we should always remember chronic thromboembolic pulmonary hypertension in the differential diagnosis. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension is a complex syndrome that encompasses a diverse group of pathophysiologies predisposed by different environmental and genetic factors. It is not clear to which extent the universal risk classification schemes can be applied to cohorts in individual pulmonary hypertension centers with differing environmental backgrounds, genetic pools, referral networks. Aims: To explore whether the recommended risk classification schemes could reliably be used for mortality prediction in an unselected pulmonary hypertension population of a tertiary pulmonary hypertension center. Study Design: A retrospective cross-sectional study. Methods: We retrospectively screened our hospital database for the patients with pulmonary hypertension between 2015 and 2022. The grouping of pulmonary hypertension was made as follows in accordance with current guidelines: Group 1: patients with pulmonary arterial hypertension, Group 2: patients with pulmonary hypertension associated with left heart disease, Group 3: patients with pulmonary hypertension associated with lung disease and/or hypoxia, and Group 4: patients with pulmonary hypertension associated with pulmonary artery obstructions. Then, we compared the predicted and observed mortality rates of four different risk classification schemes (REVEAL, REVEAL-Lite, ESC/ERS and COMPERA). Results: We identified 723 cases in our pulmonary hypertension database, the final study population consisted of 549 patients. The REVEAL, REVEAL-Lite and European Society of Cardiology/ European Respiratory Society risk scores significantly underestimated the mortality risk in the low-risk stratum (5.3% vs. 1.9%, P < 0.001; 5.3% vs. 2.9%, P = 0.015 and 6.3% vs. 1%, P < 0.001, respectively) and overestimated the mortality risk in the high-risk stratum (11.8% vs. 25.8%, P < 0.001; 10.4% vs. 25.1%, P < 0.001 and 13.2% vs. 30%, P < 0.001, respectively). Although the COMPERA 4-strata model significantly underestimated the risk in low- and intermediatelow risk strata (4.9% vs. 1.5%, P < 0.001 and 6.8% vs. 2.8%, P = 0.001, respectively), it was accurate in intermediate-high and highrisk groups (10.1% vs. 8.7%, P = 0.592 and 15.6% vs. 22%, P = 0.384, respectively). The analyses limited only to group 1 pulmonary hypertension patients gave similar results. Conclusion: The established risk classification schemes may not perform as good as expected in unselected pulmonary hypertension populations and this may have important implications on management decisions. Tertiary centers should not uncritically accept the published risk prediction models and consider modifying current risk scores according to their own patient characteristics. [ABSTRACT FROM AUTHOR]

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Background: Antiphospholipid syndrome is an autoimmune disease characterized by the occurrence of venous and/or arterial thrombosis. Chronic thromboembolism is one of the known established pathogenesis of pulmonary hypertension, known as chronic thromboembolic pulmonary hypertension. Pulmonary endarterectomy is the treatment of choice for chronic thromboembolic pulmonary hypertension. The aim of this study is to evaluate the efficacy and risk of pulmonary endarterectomy in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. Methods: Data were prospectively collected and retrospectively analyzed, for patients who underwent pulmonary endarterectomy between March 2011 and March 2020. Results: Seventeen patients (4 male and 13 female) were identified. Thirteen patients had primary antiphospholipid syndrome and 4 had secondary antiphospholipid syndrome. The mean age was 34.82 ± 10.07 years and the mean time interval between the diagnosis and surgery was 26.94 ± 17.35 months. Dyspnea on exertion was the main symptom in all patients. Seven patients had previous deep vein thrombosis, 5 patients had a history of recurrent abortions, and 2 patients had hemoptysis. Following surgery, mean pulmonary artery pressure decreased from 47.82 ± 13.11 mm Hg to 22.24 ± 4.56 mm Hg (P < .001), and pulmonary vascular resistance improved from 756.50 ± 393.91 dyn/s/cm-5 to 298.31 ± 132.84 dyn/s/cm-5 (P < .001). There was no in-hospital mortality with a mean follow-up of 75.29 ± 40.21 months. The functional capacity of all patients improved from 269.46 ± 111.7 m to 490 ± 105.34 m on a 6-minute walking test. Conclusions: Pulmonary endarterectomy is a safe and curative treatment in patients with antiphospholipid syndrome-associated chronic thromboembolic pulmonary hypertension. It has a favorable outcome by increasing the quality of life. A multidisciplinary experienced chronic thromboembolic pulmonary hypertension team is critical in the management of these unique patients. [ABSTRACT FROM AUTHOR]

Objective: Although the underlying pathology of chronic thromboembolic pulmonary hypertension (CTEPH) is mechanical obliteration of the major pulmonary vessels, high pulsatile stress penetrating into the normal distal pulmonary microvasculature resulting from reduced pulmonary arterial compliance (CPA) may cause progressive deterioration in pulmonary hemodynamics. Hypothetically, balloon pulmonary angioplasty (BPA) may be beneficial in reducing CPA and pulsatile stress in patients with CTEPH. Methods: In total, 26 patients with available pre- and post-BPA right heart catheterization results were included in the study. BPA was performed in a series of staged procedures by 2 experienced interventional cardiologists. Results: The median CPA showed a 59.2% increase (1.03 to 1.64 mL/mm Hg, p=0.005). The median pre-BPA pulsatile stress product decreased by 20.7% (4,266 to 3,380 mm Hg/min, p=0.003). A linear regression model established that the percent change in CPA after BPA accounted for 21.8% of the explained variability in the change in 6-minute walk test (p=0.009). Conclusion: Our results indicate that BPA decreases CPA and pulmonary pulsatile stress. These changes may be partly responsible for the improvement in functional capacity after BPA. [ABSTRACT FROM AUTHOR]

Both quality of life (QoL) and caregiver burden are essential constructs in patients with pulmonary hypertension (PH) however; their relationship has never been investigated before. The aim of this study was to evaluate if there was any relationship between patients' QoL and caregiver burden. Patients with PH and their caregivers were included. Patients' age, sex, World Health Organization (WHO) functional class, systolic pulmonary arterial pressure (sPAP), and six-minute walk distances (6MWD) were recorded. Patients' QoL was assessed using emPHasis-10 and caregiver burden with the Zarit Caregiver Burden Scale. 72 patient-caregiver dyads were included. Caregiver burden was significantly correlated with the QoL(r = 0.39 p <.003), but was not correlated with other clinical parameters. Patients' QoL showed significant negative correlation with the 6MWD(r = −0.46 p <.005). There is a moderate correlation between QoL and caregiver burden. Clinical parameters influence QoL, but they do not affect caregiver burden. [ABSTRACT FROM AUTHOR]

Skeletal and respiratory muscle dysfunction has been previously described in patients with other etiologic subgroups of pulmonary arterial hypertension (PAH) but has never been investigated in patients with PAH due to congenital heart diseases (CHD). This study aims to show the involvement of skeletal and respiratory muscles in these patients. This cross-sectional study included patients with PAH due to CHD and healthy controls. Patients' demographic properties, six-minute walk tests; shoulder abduction, handgrip, knee extension, and ankle dorsiflexion muscle strength, maximum inspiratory (MIP) and expiratory pressures (MEP) were measured. Deltoid, flexor digitorum superficialis, and profundus, tibialis anterior and rectus femoris muscles were visualized with ultrasonography and their cross-sectional areas (CSA) were also measured in both groups. Twelve patients and 12 controls were included. Mean MIP was 104.22±32.57 cm H2O for healthy participants while 61.33±29.74 cm H2O for patients (p<0.001). For mean MEP, it was 100.08±26.05 cm H2O in healthy participants and 69.75±39.79 cmH2O in controls (p=0.004). When the strength of skeletal muscles was compared, there were significant differences between the groups in all measurements except for bilateral grip strength. In the correlation analysis, MIP and MEP values showed no significant correlations with clinical parameters. They showed significant moderate correlations with skeletal muscle strength. When CSAs of the muscles were compared, there were significant differences in all measurements except for left FDS and FDP and bilateral rectus femoris. This study showed that in patients with pulmonary arterial hypertension due to CHD, respiratory muscle strength is significantly worse than healthy participants. Patients had also significantly worse skeletal muscle strength except for grip strength. [ABSTRACT FROM AUTHOR]

Objective: Sarcoidosis is a systemic granulomatous disease rarely complicated by pulmonary hypertension (PH). The prevalence of PH in sarcoidosis is unclear and has differences between ethnic groups. This study aimed to investigate the prevalence and predictors of PH in a Turkish cohort. Methods: The study included 55 patients with biopsy-proven sarcoidosis in a single center. All patients underwent detailed transthoracic echocardiography (TTE) to assess the probability of PH as recommended. Right heart catheterization (RHC) was performed for patients with intermediate-high risk of PH. Patients with mean pulmonary artery pressure >20 mm Hg by RHC were defined as PH. Demographic and clinical characteristics, laboratory data, spirometry, 6-min walk test, and TTE were compared between low and intermediate-high risk PH groups. Results: The probability of PH was low with 47 patients. Eight patients had intermediate-high probability of PH, and two of them refused to undergo RHC. Of six intermediate-high probability patients, three had PH, and all of them had post-precapillary PH. The prevalence of PH in sarcoidosis was 5.5% (3/55). Six-minute walk distance (6 MWD) and diastolic parameters (E/A ratio, E' wave, and left atrial volume) were significantly lower, and New York Heart association class and N-terminal probrain natriuretic peptide (NT-proBNP) level were higher in intermediate-high risk PH patients compared with low-risk PH patients. Conclusion: The frequency of PH in sarcoidosis was 5.5% in a Turkish cohort. NT-proBNP, 6 MWD, diastolic function parameters, and myocardial strain parameters can be useful predictors of PH in patients with sarcoidosis, besides known echocardiographic parameters. [ABSTRACT FROM AUTHOR]

Background: Patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH) are often treated with pulmonary arterial hypertension-specific drugs. However, most of these patients remain symptomatic, despite medical treatment. Balloon pulmonary angioplasty (BPA) is an emerging therapeutic intervention for patients with inoperable CTEPH. This study aimed to report the initial experience of BPA in a tertiary referral centre for CTEPH.Methods: A total of 26 consecutive patients, who underwent 91 BPA sessions, were included in the study. All patients underwent a detailed examination, including 6-minute walking distance (6MWD), and right heart catheterisation at baseline and 3 months after the last BPA session.Results: The mean age of the patients was 51±17 years. Fifteen (15) patients had inoperable CTEPH and 11 patients had residual or recurrent CTEPH post pulmonary endarterectomy (PEA). Functional class improved in 17 of 26 (65%) patients. The 6MWD increased from a mean 315±129 to 411±140 m (p<0.001), and NT pro-BNP reduced from a median 456 to 189 pg/mL (p=0.001). The number of patients who required supplemental oxygen decreased from 11 (42.3%) to five (19%) (p=0.031) after BPA treatment. The mean pulmonary artery pressure decreased from a mean 47.5±13.4 to 38±10.9 mmHg (p<0.001), the pulmonary vascular resistance decreased from a mean 9.3±4.7 to 5.8±2.8 Wood units (p<0.001), and the cardiac index increased from a mean 2.4±0.7 to 2.9±0.6 L/min/m2 (p=0.008).Conclusions: Balloon pulmonary angioplasty improved haemodynamics, 6MWD, and functional class, and reduced the requirement for supplemental oxygen, with an acceptable risk-benefit ratio in patients with inoperable CTEPH and with residual/recurrent CTEPH. [ABSTRACT FROM AUTHOR]