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Introduction Lifelong anticoagulation is the cornerstone of the chronic thromboembolic pulmonary hypertension (CTEPH) treatment regardless of the additional pulmonary endarterectomy, balloon pulmonary angioplasty, or medical treatment alone. Aim of this study was to evaluate the rate of oral anticoagulant preferences and document direct oral anticoagulants’ (DOACs’) safety, efficacy in the CTEPH population. Methods Patients’ demographic data obtained from database between September 2011 and April 2018. In-hospital events, death, venous thromboembolism (VTE) recurrence, bleeding events and anticoagulant therapy transition were recorded. Results We reviewed 501 CTEPH patients who observed 9.0 ± 8.5 years. All-cause death, all bleeding, recurrent VTE was observed in 15.6%, 31% and 12%. Forty-one patients (8.2%) were diagnosed as inoperable. Of all, 15.2% of operable patients remained as residual. All-cause mortality rates were 13.8% (57 pts.) in the warfarin group as compared with 9.7% (13 pts.) in rivaroxaban group (HR: 1.61, 95% CI, 0.89–2.99; p : 0.11). Higher bleeding events occurred with warfarin group (27.1%) as compared with rivaroxaban (24.6%; HR: 1.28, 95% CI, 0.86–1.88; p : 0.22). Major bleeding was significantly higher with warfarin group (HR: 1.94, 95% CI, 1.05–3.62; p : 0.03). Subgroup analysis of all-cause death revealed that this significance dominated by the rate of death according to bleeding events; warfarin versus those seen with rivaroxaban (4.85% vs. 2.2%; HR: 4.75, 95% CI: 1.12–20.16; p = 0.03). The rate of recurrent VTE was found 8.9% in the rivaroxaban group, 10.9% in warfarin group (HR: 1.21, 95% CI, 0.64–2.23; p : 0.55). Conclusion DOACs could be a safe and effective alternative for lifelong anticoagulant therapy in CTEPH patients. Rivaroxaban produced similar rates of thromboembolism and non-relevant bleeding compared to those associated with warfarin. The main difference was found with major bleeding that it was mainly associated with the death rate according to major bleeding. Using DOACs might be a more reasonable way to prevent bleeding events without increasing thromboembolic risk.

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objectives: We aimed to investigate the occurrence of sleep-related breathing disorders (SRBDs) in patients with chronic thromboembolic pulmonary hypertension (CTEPH) and addressed the effect of pulmonary hemodynamics and SRBD indices on the severity of nocturnal hypoxemia (NH). Methods: An overnight polysomnography (PSG) was conducted in patients with CTEPH, who were eligible for pulmonary endarterectomy. Pulmonary hemodynamics (mean pulmonary arterial pressure (mPAP), pulmonary arterial wedge pressure (PAWP), pulmonary vascular resistance (PVR) measured with right heart catheterization (RHC)), PSG variables (apnea–hypopnea index (AHI)), lung function and carbon monoxide diffusion capacity (DLCO) values, as well as demographics and comorbidities were entered into a logistic regression model to address the determinants of severe NH (nocturnal oxyhemoglobin saturation (SpO2) < 90% under >20% of total sleep time (TST)). SRBDs were defined as obstructive sleep apnea (OSA; as an AHI ≥ 15 events/h), central sleep apnea with Cheyne–Stokes respiration (CSA–CSR; CSR pattern ≥ 50% of TST), obesity hypoventilation syndrome (OHS), and isolated sleep-related hypoxemia (ISRH; SpO2 < 88% under >5 min without OSA, CSA, or OHS). Results: In all, 50 consecutive patients (34 men and 16 women; mean age 54.0 (SD 15.1) years) were included. The average mPAP was 43.8 (SD 16.8) mmHg. SRBD was observed in 40 (80%) patients, of whom 27 had OSA, 2 CSA–CSR, and 11 ISRH. None had OHS. Severe NH was observed in 31 (62%) patients. Among the variables tested, age (odds ratio (OR) 1.08, 95% confidence interval [CI] 1.01–1.15; p = 0.031), mPAP (OR 1.11 [95% CI 1.02–1.12; p = 0.012]), and AHI (OR 1.17 [95% CI 1.02–1.35; p = 0.031]) were independent determinants of severe NH. Conclusions: Severe NH is highly prevalent in patients with CTEPH. Early screening for SRBDs and intervention with nocturnal supplemental oxygen and/or positive airway pressure as well as pulmonary endarterectomy may reduce adverse outcomes in patients with CTEPH. [ABSTRACT FROM AUTHOR]

OBJECTIVES: The role of extracellular matrix collagen biomarkers in chronic thromboembolic pulmonary hypertension (CTEPH) is not well known. Our goal was to investigate the matrix metalloproteinase (MMP)-2 and -9 protein levels in patients with CTETH. METHODS: This is a prospective, cross-sectional study. Patients with CTETH who underwent pulmonary endarterectomy comprise group 1, and the control group included patients who underwent lung surgery without pulmonary hypertension (group 2) between March 2020 and March 2021. In addition to serum levels of MMP-9, the pulmonary endarterectomy and control pulmonary artery tissue samples were measured by the enzyme-linked immunosorbent assay  4pl, cubic, quadratic and Western blot techniques. Levels of MMP-2, which consist of pro MMP-2/ß-actin and active MMP-2/ß-actin and MMP-9/ß-actin, were measured only in the tissue samples. RESULTS: Forty-eight patients were enrolled consecutively in group 1 (n: 24) and group 2 (n: 24). The serum concentrations of MMP-9 were similar in both groups. Similarly, a comparison of tissue sample levels of pro MMP-2/ß-actin (P = 0.496) and active MMP-2/ß-actin (P = 0.216) showed no significant difference between the groups. The tissue samples from patients with CTETH had significantly lower amounts of MMP-9/ß-actin compared to the control group (P = 0.001). CONCLUSIONS: This study indicates that serum levels of extracellular matrix collagen biomarkers were similar in patients with CTETH who were candidates for surgery and in patients who had non-pulmonary hypertension who underwent lung surgery. Differences in levels of MMP-9/ß-actin in tissue samples may play a role in pulmonary vascular remodelling in operable patients. Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the presence of unresolved thromboembolism associated with fibrosis in the pulmonary arteries. [ABSTRACT FROM AUTHOR]

Background: Chronic thromboembolic pulmonary hypertension is a condition that occurs after mechanical obstruction of the pulmonary arteries by thrombus. Since the frequency and demographics of chronic thromboembolic pulmonary hypertension differ between countries, it is thought that genetic factors may play a role in its development. The aim of this study is to reveal the status of VKORC1, CYP2C9*3, CYP2C9*7, and fibrinogen-Aa THR312ALA gene polymorphisms in chronic thromboembolic pulmonary hypertension patients in Turkey. Methods: In this prospective cross-sectional study, a total of 46 chronic thromboembolic pulmonary hypertension patients and 106 healthy volunteers were included. Polymerase chain reaction-restriction fragment length polymorphism method was used to determine candidate gene polymorphisms for chronic thromboembolic pulmonary hypertension. The general population parameters of each locus were calculated, and the relationship between dominant, co-dominant, and recessive genotype models and chronic thromboembolic pulmonary hypertension was analyzed. Results: For the fibrinogen-Aa gene, those with the THR/THR genotype were found to have a 13.51 (95% CI: 2.688-33.333) times less susceptibility rate to the disease than those with the ALA/THR genotype, the susceptibility of THR/ALA genotype to the disease was 5.026 (95% CI: 1.774-14.242) times more than those with ALA/ALA genotype. There was no difference between patient groups for VKORC1, CYP2C9*3 genes (P > .05). Since the CYP2C9*7 patient group was monomorphic for the ILE allele, the patient/control odds ratio and 95% CI could not be calculated. Conclusion: This study shows that there is an association between the fibrinogen-Aa gene ALA polymorphism at the amino acid position of 312 and the development of chronic thromboembolic pulmonary hypertension, but not between the CYP2C9 and VKORC1 gene polymorphisms. [ABSTRACT FROM AUTHOR]

Copyright of Archives of the Turkish Society of Cardiology / Türk Kardiyoloji Derneği Arşivi is the property of KARE Publishing and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

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]

Copyright of Molecular Imaging & Radionuclide Therapy is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Objective: Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the leading causes of pulmonary hypertension (PH). We aimed to investigate the outcome of CTEPH patients who were followed-up by a PH outpatient clinic. Methods: We screened the medical records of 29 PH patients who were followed-up by a PH outpatient clinic between 2009 and 2018. The patients' demographics and their clinical, echocardiographic, and hemodynamic characteristics were recorded. Results: Our study group consisted of 16 females (55.2%) and 13 males (44.8%). The mean age was 59.5±13.7 years and the median follow-up duration was 44 months (1-113 months). The mean initial 6-minute walking distance (6MWD) was 321.4±119.9 m. The initial median N-terminal pro brain natriuretic peptide (NT-proBNP) level was 2468 pg/mL (46.1-20.564 pg/mL). All patients were on oral anticoagulant therapy. Pulmonary endarterectomy (PEA) was performed in 17 of 29 patients (58.6%). Twelve patients (41.4%) were not operated upon due to distal disease, comorbidities, or their own preference. The operated patients were younger than the non-operated patients (55 years & 65 years, p=0.04). At the follow-up, the 6MWD in the operated patients increased (+76 m) and decreased in non-operated patients (-46 m). The survival rate at 10-year follow-up was 58.6% for the whole group. Twelve patients died during the follow-up period. While 7 of 12 not-operated patients died (58.3%), just 5 of 17 operated patients (4 perioperatively and 1 at follow-up) died (29%). Advanced-stage final functional capacity (FC) [New York Heart Association (NYHA) III-IV], inoperability, lower final 6MWD, higher final NT-proBNP, and reduced tricuspid annular plane systolic excursion (TAPSE) were associated with an increased mortality rate. Univariate Cox regression analysis showed that patients with NYHA I-II final FC showed a 166-fold decreased mortality rate. Conclusion: The long-term prognosis of operated patients is better than the outcome of not-operated patients. The strongest predictor associated with mortality was a worse final FC (NYHA III-IV). [ABSTRACT FROM AUTHOR]

Objective: Chronic thromboembolic disease (CTED) is characterized by persistent pulmonary thromboembolic occlusions without pulmonary hypertension. Early surgical treatment by performing pulmonary endarterectomy (PEA) may improve symptoms. The goal of the study was to review our experience and early outcome of PEA in patients with CTED. Methods: Data were prospectively collected on all patients who underwent PEA between 2011 and 2015. Patients with CTED and a mean pulmonary artery pressure (mPAP) of <25 mm Hg were identified. All patients were in New York Heart Association (NYHA) functional class II or III. Measured outcomes were in-hospital complications, improvement in cardiac function and exercise capacity, and survival after PEA. Patients were reassessed at 6 months following surgery. Results: A total of 23 patients underwent surgery. There was no in-hospital mortality, but complications occurred in six patients (26%). At 6 months following surgery, 93% of the patients remained alive. Following PEA, the mPAP fell significantly from 21.0±2.7 mm Hg to 18.2±5.5 mm Hg (p<.001). Pulmonary vascular resistance also significantly decreased from 2.2±0.7 wood to 1.5±0.5 wood (p<.001). The 6-min walking distance significantly increased from 322.6±80.4 m to 379.9±68.2 m (p<.001). There was a significant symptomatic improvement in all survivors in NYHA functional classes I or II at 6 months following surgery (p=.001). Conclusion: PEA in selected patients with CTED resulted in significant improvement in symptoms. The selection of patients for undergoing PEA in the absence of pulmonary hypertension must be made based on patients' expectations and their acceptance of the perioperative risk. [ABSTRACT FROM AUTHOR]