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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]

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]

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]

Introduction: Chronic thromboembolic pulmonary hypertension (CTEPH) is a severe disease treated with pulmonary endarterectomy. Our study aims to reveal the differences in liquid modalities and operation modifications, which can affect the patients' mortality and morbidity. Methods: One hundred twenty-five patients who were diagnosed with CTEPH and underwent pulmonary thromboendarterectomy (PTE) at our center between February 2011 and September 2013 were included in this retrospective study with prospective observation. They were in New York Heart Association functional class II, III, or IV, and mean pulmonary artery pressure was > 40 mmHg. There were two groups, the crystalloid (Group 1) and colloid (Group 2) liquid groups, depending on the treatment fluids. P-value < 0.05 was considered statistically significant. Results: Although the two different fluid types did not show a significant difference in mortality between groups, fluid balance sheets significantly affected the intragroup mortality rate. Negative fluid balance significantly decreased mortality in Group 1 (P<0.01). There was no difference in mortality in positive or negative fluid balance in Group 2 (P>0.05). Mean duration of stay in the intensive care unit (ICU) for Group 1 was 6.2 days and for Group 2 was 5.4 days (P>0.05). Readmission rate to the ICU for respiratory or non-respiratory reasons was 8.3% (n=4) in Group 1 and 11.7% (n=9) in Group 2 (P>0.05). Conclusion: Changes in fluid management have an etiological significance on possible complications in patient follow-up. We believe that as new approaches are reported, the number of comorbid events will decrease. [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]

Purpose: Right ventricular (RV) function is an important factor in the prognosis of chronic thromboembolic pulmonary hypertension (CTEPH) in patients. In our study, we aimed to evaluate the timing and magnitude of regional RV function before and after balloon pulmonary angioplasty (BPA) using speckle tracking echocardiography (STE) and their relation to clinical and hemodynamic parameters in patients with CTEPH. Material and Method: We enrolled 20 CTEPH patients and 19 healthy subjects in our study. Enrolled patients underwent echocardiography, right heart catheterization (RHC), and 6‐minute walk distance (6MWD) test at baseline and after the BPA. Results: In hemodynamic RHC measurements and clinical evaluations, mean pulmonary artery pressure (median: 53.5 mm Hg vs 37.0 mm Hg, P = .001) and pulmonary vascular resistance (median: 12 Wood units [WU] vs 7 WU, P = .001) and pro‐brain natriuretic peptide level decreased and 6MWD increased after BPA sessions. There was no statistically significant difference between before and after the BPA sessions in conventional echocardiographic measurements. In STE analysis, the electromechanical delay (EMD) between RV free wall (RVF) and LV lateral wall (LVL) (median: 65 ms vs 47.5 ms, P = .01) and RV peak systolic strain dispersion index (52 ms vs 29 ms, P = .001) were higher in patients with CTEPH than healthy controls before the BPA. Both these parameters decreased significantly after BPA. Conclusion: Chronic thromboembolic pulmonary hypertension was associated with RV electromechanical delay and dispersion based on the STE analysis. Balloon pulmonary angioplasty might have an important impact on the improvement of both RV function and hemodynamics. [ABSTRACT FROM AUTHOR]

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease characterized by the persistence of thromboemboli obstructing the pulmonary arteries as an organized tissue. The consequence is an increase in pulmonary vascular resistance resulting in pulmonary hypertension (PH) and progressive right heart failure. Pulmonary thromboendarterectomy (PTE) is the treatment of choice of chronic thromboembolic pulmonary disease. Pulmonary thromboendarterectomy is performed under hypothermia and total circulatory arrest. This procedure is associated with a low mortality rate in experienced centers and results in a dramatic improvement of hemodynamic parameters, functional status and survival rate. This surgery should be performed as early as possible in the history of this disease, preferentially before development of arteriolitis in the non obstructed territories. Because of the absence of clinically evident acute pulmonary embolism history in >50% of patients, the diagnosis of CTEPH can be difficult. Lung scintigraphic scan showing segmentary mismatched perfusion defects is the best diagnostic tool to detect CTEPH. Pulmonary angiography and high-resolution computerized tomography scan are required to establish the diagnosis and to assess the operability. A diagnostic right heart catheterization is required to confirm the diagnosis of PH and to determine the degree of hemodynamic impairment. If there is a good correlation between the pulmonary vascular resistance and the anatomical obstruction, pulmonary thromboendarterectomy should be proposed. Pulmonary or cardiopulmonary transplantation is indicated in this setting only when the lesions are too distal and thus inaccessible to endarterectomy. [ABSTRACT FROM AUTHOR]

Immune thrombocytopenic purpura (ITP) patients are at high risk for bleeding complications regarding surgeries involving cardiopulmonary bypass. We report an ITP patient with chronic thromboembolic pulmonary hypertension who underwent uncomplicated pulmonary endarterectomy with receiving postoperative intravenous immunoglobulin (IVIG) therapy. The positive outcome of this case may suggest that pulmonary endarterectomy surgery is performed safely for ITP patients. [ABSTRACT FROM AUTHOR]

OBJECTIVE Pulmonary endarterectomy (PEA) is the treatment of choice for patients with chronic thrombo-embolic pulmonary hypertension (CTEPH). The aim of this study was to review our initial experience since the implementation of our program. METHODS Data were collected prospectively on all patients who underwent PEA between March 2011 and March 2012. RESULTS Forty-nine patients (20 male, 29 female, mean age 47.7 years) underwent surgery. The preoperative New York Heart Association class distribution showed the majority to be in class III or IV (n = 40). Mortality rate was 14.2% (n = 7) and the morbidity rate was 26.5% (n = 13). After PEA, the durations of mechanical ventilation, intensive care stay and hospital stay before discharge were 49.7 ± 46.1 h, 6.5 ± 5.0 days and 12.9 ± 7.5 days, respectively. The systolic and mean pulmonary artery pressure (PAP) fell significantly from 87.0 ± 26.6 mmHg and 53.8 ± 14.5 before, to 41.5 ± 12.4 mmHg and 28.5 ± 10.5 after surgery (P < 0.001 and P < 0.001, respectively). Pulmonary vascular resistance (PVR) also improved significantly from 808 ± 352.0 to 308 ± 91 dyn•s•cm−5 (P < 0.001). Univariate analysis showed that preoperative systolic PAP, tricuspid annular plane systolic excursion, right atrial volume, right atrial pressure, forced expiratory volume in 1 s, forced vital capacity, preoperative PVR, postoperative PVR, the duration of circulatory arrest and postoperative use of extracorporeal membrane oxygenation were risk factors for mortality (P < 0.05). According to multivariate analyses, only prolonged mechanical ventilation was selected as predictive risk factor for morbidity (P = 0.005). After a median follow-up of 6.1 months, two patients died due to cerebrovascular disease and one patient needed targeted pulmonary hypertension therapy. The rest of the 39 patients showed marked improvements in their clinical status. CONCLUSIONS Starting a pulmonary endarterectomy program with acceptable mortality and morbidity rates and satisfactory early-term outcomes increases awareness of the CTEPH and surgery. Preoperative factors can primarily predict postoperative outcome after PEA. Identifying the risk factors in order to achieve a good result is important for the success of a PEA program. Therefore all patients diagnosed with CTEPH should be referred for consideration of PEA in a specialized centre. [ABSTRACT FROM AUTHOR]

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a form of pulmonary embolism, and pulmonary endarterectomy (PEA) is the surgical treatment. Asymmetric dimethylarginine (ADMA) levels are increased in pulmonary hypertension. This study aimed to investigate serum ADMA levels in patients with CTEPH, the effect of PEA on ADMA, and its prognostic value in long-term mortality.Method: Eighty (80) patients with CTEPH and 32 healthy controls were included. Preoperative serum ADMA levels, determined using an enzyme-linked immunosorbent assay, were compared between patients with CTEPH and controls. Of 80 patients, 64 had PEA. Pre- and 6-month postoperative serum ADMA levels, 6-minute walk distance (6MWD), and haemodynamic parameters were collected from patients undergoing PEA. Patients were followed-up for survival analysis.Results: Mean ± standard deviation serum ADMA levels were significantly higher in patients with CTEPH compared with controls (0.79±0.32 μmol/L vs 0.52±0.12 μmol/L; p=0.0001). Statistically significant differences were observed between preoperative and postoperative serum ADMA levels (0.78±0.30 μmol/L vs 0.62±0.22 μmol/L; p=0.0001), 6MWD (p=0.0001), and pulmonary vascular resistance (p=0.0001) in 60 patients who underwent and survived PEA. The decrease in serum ADMA levels and increase in 6MWD were significantly correlated (r=-0.286, p=0.027). No other correlation was found. Perioperative mortality was 6.3%, and the survival rate with a mean follow-up of 34.57±8.20 months was 93.3%. Patients with serum ADMA levels >0.8 μmol/L had a significantly lower survival rate (logrank: 5.86; p=0.015).Conclusions: Levels of circulating ADMA might add diagnostic and prognostic information in CTEPH. Pulmonary endarterectomy is associated with an improvement in serum ADMA levels. Preoperative serum ADMA levels may be useful for estimating the outcome of PEA. [ABSTRACT FROM AUTHOR]