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Abstract Background Patients with chronic thromboembolic pulmonary hypertension (CTEPH) in countries with limited resources have, to date, been poorly represented in registries. Objective This work assesses the epidemiology, diagnosis, hemodynamic and functional parameters, and treatment of CTEPH in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia. Methods A prospective, cohort, phase IV, observational registry with 3-year follow-up (n = 212) in patients aged ≥ 18 years diagnosed with CTEPH was created. Clinical, hemodynamic, and functional parameters were obtained at an initial visit, follow-up visits, and a final visit at the end of 3 years’ observation or end of follow-up. Data were recorded on electronic case report forms. Parameters evaluated included 6-minute walking distance (6MWD), use of pulmonary endarterectomy (PEA), balloon pulmonary angioplasty (BPA), pulmonary hypertension (PH)-targeted therapy, and survival. All statistical analyses were exploratory and descriptive, and were performed in the overall population. Results The most common symptoms were typical of those expected for CTEPH. Almost 90% of patients underwent right heart catheterization at diagnosis or initial study visit. In total, 66 patients (31%) underwent PEA before the initial visit; 95 patients (45%) were considered operable, 115 (54%) were inoperable, and two (1%) had no operability data. Only 26 patients (12%) had been assessed for BPA at their initial visit. PH-targeted therapy was documented at diagnosis for 77 patients (36%), most commonly a phosphodiesterase type 5 inhibitor (23%). Use of PH-targeted therapy increased to 142 patients (67%) at the initial visit, remaining similar after 3 years. Use of riociguat increased from 6% of patients at diagnosis to 38% at 3 years. Between baseline and end of observation, results for patients with paired data showed an increase in 6MWD. Survival at the end of observation was 88%. Conclusions These data highlight the current diagnosis and management of CTEPH in the participating countries. They show that early CTEPH diagnosis remains challenging, and use of off-label PH-targeted therapy is common. ClinicalTrials.gov: NCT02637050; registered December 2015. Graphical Abstract

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 intermediate-low 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 high-risk 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.

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

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.

Acute heart failure (HF) is one of the most common cardiac emergencies. Pulmonary edema caused by HF may mimic an exudative disease on chest computed tomography scans. Coronavirus disease 2019 (COVID-19) emerged in China in December 2019 and quickly spread around the world. During this pandemic period, the need to exclude the possibility of COVID-19 pneumonia in patients with acute dyspnea may cause a delay in the diagnosis and treatment of patients with acutely decompensated HF who have similar symptoms. This case report describes a diabetic patient admitted with dyspnea one week after she suffered an acute myocardial infarction. The objective of this report is to draw attention to the differential diagnosis of HF and COVID-19 pneumonia.

Objective: This was an investigation of the relationship between the N-terminal pro-brain natriuretic peptide (NT-proBNP) level and mortality in patients with stage 3-4 chronic kidney disease (CKD). Methods: This study was designed as a subgroup analysis of the Heart Failure Prevalence and Predictors in Turkey (HAPPY) study. The HAPPY study included 4650 randomly selected individuals from the 7 geographical regions of Turkey. A total of 191 subjects from the original cohort with an estimated glomerular filtration rate (eGFR)

Objective: Right ventricular (RV) dysfunction in acute pulmonary embolism (APE) has been associated with increased mortality and morbidity. The aim of the present study was to assess the timing and magnitude of regional RV functions using speckle-tracking echocardiography (STE) and their relationship to early hospital mortality in patients with APE. Methods: One hundred forty-two patients were prospectively studied at the onset of an acute episode and after a median follow-up period of 30 days. Their clinical and laboratory characteristics were recorded. For all patients, conventional two-dimensional echocardiography and STE were performed within 24 h after the diagnosis of APE. Results: Twenty-eight (19.7%) patients died during the hospitalization follow-up. Patients who died during hospitalization were older and had higher high sensitivity cardiac troponin T levels, and a higher percentage of patients had simplified Pulmonary Embolism Severity Indexes. In STE analyses, they had lower RV free wall peak longitudinal systolic strain (PLSS) and higher RV peak systolic strain dispersion indexes. The time to PLSS difference between RV free wall and LV lateral was longer in patients who died during hospitalization than in those who survived, and this was an independent predictor of early hospital mortality with 85.7% sensitivity and 75.0% specificity in patients with APE. Conclusion: APE was associated with RV electromechanical delay and dispersion. Electromechanical delay index might be useful to predict early hospital mortality in patients with APE.

Background: The aim of the present study was therefore to evaluate the evolution of electrocardiography (ECG) markers indicator of morbidity and mortality after pulmonary endarterectomy (PEA). It may be a good predictor of mortality and morbidity in chronic thromboembolic pulmonary hypertension (CTEPH) with patients who underwent PEA. PEA may be reduced risk of arrhythmia in patients with CTEPH. However, this claim must to be supported with long-term results. Materials and Methods: We collected demographic, ECG, and echocardiographic parameters data (baseline and after the operation) in patients undergoing PEA for CTEPH at our institution from 2009 to 2013.We assessed 62 CTEPH patients who underwent PEA. Results: P wave amplitude in DII, PR interval, P and QT dispersion changed significantly at 3 months after surgery. The P dispersion (17.66 ± 6.2, P < 0.04) and QT dispersion (23.75 ± 11.37, P < 0.015) were longer in before operation than in after operation. Conclusions: In our study, we found in ECG analyses of CTEPH with patients who are undergoing PEA that P dispersion, QT dispersion were changed when compared with before operation. For this reason, we think that PEA reduces the risk of atrial fibrillation and malignant arrhythmia.

Objective: Pre- and postoperative changes in echocardiographic parameters and results of 6-minute walking test (6-MWT) were investigated in the present study. Methods: Seventy-six patients (32 males, 44 females; mean age 45.9+-15.1 years) were included. Before and after surgery, 6-MWT and echocardiography were performed. Changes in postoperative parameters were compared to basal walking test and other basal parameters. Results: Distance covered in 6-MWT significantly increased after surgery (p

A fifty-four-year-old female patient was admitted to our unit with exertional chest pain of six months duration. Transthoracic echocardiography showed apical hypertrophy. Upon further investigation, cardiac magnetic resonance imaging revealed apical hypertrophic cardiomyopathy. The patient underwent myocardial perfusion scintigraphy which showed anterior ischemia. Coronary angiography revealed an arteriovenous fistula (AVF) from the left anterior descending artery to the pulmonary artery. The patient's chest pain was attributed to a coronary steal syndrome secondary to the coronary AVF. The AVF fistula was closed with a coil and the patient's chest pain improved. In conclusion, coronary steal syndrome may lead to myocardial ischemia in patients with a coronary AVF.

Objectives: The underlying mechanisms for the development of chronic thromboembolic pulmonary hypertension and prognostic biomarkers are not clear yet. Thus, our aim is to assess and identify new biomarkers for the expression of 84 key genes linked to angiogenesis. Methods: Patients who had levels more than 1000 dynes·sec·cm−5 were included in the test group, and the other patients were included in the control group. Twelve specimens were taken from the patients. RT2 Profiler PCR Array (Qiagen) was used to quantify the expression of the 84 key genes. Results: Eight patients (6 male, 2 female, median age 54.4 ± 13.1 years) who underwent pulmonary endarterectomy were included. Pulmonary vascular resistance improved significantly from 811 ± 390 dyn/s/cm−5 to 413.3 ± 144.9 dyn/s/cm−5 (P .005) after surgery. Median length of hospital stay was 11.62 ± 2.97 days. The test group had a distinct pattern of impaired angiogenic and antiangiogenic genes. The expression levels of TGFA, TGFB1, THBS2, THBS1, TGFBR1, SERPINE1, SERPINF1, TGFB2, TIMP2, VEGFC, IFNA1, TNF, CXCL10, NOS3, IGF1, and MMP14 were downregulated in the specimens from the patients who had higher pulmonary vascular resistance values, whereas some genes, including PDGFA, showed upregulation that was statistically nonsignificant in the same group. Conclusions: These results can lead to the development of new markers that could predict adverse outcomes of patients with CTEPH. Identification of new markers that are related to worse outcomes would enable screening patients for early diagnosis and treatment.

The aim of present study is to evaluate left ventricular (LV) functions using speckle tracking echocardiography (STE) after iron therapy in patients with iron deficiency anemia (IDA). We consecutively enrolled 92 patients with IDA who needed iron therapy and 82 age- and gender-matched healthy volunteers in our study. Their clinical, laboratory, conventional two-dimensional echocardiography (2DE), and STE examinations were performed to all patients before and after iron therapy. Echocardiographic measurements were compared with healthy controls. The hemoglobin level increased after iron therapy (7.2±2.9 vs. 12.1±2.0, p [Med-Science 2019; 8(4.000): 947-52]

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a progressive disease of pulmonary hypertension (PH) as a consequence obstructive of pulmonary arteries with thromboembolism. Augmented pulmonary vascular resistance and pulmonary artery pressure (PAP) can ultimately lead to the right ventricular dysfunction, which leads to adaptive and maladaptive changes. Right ventricle (RV) remodeling can cause clinical deterioration and RV failure. Aims and Objectives: We aimed to evaluate the RV functions with a new diagnostic modality 2-deoxy-2-(18F)-fluoro-D-glucose (FDG)-positron emission tomography (PET) in patients with CTEPH. Materials and Methods: We included 53 patients diagnosed with CTEPH who were planned to have pulmonary thromboendarterectomy (PEA) and did not have any contraindication for cardiac PET/computed tomography (CT). We performed transthoracic echo, cardiac PET/CT, 6-minute walk distance (6-MWT), and right heart catheterization 1 week before PEA surgery. Results: The patients divided into two groups according to RV/left ventricle (LV) FDG-PET uptake ratio, RV/LV ≤1, and RV/LV >1. Six-MWD was significantly higher in the RV/LV ≤1 group (P = 0.005). Pro-BNP was considerably higher in the RV/LV >1 group (P = 0.041). Conclusion: The present study aims to demonstrate the RV/LV FDG-PET uptake ratio could be used in the noninvasive diagnostic method in diagnosing, treatment strategy, and clinical follow-up in patients with CTEPH.

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. 12 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

In acute myocarditis, thrombus formation is an important prognostic factor. Early diagnosis and treatment of intracardiac thrombus is critical, especially when there are multiple thrombi. When a patient presents with multiple cardiac thrombi not only cardiac disorders, but other diseases such as malignancies, rheumatologic disorders and thrombophilia must be considered in the differential diagnosis. Although the presence of hypercoagulable states does not generally affect the treatment choice, it may have an impact on continuation and duration of the anticoagulant therapy. In this paper, we present two cases of acute myocarditis with multiple intracardiac thrombi. Additionally, these cases had hypercoagulable states which might have contributed to the thrombus formation.

Renal sympathetic denervation is safe and effective in patients with resistant hypertension. In all of the studies of renal artery denervation, patients with accessory renal arteries are excluded. So there is not any data regarding renal sympathetic denervation applied to the accessory renal arteries. We present a young female patient with resistant hypertension despite use of five different antihypertensive drugs. The patient had a well developed (diameter >4 mm) left renal accessory. We believe that if we omitted the well developed accessory renal artery, we would not have maintained adequate blood pressure control. Thus, we applied radiofrequency ablation to both renal arteries and left accessory artery. Immediately after the procedure, the patient′s blood pressure was reduced to 110/60 mmHg and this effect was continued during the first month of follow-up.