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Objectives: We aimed to analyze the prevalence, mortality, and prognostic factors in systemic sclerosis (SSc) patients with pulmonary hypertension (PH) with or without interstitial lung disease (ILD)., Methods: The associations between mortality and demographics, transthoracic echocardiography, right heart catheterization (RHC), pulmonary functional parameters at baseline, and treatment modalities in two groups; patients with pulmonary arterial hypertension (PAH, PH without significant ILD) and PH + ILD were evaluated., Results: The mean age of the patients was 56.6±13.5 (range: 34-82, 44 women/ 2 men), and 23 (52.3%) were deceased during a median follow-up of 45 months. The survival rates of PH-SSc patients were 91% for the first year, 75% for 2 years, 68% for 3 years, and 43.1% for 5 years. The majority of deceased patients were in the PH + ILD group (p=0.007). The PH + ILD group had more diffuse skin involvement, anti-Scl-70 positivity, high C-reactive protein, low FVC, and lower DLCO values. The deceased patients had higher ePASP, low CO, and FVC values compared to surviving patients. Median survival time was significantly better in patients on combined therapy (44 vs. 61 months, p=0.01). The mortality-related factors in the PH + ILD group were decreased initial FVC, high ePASP on echocardiography, low cardiac output on RHC, deteriorated functional class, and monotherapy., Conclusion: This is the first reported SSc-PH cohort from Turkey by a multidisciplinary team after the implementation of PAH-specific drugs. SSc-PH is a severe complication of SSc with high mortality especially in patients with accompanying severe ILD., (© Japan College of Rheumatology 2024. Published by Oxford University Press.)

Background: The six-minute walk test (6MWT) is widely used to assess functional capacity and prognosis in patients with idiopathic pulmonary fibrosis (IPF). However, studies on oxygen saturation recovery after the 6MWT in patients with IPF are rare. In our study, we investigated the relationship between oxygen saturation recovery time and dyspnea, fatigue, quality of life, prognostic markers and pulmonary hypertension (PH)., Methods: In this cross-sectional study, IPF patients diagnosed according to current guidelines and followed up in our Interstitial Lung Disease Outpatient Clinic between 2021 and 2022 were included. Demographics, data from spirometry, diffusion capacity measurement, arterial blood gas analysis, transthoracic echocardiography and the 6MWT were recorded. The oxygen saturation recovery time, distance saturation product (DSP), gender-age-physiology (GAP) index and composite physiological index (CPI) scores were calculated. Dyspnea severity was assessed by the modified Medical Research Council (mMRC) and Dyspnoea-12 (D-12) scales, fatigue severity by the Multidimensional Fatigue Inventory (MFI-20) and quality of life by the St George's Respiratory Questionnaire (SGRQ)., Results: Fifty IPF patients (34 men, 16 women, age: 66.8 ± 7.3 years) were included in the study. The mean FVC was 77.8 ± 19.3%, the DLCO was 52.9 ± 17.1%, the 6-minute walk distance (6MWD) was 385.7 ± 90.6 m, the GAP index was 3.5 ± 1.5, and the CPI was 43.7 ± 14.1. Oxygen saturation after the 6MWT reached pretest values at an average of 135.6 ± 73.5 s. The oxygen saturation recovery time was longer in patients with higher GAP index scores (Rs = 0.870, p < 0.001), CPI scores (Rs = 0.906, p < 0.001), desaturation (Rs = 0.801, p < 0.001), FVC%/DLCO% (Rs = 0.432, p = 0.002), sPAP (Rs = 0.492, p = 0.001), TRV (Rs = 0.504, p = 0.001), mMRC (Rs = 0.913, p < 0.001), MFI-20 (Rs = 0.944, p < 0.001), D-12 scale (Rs = 0.915, p < 0.001) and SGRQ scores (Rs = 0.927, p < 0.001); lower FVC (%) (Rs=-0.627, p < 0.001), DLCO (%) (Rs=-0.892, p < 0.001), PaO 2 (Rs=-0.779, p < 0.001), DSP (Rs=-0.835, p < 0.001), and 6MWD (Rs=-0.763, p < 0.001). A total of twenty patients (40%) exhibited an increased risk of PH. According to our multiple regression analysis, oxygen saturation recovery time was independently associated with the GAP index (p = 0.036), the lowest oxygen saturation occurring during the 6MWT (p = 0.011) and the SGRQ score (p < 0.001)., Conclusions: Our results showed that oxygen saturation recovery time is associated with dyspnea, fatigue, quality of life, increased risk of PH and prognostic markers in IPF. Therefore, we recommend continuous measurement of oxygen saturation after 6MWT until pretest values are reached., (© 2024. The Author(s).)

Pulmonary arterial hypertension (PAH) is driven by pathologies associated with increased metabolism such as pulmonary revascularization, vasoconstriction and smooth muscle cell proliferation in pulmonary artery wall. 18-fluorodeoxyglucose positron emission tomography (18FDG-PET) is an imaging technique sensitive to glucose metabolism and might be considered as a non-invasive method for diagnosis due to significant role of inflammation in idiopathic pulmonary artery hypertension (IPAH) and chronic thromboembolic pulmonary hypertension (CTEPH). The present study aimed to investigate the role of PET/CT imaging of patients with IPAH and CTEPH as an alternative diagnosis method. Demographic characteristics, FDG uptake in lungs, pulmonary artery and right ventricle (RV) of 17 patients (10 IPAH, 7 CTEPH), and 30 controls were evaluated. PET scanning, 6-min walk test, pro-BNP level, right heart catheterization of patients were performed both at the onsert and after 6-month PAH specific treatment. IPAH and CTEPH patients had significantly higher left lung FDG ( p  = 0.006), right lung FDG ( p  = 0.004), right atrial (RA) FDG ( p  < 0.001) and RV FDG ( p  < 0.001) uptakes than controls. Positive correlation was detected between the RV FDG uptake and the mean pulmonary artery pressure (mPAP) ( r  = 0.7, p  = 0.012) and between the RA FDG uptake and the right atrial pressure (RAP) ( r  = 0.5, p  = 0.02). Increased RV FDG and RA FDG uptakes predicts the presence of pulmonary hypertension and correlates with mPAP and RAP, respectively, which are important indicators in the prognosis of PAH. Further studies are required whether FDG PET imaging can be used to diagnose or predict the prognosis of pulmonary hypertension., Competing Interests: The authors declare no conflicts of interest., (© 2024 The Authors. Pulmonary Circulation published by John Wiley & Sons Ltd on behalf of Pulmonary Vascular Research Institute.)

Background: Annotation of earliest depolarization which depends on maximum dV/dt of unipolar-electrograms and unipolar QS morphology identify site of origin for ventricular premature contractions (VPC). However, identification of unipolar QS morphology has limitations due to low spatial resolution. This study aims to compare electrogram characteristics at successful ablation site in patients with outflow tract (OT) VPC., Methods: Local activation time (LAT), duration, and voltage data of each bipolar- and unipolar-electrogram at the successful ablation sites from the right ventricle OT (RVOT) and the left ventricle OT (LVOT) cases were analyzed., Results: Forty-four of 60 (73%) of patients were ablated from RVOT and in 16/60 (27%) required ablation from both sides. All patients had acute VPC suppression. Bipolar-electrogram-QRS onset was earlier (36.4 ± 14.5 ms vs 26.3 ± 7.4 ms, p = 0.01), duration of bipolar-electrogram was shorter (56.9 ± 18.9 ms vs 78.9 ± 21.8 ms, p = 0.002), and bi-voltage amplitude was higher (3.2 ± 2.3 mV vs 1.4 ± 1.1 mV, p = 0.07) for patients with RVOT-only ablation. Mean bipolar-unipolar-electrogram difference was 4.4 ± 4.5 ms in the RVOT group vs 12.8 ± 4.9 ms in RVOT + LVOT group (p < 0.001). Unipolar QS morphology was recorded in 3.0 ± 3.9 vs 3.6 ± 1.8 cm 2 in RVOT and RVOT + LVOT group, respectively (p = 0.41). Unipolar-electrogram revealed W pattern in 3/44 of RVOT vs 5/16 of RVOT + LVOT group, respectively (p = 0.01). In 18/60 (30%) of patients, unipolar QS was not identified at successful ablation site., Conclusion: QS in unipolar-electrogram was not a perfect predictor for successful ablation sites. Analysis of bipolar voltage amplitude and duration with bipolar-unipolar-electrogram time difference may identify presence of a deeper source., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Objective: The electromechanical window (EMW) was investigated as a new predictor of arrhythmia in the presence of long QT. However, the use of EMW to predict idiopathic frequent ventricular premature complexes (PVCs) in those with normal QT intervals has not been clarified., Patients and Methods: This single-center study included consecutive patients who presented to the Cardiology Clinic with palpitations and were found to have idiopathic PVC on 24-hour Holter monitoring. Those with a PVC/24-hour frequency of < 1% were defined as group 1, 1-10% as group 2, and > 10% as group 3. The EMW was defined as the time difference (in ms) between the aortic valve closure and the end of the QT interval, measured from an ECG on the concurrent echocardiogram., Results: A total of 148 patients were included in the study, 64% (n = 94) of which were female. The patients' mean age was 50.11 ± 14.7. The groups were similar in terms of the patients' age, BMI, and comorbidities. There was a statistically significant difference between the three groups in terms of the EMW measurements (group 1: 3.78 ± 19.6, group 2: -7 ± 30.9, group 3: -34.83 ± 55.2 ms: p < 0.001). In the multivariate regression analysis, the EMW (OR 0.971, p = 0.007) and every 10-ms decrease in the EMW (OR 1.254, p = 0.011) were thus determined to be independent predictors of PVC > 10%. An EMW value of ≤ -15 ms was associated with the frequency of 24-h PVC > 10%, with a sensitivity of 70% and a specificity of 70% (AUC 0.716, 95% CI: 0.636-0.787 p < 0.001)., Conclusions: The results showed that a negative increase in the EMW may be associated with frequent idiopathic PVCs.

This study aimed to determine the effect of ground-based walking training on exercise capacity, physical activity, quadriceps muscle strength, and quality of life (QoL) in patients with pulmonary hypertension. A total of 24 patients were included in the study. Patients were randomly assigned to 2 groups as the walking group or the control group. The walking group participated in 30-minute supervised ground-based walking training 2 days/week for 8 weeks. Also, they walked unsupervised at least 1 day/week. The control group received no intervention. The number of weekly steps taken in both groups was recorded using a pedometer. In addition to the sociodemographic and clinic characteristics of the patients, the endurance shuttle walk test, incremental shuttle walk test, and 6-minute walk test were used for the evaluation of exercise capacity, and an activity monitor and pedometer for physical activity, a dynamometer for quadriceps muscle strength, and emPHasis-10 for QoL. After 8 weeks, endurance capacity, maximal exercise capacity, and the number of steps significantly improved in the walking group (p <0.05). The 6-minute walk distance, physical activity, quadriceps muscle strength, and QoL were similar in both groups (p >0.05). The results of the study showed that ground-based walking could improve endurance capacity, maximal exercise capacity, and the number of steps. Quadriceps muscle strength also improved in the walking group. No adverse effects were reported during the training period. Ground-based walking training can be performed safely in patients with pulmonary hypertension., Competing Interests: Disclosures The authors have no conflicts of interest to declare., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: MicroRNAs are small, endogenous, non-coding RNAs that regulate the expression of many genes. It has recently been shown that circulating microRNAs may be biomarkers of hypertrophy and fibrosis in patients with hypertrophic cardiomyopathy (HCM)., Objective: To determine whether circulating levels of microRNAs involved in HCM are associated with electrocardiographic and echocardiographic parameters., Methods: This study enrolled 20 patients with familial HCM and 20 blood donors. Peripheral serum levels of miR-29a-3p, miR-199a-5p and miR-451a were assessed by quantitative real-time polymerase chain reaction and compared with levels in the control group. Whether circulating levels of miRNAs in HCM patients correlated with electrocardiographic and echocardiographic parameters was also assessed., Results: Median circulating levels of miR-29a and miR-451a were significantly higher in HCM than the control group. Median miR-199a levels did not differ between groups. However, circulating levels of miR-199a negatively correlated with corrected QT duration (Bazett formula). Median miR-29a levels positively correlated with QRS duration. In addition, circulating levels of miR-29a correlated with maximal wall thickness, left ventricular mass index and left atrial volume index., Conclusions: The data suggested that serum levels of miR-29a and miR-451a were significantly increased in HCM patients. As the circulating level of miR-29a correlated with QRS duration, left ventricular hypertrophy and left atrial dilatation, the serum miR-199a level negatively correlated with corrected QT duration. These miRNAs may be seen as potential biomarkers for further research in HCM pathophysiology., (Copyright © 2021 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.)

Objective: The aim of this cross-sectional, retrospective, descriptive study was to review and classify cardiac masses systematically and to determine their frequencies., Methods: The medical records of 64,862 consecutive patients were investigated within 12 years. Every patient with a cardiac mass imaged by transthoracic echocardiography (TTE) and confirmed with an advanced imaging modality such as transesophageal echocardiography (TEE), computed tomography (CT) and/or cardiac magnetic resonance imaging (CMR) was included. Acute coronary syndromes triggering thrombus formation, vegetations, intracardiac device and catheter related thrombi were excluded., Results: Data demonstrated 127 (0.195%) intracardiac masses consisting of 33 (0.050%) primary benign, 3 (0.004%) primary malignant, 20 (0.030%) secondary tumors, 3 (0.004%) hydatid cysts and 68 (0.104%) thrombi respectively. The majority of primary cardiac tumors were benign (91.67%), predominantly myxomas (78.79%), and the less malignant (8.33%). Secondary cardiac tumors were common than the primary malignant tumors (20:3), with male dominancy (55%), lymphoma and lung cancers were the most frequent. Intracardiac thrombi was the majority of the cardiac masses, thrombi accompanying malignancies were in the first range (n = 17, 25%), followed by autoimmune diseases (n = 13, 19.12%) and ischemic heart disease with low ejection fraction (n = 12, 17.65%)., Conclusions: This retrospective analysis identified 127 patients with cardiac masses. The majority of benign tumors were myxoma, the most common tumors that metastasized to the heart were lymphoma and lung cancers, and the thrombi associated with malignancies and autoimmune diseases were the most frequent., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2021 Published by Elsevier Inc.)

Objective: In this study, we aimed to compare the functional adaptations of the left ventricle in variant forms of left ventricular hypertrophy (LVH) and to evaluate the use of two-dimensional speckle tracking echocardiography (2D-STE) in differential diagnosis and prognosis., Methods: This was a prospective cohort study of 68 patients with LVH, including 20 patients with non-obstructive hypertrophic cardiomyopathy (HCM), 23 competitive top-level athletes free of cardiovascular disease, and 25 patients with hypertensive heart disease (HHD). All the subjects underwent 2D transthoracic echocardiography (TTE) and 2D-STE. The primary endpoint was all-cause mortality. Global longitudinal strain (GLS) below -12.5% was defined as severely reduced strain, -12.5% to -17.9% as mildly reduced strain, and above -18% as normal strain., Results: The mean LV-GLS value was higher in athletes than in patients with HCM and HHD with the lowest value being in the HCM group (HCM: -11.4±2.2%; HHD: -13.6±2.6%; and athletes: -15.5±2.1%; p<0.001 among groups). LV-GLS below -12.5% distinguished HCM from others with 65% sensitivity and 77% specificity [area under curve (AUC)=0.808, 95% confidence interval (CI): 0.699-0.917, p<0.001]. The median follow-up duration was 6.4±1.1 years. Overall, 11 patients (16%) died. Seven of these were in the HHD group, and four were in the HCM group. The mean GLS value in patients who died was -11.8±1.5%. LV-GLS was significantly associated with mortality after adjusting age and sex via multiple analysis (RR=0.723, 95% CI: 0.537-0.974, p=0.033). Patients with GLS below -12.5% had a higher risk of all-cause mortality compared with that of patients with GLS above -12.5% according to Kaplan-Meier survival analysis for 7 years (29% vs. 9%; p=0.032). The LV-GLS value predicts mortality with 64% sensitivity and 70% specificity with a cut-off value of -12.5 (AUC=0.740, 95% CI: 0.617-0.863, p=0.012)., Conclusion: The 2D-STE provides important information about the longitudinal systolic function of the myocardium. It may enable differentiation variable forms of LVH and predict prognosis.