(18)F-fluorodeoxyglucose uptake by positron emission tomography in patients with IPAH and CTEPH.

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.

Asthma-chronic obstructive pulmonary disease overlap: Results from a national-multicenter study.

Introduction: Patients with asthma-chronic obstructive pulmonary disease (COPD) overlap (ACO) have a greater disease burden than those with COPD or asthma alone. In this study, it was aimed to determine the prevalence, risk factors, and clinical features of ACO because there are limited national data in Türkiye. Materials and Methods: The study was conducted in a cross-sectional design in nine tertiary-care hospitals. The patients followed with a diagnosis of asthma or COPD for at least one year were enrolled in the study. The frequency of ACO and the characteristics of the patients were evaluated in the asthma and COPD groups. Result: The study included 408 subjects (F/M= 205/203, mean age= 56.24 ± 11.85 years). The overall prevalence of ACO in both groups was 20.8% (n= 85). The frequency was higher in the COPD group than in the asthma group (n= 55; 33.3% vs. n= 22; 9.8%), respectively (p= 0.001). Patients with ACO had similarities to patients with COPD in terms of advanced age, sex, smoking, exposure to biomass during childhood, being born in rural areas, and radiologic features. Characteristics such as a history of childhood asthma and allergic rhinitis, presence of chronic sinusitis, NSAID hypersensitivity, atopy, and high eosinophil counts were similar to those of patients with asthma (p<0.001). The annual decline in FEV1 was more prominent in the ACO group (mean= -250 mL) than in the asthma (mean change= -60 mL) and COPD (mean change= -230 mL) groups (p= 0.003). Conclusions: This study showed that ACO was common among patients with asthma and COPD in tertiary care clinics in our country. ACO should be considered in patients with asthma and COPD who exhibit the abovementioned symptoms.

Diagnosis and Treatment Patterns of Chronic Thromboembolic Pulmonary Hypertension in Russia, Kazakhstan, Turkey, Lebanon, and Saudi Arabia: A Registry Study.

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.

Impact of Promising Biomarkers on Severity and Outcome of Acute Pulmonary Embolism.

Background: Acute pulmonary embolism (APE) is a common clinical condition. Its severity ranges from asymptomatic radiological findings to fatal obstructive shock. The potential circulating biomarkers have been studied to predict APE outcomes. This study aimed to explore their predictive power on prognosis in APE. Material and Method: It was a prospective observational study between March 2008 and April 2010. All consecutive patients diagnosed with APE were categorized as massive/high-risk, submassive/moderate-risk, and non-massive/low-risk. Cardiac troponin T (cTnT), myoglobin, N-terminal pro-brain natriuretic peptide (NT-proBNP), heart-type fatty acid-binding protein (H-FABP), growth differentiation factor-15 (GDF-15), and D-dimer levels were measured. Results: Of these patients, 14 (29.8%), 16 (34.0%), and 17 (36.2%) patients were categorized as low-risk, moderate-risk, and high risk-patients, respectively. There was no significant difference between the patient groups categorized based on the risk stratification in terms of demographic and clinical characteristics. The cTnT, myoglobin, HFABP, and D-dimer levels have also not differed significantly between the groups. There was a significant difference between the groups in respect of NT-proBNP and GDF-15 levels (p=0.009 and p=0.037, respectively). Nine (19.1%) patients had died by the 3rd-month follow-up. Adverse events were seen in 26 (55.3%) patients. GDF-15 had the highest area under the curve (AUC) value for predicting any adverse event (cut-off value=9.3 ng/mL, AUC=0.796, CI (confidence interval) 95%: 0.653-0.899). NT-ProBNP was determined as the best predictor for mortality (cut-off value=229.2 pg/mL, AUC=0.889, CI 95%: 0.756-0.964). Conclusion: Higher levels of NT-proBNP and GDF-15 were found to be associated with more severe APE, worse outcomes, and mortality.

Common but neglected problem in chronic spontaneous urticaria: Sleep disturbance.

Background: Association of chronic spontaneous urticaria (CSU) with sleep disturbance has not been evaluated in studies that involve a large number of patients. Objective: In this study, we aimed to evaluate the sleep attitude and circadian rhythm in patients with CSU. Methods: As the patient group, recently diagnosed 100 patients with CSU, 100 patients with allergic rhinitis (AR) as the patient control group, and 100 healthy controls (HCs) were included. The Pittsburgh Sleep Quality Index (PSQI) questionnaire, sleep hygiene index (SHI), Epworth Sleepiness Scale (ESS) questionnaire, and the morningness-eveningness questionnaire (MEQ) were filled to assess sleep quality and circadian rhythm. CSU disease activity was evaluated by urticaria activity score-7 (UAS-7). Patients with concomitant diseases, e.g., psychiatric illnesses, that possibly affect sleep status or those who use related medications and at moderate or high risk of obstructive sleep apnea according to the STOP-Bang questionnaire were excluded from the study. Results: PSQI, SHI, and ESS scores were higher, and the MEQ score was lower in patients with CSU and patients with AR than those in the HCs (p < 0.001, for each score). However, the scores were not different among the patients with CSU and the patients with AR. UAS-7 was only correlated with PSQI scores (r = 0.402, p < 0.001). In addition, blood eosinophil counts and the serum C Reactive Protein (CRP) level were correlated with sleep quality (p = 0.02). Conclusion: The poor sleep quality, impaired sleep hygiene, increased daytime sleepiness, and intermediate type of circadian rhythm were observed in the patients with CSU and the patients with AR. Physicians should be aware of sleep problems in patients with CSU that might affect their quality of life and the success of their treatment.

Importance of oxidative stress in the evaluation of acute pulmonary embolism severity.

BACKGROUND: Pulmonary embolism (PE) is a common and potentially life-threatening disorder. Our study was aimed to investigate whether oxidative stress markers can be used as clinical markers in the evaluation of acute PE (APE) severity. METHODS: 47 patients with objectively documented diagnosis of APE were recorded. Of these patients, 14 had low-risk PE, 16 had moderate-risk PE, and 17 had high-risk PE. 21 healthy subjects were also enrolled in this study. Ischemia-modified albumin (IMA), prooxidants-antioxidants balance (PAB), advanced protein oxidation products (AOPPs), and ferric reducing antioxidant power (FRAP) were measured as oxidative stress parameters to evaluate the role of oxidative stress. RESULTS: In the low-risk and moderate-risk APE groups, AOPPs and PAB levels were significantly higher and FRAP levels were significantly lower than those in the control group. AOPPs and IMA levels in the patients with high-risk PE were significantly higher than those in both the low-risk and moderate-risk APE patients. There was a significant correlation between levels of AOPPs and the levels of both IMA (r: 0.462, p < 0.001) and PAB (r:0.378, p < 0.005). Serum FRAP levels were negatively correlated with PAB (r:- 0.683, p < 0.001) and AOPPs levels (r:- 0,384, p < 0.001). There was also a significant positive correlation between the serum IMA and PAB levels. CONCLUSIONS: We clearly demonstrated that reactive oxygen species formation is significantly enhanced in APE. IMA and AOPPs may be used as clinical markers in the evaluation of APE severity in clinical practice. However, further studies with larger patient populations and longer follow-up periods are required to confirm the mechanisms underlying these findings.

Effect of Ground-Based Walk Training in Pulmonary Hypertension.

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.

Clinical efficacy, safety, tolerability, and survival outcome of long-term inhaled iloprost treatment in the management of pulmonary arterial hypertension: Data from prospective multicenter observational OPTION study.

OBJECTIVE: To evaluate clinical efficacy, safety and tolerability of long-term inhaled iloprost treatment in the daily practice for the management of pulmonary arterial hypertension (PAH). METHODS: A total of 115 patients with PAH on inhaled iloprost treatment were included. New York Heart Association (NYHA) functional class, brain natriuretic peptide (BNP) and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels, and 6-minute walk distance (6MWD) were recorded at baseline and at 3rd to 24th month visits. Safety and tolerability of iloprost treatment were also evaluated during follow-up, as were the survival, clinical worsening, and the related risk factors. RESULTS: The treatment was associated with an increase in the percentage NYHA functional class II (from 0.0% at enrolment to 36.2% at 24th month visit) patients but no significant difference was noted in 6MWD values. Clinical worsening was observed in 63.5% patients, while survival rate was 69.6%. NT-proBNP levels were significantly higher in non-survivors than in survivors (p=0.042). Cox regression analysis revealed the association of female sex [odds ratio (OR)=0.318; 95% confidence interval (CI), 0.128-0.792; p=0.014] and scleroderma-related PAH (OR=0.347; 95% CI, 0.140-0.860; p=0.022) with significantly lower risk (3.14 fold and 2.88 fold, respectively) of mortality. CONCLUSION: Our findings indicate favorable efficacy, safety, and tolerability of long-term iloprost treatment in the management of PAH, whereas improved NYHA functional class was not accompanied with a significant change in 6MWD values. Patient age was a risk factor for clinical worsening, while female sex, scleroderma subtype, and lower NT-proBNP levels were associated with significantly lower mortality risk.

Factors affecting the recurrence in patients with venous thromboembolism: A retrospective cohort study.

BACKGROUND: The aim of the study was to evaluate the frequency of recurrence and the risk factors for recurrence in patients who were diagnosed with venous thromboembolism. METHODS: Between January 2005 and January 2015, a total of 412 venous thromboembolism patients (164 males, 248 females; mean age: 53.5±16.6 years; range: 19 to 95 years) were retrospectively analyzed. The demographics, underlying risk factors, comorbidities, imaging findings, and treatment data of the patients were recorded. RESULTS: At least one transient/permanent risk factor was found in 341 (82.7%) of the index events, and the other 71 (17.2%) were idiopathic. Recurrence developed in 76 (18.4%) of the patients. The duration of the treatment in the first event was significantly longer in recurrent cases (p=0.007). The recurrence rate in patients diagnosed with only deep vein thrombosis or patients diagnosed with pulmonary thromboembolism + deep vein thrombosis was significantly higher than the patients diagnosed with only pulmonary thromboembolism (24% vs. 14.2%, respectively; p=0.007). The rate of idiopathic venous thromboembolism was higher in recurrent cases than in non-recurrent cases (26.3% vs. 15.2%, respectively; p=0.028). At the end of the first year, the mean D-dimer levels were higher in recurrent cases (p=0.034). Hereditary risk factors were also higher in recurrent cases (39.5% vs. 19.3%, respectively; p=0.031). There was no significant correlation between recurrence and mortality. CONCLUSION: The presence of deep vein thrombosis, idiopathic events, high D-dimer levels at the end of the first year and hereditary risk factors seem to be associated with recurrence.

Frequency of Obstructive Sleep Apnea in Stage I and II Sarcoidosis Subjects Who Had No Corticosteroid Therapy.

OBJECTIVES: The number of studies on the frequency of obstructive sleep apnea (OSA) in subjects with sarcoidosis is low. Therefore, we aimed to investigate the frequency and predictors of OSA in subjects with clinically stable stage I and II sarcoidosis who were not taking corticosteroid and/or immunosuppressive drugs. We also evaluated restless legs syndrome (RLS) and periodic leg movements in sleep (PLMS). MATERIALS AND METHODS: Subjects with clinically stable stage I and II sarcoidosis and not receiving corticosteroid and/or immunosuppressive therapy were included in the study. Upper airway examination, lung function tests (forced vital capacity [FVC], forced expiratory volume in 1 second [FEV1], diffusing capacity of the lungs for carbon monoxide [DLCO]), and polysomnography were performed on all subjects. In addition, subjects' Epworth Sleepiness Scale (ESS) scores and the Pittsburgh Sleep Quality Index (PSQI) were recorded. RESULTS: Of the total number of 46 sarcoidosis subjects (35 women, 11 men; age: 44.4±10.7 years; body mass index (BMI): 29.3±5 kg/m2), 28 (60.9%) were detected with OSA (67.8% mild OSA). The recorded ESS score of the subjects was low (2.6±3.2), whereas the sleep quality was poor in 36.9% of these subjects. Rapid eye movements (REM) related OSA was diagnosed in 14.2% of the OSA subjects. Age was the only factor related to OSA diagnosis in a logistic regression analysis (p=0.048). None of the subjects were diagnosed with RLS and PLMS. CONCLUSION: OSA is common in stage I and II sarcoidosis subjects who did not receive corticosteroid therapy. The frequency of OSA diagnosis increases as the age of the subjects increases. Therefore, sarcoidosis subjects should be evaluated for OSA throughout the follow-up.

The relationship between T-wave peak-to-end interval and hemodynamic parameters in patients with pulmonary arterial hypertension.

BACKGROUND: T-wave peak-to-end interval (TPEI) is a measure of repolarization dispersion on surface electrocardiogram (ECG). TPEI has been reported as a prognostic parameter with heart disorders. In this study, we aimed to evaluate the relationship between echocardiogram-derived right heart parameters, right heart catheterization (RHC) measurements, and TPEI in patients with precapillary pulmonary arterial hypertension (PAH). METHODS: Thirty-eight patients (29 females and 9 males, mean age of 54.9 ± 10.9 years) who had undergone RHC for a preliminary diagnosis of pulmonary hypertension (PH) were included in the study. We performed transthoracic echocardiography (TTE), and resting 12-lead ECG was recorded before RHC. TPEI was measured from leads of V1-V6, DII, DIII, and aVF, and these values are averaged to obtain the global TPEI. RESULTS: Duration of TPEI was significantly correlated with mean PAP, pulmonary vascular resistance (PVR), and cardiac index (CI). Longer TPEI was associated with higher N terminal probrain natriuretic peptide (NT pro-BNP) level, lower 6-min walk distance (6MWD), and lower tricuspid annular plane systolic excursion (TAPSE). CONCLUSION: Prolongation of TPEI could be a new predictor of adverse outcome in PAH and may provide additional prognostic information for patients with PAH.

Survey of Physician's Knowledge, Attitudes, and Practices Regarding Idiopathic Pulmonary Fibrosis in Turkey.

OBJECTIVES: The aim of this study is to evaluate the approaches of Turkish pulmonologists to the diagnosis and treatment of idiopathic pulmonary fibrosis (IPF) in daily clinical practice. MATERIALS AND METHODS: A questionnaire containing 38 questions about the IPF diagnosis and treatment was given to pulmonologists between January 22 and 29, 2018, and the data of 158 physicians who responded to the questionnaire were evaluated. RESULTS: This survey showed that the mean number of patients that physicians followed up and managed annually was 8.3 and 5, respectively. The mean symptom duration before the diagnosis was 9-12 months. Patients were seen on average by three physicians prior to confirmed diagnosis. Almost 80% of the physicians have an opportunity to access a pathologist and radiologist specialized in IPF. However, only 26% of them have an opportunity to access regular multidisciplinary meetings. Although antifibrotics were the most commonly prescribed drugs, approximately 10% of patients were prescribed steroids, N-acetylcysteine, and immunosuppressants. Most of the physicians (81%) were aware of international guidelines; however, the Turkish Thoracic Society IPF Diagnosis and Treatment Consensus Report was read by only 41% of them. CONCLUSION: This survey may lead to the IPF awareness in Turkey, and it may help to close the gaps regarding the diagnosis and treatment.

A randomized controlled trial on inspiratory muscle training in pulmonary hypertension: Effects on respiratory functions, functional exercise capacity, physical activity, and quality of life.

BACKGROUND: Impaired respiratory muscle function may be one of the causes of increased dyspnea, reduced exercise capacity, and physical activity (PA), and poor quality of life in pulmonary hypertension (PH). OBJECTIVE: To investigate the effects of threshold inspiratory muscle training (TIMT) on respiratory functions, functional exercise capacity, PA, and QoL in patients with PH. METHODS: Thirty patients with PH were randomly allocated to a TIMT (n = 15) and sham group (n = 15). Three patients in the sham group could not participate in the program. The TIMT group (n = 15) trained at 30% of the maximal inspiratory pressure (MIP), and the sham group (n = 12) performed at lowest pressure without change in threshold pressure. In both groups, patients performed TIMT at home for 15 min, twice per day, with the MIP load determined by the trainer, and were supervised once weekly at the hospital for eight weeks. The primary outcomes were MIP and maximal expiratory pressure (MEP). The secondary outcome measures included spirometric measurements, six-minute walking distance (6MWD), PA (SenseWear armband and International Physical Activity Questionnaire-Short Form-IPAQ-Short Form), and QoL (Minnesota Living with Heart Failure-MLHF). RESULTS: After the training, changes in MIP (p = 0.023) were higher in the intervention group compared with the sham group. Differences in MEP, FEV1 (%), FVC (%), FEV1/FVC (%), 6MWD, %6MWD, IPAQ-SF, MLHFQ, and armband parameters were not significantly different between the groups (p > 0.05). CONCLUSIONS: The results of the study demonstrated that TIMT could increase MIP and did not improve other parameters of respiratory functions, functional exercise capacity, PA, and QoL in patients with PH.

Comparison of arterial and venous blood gases in patients with obesity hypoventilation syndrome and neuromuscular disease.

OBJECTIVES: Obesity hypoventilation syndrome (OHS) and some neuromuscular diseases (NMD) present with hypercapnic respiratory failure. Arterial blood gas (ABG) analysis is important in the diagnosis, follow-up, and treatment response of these diseases. However, ABG sampling is difficult in these patients because of excessive subcutaneous fat tissue, muscle atrophy, or contracture. The aim of this study is to investigate the value of venous blood gas (VBG), which is an easier and less complicated method, among stable patients with OHS and NMD. METHODS: The study included stable OHS and NMD patients who had been previously diagnosed and followed up between March 2017 and May 2017 in the outpatient clinic. ABG was taken from all patients in room air, and peripheral VBG was taken within 5 min after ABG sampling. RESULTS: Thirty-six patients with OHS and 46 patients with NMD were included in the study. There was a moderate positive correlation between arterial and venous pH values for all patients (r s = 0.590, P < 0.001). There were a strong and very strong positive correlations between arterial and venous pCO2 and HCO3 values (r s = 0.725 and r s = 0.934, respectively) (P < 0.001). There was no correlation between arterial and venous pO2 and saturation values. There was an agreement in Bland-Altman method for the values of ABG and VBG (pH, pCO2, and HCO3). CONCLUSIONS: There was a correlation between ABG and VBG values (pH, pCO2, and HCO3). VBG parameters (pH, pCO2, and HCO3) can be used safely instead of ABG parameters which have many risks, during treatment and follow-up of patients with OHS and NMD.

Leptin and adiponectin levels in obstructive sleep apnea phenotypes.

Objective: To examine the serum levels of leptin and adiponectin in different obstructive sleep apnea (OSA) phenotypes. Methods: Obese patients who were admitted to our sleep laboratory were included. All patients underwent spirometry, daytime arterial blood gas analysis, polysomnography and transthoracic echocardiography. Serum levels of adiponectin and leptin were recorded. Results: Analysis included 146 OSA patients (81 females, 65 males, age: 49.8 ± 10.7 years, body mass index: 40.3 ± 4.9 kg/m2, 47.9% severe OSA, 42.5% severe obesity). Females had higher leptin and adiponectin levels (p < 0.001; p < 0.001, respectively). Leptin levels were higher in patients with severe obesity (p < 0.001). Severe OSA patients had lower leptin and adiponectin levels (p = 0.023; p = 0.035, respectively). Conclusion: Adipokine levels were different especially in OSA patients with severe obesity, female gender and severe OSA.

Validity and reliability of the Turkish version of the EmPHasis-10 questionnaire in patients with pulmonary hypertension.

RATIONALE, AIMS, AND OBJECTIVES: The aim of the study was to evaluate the reliability and validity of the Turkish version of the EmPHasis-10 questionnaire to ensure cultural adaptation. METHODS: This study involved translation, back translation, and cross-cultural adaptation. One hundred and one patients who were diagnosed as having pulmonary hypertension (PH) for at least 6 months were evaluated using the Turkish version of EmPHasis-10. Turkish version of the Minnesota Living with Heart Failure Questionnaire (MLHFQ) was used as gold standard to assess the validation of the Turkish version of the EmPHasis-10 questionnaire. Relationship between MLHFQ and EmPHasis-10 was analysed using Spearman correlation analysis to assess the validation. Cronbach alpha (internal consistency) and exploratory factor analyses were used to assess the questionnaire's reliability. RESULTS: The statistical analysis showed that the EmPHasis-10 questionnaire showed a high validity with MLHFQ (r = 0.85) (P = 0.001). Reliability analysis showed that EmPHasis-10 had a high level of Cronbach alpha (α = 0.98) and internal consistency (ICC = 0.97). CONCLUSIONS: The Turkish version of EmPHasis-10 is a quality of life questionnaire specific to PH. It has a high-level validity and reliability questionnaire that can be used by researchers and physicians.

Respiratory muscle strength in patients with pulmonary hypertension: The relationship with exercise capacity, physical activity level, and quality of life.

INTRODUCTION: Pulmonary hypertension (PH) is characterized by exertional dyspnea, fatigue, chest pain, dizziness, and syncope. Physical activity, peripheral, and respiratory muscle strength reduces in pateints with PH. Little is known about respiratory muscle weakness and related outcomes. OBJECTIVES: The aims of the study were to determine respiratory muscle strength and to investigate the relationship between respiratory muscle strength and spirometric measurements, exercise capacity, physical activity level, quality of life, and pulmonary hemodynamics in patients with PH. METHODS: In total, 33 patients aged 25-80 years who were diagnosed as having PH and 24 healthy volunteers were included in the study. To measure respiratory function, spirometry, maximal inspiratory (MIP), and expiratory pressures (MEP) were used. Physical activity level was determined with activity monitoring (SenseWear Armband) and the International Physical Activity Questionnaire-Short Form. Exercise capacity was determined using the 6-minute walk test. Quality of life was evaluated with the Minnesota Living with Heart Failure Questionnaire (MLHFQ). RESULTS: Maximal inspiratory pressure and MEP values of the patients with PH were significantly lower than the age- and sex-matched healthy controls (P < .0001). Significant relationships were found between the MIP and six MWD (r = .40, P = .02), vigorous physical activity (r = .38, P = .03), moderate physical activity (r = 61, P < .001), and arm band-average metabolic equivalent (r = .39, P = .02). CONCLUSION: The relationship between maximum inspiratory pressure, exercise capacity, and physical activity level showed that a decrease in exercise capacity or physical activity level may be a predictor for decreased MIP.

Fractional Exhaled Nitric Oxide Measurement in Pulmonary Hypertension: A Follow-Up Study.

Pulmonary hypertension (PH) is a fatal disease although significant improvements in treatment are achieved. Easily implemented and noninvasive prognostic techniques are needed while following-up these patients. The aim was to investigate the role of fractional exhaled nitric oxide (FeNO) in follow-up for patients with PH. In this longitudinal study, patients with pulmonary arterial hypertension (PAH) and chronic thromboembolic PH (CTEPH) who were seen in PH Outpatient Clinic, Istanbul Faculty of Medicine, Istanbul University, were enrolled in the study. Echocardiography, 6-minute walking test, brain natriuretic peptide, and FeNO measurements were performed, and World Health Organization functional class was evaluated to all patients at baseline, and third, and sixth months. Right-heart catheterization and pulmonary function tests at the time of diagnosis were recorded. The study comprised 31 patients (23 women, 8 men; mean age: 53.4 ± 17.1 years) with PAH (n = 19) and CTEPH (n = 12) and 80 healthy controls. Patients with PH had lower FeNO values than the control group (16.5 ppb vs 19.8 ppb; P < .05). Fractional exhaled nitric oxide values did not change during follow-up and did not correlate with other follow-up measures except tricuspid annular plane systolic excursion values. Fractional exhaled nitric oxide was higher in the idiopathic PAH subgroup at baseline and at third month than patients with PAH associated with other diseases. Fractional exhaled nitric oxide did not change in patients who had clinical deterioration. As a conclusion; Patients with PH had lower FeNO values than healthy controls, but FeNO did not change significantly during follow-up. Large-scale studies with prolonged follow-up periods are needed to understand the role of FeNO in the follow-up of the patients with PH.

The role of an activity monitor in the objective evaluation of patients with pulmonary hypertension.

INTRODUCTION: Patients with pulmonary hypertension (PH) show no symptoms at rest, but symptoms are triggered by physical activities. OBJECTIVES: The primary aim of our study was to assess physical activity of patients with PH by using an activity monitor. The secondary aim was to evaluate the correlation between the activity monitor parameters and 6-min walk distance (6MWD), activity of daily living (ADL), quality of life, WHO functional class and PH classification. METHODS: Thirty-eight patients with pulmonary arterial hypertension (PAH) and seven patients with chronic thromboembolic PH were included in the study. Physical activity was assessed using a SenseWear arm band. A 6-min walk test was performed. Daily living activities were assessed using the 'nottingham extended activity of daily living index' (NEADL). For quality of life assessments, 'Minnesota living with heart failure' (MLHF) and 'Short form-36' (SF-36) surveys were used. RESULTS: Physical activity, exercise capacity, quality of life and contribution to ADL were lower in patients with PH. These data were associated with the 6MWD, quality of life and ADL index scores. We also found weak and moderate correlations between activity monitor data and SF-36 sub-group scores, MLHF and NEADL scores (P < 0.05). For the group with PAH, idiopathic PAH patients had more exercise capacity and total energy expenditure compared with patients with scleroderma-associated PAH. CONCLUSION: Correlation between activity monitor data and 6MWD, most of SF-36 sub-group scores, MLHF scores and NEADL index scores suggest that activity monitor can be used in the evaluation of patients with PH.

Follow-up of 1887 patients receiving tumor necrosis-alpha antagonists: Tuberculin skin test conversion and tuberculosis risk.

OBJECTIVES: To evaluate the characteristics of patients who developed tuberculosis while receiving tumor necrosis factor-alpha (TNF-α) antagonists and the related factors with tuberculosis. METHODS: Patient's demographics, tuberculin skin test (TST), isoniazid prophylaxis and type of TNF-α antagonist were recorded. TST conversion (≥5 mm increase) was evaluated for patients who had baseline and 1-year TST. RESULTS: Files of 1887 patients who were receiving TNF-α antagonists between August 2005 and June 2015 were evaluated. TST significantly increased at the end of 1 year (n = 748 baseline:7.36 ± 7.2 mm vs. 1 year:9.52 ± 7.5 mm, P < 0.001). One-third of patients (31.2%) who had negative TST at baseline had positive TST at 1 year. Tuberculosis developed in 22 patients (1.16%). The annual incidence of tuberculosis was 423/100 000 patient-year. TNF-α antagonist indications were ankylosing spondylitis (n = 8), inflammatory bovel diseases (n = 7) and rheumatoid arthritis (n = 4). Ten (45.5%) patients received infliximab, six (27.3%) patients received etanercept and six (27.3%) patients received adalimumab. Nineteen (86.4%) patients were under isoniazid prophylaxis. Twelve patients had extrapulmonary tuberculosis (54.5%; four lymph node, three pleura, two periton, one pericarditis, one intestinal, one joint). Atypical mycobacterium was detected in one patient. Adalimumab treatment (9.5× increase), male sex (15.6× increase) and previous tuberculosis disease history (11.5× increase) were risk factors for active tuberculosis. Conversion of TST was not found related with tuberculosis. CONCLUSIONS: Despite the high proportion of isoniazid prophylaxis, the incidence of tuberculosis in our patients receiving TNF-α antagonist was higher than the literature. Adalimumab treatment, male sex and previous tuberculosis disease history were found as risk factors for tuberculosis.