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.

Heritable Pulmonary Arterial Hypertension Diagnosed during the Postpartum Period: A Case Report and Literature Review.

Approximately one-third of bone morphogenic protein receptor-2 (BMPR2) mutation carriers develop pulmonary arterial hypertension (PAH), which indicates that additional risk factors are needed for the manifestation of the disease. It is questionable whether pregnancy is a risk factor for PAH development in these patients. We represent a 30-year-old woman with a heterozygous BMPR2 mutation who was diagnosed with PAH during the postpartum period and reviewed the literature in this report. We also discussed the possible underlying mechanisms that might have resulted in PAH development during pregnancy in BMPR2 mutation carriers.

The Usefulness of the TAPSE/sPAP Ratio for Predicting Survival in Medically Treated Chronic Thromboembolic Pulmonary Hypertension.

OBJECTIVE: The ventriculoarterial uncoupling has been linked with unfavorable results as measured noninvasively by tricuspid annular plane systolic excursion divided by systolic pulmonary artery pressure (TAPSE/sPAP). However, its prognostic importance in chronic thromboembolic pulmonary hypertension (CTEPH) is limited. Thus, we determine the effect of the TAPSE/sPAP ratio on outcomes and predictors of all-cause mortality in these patients. METHODS: We analyzed 56 subjects with medically treated CTEPH. Two-dimensional echocardiographic examination and right heart catheterization findings were recorded from the hospital database. Baseline New York Heart Association functional class (NYHA-FC), 6-min walk distance (6MWD), and brain natriuretic peptide (BNP) test results were recorded. RESULTS: The median age was 65.5 years. Over a median follow-up time of 27 months, 29 (51.8%) patients died. BNP values were higher (P = 0.008), 6MWD values were lower (P = 0.004), and NHYA-FC (P = 0.0001) was worse in the non-survivor group. TAPSE (P = 0.0001) and TAPSE/sPAP ratio (P = 0.001) were significantly lower and pulmonary vascular resistance (PVR) was higher in the non-survivor group (P = 0.03). The best cut-off value for the TAPSE/sPAP ratio for predicting mortality was 0.20 mm/mmHg and the survival rates were significantly lower in the TAPSE/sPAP ratio ≤0.20 group (log-rank P = 0.012). 6MWD (P = 0.005), NHYA-FC III-IV (P = 0.0001), TAPSE/sPAP ratio ≤0.20 (P = 0.017), PVR (P = 0.008), and TAPSE/sPAP ratio ≤0.20 combined with NYHA-FC III-IV (P = 0.0001) were significant determinants and TAPSE/sPAP ratio ≤0.20 combined with NYHA-FC III-IV was the only independent predictor of mortality (P = 0.003). CONCLUSION: Medically treated CTEPH patients with a TAPSE/sPAP ratio ≤0.20 had lower survival rates. TAPSE/sPAP ratio≤0.20 combined with NYHA-FC III-IV was the independent predictor of poor prognosis.

Effects of Inspiratory Muscle Training in Patients With Pulmonary Hypertension.

The study aimed to examine the effects of inspiratory muscle training (IMT) in patients with pulmonary hypertension (PH). A total of 24 patients with PH were included in the randomized controlled evaluator-blind study. IMT was performed at 40% to 60% of the maximal inspiratory pressure for 30 min/d, 7 d/wk (1 day supervised) for 8 weeks. Respiratory muscle strength, dyspnea, diaphragm thickness (DT), pulmonary functions, 24-hour ambulatory blood pressure (BP), arterial stiffness, exercise capacity, upper extremity functional exercise capacity, physical activity levels, fatigue, anxiety-depression levels, activities of daily living (ADL), and quality of life were evaluated. A total of 24 patients (treatment = 12, control = 12) completed the 8-week follow-up. There was no significant difference between the patient groups in terms of demographic and clinical characteristics (p >0.05). Considering the change between the groups in the treatment and control groups, brachial and central BP, dyspnea, respiratory muscle strength, DT in total lung capacity, knee extension muscle strength, functional exercise capacity, upper extremity functional exercise capacity, physical activity, ADL, fatigue, anxiety, and quality of life improved in favor of the IMT group (p <0.05). In conclusion, IMT has improved brachial and central BP, dyspnea, respiratory muscle strength, DT in total lung capacity, knee extension muscle strength, functional exercise capacity, upper extremity functional exercise capacity, physical activity, ADL, fatigue, anxiety, and quality of life compared with the control group. IMT is an effective method in cardiopulmonary rehabilitation for patients with PH.

Obstructive Sleep Apnea and Cardiovascular Disease: Where Do We Stand?

Obstructive sleep apnea is common in adults with cardiovascular disease. Accumulating evidence suggests an association between obstructive sleep apnea and cardiovascular disease independent of the traditionally recognized cardiovascular disease risk factors. Observational studies indicate that obstructive sleep apnea is a risk factor for development of cardiovascular disease and that alleviation of obstructive events with positive airway pressure may improve cardiovascular disease outcomes. However, recent randomized controlled trials have not supported the beneficial effect of positive airway pressure in cardiac populations with concomitant obstructive sleep apnea. Some evidence suggests that the relationship between obstructive sleep apnea and traditionally recognized cardiovascular disease risk factors is bidirectional, suggesting that patients with cardiovascular disease may also develop obstructive sleep apnea and that efficient treatment of cardiovascular disease may improve obstructive sleep apnea. Recent data also indicate that the apnea-hypopnea index, which is commonly used as a diagnostic measure of obstructive sleep apnea severity, has limited value as a prognostic measure for cardiovascular disease outcomes. Novel markers of obstructive sleep apnea-associated hypoxic burden and cardiac autonomic response seem to be strong predictors of adverse cardiovascular disease outcomes and response to treatment of obstructive sleep apnea. This narrative review and position paper from the Turkish Collaboration of Sleep Apnea Cardiovascular Trialists aims to update the current evidence about the relationship between obstructive sleep apnea and cardiovascular disease and, consequently, raise awareness for health professionals who deal with cardiovascular and respiratory diseases to improve the ability to direct resources at patients most likely to benefit from treatment of obstructive sleep apnea and optimize treatment of the coexisting cardiovascular diseases. Moreover, the Turkish Collaboration of Sleep Apnea Cardiovascular Trialists aims to contribute to strengthening the efforts of the International Collaboration of Sleep Apnea Cardiovascular Trialists in this context.

Effects of high intensity interval-based inspiratory muscle training in patients with heart failure: A single-blind randomized controlled trial.

BACKGROUND: Given the promising effects of inspiratory muscle training (IMT), determining the most appropriate IMT protocol will optimize the training benefits. OBJECTIVES: The objective of this study was to determine the effects of high intensity interval-based inspiratory muscle training (H-IMT) on cardiovascular, pulmonary, physical, and psychosocial functions in patients with heart failure and reduced ejection fraction (HFrEF). METHODS: Thirty-four patients with HFrEF were randomly assigned to the H-IMT or control group for 3 days/week, 8 weeks training period. The H-IMT group performed IMT at least 70% of the maximal inspiratory pressure, whereas the control group performed unloaded IMT. Each session occurred 7 sets with a total of 21 min consisting of 2-min training and 1-min interval. Heart rate variability (HRV), arterial stiffness, respiratory muscle strength and endurance, diaphragm thickness, quadriceps strength, functional capacity, frailty, dyspnea, fatigue, disease-specific health-related quality of life (HRQoL), and generic HRQoL were evaluated at baseline and after 8 weeks training period by blinded assessors. RESULTS: Statistically significant between-group differences were observed in the time domain parameters of HRV, arterial stiffness, inspiratory and quadriceps muscle strength, respiratory muscle endurance, diaphragm thickness, functional capacity, frailty, dyspnea, fatigue, and disease-specific HRQoL in favor of the H-IMT group (p<0.05). CONCLUSIONS: H-IMT is an effective protocol for improving cardiac autonomic function, arterial stiffness, inspiratory and quadriceps muscle strength, respiratory muscle endurance, diaphragm thickness, functional capacity, frailty, dyspnea, fatigue, and disease-specific quality of life in patients with HFrEF. CLINICAL TRIAL REGISTRATION: NCT04839211.

Comparison of physiological responses after incremental shuttle walking test and 6-minute walk test in patients with systemic sclerosis.

BACKGROUND: The 6­minute walk test (6MWT) is a commonly used to evaluate exercise capacity in patients with systemic sclerosis (SSc), but there was no study using the incremental shuttle walking test (ISWT) for assessing exercise capacity and comparing the patient's cardiorespiratory responses to these tests. The aim was to investigate the usability and determinants of the ISWT in patients with SSc and compare the physiological responses after the ISWT and 6MWT. METHODS: A total of thirty four female patients with SSc were included. Dyspnea during daily activities and knee extensor muscle strength was assessed, skin fibrosis and disease severity were recorded, and 6MWT and ISWT were carried out for the exercise capacity measurement. Pulmonary function test results were recorded from the individuals' medical records for SSc with interstitial lung disease (SSc-ILD) patients. RESULTS: The ISWT distance was significantly correlated with the 6MWT distance (p < 0.001). The 6MWT was correlated with age, modified Rodnan skin score, Medsger severity score, modified British Medical Research Council Questionnaire (mMRC) score, and knee extensor muscle strength (p < 0.05). The 6MWT was correlated with the forced expiratory volume in the first second (FEV1) (lt) and forced vital capacity (FVC) (lt) in patients with SSc-ILD (p < 0.05). The ISWT distance was correlated with age, modified Rodnan skin score, mMRC score, and knee extensor muscle strength (p < 0.05). Age, mMRC, and knee extensor muscle strength explained 33.8% of the variance in 6MWT distance, while age, mMRC, and knee extensor muscle strength explained 51.7% of the variance in the ISWT distance. CONCLUSION: Because of the higher cardiopulmonary responses, and having a more standardized procedure, the ISWT may be preferable for investigating symptom-limited exercise capacity in patients with SSc. Age, dyspnea, and knee extensor muscle strength were the determinants of exercise capacity in patients with SSc.

The Comparison of Quantitative Evaluation Results of the MPS SPECT/CT and Coronary Angiography: Determining the Most Valuable Quantitative Evaluation Score

Objectives: This study aimed to determine the most important perfusion score in patient selection for coronary angiography (CA) by quantitatively evaluating myocardial perfusion scintigraphy (MPS). Methods: Patients who underwent MPS single-photon emission computerized tomography/computed tomograph imaging in our clinic between December 2017 and January 2019, without coronary artery disease (CAD) history, followed by CA were included in the study. CA was considered positive when there is a stenosis of 70% or more in at least one coronary vessel. The summed stress score, rest score, and differential score; total perfusion deficit (TPD); and the defect's extent obtained from non-attenuation-corrected (NC) and attenuation-corrected (AC) images of 80 patients were evaluated using the Mann-Whitney U test. A p value of <0.05 was considered significant. Receiver operating characteristic (ROC) analysis was performed. Results: The scores obtained from NC and AC images showed a significant difference between the two groups for all scores except for the extent and TPD scores at rest from AC images. The applied ROC curves' highest diagnostic value was determined as the TPD score at stress (TPDS) obtained from NC images (area under the curve: 0.880, 95% confidence interval, 0.807-0.952, p<0.001). The cut-off value obtained for the TPDS from the ROC curve was found to be 5.5. Conclusion: The scores obtained from NC images have more power to detect CAD than those obtained from AC images. Patients with no prior CAD history with TPDS score higher than 5 in MPS should be referred for CA with priority.

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.

Determinants of gait speed in patients with heart failure with reduced ejection fraction.

OBJECTIVE: Given the prognostic significance of gait speed, there is insufficient evidence about possible functional determinants of gait speed in patients with heart failure with reduced ejection fraction (HFrEF). Therefore, the objective of this study was to investigate the functional determinants of gait speed in patients with HFrEF. METHODS: Fifty-nine patients with HFrEF participated in this cross-sectional study. Demographic and clinical characteristics were recorded. The gait speed was determined with a 4-meter walking test. Dyspnea perception was assessed with the modified medical research council (mMRC) scale. Functional capacity was evaluated with a 6-minute walk test (6MWT). The five times sit-to-stand (5-STS) test and the Berg Balance Scale (BBS) were used to measure functional mobility and balance. Physical activity was evaluated with the International Physical Activity Questionnaire (IPAQ) Short-Form. RESULTS: Gait speed was correlated with age (r=-0.368, p=0.004), NYHA functional class (r=-0.438, p=0.001), mMRC score (r=-0.422, p=0.001), 6MWT (r=0.650, p<0.001), 5STS (r=-0.506, p<0.001), BBS (r=0.586, p<0.001), IPAQ (r=0.305, p=0.019) and IPAQ-Sitting time (r=-0.327, p=0.011). On multiple linear regression analysis, the 6MWT distance and BBS were independent determinants of the usual gait speed in patients with HFrEF, accounting for 44.4% of the variance. CONCLUSION: This study indicates that functional capacity and balance are independent functional determinants of gait speed in patients with HFrEF.

Assessment of psychometric properties, cross-cultural adaptation, and translation of the Turkish version of the ICU mobility scale

Background/aim: The aim of the study was to carry out the cultural adaptation and translation of the ICU mobility scale (IMS) into Turkish and research the psychometric properties. Materials and methods: This study was based on methodological design. The IMS was translated from English to the Turkish through a regularised translation process. Two physiotherapists assessed patients independently in the coronary intensive care unit. The measures such as construct validity, intra and interrater reliability, and internal consistency of the IMS Turkish version were assessed. Results: A total of 70 intensive care patients were included in the study. The intrarater and interrater reliability of the IMS was excellent. The weighted Kappa value was 0.92 (0.87­0.96) for the intrarater reliability, and 0.87 (0.80­0.93) for the interrater reliability. There were significant correlations between the IMS and functional status score for the intensive care unit (r = 0.83), Perme intensive care unit mobility score (r = 0.84), Katz activities of daily living (r = 0.73), handgrip strength (r = 0.62), knee extension strength (r = 0.46), and age (r = ­0.44). Conclusion: This study suggests that the IMS Turkish version is a reliable and valid scale for assessing functional status and mobility level in ICU patients.

Balance performance in patients with heart failure.

BACKGROUND: It has been suggested that patients with heart failure (HF) have an increased fall rate. Although balance is one of the most important risk factors for fall, there is not sufficient information about balance in HF. OBJECTIVE: To compare static, dynamic and functional balance between patients with HF and healthy controls. METHODS: Twenty-seven patients with HF and 22 healthy controls were recruited in this study. The Unilateral Stance (US) and Limits of Stability (LOS) tests were used to measure static and dynamic balance, respectively. Functional balance was assessed with Berg Balance Scale. RESULTS: There was no significant difference in age, gender and body mass index between the groups (p > 0.05). There was a significant difference in US with open eyes between the groups (p < 0.05). Reaction time (backward and left), endpoint excursion (backward), maximum excursion (forward and backward) and directional control (forward and right) variables of LOS were significantly different between the groups (p < 0.05). CONCLUSIONS: Patients with HF have impaired static, dynamic and functional balance. Considering the balance impairment, a comprehensive balance assessment performed and balance training should be included in the management of HF as a part of the cardiac rehabilitation program.

Effects of neuromuscular electrical stimulation in patients with pulmonary arterial hypertension: a randomized controlled pilot study.

BACKGROUND: Patients with pulmonary arterial hypertension (PAH) present impairments in muscle strength and exercise capacity. There is growing evidence about the benefits of neuromuscular electrical stimulation (NMES) in patients with respiratory diseases, except in patients with PAH. The aim of this study was to investigate the effects of NMES on muscle strength, and other physical and psychosocial variables in patients with PAH. METHODS: Patients with PAH were randomly divided into two groups as NMES and control. The NMES was applied to the bilateral deltoid and quadriceps femoris muscles with 50 Hz for 3 days/week, 8 weeks for the NMES group. Muscle strength, muscle cross-sectional area and thickness, arterial stiffness, exercise capacity, functional mobility and balance, balance confidence, fatigue, physical activity, and quality of life were assessed at baseline and after 8 weeks by blinded assessors. RESULTS: There was no significant difference in the demographic and clinical characteristics between the patient groups (p > 0.05). The improvements in muscle strength, muscle cross-sectional area and thickness, pulse wave velocity, exercise capacity, functional mobility and balance, balance confidence, fatigue, physical activity, and quality of life were significantly higher in the NMES group compared to the control group (p < 0.05). CONCLUSIONS: This study suggests that NMES intervention is safe and effective for patients with PAH.

Test-retest reliability and validity of the timed up and go test and 30-second sit to stand test in patients with pulmonary hypertension.

BACKGROUND: Timed up and go (TUG) and sit to stand (STS) tests that required less space and easier to be performed in respiratory and cardiac diseases for assessing functionality. Aim was to test the reliability of TUG and 30-second STS (30STS) tests and determine the validity of TUG and 30STS tests in patients with Pulmonary Hypertension (PH). METHODS: Thirty-eight patients with diagnosed PH were included. We collected TUG, 30STS, quadriceps muscle strength, physical activity level, and 6MWT. Intra-class correlation coefficient (ICC) was used to determine test-retest reliability and correlations with quadriceps muscle strength, physical activity level and 6MWT for validity of the TUG and 30STS tests. RESULTS: The TUG and 30STS tests were associated with age, functional class, muscle strength, physical activity and functional exercise capacity in patients with PAH (p < 0.05). 6MWT was associated with age, functional class, muscle strength, physical activity and functional exercise capacity (p < 0.05). ICC (95%) for TUG test and 30STS were 0.96 (0.93-0.98) and 0.95 (0.90-0.97), respectively. CONCLUSIONS: The TUG and 30STS tests were reliable and valid tests for measuring physical performance in PH. This study supports using the TUG and 30STS tests as practical assessment tools in patients with PH.

Turkish translation, cross-cultural adaptation, and assessment of psychometric properties of the Functional Status Score for the Intensive Care Unit.

Purpose: To translate and cross-culturally adapt the Functional Status Score for the Intensive Care Unit instrument to Turkish and investigate its psychometric properties.Methods: An expert committee supervised forward and backward translation. Thirteen participants reviewed the pre-final version of Turkish Functional Status Score for the Intensive Care Unit instrument providing minor revisions to improve its readability. Two physiotherapists assessed patients (N = 50) from a coronary intensive care unit using the Turkish Functional Status Score for the Intensive Care Unit instrument.Results: Internal consistency was excellent (Cronbach's α = 0.949). Inter-rater reliability and intra-rater reliability were excellent for each of five functional tasks and total scores (intra-class correlation coefficient = 0.955-0.996). The Turkish Functional Status Score for the Intensive Care Unit score had moderate to high correlations with other functional measures as follows: Perme Intensive Care Unit Mobility Score (Spearman's r = 0.92), Katz Activities of Daily Living (r = 0.80), handgrip strength (r = 0.76-0.77), and knee extension strength (r = 0.70-0.71).Conclusion: The Functional Status Score for the Intensive Care Unit instrument was translated and culturally adapted to Turkish and demonstrated strong psychometric properties, including internal consistency, intra-rater and inter-rater reliability, construct validity, and floor and ceiling effects.Implications for rehabilitationRehabilitation professionals strive to assess and document patient status using validated and reliable outcome measures as part of good clinical practice.Longitudinal evaluation of physical function in the intensive care units is important.The Functional Status Score for the Intensive Care Unit is a validated and reliable physical functioning measurement instrument suitable for the intensive care units.The Functional Status Score for the Intensive Care Unit was translated and culturally adapted to Turkish, and demonstrated strong psychometric properties, including internal consistency, intra-rater reliability, inter-rater reliability, construct validity, and floor and ceiling effects.

Whole blood viscosity in systemic sclerosis: a potential biomarker of pulmonary hypertension?

OBJECTIVE: Our goal was to determine if whole blood viscosity (WBV) can be used to predict the risk of pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc). METHODS: Patients with SSc were analyzed. Out of 107 patients, 26 patients, found to have confirmed diagnosis of PAH, were classified as those with (n = 26, PAH group) and without PAH (n = 81, non-PAH group). We calculated estimated WBV at both high (HSR) and low shear rates (LSR) from hematocrit and total serum protein levels. RESULTS: Total protein levels were significantly higher and the anti-centromere antibody (ACA) was more frequent in the PAH group. Furthermore, anti-topoisomerase antibody (anti-scl-70) was significantly less frequent in the PAH group. The WBV values were significantly higher at HSR (16.68 ± 0.38 vs. 16.24 ± 0.58; p < 0.001) and at LSR (51.81 ± 7.21 vs. 42.97 ± 11.76; p < 0.001) in PAH group. The multivariate analysis revealed that the WBV at both shear rates independently designated the presence of PAH in SSc patients. The ROC curve showed that the sensitivity and specificity of LSR and HSR were 92.3% and 61.7% (AUC 0.759, p < 0.001), and 88.5% and 65.4% (AUC 0.770, p < 0.001) with a cutoff value of 43.56 and 16.32 for WBV, respectively. CONCLUSION: Higher WBV levels in SSc patients were an independent indicator for PAH development in this cohort. WBV-LSR and WBV-HSR values might help exclude the PAH possibility in patients diagnosed with SSc and remain as an independently associated biomarker for follow-up of these patients for future risk of PAH development. Findings remain to be confirmed by other cohorts.Key Points• The most important cause of morbidity and mortality in systemic sclerosis patients is considered to be pulmonary arterial hypertension.• When the symptoms of PAH are not recognized earlier in the course of the SSc, the prognosis might be worse.• Higher whole blood viscosity levels in scleroderma patients with PAH was an independent indicator for PAH development.

Association Between Non-Dipping and Fragmented QRS Complexes in Prehypertensive Patients / Associação entre Ausência de Descenso da Pressão Arterial Durante o Sono e a Fragmentação do Complexo QRS em Pacientes Pré-Hipertensos

Abstract Background: Fragmented QRS (fQRS) is a sign of adverse cardiovascular events in various cardiovascular diseases. It is also associated with increased blood pressure and non-dipping in hypertensive patients. However, no study has investigated the importance of fQRS in prehypertensive patients. Objectives: The aim of our study is to investigate the relationship between fQRS and non-dipper status in prehypertensive patients. Methods: Two hundred and sixteen eligible, newly diagnosed prehypertensive patients who underwent 24-hour ambulatory blood pressure monitoring (ABPM) for further evaluation of blood pressure between June 2015 and July 2016 were included into the study. Patients were divided into three groups according to ABPM results: normotensives, dipper prehypertensives, and non-dipper prehypertensives. Groups were compared regarding presence of fQRS on electrocardiography. Additionally, multinomial logistic regression analysis was used to determine the relationship between fQRS and blood pressure pattern in prehypertensive patients. Results: According to ABPM recordings, 61 patients had normotensive blood pressure pattern (systolic blood pressure < 120 mmHg and diastolic blood pressure < 80 mmHg). Of the remaining 155 prehypertensive patients, 83 were dippers and 72 were non-dippers. Non-dipper prehypertensives had a significantly higher frequency of fQRS compared to normotensives (p = 0.048). Furthermore, multinomial logistic regression analysis revealed that fQRS is an independent predictor of non-dipping blood pressure pattern in prehypertensive patients (p = 0.017, OR: 4.071, 95% CI: 1.281-12.936). Conclusions: We found that fQRS is a predictor of non-dipping in prehypertensives. As a marker of fibrosis and higher fibrotic burden within myocardium, fQRS may be useful in identifying high-risk prehypertensive patients before the development of hypertension.

Resumo Fundamento: A fragmentação do QRS (fQRS) é um sinal de eventos cardiovasculares adversos em várias doenças cardiovasculares. É associado também à pressão arterial elevada e à ausência de descenso da pressão arterial durante o sono (non-dipping) em pacientes pré-hipertensos. Objetivos: O objetivo do estudo presente é investigar a relação entre fQRS e ausência de descenso da pressão arterial durante o sono em pacientes pré-hipertensos. Métodos: Duzentos e dezesseis pacientes elegíveis, recém-diagnosticados com pré-hipertensão, que foram submetidos a monitorização ambulatorial da pressão arterial (MAPA) durante 24 horas para avaliação mais aprofundada da pressão arterial entre junho de 2015 e julho de 2016, foram incluídos no estudo. De acordo com os resultados da MAPA, os pacientes foram divididos em três grupos: normotensos, pré-hipertensos com descenso da pressão arterial durante o sono (padrão dipping) e pré-hipertensos com ausência de descenso da pressão arterial durante o sono (padrão non-dipping). Os grupos foram comparados quanto à presença de fQRS no eletrocardiograma. Adicionalmente, utilizou-se a análise de regressão logística multinomial para determinar a relação entre a fQRS e o padrão de pressão arterial em pacientes pré-hipertensos. Resultados: De acordo com os registos da MAPA, 61 pacientes apresentavam padrão de pressão arterial normotenso (pressão arterial sistólica < 120 mmHg e pressão arterial diastólica < 80 mmHg). Dos 155 pacientes pré-hipertensos, 83 tinham padrão dipping e 72 tinham padrão non-dipping. Os pacientes pré-hipertensos com padrão non-dipping tinham uma frequência significativamente mais alta de fQRS em comparação com os pacientes normotensos (p = 0,048). Além disso, a análise de regressão logística multinomial revelou que fQRS é um preditor independente do padrão non-dipping de pressão arterial em pacientes pré-hipertensos (p = 0,017, OR: 4,071, 95 % CI: 1,281-12,936). Conclusões: Verificamos que a fQRS é um preditor do padrão non-dipping em pacientes pré-hipertensos. Como marcador de fibrose e aumento na carga fibrótica do miocárdio, a fQRS pode ser útil na identificação de pacientes pré-hipertensos de alto risco antes do desenvolvimento da hipertensão.

Association Between Non-Dipping and Fragmented QRS Complexes in Prehypertensive Patients.

BACKGROUND: Fragmented QRS (fQRS) is a sign of adverse cardiovascular events in various cardiovascular diseases. It is also associated with increased blood pressure and non-dipping in hypertensive patients. However, no study has investigated the importance of fQRS in prehypertensive patients. OBJECTIVES: The aim of our study is to investigate the relationship between fQRS and non-dipper status in prehypertensive patients. METHODS: Two hundred and sixteen eligible, newly diagnosed prehypertensive patients who underwent 24-hour ambulatory blood pressure monitoring (ABPM) for further evaluation of blood pressure between June 2015 and July 2016 were included into the study. Patients were divided into three groups according to ABPM results: normotensives, dipper prehypertensives, and non-dipper prehypertensives. Groups were compared regarding presence of fQRS on electrocardiography. Additionally, multinomial logistic regression analysis was used to determine the relationship between fQRS and blood pressure pattern in prehypertensive patients. RESULTS: According to ABPM recordings, 61 patients had normotensive blood pressure pattern (systolic blood pressure < 120 mmHg and diastolic blood pressure < 80 mmHg). Of the remaining 155 prehypertensive patients, 83 were dippers and 72 were non-dippers. Non-dipper prehypertensives had a significantly higher frequency of fQRS compared to normotensives (p = 0.048). Furthermore, multinomial logistic regression analysis revealed that fQRS is an independent predictor of non-dipping blood pressure pattern in prehypertensive patients (p = 0.017, OR: 4.071, 95% CI: 1.281-12.936). CONCLUSIONS: We found that fQRS is a predictor of non-dipping in prehypertensives. As a marker of fibrosis and higher fibrotic burden within myocardium, fQRS may be useful in identifying high-risk prehypertensive patients before the development of hypertension.