Purpose: Our study aimed to assess the validity, reliability, and minimal detectable change (MDC) of the 6-Minute Pegboard and Ring Test (6PBRT) in patients with heart failure (pwHF). Material and Methods: This cross-sectional study included 37 pwHF. Upper extremity functional capacity was evaluated using the 6PBRT. The 6PBRT was executed twice to investigate within-session reliability. The upper extremity muscle strength was measured using a dynamometer. Functional capacity, activities of daily living, and quality of life were assessed with the Milliken Activities of Daily Living Scale (MAS), six-minute walk test (6MWT) and Minnesota Living with Heart Failure Questionnaire (MLHFQ), respectively. Results: Reliability was excellent for 6PBRT, with an ICC of 0.957 (95% CI, 0.863 to 0.982). The MDC of the 6PBRT was 23.75 rings. The 6PBRT was strongly correlated with functional class (rho=-0.778, p<0.001), MAS (r=0.670, p<0.001) and 6MWT distance (r=0.626, p<0.001), and moderately correlated with age (r=-0.513, p=0.001), shoulder abductor (dominant r=0.580, p<0.001), shoulder flexor (dominant r=0.543, p=0.001), elbow extensor (dominant r=0.428, p=0.008), elbow flexor muscle strength (dominant rho=0.469, p=0.003), and MLHFQ (rho=-0.442, p=0.006). Conclusion: The 6PBRT provides a valid and reliable assessment of upper extremity functional capacity in pwHF. Executing 6PBRT at least twice is recommended to avoid the learning effect.

BACKGROUND:Myocardial infarction with nonobstructive coronary arteries (MINOCA) is a heterogeneous syndrome presenting as acute infarction despite < 50% epicardial stenosis. Recognition remains low, and optimal management is unclear. AIMS:This study aims to analyze the clinical profile of MINOCA patients compared to those with myocardial infarction with obstructive lesions, and evaluate MINOCA patients. METHODS:A total of 1421 consecutive patients with acute MI admitted to our hospital between January 2016 and March 2019 were retrospectively screened. Patients were classified into two groups: MINOCA, comprising patients with no significant lesions on angiography, and MI-CAD, consisting of patients with lesions of the coronary artery. A total of 130 patients with MINOCA and 210 patients with MI-CAD were enrolled. Demographics, laboratory parameters, imaging findings, etiologies, in-hospital outcomes, and discharge therapies were analyzed. RESULTS:The prevalence of MINOCA was 9.7%. Patients with MINOCA were more frequently women (especially premenopausal women) and had a younger age (54.65 ± 18.4 to 63.2 ± 12.3, p < 0.001). The prevalence of traditional CAD risk factors was lower in MINOCA patients. Patients with MINOCA were more likely to have a history of upper-respiratory-tract infections (URIs) and use of antidepressant drugs compared to MI-CAD at admission. In-hospital mortality and event rates were comparable to those with MI-CAD. However, antiplatelets, statins, renin-angiotensin system blockers, and β-blockers were less frequently prescribed to patients with MINOCA at discharge. CONCLUSIONS:Patients with MINOCA constitute a population that differs from the classical MI profile. Compared with MI-CAD, MINOCA is accompanied by fewer traditional risk factors for CAD.

PURPOSE:The link between arterial stiffness and physical activity remains unknown in patients with heart failure with reduced ejection fraction (HFrEF). This study aimed to assess the association between arterial stiffness and physical activity in patients with HFrEF. METHODS:Seventy-six patients with HFrEF (mean age: 61.40 ± 8.56 years) were recruited in this cross-sectional study. Arterial stiffness was evaluated with carotid-femoral pulse wave velocity (cfPWV). Physical activity was objectively assessed with an accelerometer over 7 days. Physical activity intensity was classified based on the metabolic equivalents of task (MET) as light-intensity physical activity (LPA; 1.5-2.9 MET), moderate-intensity physical activity (MPA; 3.0-6.0 MET), and vigorous-intensity physical activity (VPA; >6.0 MET). RESULTS:There was a significant correlation between cfPWV and the time spent per week in LPA ( r = -0.478, P < .001), MPA ( r = -0.547, P < .001), total physical activity ( r = -0.579, P < .001), and step count ( r = -0.489, P < .001). After adjusting for age, sex, body mass index, mean arterial pressure, and New York Heart Association functional class, the correlations remained significant. When patients were categorized into inactive, active, and highly active groups according to weekly moderate-to-vigorous intensity physical activity (sum of MPA and VPA), there was a significant difference in cfPWV between the groups ( P < .001). CONCLUSIONS:This study indicates that LPA, MPA, total physical activity, and step count are independently associated with arterial stiffness in patients with HFrEF. Arterial stiffness is lower in patients who are active or highly active compared to those who are inactive.

Objectives: This study aimed to compare the data on mortality of the clinical scoring system that predicts the risk of ischemia-bleeding under dual therapy. Materials and Methods: The records of the patients were retrospectively examined through the hospital information system and archival records. The prepared case data registration form and the Morisky Medication Adherence scale drug compliance scale were filled out. With these data, the patients’ predicting bleeding complications in Patients Undergoing Stent Implantation and Subsequent dual antiplatelet therapy (PRECISE-DAPT) and DAPT scores were calculated. Results: A total of 260 patients were included in the study. The PRECISE-DAPT and DAPT scores were calculated for the patients with acute coronary syndrome. A total of 62 patients (23.8%), exhibited a PRECISE-DAPT score of ≥25. The number of patients with a DAPT score ≥2 was found to be 193 (74.2%). In terms of mortality, patients with PRECISE-DAPT ≥25 and those with PRECISE-DAPT <25 were compared with another group (score should be specified here), and mortality was significantly higher in the high-score group [p=0.001 odds ratio: 6.94 confidence interval: (3.53-13.62)]. Patients were divided into 4 groups based on PRECISE-DAPT and DAPT scores and compared with each other (PRECISE-DAPT <25 and DAPT ≥2, PRECISE-DAPT ≥25 and DAPT ≥2, PRECISE-DAPT <25 and DAPT <2, PRECISE-DAPT ≥25 and DAPT <2). Patients with a high PRECISE-DAPT score had a significantly higher mortality rate compared to those with a lower DAPT score (p<0.001). Conclusion: In our study, we discovered that the bleeding risk score was insufficient for predicting bleeding events, but it could identify high-risk patients in terms of mortality.

BACKGROUND:Cardiac amyloidosis (CA) is an increasingly recognized disease. Several recent advanced imaging techniques and parameters have been introduced into the diagnosis of CA. However, the first step in using those techniques is clinical suspicion. Left ventricular hypertrophy (LVH) is the main entity in rising the suspicion of CA in routine echocardiography, although it is not a diagnosis for CA. The aim of this study is to investigate the prevalence of CA and its subtypes and predictive value of clinical and echocardiographic red flags of CA among consecutive adult patients with LVH identified during routine echocardiographic examination in 25 tertiary institutions in Türkiye. METHODS:This was a prospective observational multicenter, national registration study. Patients with LVH (interventricular septum thickness ≥13 mm or >15 mm in those with hypertension) were screened for CA stepwise. The first step was a clinical questionnaire for the red flags of CA. Those having ≥2 red flags were further analyzed by detailed echocardiography, blood tests, Tc-pyrophosphate (PYP) bone scintigraphy, and histopathological examination if needed. Parameters associated with CA were evaluated via univariate and multivariate analyses. Wild-type transthyretin (wTTR) vs. mutant-type TTR (mTTR), CA discriminators were also evaluated in the same manner. RESULTS:A total of 420 patients meeting these criteria were included in the study. With a standardized algorithmic approach, 27.1% (114) of patients received a CA diagnosis. Among these patients with CA, 50.8% (58) were diagnosed with immunoglobulin free chain (AL) CA, 38.6% (44) with wTTR CA, and 7% (8) with mTTR CA. Left ventricular apical sparing pattern and restrictive type LV filling on echocardiography, low QRS voltage on ECG, bilateral carpal tunnel syndrome, low blood pressure, right ventricular diameter, and an increased basal heart rate (HR) were independent predictors for CA diagnosis. When it comes to diagnosis of wTTR CA; advanced age (age >75), lower troponin values, absence of pericardial effusion and absence of proteinuria were the independent predictors. CONCLUSION:Cardiac amyloidosis is highly prevalent in a patient population with LVH and 2 red flags who underwent a standardized algorithmic approach, in which apical sparing, restrictive filling pattern, low QRS voltage, carpal tunnel syndrome, low blood pressure, and increased HR are the highly suggestive signs of CA. Among this pool of newly diagnosed CA patients in Türkiye, AL-CA constituted 50.8%, wTTR CA 38.6%, and mTTR CA 7%, emphasizing that approximately 1 in 2 patients diagnosed with CA may have TTR CA.

BACKGROUND/OBJECTIVES:There is limited information about which frequency of the mechanical vibration is more effective in whole-body vibration training in the hypertensive population. The aim of this study was to compare the acute effects of whole-body vibration training on arterial stiffness, heart rate variability, blood pressure, perceived exertion, energy expenditure, and muscle strength using different mechanical vibration frequencies in hypertensive patients. METHODS:Thirty-eight individuals diagnosed with hypertension between the ages of 40 and 70 years old participated in this crossover study. Whole-body vibration intervention were performed in random order on different days, with an amplitude of 2-4 mm and three different mechanical vibration frequencies of 0, 25, and 40 Hz. The mechanical vibration device used in the study produced vertical oscillations. A total of five additional exercises were performed in a single session, and each session lasted 15 min. Arterial stiffness was assessed using a noninvasive method, which can perform automatic measurements with a cuff. Heart rate variability was assessed using a 5-min electrocardiography recording. Lower-extremity muscle strength and energy expenditure were assessed using a digital dynamometer and an activity monitor, respectively. RESULTS:There was a significant main effect of time for heart rate, respiratory rate, perceived effort, systolic blood pressure, augmentation index at a heart rate of 75 beats per minute, pulse pressure, mean arterial pressure, high-frequency power, and quadriceps muscle strength (p < .05). However, there was no significant frequency effect for changes in all variables (p > .05). CONCLUSION:Following whole-body vibration application at mechanical vibration frequencies of 0, 25, and 40 Hz; acute responses on heart rate, blood pressure, respiratory rate, perceived exertion, arterial stiffness, heart rate variability, muscle strength, and energy expenditure produced similar effects. Significance/Implications: More studies investigating the frequency effects of whole-body vibration training applied at different mechanical vibration frequencies on acute responses will provide further guidance for future research.

Objective: Limited information is available regarding the associations between upper extremity function, activities of daily living (ADLs), and functional capacity in patients with heart failure with reduced ejection fraction (HFrEF).This study aimed to investigate the associations between upper extremity function, ADLs, and functional capacity in patients with HFrEF.Methods: This cross-sectional study included 31 patients with HFrEF.Demographic, anthropometric, and clinical data were recorded.Upper extremity function and ADLs were evaluated using the 6-Minute Pegboard and Ring Test (6PBRT) and the Glittre Activities of Daily Living Test (TGlittre), respectively.The 6-Minute Walk Test (6MWT) was administered to measure functional capacity.Heart rate (HR), systolic blood pressure (SBP), diastolic blood pressure (DBP), peripheral oxygen saturation (SpO 2 ), dyspnea, and fatigue were assessed at the beginning and end of each test. Results:The 6PBRT was significantly correlated with TGlittre (rho = -0.718,P < 0.001) and 6-minute walk distance (6MWD) (r = 0.546, P = 0.001).A significant correlation was also found between TGlittre and 6MWD (rho = -0.810,P < 0.001).Changes in HR, SBP, and dyspnea were significantly different across the 6PBRT, TGlittre, and 6MWT (P < 0.05). Conclusion:This study indicates that upper extremity function is associated with ADLs and functional capacity in patients with HFrEF.The 6PBRT requires lower cardiopulmonary demand than TGlittre and 6MWT in this patient population.

Background: We aimed to evaluate the correlation and agreement between the Systemic Coronary Risk Estimation 2 (SCORE2) and Predicting Risk of CVD EVENTs (PREVENT) 10-year ASCVD risk scores by incorporating computed tomographic (CT) data to assess differences between the scoring systems. Methods: The PREVENT risk score was calculated for 171 patients, while the SCORE2 and SCORE2 Older Persons (OP) risk scores were calculated for 113 patients. Coronary artery calcium (CAC) scores were calculated, and the grading of coronary artery disease (CAD) was assessed according to these scores. Results: According to the PREVENT risk category, 79 patients (46.2%) were in the low-risk category, 32 (18.7%) were in the borderline-risk category, and 51 (29.8%) were in the intermediate-risk category. In contrast, the SCORE2 systems placed 32 patients (28.3%) in the low- to moderate-risk categories. Only 9 patients (5.3%) were classified as being at high risk by PREVENT, while SCORE2 categorized 39 patients (34.5%) as being at high risk and 42 patients (37.2%) as being at very high risk. There was a strong correlation between the scores (r = 85, p < 0.001), with a Bland–Altman plot analysis showing a bias of −3.71 points and the limits of agreement ranging from −16.06 to 8.64. The total CAC score and CAD grading were significantly different across the PREVENT risk groups (p < 0.001 for all) but were similar across the SCORE2 groups (p = 0.3 and p = 0.051, respectively). Conclusions: There is a strong correlation and agreement between the two risk scores. However, SCORE2 tends to categorize more patients as high-risk than PREVENT does. Additionally, the PREVENT risk categories are more effective than SCORE2 in determining the likelihood of CAD based on CT results.

AIMS:Right ventricular (RV) failure is one of the leading causes of death in patients with pulmonary hypertension (PH). Conventional echocardiographic parameters are not included in risk stratification and follow-up for prognostic assessment due to PH's diverse nature and the RV's complex geometry. RV outflow tract velocity time integral (RVOT VTI) is a simple, non-invasive estimate of pulmonary flow and an echocardiographic surrogate of RV stroke volume. In this study, we aimed to define the prognostic value of RVOT VTI in PH patients. METHODS:Sixty-three subjects with idiopathic PAH (IPAH) (n = 23), connective tissue disease-associated PAH (CTD-associated PAH) (n = 19) and chronic thromboembolic pulmonary hypertension (CTEPH) (n = 21) were retrospectively included. A comprehensive two-dimensional echocardiographic evaluation, including RVOT-VTI measurement, was performed during the follow-up and the New York Heart Association functional class (NYHA FC), 6 min walk distance (6MWD) and brain natriuretic peptide (BNP) levels were recorded. RESULTS:The median age of the whole cohort was 63 years (52-68), and 47 (74.6%) of the patients were women. The median follow-up period was 20 months (11-33), and 20 (31.7%) patients died in this period. BNP values were higher [317 (210-641) vs 161 (47-466), P = 0.02], and 6MWD values were lower [197.5 ± 89.5 vs 339 ± 146.3, P < 0.0001] in the non-survivor group, and the non-survivor group had a worse NYHA-FC (P = 0.02). Among echocardiographic data, tricuspid annular plane systolic excursion (TAPSE) (15.4 ± 4.8 vs 18.6 ± 4.2, P = 0.01) and RVOT VTI (11.9 ± 4.1 vs 17.2 ± 4.3, P < 0.0001) values were lower whereas right atrial area (RAA) (26.9 ± 10.1 vs 22.2 ± 7.1, P = 0.04) values were higher in the non-survivor group. The area under curve of the RVOT VTI for predicting mortality was 0.82 [95% confidence interval (CI) 0.715-0.940, P < 0.0001], and the best cut-off value was 14.7 cm with a sensitivity of 80% and specificity of 77%. Survival was significantly lower in subjects with RVOT VTI ≤ 14.7 cm (log-rank P < 0.0001). Survival rates for patients with RVOT VTI ≤ 14.7 cm were 70% at 1 year, 50% at 2 years, %29 at 3 years and 21% at 5 years. The univariate determinants of all-cause mortality were BNP [hazard ratio (HR) 1.001 (1.001-1.002), P = 0.001], 6MWD [HR 0.994 (0.990-0.999), P = 0.012] and NYHA-FC III-IV [HR 3.335 (1.103-10.083), P = 0.03], TAPSE [HR 0.838 (0.775-0.929), P = 0.001], RAA [HR 1.072 (1.013-1.135), P = 0.016] and RVOT VTI [HR 0.819 (0.740-0.906), P < 0.0001]. RVOT VTI was found to be the only independent determinant of mortality [HR 0.857 (0.766-0.960), P = 0.008]. CONCLUSIONS:The decreased RVOT VTI predicts mortality in patients with PH and each 1 mm decrease in RVOT VTI increases the risk of mortality by 14.3%. This parameter might serve as an additional parameter in the follow-up of these patients especially when 6MWD and NYHA-FC could not be determined.

Spontaneous coronary artery dissection (SCAD) is an atypical cause of myocardial infarction, predominantly seen in women.Among various predisposing factors, genetic vasculopathies such as connective tissue diseases significantly contribute to SCAD.This report discusses a 36-year-old male diagnosed with vascular type Ehlers-Danlos syndrome following an anterior myocardial infarction and explores relevant literature.

Cardiomyopathy, which is shortly defined as a disease of the myocardium, has a broad definition that includes many different diagnoses. Recent advances in cardiac imaging techniques, including basic and advanced echocardiography, computed tomography, nuclear medicine, and cardiac magnetic resonance, allow for a more accurate evaluation of volumes and thickness of cardiac chambers, systolic and diastolic function of the ventricules, and tissue structure. Multimodality imaging often provides the first clinical suspicion for specific etiologies, especially when the medical and family history is unclear, by identification of red flags of underlying systemic diseases. In this review, we aimed to evaluate the role of multimodality imaging in diagnosis of cardiomyopathies with key images and discussed the effects of genetics on the diagnostic, prognostic, and therapeutic guidance of cardiomyopathies.

The 6‑minute 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. 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. 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

Introdução: Foram identificados preditores de arritmias e morte cardíaca súbita em pacientes com cardiomiopatia hipertrófica (CMH). No entanto, não foram validados após ablação septal alcoólica (ASA), uma vez que há uma escassez de dados sobre a predição da ocorrência de arritmia após o procedimento. Objetivos: Analisar retrospectivamente pacientes submetidos à ASA e determinar os preditores de arritmia ventricular pós-operatória. Pacientes e Metodologia: Um total de 53 pacientes consecutivos com CMH, submetidos ao procedimento de ASA devido à obstrução sintomática da via de saída do ventrículo esquerdo (VSVE), foram inscritos retrospectivamente, apesar do tratamento médico tolerado ao máximo entre janeiro de 2010 e dezembro de 2022. Um valor de p < 0,05 foi considerado estatisticamente significativo. Resultados: A idade média dos pacientes foi de 56,45 anos e 55% eram do sexo masculino. Os pacientes foram submetidos à ASA bem-sucedida, com uma média de 1,76 cc de álcool sendo usada. Uma redução de 70,81% foi obtida nos gradientes da VSVE. Nenhum gradiente patológico da VSVE foi observado em nenhum paciente após o procedimento. Durante o acompanhamento pós-procedimento, o bloqueio atrioventricular total foi detectado em 12 pacientes e o implante de cardioversor desfibrilador implantável (CDI) foi realizado nesses pacientes. Quando pacientes com arritmias ventriculares pós ASA bem-sucedida foram comparados com um grupo sem procedimento, o escore de risco de cardiomiopatia hipertrófica de morte cardíaca súbita (HCMSCD) pré-operatório > 6 e a espessura da parede inferolateral do ventrículo esquerdo pré-operatória foram estatisticamente diferentes entre os dois grupos (p: 0,049, p: 0,006, respectivamente). Quando a regressão multilogística foi realizada, a espessura da parede inferolateral do ventrículo esquerdo basal > 15,5 mm foi considerada um fator de risco independente para arritmia ventricular pós ASA (p: 0,027). Conclusões: A espessura da parede inferolateral do ventrículo esquerdo é um preditor independente de arritmia ventricular após ASA, podendo ser usada no acompanhamento pós-procedimento do paciente e na tomada de decisão para implantação do CDI.

ObjectivesThis study has been conducted to investigate the non-invasive diagnostic journey of patients with a transthyretin amyloid cardiomyopathy (aTTR-CM) in Turkey, identify the challenges and uncertainties encountered on the path to diagnosis from the perspectives of expert physicians, and develop recommendations that can be applied in such cases.MethodsThis study employed a three-round modified Delphi method and included 10 cardiologists and five nuclear medicine specialists. Two hematologists also shared their expert opinions on the survey results related to hematological tests during a final face-to-face discussion. A consensus was reached when 80% or more of the panel members marked the “agree/strongly agree” or “disagree/strongly disagree” option.ResultsThe panelists unanimously agreed that the aTTR-CM diagnosis could be established through scintigraphy (using either 99mTc-PYP, 99mTc-DPD, or 99mTc-HMPD) in a patient with suspected cardiac amyloidosis (CA) without a further investigation if AL amyloidosis is ruled out (by sFLC, SPIE and UPIE). In addition, scintigraphy imaging performed by SPECT or SPECT-CT should reveal a myocardial uptake of Grade ≥2 with a heart-to-contralateral (H/CL) ratio of ≥1.5. The cardiology panelists recommended using cardiovascular magnetic resonance (CMR) and a detailed echocardiographic scoring as a last resort before considering an endomyocardial biopsy in patients with suspected CA whose scintigraphy results were discordant/inconclusive or negative but still carried a high clinical suspicion of aTTR-CM.ConclusionThe diagnostic approach for aTTR-CM should be customized based on the availability of diagnostic tools/methods in each expert clinic to achieve a timely and definitive diagnosis.

Systemic sclerosis (SSc) is a complex immune-mediated connective tissue disease, involving skin manifestations, vascular features, and organ-based complications that may affect functional capacity and physical activity. Functional capacity and physical activity are associated with arterial stiffness; however, this relationship has not been evaluated in patients with SSc. Therefore, the objective of this study was to investigate the association of functional capacity and physical activity with arterial stiffness in patients with SSc. Sixty-five patients with SSc were enrolled in this cross-sectional study. Arterial stiffness was evaluated with carotid-femoral pulse wave velocity (cf-PWV). Functional capacity and physical activity were assessed with a six-min walk test (6MWT) and International Physical Activity Questionnaire-Short Form (IPAQ-SF), respectively. All participants were women, and the mean age was 54.91 ± 11.18 years. 6MWT distance and IPAQ-SF were inversely associated with cf-PWV in crude analysis (p < 0.05). The relationship between 6MWT distance and cf-PWV was maintained in the fully adjusted model (β = − 0.007, 95

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.

Amaç: Çalışmamızın amacı Vazovagal senkoplu hastalarda hemodinamik mekanizmayı etkileyebilecek kardiyovasküler otonomik modülasyonlardan biri olan arteriyel sertlik parametrelerini değerlendirmek. Gereç ve yöntemler: Çalışmamız retrospektif bir çalışmadır. Ocak 2012-Ocak 2016 tarihleri arasında kardiyoloji polikliniğine başvuran en az iki senkop atağı olan ve eğik masa testi pozitif olan 42 hastanın eğik masa testi sonuçlarını ve arteriyel sertlik parametrelerini topladık. Kontrol grubu olarak da yaş ve cinsiyete göre eşleştirilmiş 41 kişi aldık. Hastalardan damar özelliklerini etkileyebilecek durumları olan, ritim bozukluğu ve psikiyatrik bozukluğu olan hastaları çalışma dışı bıraktık. Hastaların arteriyel sertlik ölçümleri, tilt testi sonuçları, tıbbi öyküleri, sosyodemografik ve klinik bilgileri ile ilgili veriler tıbbi kayıtlarından elde edildi. Arteriyel sertliğinin belirteçleri olarak augmentasyon indeksi, merkezi nabız basıncı ve karotid ila radyal nabız dalga hızı dahil olmak üzere merkezi aort basıncı parametreleri elde edildi. Ardından 2 grup arasında değişkenler karşılaştırıldı. Bulgular: Periferik sistolik (125 ± 16,6 ve 121,1 ± 14,9, p: 0,31) ve diyastolik kan basınçları (79,6 ± 8,9 ve 77,7 ± 9,7, p: 0,38) VVS ve kontrol grupları arasında benzerdi. Merkezi sistolik kan basınçları ve merkezi güçlendirme basınçları da gruplar arasında anlamlı farklılık göstermedi. Vazovagal senkoplu hastalarda nabız dalga hızı sağlıklı kontrollere göre anlamlı derecede düşüktü (5.4 (1.4) and 5.9 (1.8), p= 0.04). Aort augmentasyon indeksi gruplar arasında istatistiksel olarak farklı olmasa da hasta grubunda daha yüksek bulduk. Sonuç: Çalışmamızda VVS grubunda nabız dalga yayılımı sağlıklı gruba göre daha düşük bulundu. Bu sonuçlar VVS hastalarında patofizyolojinin anlaşılmasına katkıda bulunabilir. Vazovagal senkoplu hastalarda nabız dalga hızının önemini anlamak için daha ileri çalışmalara ihtiyaç vardır.

Background:The present study aimed to identify the frequency of Fabry disease in patients with cardiac hypertrophy of unknown etiology and to evaluate demographic and clinical characteristics, enzyme activity levels, and genetic mutations at the time of diagnosis.Methods: This national, multicenter, cross-sectional, single-arm, observational registry study was conducted in adult patients with a clinical echocardiographic diagnosis of left ventricular hypertrophy and/or the presence of prominent papillary muscle.In both genders, genetic analysis was performed by DNA Sanger sequence analysis.Results: A total of 406 patients with left ventricular hypertrophy of unknown origin were included.Of the patients, 19.5% had decreased enzyme activity (≤2.5 nmol/mL/h).Although genetic analysis revealed GLA (galactosidase alpha) gene mutation in only 2 patients (0.5%), these patients were considered to have probable but not "definite Fabry disease" due to normal lyso Gb3 levels and gene mutations categorized as variants of unknown significance. Conclusion:The prevalence of Fabry disease varies according to the characteristics of the population screened and the definition of the disease used in these trials.From cardiology perspective, left ventricular hypertrophy is the major reason to consider screening for Fabry disease.Enzyme testing, genetic analysis, substrate analysis, histopathological examination, and family screening should be performed, when necessary, for a definite diagnosis of Fabry disease.The results of this study underline the importance of the comprehensive use of these diagnostic tools to reach a definite diagnosis.The diagnosis and management of Fabry disease should not be based solely on the results of the screening tests.