Six-Minute Walk Test: How to do Guide for Performing a Reliable Test, Interpretation, and Prognostication in Pulmonary Hypertension.

Six-minute walk test (6MWT) is the most widely used exercise capacity measurement worldwide in patients with pulmonary hypertension (PH). Although cardiopulmonary exercise testing (CPET) is the gold standard for the assessment of exercise capacity in cardiovascular diseases; the limited accessibility of the device, the need for experience in interpreting the results, and the difficulties in performing CPET in advanced PH have aroused the interest in the application of easier methods for the measurement of exercise capacity. Since then, accumulated data proved that; 6-minutes walking distance (6MWD) can be used to determine exercise capacity and is highly correlated with maximum oxygen consumption (peak VO2) detected by CPET in patients with heart failure and/or PH. Moreover, 6MWT is very easy and practical to apply in all PH subgroups. This review is focused on the application of a reliable 6MWT and the interpretation of the results in patients with PH.

The Frequency of Fabry Disease in Patients with Cardiac Hypertrophy of Various Phenotypes Including Prominent Papillary Muscle: The TUCARFAB Study in Turkey.

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.

Prognostic performance of copeptin among patients with acute decompensated heart failure.

BACKGROUND: In heart failure (HF), various biomarkers have been established for prognosis. However, little is known about the relevance of copeptin measurements to HF. This study aimed to explore the prognostic value of copeptin for predicting cardiovascular (CV) death or HF-related re-hospitalisation in patients with acute decompensated HF. MATERIALS AND METHODS: We prospectively enrolled 155 consecutive patients with acute signs and symptoms of HF. Plasma copeptin and N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels were measured at admission. Patients were monitored for 90 days regarding the composite endpoint of CV death or acute HF-related re-hospitalisation. RESULTS: Of the 155 patients enrolled, 40 reached the endpoint, and 115 were in a stable condition during follow-up. Patients who reached an adverse endpoint showed higher NT-proBNP and copeptin levels compared to patients in stable condition. Receiver operating characteristic curve analysis revealed that the area under curve of copeptin 0.844 (95% CI, 0.753-0.935) was superior to that of NT-proBNP 0.809 (95% CI, 0.729-0.890) for the prediction of adverse events within 90 days. Meanwhile, compared to the group with lower copeptin levels (<34 pmol/L), patients with higher copeptin levels (≥34 pmol/L) were at a 10.672-times higher risk of CV death or acute HF-related re-hospitalisation. Multivariate Cox proportional hazards regression analysis revealed that increased copeptin level was a significantly independent predictor of adverse events (risk ratio, 1.051; 95% CI, 1.020-1.083; p < 0.001). CONCLUSION: Copeptin was found to be a strong, novel marker for predicting CV death or HF-related re-hospitalisation in patients with acute decompensated HF.

Is there any link between joint hypermobility and mitral valve prolapse in patients with fibromyalgia syndrome?

The objective of the present study is to determine whether benign joint hypermobility syndrome (BJHS) modifies the risk of mitral valve prolapse (MVP) in patients with fibromyalgia (FM). Female patients fulfilling the 1990 American College of Rheumatology (ACR) diagnostic criteria for FM were included into the study. Joint hypermobility and BJHS were assessed using Beighton's scoring system and Brighton criteria, respectively. Echocardiograpic evaluation was performed in order to test the presence of MVP. Of the 75 female FM patients, 68.0 % (n = 51) and 20.0 % (n = 15) were diagnosed with BJHS and MVP, respectively. The frequencies of both MVP and BJHS seemed higher than the general population prevalence (p = 0.000 for both). The frequency of MVP was significantly higher in patients with BJHS than that in patients without BJHS (p = 0.028). In addition, BJHS was found to increase the risk of MVP approximately ninefold [odds ratio (OR) 8.7, 95 % confidence interval (CI) 1.1-70.7]. As a result, BJHS and MVP are both common in female patients with FM. Moreover, among the female patients with FM, those with BJHS are about nine times more prone to MVP than those without BJHS. Cardiologic assessment might be added to the routine follow-up strategies in FM patients with BJHS in order to exclude the cardiac pathologies, especially MVP.

Electrocardiographic markers of left ventricular systolic dysfunction in patients with left bundle branch block.

BACKGROUND: Although some patients with left bundle branch block (LBBB) have structural heart diseases, some patients with LBBB have "normal hearts". The electrocardiography (ECG) criteria of LBBB in reduced left ventricular ejection fraction (LVEF) have not been defined completely. AIM: The main purpose of this study was to differentiate patients with reduced LVEF from patients with normal left ventricular systolic function simply by analysing 12-lead ECG. METHODS: Subjects admitted to our hospital with LBBB in their ECG were included in the study. The patients were categorised according to their left ventricular systolic function as group 1 (LVEF ≥ 50%) and group 2 (LVEF < 50%). Duration of the QRS complex, residual conduction of left bundle branch, and concordance/discordance of T waves in leads V5, V6, or D1 were recorded. The ECG findings of the two groups were compared. RESULTS: One hundred consecutive patients with LBBB were included in the study (male/female: 56/44, age: 66 ± 15 years). In the whole group, there were 35 patients with normal left ventricular systolic function (LVEF ≥ 50%), and 65 patients had LVEF below 50%. 80% of male patients with LBBB and 45% of female patients with LBBB had their LVEF below 50% (p < 0.001). Mean QRS durations of group 1 and group 2 were 132 ± 10 ms vs. 152 ± 22 ms, respectively (p < 0.001). The QRS duration of 140 ms was found to be the cut-off value to differentiate group 1 from group 2, with sensitivity and specificity of 72% and 75%, respectively. Twenty-one per cent of patients in group 1 and 69% in group 2 had discordant LBBB (p < 0.001). Residual conduction of left bundle branch was more frequent in group 2 (29% in group 1 vs. 52% in group 2, p = 0.03). CONCLUSIONS: Male gender, QRS duration greater than 140 ms, discordant LBBB, and residual conduction in the left bundle branch seem to be markers of reduced LVEF in patients with LBBB.