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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.

Objective. The aim of the study was to determine an association between the plasma YKL-40 level and echocardiographic left ventricle systolic and diastolic function parameters in patients with acute myocardial infarction. Subjects and Methods. The study included 46 patients with acute myocardial infarction. Serum brain natriuretic protein (BNP) and YKL-40 levels were analyzed on admission and after one month. Left ventricle systolic and diastolic functions and Tei index were computed by transthoracic echocardiography. Results. Plasma YKL-40 was significantly higher in patients with acute myocardial infarction (AMI) (101.7 μg/L versus 34 μg/L, resp., p

Percutaneous edge-to-edge mitral valve repair using the MitraClip (Abbot Vascular, USA) system is a promising technique for mitral regurgitation treatment in select high-risk surgical patients. Although the safety and efficacy of the technique have been demonstrated, a few complications of the MitraClip device have been reported. In this report, we present a rare complication that recently occurred during the performance of a MitraClip procedure in a patient with severe functional mitral regurgitation. One MitraClip arm got stuck inside the guide catheter in the left atrium and a decision was made to discontinue percutaneous intervention because the problem could not be resolved.

Hypertrophic biventricular cardiomyopathy is a rare finding and generally caused by systemic infiltrative diseases. Its association with pulmonary stenosis in same patient is even rarer. We report a case report of male patient with biventricular hypertrophy coexisting with pulmonary valve stenosis and systemic hypertension.

Abstract Fundamento: The role of papillary muscle function in severe mitral regurgitation with preserved and reduced left ventricular ejection fraction and the method of choice to evaluate PM have still been the subjects of controversy. Objectives: To evaluate and compare papillary muscle function in and between patients with severe degenerative and functional mitral regurgitation by using the free strain method. Methods: 64 patients with severe mitral regurgitation - 39 patients with degenerative mitral regurgitation (DMR group) and 25 patients with severe functional mitral regurgitation (FMR group) - and 30 control subjects (control group) were included in the study. Papillary muscle function was evaluated through the free strain method from apical four chamber images of the anterolateral papillary muscle (APM) and from apical three chamber images of the posteromedial papillary muscle (PPM). Global left ventricular longitudinal and circumferential strains were evaluated by applying 2D speckle tracking imaging. Results: Global left ventricular longitudinal strain (DMR group, -17 [-14.2/-20]; FMR group, -9 [-7/-10.7]; control group, -20 [-18/-21] p < 0.001), global left ventricular circumferential strain (DMR group, -20 [-14.5/-22.7]; FMR group, -10 [-7/-12]; control group, -23 [-21/-27.5] p < 0.001) and papillary musle strains (PPMS; DMR group, -30.5 [-24/-46.7]; FMR group, -18 [-12/-30]; control group; -43 [-34.5/-39.5] p < 0.001; APMS; DMR group, (-35 [-23.5/-43]; FMR group, -20 [-13.5/-26]; control group, -40 [-32.5/-48] p < 0.001) were significantly different among all groups. APMS and PPMS were highly correlated with LVEF (p < 0.001, p < 0.001; respectively), GLS (p < 0.001, p < 0.001; respectively) and GCS (p < 0.001, p < 0.00; respectively) of LV among all groups. No correlation was found between papillary muscle strains and effective orifice area (EOA) in both groups of severe mitral regurgitation. Conclusions: Measuring papillary muscle longitudinal strain by the free strain method is practical and applicable. Papillary muscle dysfunction plays a small role in severe MR due to degenerative or functional causes and papillary muscle functions in general seems to follow left ventricular function. PPM is the most affected PM in severe mitral regurgitation in both groups of DMR and FMR.

[This corrects the article DOI: 10.1371/journal.pone.0282054.].

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.

Background: Tricuspid regurgitation is a condition that affects 1.6 million patients in the United States and is independently associated with morbidity and mortality. The TriClip™ procedure repairs the tricuspid valve without the need for open-heart surgery. The aim of this study is to evaluate the cost-effectiveness of TriClip™ treatment in patients with advanced tricuspid regurgitation from the Turkish reimbursement agency perspective. Methods: Within the scope of this study, the general literature was searched in order to reach data on tricuspid regurgitation. The utilization of health care services used in the expert panel was re-calculated with the current reimbursement costs to determine the cost of heart failure in Turkey. In this study, Markov analysis, Tornado analysis, cost-effectiveness analysis, and partitioned survival analysis have been performed to determine whether TriClip™ is an effective treatment method compared to medication treatment. Results: In according to calculations, 5-year survival rate was found as 49.91% for medication treatment and 57.64% for TriClip™ treatment. According to the analysis performed, the cost of medication treatment was calculated as €3879.72 and TriClip™ Transcatheter Tricuspid Valve Repair System treatment as €25 661.15 for a 60-month period in patients with tricuspid regurgitation and New York Heart Association III-IV. In the calculation, it was found that TriClip™ treatment gave patients an average of 1.64 life years and it was found to be cost-effective compared to medication treatment. Conclusions: Considering the positive effect of TriClip™ treatment on patients with tricuspid regurgitation in terms of mortality and regression of the heart failure stage, as recommended in the guidelines, widespread of its use has great importance.

BackgroundPapillary muscle free strain has not been evaluated previously in hypertrophic cardiomyopathy (HCMP) patients. Our aim was to evaluate free papillary muscle free strain in HCMP patients and to find whether it has a value for prediction of sudden cardiac death (SCD) risk score.MethodsTransthoracic echocardiography with tissue Doppler imaging, 2-D speckle tracking imaging (STI) of 55 HCMP patients and 45 controls were performed. HCMP patients were further divided into two groups according to their SCD risk score. Patients with risk score of less than 6 points constituted low/intermediate risk group, whereas patients with risk score of greater or equal to 6 points constituted high risk group.ResultsInterventricular septum, posterior wall, and left ventricular mass index were significantly higher, whereas mitral E/A ratio was significantly lower in HCMP patients compared to controls. Longitudinal apical 4C, 2C, 3C, global longitudinal LV strain, anterolateral papillary muscle (ALPM), posteromedial papillary muscle (PMPM) free strain were significantly reduced in HCMP group compared to control group. Global longitudinal strain and ALPM free strain were significantly lower in patients with high SCD risk score (-14.6 (-17.4 - -13.1) vs -11.6 (-13.2 - -10.1), p = 0.001 and -17.1 (-20.3 - -14.0) vs -9.2 (-12.6 - -7.5), pConclusionPapillary muscle free strain was reduced in HCMP patients. It might be used in risk stratification of these patients.

Çalışmanın amacı okulların örgütsel narsizm düzeyleri ile öğretmenlerin okul iklimi algıları arasındaki ilişkinin incelenmesi ve bu ilişkinin demografik değişkenler açısından anlamlı farklılık gösterip göstermediğinin değerlendirilmesidir. Rize’nin Çayeli ilçesinde ilkokul ve ortaokullarda görev yapan 233 öğretmen araştırmanın çalışma grubunu oluşturmaktadır. İlişkisel tarama modelinde desenlenen araştırmanın verilerinin toplanmasında “Örgütsel Narsizm” ve “Okul İklimi” ölçekleri kullanılmıştır. Analiz sürecinde SPSS programı kullanılarak, korelasyon ve regresyon analizleri gerçekleştirilmiş ve t- Testi, Tek Yönlü Varyans Analizi (ANOVA); Mann Whitney U testi, Kruskal Wallis H testi yapılmıştır. Verilerin analizi sonucunda, öğretmenlerin örgütsel narsizm düzeyleri ile okul iklimi algıları arasında pozitif yönlü orta düzeyde anlamlı bir ilişkinin varlığı tespit edilmiş ve örgütsel narsizmin okul ikliminin anlamlı bir yordayıcısı olduğu sonucuna varılmıştır.

Primary tricuspid valve regurgitation may be encountered in daily practice as a result of multiple etiologies. Described herein are the cases of 2 patients with severe primary tricuspid regurgitation. The underlying mechanism was posterior leaflet prolapse due to spontaneous chordae rupture in 1 case, and iatrogenic posterior leaflet tissue loss during removal of a permanent pacemaker in the other. Transthoracic and transesophageal echocardiography, which permit assessment of the tricuspid valve with multilevel imaging, are the techniques of choice for accurate detection and understanding of the etiology, the severity of valve regurgitation, and the determination of treatment options, in addition to providing assistance with timing and guidance during intervention. Three-dimensional echocardiography offers the ability to visualize the entire tricuspid valve and to identify which leaflets are affected by the pathology.

Background: In the early stages of atrial remodeling, aortic stiffness might be an indication of an atrial myopathy, in particular, atrial fibrosis. This study aimed to investigate the association between left atrial (LA) mechanical function, assessed by two-dimensional speckle tracking echocardiography, and aortic stiffness in middle-aged patients with the first episode of nonvalvular atrial fibrillation (AF). Methods: This prospective study included 34 consecutive patients with the first episode of AF, who were admitted to Kartal Koşuyolu Research and Training Hospital between May 2013 and October 2015, and 31 age- and gender-matched healthy controls. During the 1 st month (mostly in the first 2 weeks) following their first admission, 34 patients underwent the first pulse wave measurements. Then, 21 patients were recalled for their second pulse wave measurement at 11.8 ± 6.0 months following their initial admission. Echocardiographic and pulse wave findings were compared between these 34 patients and 31 healthy controls. We also compared the pulse wave and echocardiographic findings between the first and second measurements in 21 patients. Results: Pulse wave analysis showed no significant differences between the AF patients and healthy controls with respect to PWV (10.2 ± 2.5 m/s vs. 9.7 ± 2.1 m/s; P = 0.370), augmentation pressure (9.6 ± 7.4 mmHg vs. 9.1 ± 5.7 mmHg; P = 0.740), and aortic pulse pressure (AoPP; 40.4 ± 14.0 mmHg vs. 42.1 ± 7.6 mmHg, P = 0.550). The first LA positive peak of strain was inversely related to the augmentation pressure (r = −0.30; P = 0.02) and aortic systolic pressure (r = −0.26, P = 0.04). Comparison between the two consecutive pulse wave measurements in 21 patients showed similar results, except for AoPP. In 21 patients, the AoPP at the second measurement (45.1 ± 14.1 mmHg) showed a significant increase compared with AoPP at the first measurement (39.0 ± 10.6 mmHg, P = 0.028), which was also higher than that of healthy controls (42.1 ± 7.6 mmHg, P = 0.000). Conclusion: The association between aortic stiffness with reduced atrial strain and the key role of AoPP in the development of AF should be considered when treating nonvalvular AF patients with normal LA sizes.

Objective: Mitral valve regurgitation (MR) is the second most common heart valve disease in Europe. Without intervention, prognosis of severe symptomatic MR is poor. Percutaneous edge-to-edge mitral valve repair with MitraClip is a promising mitral regurgitation treatment technique in select, high-surgical-risk patients. The present objective was to describe the experience of a single center with MitraClip use in a high-risk series in Turkey. Methods: Between May 2013 and September 2014, 28 high-surgical-risk patients with MR of at least grade 3+ and mean EuroSCORE of 26% underwent MitraClip implantation at our institution. In-hospital and follow-up safety and efficacy results are presented. Results: Mean patient age was 58 years, and 75% were male. Grade 3 or 4 MR was present in all patients, and was primarily the result of restrictive functional mitral regurgitation (in 89% of cases). Mean left ventricular ejection fraction (LVEF) was 27% and New York Heart Association (NYHA) classification was III or IV in 89% of the population. Acute procedural success was 89%, with 47% of patients receiving a single clip, 39% receiving 2 clips, and 14% receiving 3 clips. One periprocedural death occurred, and 2 deaths occurred during follow-up (mean: 13.9 months). After 1 year, more than 75% of patients had MR severity of ≤2+ and NYHA classification of I or II, but no significant change in left ventricular volume or systolic function. Significant improvement in 6-minute walk test and quality of life was also observed. Conclusion: Initial experience with the MitraClip system showed promising results in patients considered high-surgical-risk, particularly in those with end-stage heart failure.

Objectives: Typical atrioventricular nodal reentrant tachycardia (AVNRT) can be cured with slow pathway ablation. This study was designed to assess the alterations in atrial and ventricular functioning using speckle tracking echocardiography in consecutive patients with typical AVNRT who underwent slow pathway radiofrequency (RF) ablation. Study design: Included in this study were 23 consecutive patients with symptomatic drug-resistant typical (slow-fast) AVNRT, all of whom underwent an invasive electrophysiology study and RF ablation. Patients underwent transthoracic echocardiographic evaluation 24 hours before and 24 hours after the ablation procedure. Results: AVNRT was induced during the electrophysiological study, and RF ablation successfully eliminated tachyarrhythmia in 23 (100%) patients. The atrial-His (A-H) interval was decreased in the post-ablation period compared to the pre-ablation period without the occurrence of immediate conduction disturbances. Peak left atrial longitudinal strain during the reservoir phase was increased in the post-ablation period compared to the pre-ablation period (48.24+-16.45 vs. 38.07+-15.72, p

Objectives: The aim of this study was to investigate the value of plasma D-dimer (DD) levels for predicting systemic embolism in patients with infective endocarditis (IE). Study design: A total of 42 patients (mean age: 46+-16 years; 78% males) with IE were included. Clinical, laboratory and echocardiographic findings of the patients were evaluated. Results: Increased plasma DD levels were determined in 13 patients with systemic embolism (p=0.016). Moreover, when patients were divided in two groups as DD >500 ng/dl and DD 500 ng/dl group (p=0.036). Receiver operating characteristics (ROC) curve analysis was performed to detect the best cut-off value of DD in the prediction of systemic embolism. DD >425 ng/dl yielded an area under the curve (AUC) value of 0.735 (95% CI 0.560-0.909, p=0.016). DD >425 ng/dl demonstrated a sensitivity of 77% and specificity of 62% for the prediction of clinical embolism. Hematocrit (r=-0.31, p=0.045), platelet count (r=-0.40, p=0.009), albumin (r=-0.37, p=0.026), and globulin (r=0.38, p=0.028) levels were correlated with DD levels. Conclusion: Plasma DD levels are increased in patients with IE who suffered from clinically significant systemic embolism. Further studies are needed to determine the predictive value of DD levels for clinically silent systemic embolism.

Aims Diastolic tissue Doppler (TD) parameters allow prediction of patients with hypertrophic cardiomyopathy (HC) at risk of sudden death, ventricular tachycardia, or cardiac arrest. The aim of this study was to assess the value of TD imaging in predicting the clinical course of patients with HC. Methods and results Eighty-six HC patients were prospectively included in the study and followed-up for clinical endpoints (cardiovascular death or hospitalization due to worsening of heart failure symptoms). Patients with clinical endpoints (n = 25) had larger left atrium diameters, thicker left ventricle (LV) walls, more often LV outflow obstruction and lower TD velocities of LV. LV outflow tract obstruction (r=0.54, R²=0.29, Pr=0.50, R²=0.25, P 4 cm/s were significantly free of clinical endpoints. Conclusion In conclusion, LMSa seems to be a reliable parameter that can be used in predicting the HC patients at risk for clinical deterioration or death at long-term follow-up. [ABSTRACT FROM AUTHOR]