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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.

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.

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.

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.

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.