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The article presents a case study of a 63-year-old woman with Gaucher disease (GD1) at an advanced stage of the disease. Topics include pulmonary involvement being a rare life-threatening disorder and pulmonary hypertension (PH) showing the most serious pulmonary complication in GD1; and early menopause, hip fracture and related operation, and oxygen therapy for the last 2 years due to bronchiectasis.

Objective: Allograft rejection is still an important cause of morbidity and mortality after heart transplantation (HTx). Many techniques in cardiac magnetic resonance imaging (CMR) were investigated to diagnose acute cellular rejection (ACR). However, there is not enough information about late gadolinium enhancement (LGE) in the myocardium and ACR. Methods: We prospectively analyzed our consecutive 41 heart transplant recipients who were admitted for routine endomyocardial biopsies. CMR was performed maximum 6 h before the scheduled endomyocardial biopsy. Correlation between LGE in the myocardium and ACR was investigated. Results: Twenty-seven patients showed no rejection, and nine of them had LGE in the myocardium. Fourteen patients had LGE in the left ventricle (LV), and two patients had LGE also in the right ventricle (RV). There was no correlation between LGE and ACR (p=0.879). There was no difference in the left ventricular ejection fraction (LVEF), right ventricular fractional area change (RVFAC), and cardiac ischemic time between the groups (p=0.825, p=0.370, and p=0.419, respectively). LGE in the myocardium could be due to previous rejection episodes; therefore, all patients were retrospectively searched for previous rejection grades and number of episodes. Thirty-eight of the 41 patients had a history of one ACR episode, but none of them had a statistically significant correlation with LGE (for grade 1R, p=0.964 and grade 3R, p=1) There was also no correlation between number of rejection episodes history and LGE. Conclusion: LGE is not suitable to detect ACR in heart transplant patients. LGE and the history of ACR have no correlation. [ABSTRACT FROM AUTHOR]

Pulmonary arterial hypertension (PAH) is an uncommon disorder that may be hereditable, idiopathic or associated with conditions like drug exposure, connective tissue disease, HIV infection or congenital heart disease. Familial disease are usually due to mutations in the bone morphogenic protein receptor type 2 (BMPR2), activin-like kinase-type 1 (ALK1) and endoglin (ENG). Functional and structural changes in the pulmonary vasculature lead to increased pulmonary vascular resistance. Vascular remodeling involves endothelial dysfunction, activation of fibroblasts and smooth muscle cells and recruitment of circulating progenitor cells. Vasoconstriction has also been shown to affect the remodeling process. Genetics, cellular and molecular basis of PAH are discussed in the paper. [ABSTRACT FROM AUTHOR]

Background: Pulmonary hypertension guidelines recommend invasive right heart catheterization for diagnosis and clinical follow-up. Our aim was to compare non-invasive impedance cardiography with invasive techniques for cardiac index measurements and mortality prediction in patients with pulmonary hypertension. Methods: Between 2008 and 2018, 284 right heart catheterizations were performed for the diagnosis of pulmonary hypertension in 215 patients with mean pulmonary artery pressure >25 mm Hg, and at least 2 methods used for cardiac output measurement were included in the study retrospectively. Patients were evaluated with Pearson’s correlation in 3 groups: estimated Fick (eFick) method and thermodilution (group 1), eFick method and impedance cardiography (group 2), and thermodilution and impedance cardiography (group 3). We also compared the predictive power of cardiac index measured by different methods for 1-year overall mortality and hospitalizations. Results: There were strong and moderate positive correlations in groups 1 and 3, respectively (r=0.634, P < .001, r=0.534, P=.001), and the weakest correlation was in group 2 (r=0.390, P=.001). The mean difference (bias) between eFick method versus impedance cardiography, impedance cardiography vs. thermodilution, and eFick method vs. thermodilution was 0.6 mL/min, 0.47 mL/min, and −0.2 mL/min respectively, but limits of agreement were wide. In both groups, cardiac index <2.5 L/min/m² as measured by thermodilution significantly predicted 1-year mortality. Also, impedance cardiography was better than eFick method in predicting mortality (P=.02). Conclusions: Our single-center real-life data showed that for cardiac output and cardiac index measurements, impedance cardiography provides a moderate correlation with thermodilution and is fair with eFick method methods. Moreover, thermodilution appeared superior to both eFick method and impedance cardiography, while impedance cardiography was even better than eFick method in predicting 1-year adverse events, including total mortality and hospitalization, in patients with pulmonary hypertension. [ABSTRACT FROM AUTHOR]

The article presents case study of a 61-year-old patient hospitalized with the diagnosis of decompensated heart failure and given diuretic treatment. It mentions the hemodynamic transesophageal echocardiography (hTEE) technology used in the follow-up of postoperative patients in the cardiovascular surgery intensive care units. It adds the left ventricular ejection fraction (LVEF).

Objective: The purpose of this study is to compare the analysis of right ventricular (RV) free wall strain via 2D speckle tracking echocardiography with conventional echocardiography and clinical parameters in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) receiving specific treatment. This study also aims to describe the differences between patients with repaired and unrepaired defects. Methods: This prospective study included 44 adult patients with PAH-CHD who were receiving PAH-specific treatment in a single center. This study excluded patients with complex congenital heart disease. The authors studied the conventional echocardiographic parameters, such as RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), right atrial (RA) area, Tricuspid S', and hemodynamic parameters, such as functional class, 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. Results: The mean age of participants was 33.8±11.6 years, and 65.9% of participants were female. The mean RV free wall strain was -14.8±4.7%. Majority of the patients belonged to WHO functional class 2 (61.4%) with a mean NT-proBNP level of 619.2±778.4 and mean 6MWD of 400.2±86.9 meters. During the follow-up of 30.8±9.0 months, 6 patients (13.6%) developed clinical right heart failure, whereas 9 (20.5%) of them died. There was a positive and significant correlation between RV free wall strain and WHO functional class (r=0.320, p=0.03), whereas there was a negative correlation between RV free wall strain and FAC (r=-0.392, p=0.01), TAPSE (r=-0.577, p=0.0001), and Tricuspid S' (r=-0.489, p=0.001). There was no significant correlation of RV free wall strain with either RA area or 6MWD. Patients with repaired congenital heart defects had worse RV functional parameters and RV free wall strain than patients with unrepaired defects. Conclusion: The assessment of RV free wall strain via 2D speckle tracking echocardiography is a feasible method and correlates well with conventional echocardiography and clinical parameters in patients with PAH-CHD receiving specific treatment. [ABSTRACT FROM AUTHOR]

Background: Risk assessment is recommended for patients with congenital heart disease-associated pulmonary arterial hypertension. This study aims to compare an abbreviated version of the risk assessment strategy, noninvasive French model, and an abridged version of the Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management 2.0 risk score calculator, Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2. Methods: We enrolled a mixed prevalent and incident cohort of patients with congenital heart disease-associated pulmonary arterial hypertension (n = 126). Noninvasive French model comprising World Health Organization functional class, 6-minute walk distance, and N-terminal pro-hormone of brain natriuretic peptide or brain natriuretic peptide was used. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 includes functional class, systolic blood pressure, heart rate, 6-minute walk distance, brain natriuretic peptide/N-terminal pro-hormone of brain natriuretic peptide, and estimated glomerular filtration rate. Results: The mean age was 32.17 ± 16.3 years. The mean follow-up was 99.41 ± 58.2 months. Thirty-two patients died during follow-up period. Most patients were Eisenmenger syndrome (31%) and simple defects (29.4%). Most patients received monotherapy (76.2%). Most patients were World Health Organization functional class I-II (66.6%). Both models effectively identified risk in our cohort (P = .0001). Patients achieving 2 or 3 noninvasive low-risk criteria or low-risk category by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 at follow-up had a significantly reduced risk of death. Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2 approximates noninvasive French model at discriminating among patients based on c-index. Age, high risk by Registry to Evaluate Early and Long-term Pulmonary Arterial Hypertension Disease Management Lite 2, and the presence of 2 or 3 low-risk criteria by noninvasive French model emerged as an independent predictors of mortality (multivariate hazard ratio: 1.031, 95% CI: 1.005-1.058, P = .02; hazard ratio: 4.258, CI: 1.143-15.860, P = .031; hazard ratio: 0.095, CI: 0.013-0.672, P = .018, respectively). Conclusions: Both abbreviated risk assessment tools may provide a simplified and robust method of risk assessment for congenital heart disease-associated pulmonary arterial hypertension. Patients not achieving low risk at follow-up may benefit from aggressive use of available therapies. [ABSTRACT FROM AUTHOR]

Natriuretic peptides are widely used in all types of heart failure. Previously, we defined heart failure with non-reduced ejection fraction as patients with heart failure symptoms and/or signs and who have left ventricular ejection fraction > 40%.1 For the diagnosis of heart failure with preserved ejection fraction, the presence of raised natriuretic peptides is one of the major components of the diagnosis, and raised natriuretic peptides make the diagnosis more likely in patients with heart failure with mildly reduced ejection fraction.2 The majority of the existing studies have described the utility of natriuretic peptides in patients with heart failure with reduced ejection fraction, but there is not enough data on natriuretic peptides in heart failure patients with heart failure with non-reduced ejection fraction. Despite the insufficient information regarding the usage of natriuretic peptides in heart failure with non-reduced ejection fraction, it is obvious that there is an unmet need to guide how to use natriuretic peptides in these patients. The main goal of this article is to discuss the role of natriuretic peptides in diagnosis, prognosis, and guidance of heart failure treatment in patients with heart failure with non-reduced ejection fraction. The present review discusses the role of natriuretic peptides in heart failure with non-reduced ejection fraction focusing on: the characteristics of natriuretic peptides, primary prevention of heart failure, diagnosis of heart failure with non-reduced ejection fraction in different patient characteristics and co-morbidities, prognosis of heart failure, monitoring of heart failure treatment and, how to use in worsening heart failure. [ABSTRACT FROM AUTHOR]

Objective: Germline mutations in the bone morphogenetic protein receptor type-2 (BMPR2) gene are considered to be a major risk factor for pulmonary arterial hypertension (PAH). BMPR2 mutations have been reported in 10%-20% of idiopathic PAH and in 80% of familial PAH cases. The aim of this study was to evaluate the frequency of mutations in the serine/threonine kinase domain of the BMPR2 gene in a group of patients from a single PAH referral center in Turkey. Methods: This cross-sectional study used a DNA-sequencing method to investigate BMPR2 mutations in the serine-threonine-kinase domain in 43 patients diagnosed with PAH [8 with idiopathic PAH and 35 with congenital heart disease (CHD)] from a single PAH referral center. Patients were included if they had a hemodynamically measured mean pulmonary arterial pressure of >25 mm Hg with a mean pulmonary capillary wedge pressure of ⩽15 mm Hg. Patients with severe left heart disease and/or pulmonary disease that could cause pulmonary hypertension were excluded. Associations between categoric variables were determined using the chi-square test. Differences between idiopathic and CHD-associated PAH groups were compared with the unpaired Student's t-test for continuous variables. Results: We detected a missense mutation, [p.C347Y (c.1040G>A)], in one patient with idiopathic PAH in exon 8 of the BMPR2 gene. The mutation was detected in a 27-year-old female with a remarkable family history for PAH. She had a favorable response to endothelin receptor antagonists. No mutations were detected in the exons 5-11 of the BMPR2 gene in the PAH-CHD group. Conclusion: A missense mutation was detected in only one of the eight patients with idiopathic PAH. The BMPR2 missense mutation rate of 12.5% in this cohort of Turkish patients with idiopathic PAH was similar to that seen in European registries. The index patient was a young female with a family history remarkable for PAH; she had a good long-term response to PAH-specific treatment, probably due to the early initiation of the treatment. Genetic screening of families affected by PAH might have great value in identifying the disease at an early stage. [ABSTRACT FROM AUTHOR]

OBJECTIVE: We retrospectively analyzed case records to identify risk factors for mortality in heart transplant recipients in our center. METHODS: Data of 123 patients (mean age 40.6±14 years and 97 male) transplanted from February 1998 to April 2009 were studied and analyzed. Pre- and intra-operative variables of the 73 patients who survived (Group 1, mean age 39.1±14.6, 58 male) and 50 patients who died (Group 2, mean age 42.6±12.9, 39 male) were compared. For statistical analysis Student t, Mann-Whitney U and Chi-square tests were used. Logistic regression analysis was used to determine independent risk factors for mortality and survival analysis was accomplished using Kaplan-Meier analysis. RESULTS: Overall mortality in the entire population was 40.6% (50/123). Actuarial survival was 74%, 65%, 51% and 31% at 1, 2, 5 and 10 years respectively. Major causes of death after heart transplantation were infections (30%), right ventricular failure (18%), rejection (12%) and sudden cardiac death (12%). Total ischemic time was 176.7±63.3 minutes (range 90-410 minutes). The total cardiopulmonary by-pass (CPB) time (114.3±27.7 vs 126.9±42.1 min, p=0.05), pre-operative creatinine clearance (81.0±31.5 vs 67.2±33.0 ml/min, p=0.02), urea (45.2±18.6 vs 57.8±35.2 mg/dl, p=0.02), and creatinine (1.01±0.25 vs 1.17±0.36 mg/dl, p=0.05) were significantly different between survivors and non-survivors. Group 2 patients had higher transpulmonary gradient (TPG) (9.21±5.16 vs 12.50±8.26 mmHg, p=0.02) as compared with group 1 patients. Logistic regression analysis revealed that preoperative creatinine clearance (OR 0.989, 95% CI 0.973-1.005, p=0.044), creatinine level (OR 2.028, 95% CI 0.288-14.301, p=0.027), total CPB time (OR 1.013, 95% CI 1.000-1.027, p=0.036), and TPG (OR 1.113, 95% CI 0.992 1.249, p=0.045) were the independent predictors of mortality. CONCLUSION: Our data showed that TPG, total CPB time, pre-transplant renal dysfunction are pre-operative and intra-operative risk factors for mortality after heart transplantation. [ABSTRACT FROM AUTHOR]

Objective: We retrospectively analyzed case records to identify risk factors for mortality in heart transplant recipients in our center. Methods: Data of 123 patients (mean age 40.6±14 years and 97 male) transplanted from February 1998 to April 2009 were studied and analyzed. Pre- and intra-operative variables of the 73 patients who survived (Group 1, mean age 39.1±14.6, 58 male) and 50 patients who died (Group 2, mean age 42.6±12.9, 39 male) were compared. For statistical analysis Student t, Mann-Whitney U and Chi-square tests were used. Logistic regression analysis was used to determine independent risk factors for mortality and survival analysis was accomplished using Kaplan-Meier analysis. Results: Overall mortality in the entire population was 40.6% (50/123). Actuarial survival was 74%, 65%, 51% and 31% at 1, 2, 5 and 10 years respectively. Major causes of death after heart transplantation were infections (30%), right ventricular failure (18%), rejection (12%) and sudden cardiac death (12%). Total ischemic time was 176.7±63.3 minutes (range 90-410 minutes). The total cardiopulmonary by-pass (CPB) time (114.3±27.7 vs 126.9±42.1 min, p=0.05), pre-operative creatinine clearance (81.0±31.5 vs 67.2±33.0 ml/min, p=0.02), urea (45.2±18.6 vs 57.8±35.2 mg/dl, p=0.02), and creatinine (1.01±0.25 vs 1.17±0.36 mg/dl, p=0.05) were significantly different between survivors and non-survivors. Group 2 patients had higher transpulmonary gradient (TPG) (9.21±5.16 vs 12.50±8.26 mmHg, p=0.02) as compared with group 1 patients. Logistic regres sion analysis revealed that preoperative creatinine clearance (OR 0.989, 95% CI 0.973-1.005, p=0.044), creatinine level (OR 2.028, 95% CI 0.288- 14.301, p=0.027), total CPB time (OR 1.013, 95% CI 1.000-1.027, p=0.036), and TPG (OR 1.113, 95% CI 0.992 1.249, p=0.045) were the independent predictors of mortality. Conclusion: Our data showed that TPG, total CPB time, pre-transplant renal dysfunction are pre-operative and intra-operative risk factors for mortality after heart transplantation. [ABSTRACT FROM AUTHOR]

Objective: Myocardial bridge (MB) is a congenital anomaly characterized by narrowing during systole of some of the epicardial coronary arterial segments running in the myocardium. Although, it is considered as a benign anomaly, it may lead to such complications as acute myocardial infarction, ventricular tachycardia, syncope, atrioventricular block and sudden cardiac death. In this study, we aimed to investigate demographic, clinical and angiographic characteristics of the patients with MB found on coronary angiography. Methods: The present study included 71 patients with MB found on coronary angiographies performed in our institution between January 1999 and September 2003. Based on the findings on angiography, the patients were subdivided into group A (n=41) and group B (n=30). The patients in the group A had no atherosclerotic lesion and the patients in the group B had coronary artery disease in addition to MB. Angiographic, demographic and clinical characteristics of both groups were compared. Results: There were no differences between two groups in distribution of gender and risk factors of coronary artery disease whereas mean age of the patients in the group A was lower (47±5 years vs 55±11 years, p=0.01). Frequency of two or more risk factors for coronary artery disease in a particular patient was significantly higher in the group B (55% vs 30%, p=0.03). Myocardial bridge was located at proximal or mid segments of left anterior descending artery (LAD) in 40 patients whereas its presence in both LAD and right coronary artery was found only in one patient in group A. Mean bridging percent was 43±27% in group A. Localization of MB was LAD in 29 patients of group B. One patient with severe aortic valve stenosis in this group had MB at first septal branch. Mean bridging percent was 70±25% in group B, which was significantly higher than in group A (p<0.05). Atherosclerotic narrowing developed in only LAD in 14 patients, LAD and other vessels in 7 patients and in the vessels without MB in 9 patients. In patients with MB in LAD atherosclerotic narrowing of vessel developed proximally to the MB. Clinically, stable angina pectoris was seen more frequently in group A than group B (70% vs 35%, p=0.01), whereas the frequency of acute coronary syndrome was higher in group B (65% vs 30%, p=0.04). In regard to therapeutic approach, more patients in the group A received medical management (80% vs 50%, p=0.01), while more patients in the group B underwent surgical and percutaneous interventions (50% vs 18%, p=0.04). Conclusion: Myocardial bridge probability should be considered in young patients presenting with angina or if the same symptoms are persistent in the patients without more than one risk factor for coronary artery disease. Myocardial bridge may initiate the development of atherosclerotic lesion or may facilitate progression of atherosclerosis in the proximal segment of the vessel. The risk of acute coronary syndrome rises when atherosclerosis is superimposed on MB. Myocardial bridge should be considered in the young patients, presenting with angina or its equivalents without atherosclerotic lesions on coronary angiography. [ABSTRACT FROM AUTHOR]

Natriuretic peptides have long been introduced into clinical practice. These biomarkers have certainly been shown to provide useful information in the diagnosis, prognosis and risk stratification in heart failure and also may have a role in the guidance of heart failure therapy. Although, there are some limitations in using of these markers such as lack of specificity, aging, renal dysfunction or obesity, among the huge number of candidates for heart failure biomarkers, only natriuretic peptides are currently widely used in daily clinical practice in heart failure. Recent heart failure guidelines recognize natriuretic peptides as an essential tool in the new diagnostic and therapeutic algorithms. Furthermore, natriuretic peptides are not only used in the diagnosis or prognosis of heart failure, but also these biomarkers are referred to have some potential role in primary prevention, cardio-oncology, advanced heart failure, assessment of response to cardiac resynchronization therapy, pulmonary arterial hypertension, acute coronary syndromes, atrial fibrillation and valvular heart disease. In this article, natriuretic peptides have been reviewed for their updated information and new recommendations in heart failure and also potential role of these biomarkers in the management of various clinical conditions have been addressed in the form of expert opinion based on the available data in the literature. [ABSTRACT FROM AUTHOR]

Objective: The present study was designed to evaluate the characteristics of pulmonary hypertension (PH) and adult cardiology practice patterns for PH in our country. Methods: We evaluated preliminary survey data of 1501 patients with PH (females, 69%; age, 44.8±5.45) from 20 adult cardiology centers (AdCCs). Results: The average experience of AdCCs in diagnosing and treating patients with PH was 8.5±3.7 years. Pulmonary arterial hypertension (PAH) was the most frequent group (69%) followed by group 4 PH (19%), group 3 PH (8%), and combined pre- and post-capillary PH (4%). PAH associated with congenital heart disease (APAH-CHD) was the most frequent subgroup (47%) of PAH. Most of the patients' functional class (FC) at the time of diagnosis was III. The right heart catheterization (RHC) rate was 11.9±11.6 per month. Most frequently used vasoreactivity agent was intravenous adenosine (60%). All patients under targeted treatments were periodically for FC, six-minute walking test, and echo measures at 3-month intervals. AdCCs repeated RHC in case of clinical worsening (CW). The annual rate of hospitalization was 14.9±19.5. In-hospital use of intravenous iloprost reported from 16 AdCCs in CWs. Bosentan and ambrisentan, as monotreatment or combination treatment (CT), were noted in 845 and 28 patients, respectively, and inhaled iloprost, subcutaneous treprostinil, and intravenous epoprostenol were noted in 283, 30, and four patients, respectively. Bosentan was the first agent used for CT in all AdCCs and iloprost was the second. Routine use of antiaggregant, anticoagulant, and pneumococcal and influenza prophylaxis were restricted in only two AdCCs. Conclusion: Our nationwide data illustrate the current status of PH regarding clinical characteristics and practice patterns. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to an article related to intravascular ultrasound as a method for early detection of transplant vasculopathy after heart transplantation.

Heart failure (HF) is a progressive disorder associated with impaired quality of life, high morbidity, mortality and frequent hospitalization and affects millions of people from all around the world. Despite further improvements in HF therapy, mortality and morbidity remains to be very high. The life-long treatment, frequent hospitalization, and sophisticated and very expensive device therapies for HF also leads a substantial economic burden on the health care system. Therefore, implementation of evidence-based guideline-recommended therapy is very important to overcome its worse clinical outcomes. However, HF therapy is a long process that has many drawbacks and sometimes HF guidelines cannot answers to every question which rises in everyday clinical practice. In this paper, commonly encountered questions, overlooked points, controversial issues, management strategies in grey zone and problems arising during follow up of a HF patient in real life clinical practice have been addressed in the form of expert opinions based on the available data in the literature. [ABSTRACT FROM AUTHOR]

Objective: We aimed to evaluate left atrium (LA) phasic functions and relation with N-terminal pro-B-type natriuretic peptide (NT-proBNP) levels and symptomatic states of the patients with hypertrophic cardiomyopathy (HCM). Methods: Left atrial volume was calculated at end-systole (Vmax), end-diastole and pre-atrial contraction by echocardiography in 75 patients with HCM and 75 control subjects. Left atrial ejection fraction (LAEF), expansion index (LAEI), active emptying volume index (LAAEVI) and fraction (LAAEFr), passive emptying volume index (LAPEVI) and fraction (LAPEFr) were calculated. NT-proBNP levels were measured. Results: Left atrial active emptying volume (LAAEV) positively correlated with Vmax (r=0.343, p=0.003) up to a point, but then reached a plateau with larger LA volumes in HCM group. The LAAEFr was the only variable which was similiar between asymptomatic patients and controls, but was significantly decreased in symptomatic patients (p<0.05). NT-proBNP was correlated with LAEF (r=-0.32, p=0.005), LAEI (r=-0387, p=0.001), and LAAEFr (r=-0.25, p=0.035) but not related with LAPEFr (p=0.4). In receiver operating characteristic curve analysis an NT-proBNP cut-off value of 1415 pg/mL identified reduced LAEF with 87% specificity and 59% sensitivity [AUC=0.77 (95% CI: 0.65-0.89), p=0.004], a cut-off value of 820 pg/mL predicted impaired LAEI with 81% specificity ve 67% sensitivity [AUC=0.78 (95% CI: 0.66-0.9), p<0.001]; while a cut-off value of 1320 pg/mL predicted impaired LAAEFr with 76% specificity and 67% sensitivity [AUC=0.79 (95% CI: 0.68-0.91), p=0.02]. Conclusion: In HCM, LA phasic functions alter according to the Frank-Starling mechanism indicating occurrence of a secondary atrial myopathy. Impairment of LA booster pump function seems to be associated with appearance of symptoms and NT-proBNP levels predict the deterioration of LA reservoir and pump functions in HCM population. [ABSTRACT FROM AUTHOR]

Objective: In this study, patients admitted with the diagnosis of Eisenmenger syndrome (ES) in a tertiary referral center were analyzed. Methods: The data of 20 consecutive patients (mean age: 27.6+1.8 years, 7 male and mean follow-up time: 35.6±9.1 months) with ES were retrospectively analyzed. Demographic characteristics, symptoms, physical examination, laboratory and hemodynamic parameters were analyzed at the time of first admission. Results: The most frequent underlying heart diseases were ventricular septal defect (VSD) with complex congenital disease (n:8, 40%) and isolated VSD (n:7, 35%). 6-minute walking test distance was 347.9±33.7 meters and 15 patients (75%) had a functional capacity of NYHA Class III, at the time of admission. ES was diagnosed with catheterization in all patients and mean systolic pulmonary arterial pressure measured by catheterization was 112±6.8 mmHg. Pulmonary function tests, FVC (forced vital capacity), FEV1 (forced expiratory volume), FEV1/FVC values were respectively, 3.1±0.4, 2.5±0.4 L and 76.7±3.3%. Metabolic tests were performed in all patients at the first visit. Mean VO2 max was 16.7 ±1.0 ml / kg/min and VE/VCO2 rate was 53.9±3.2%. Although PH and partial pressure of carbon dioxide levels were within normal range in blood gas analysis, oxygen saturation and partial pressure of oxygen levels were low. Conclusion: The most common underlying heart disease of ES patients is VSD. In this cases exercise capacity is restricted and this restriction is reflected in laboratory parameters. [ABSTRACT FROM AUTHOR]

Objective: To evaluate postgraduate training period, social life and problems of cardiology residents in Turkey by using a questionnaire form and to compare with the core curriculum of European Society of Cardiology for general cardiology. Methods: Overall, 529 residents of cardiology ages in range of 24-35 years (mean age: 26.5±2.0 years, 81.4% male) participated as volunteers in this cross-sectional survey study. An 86-item questionnaire form was used to evaluate the education process, capacity of knowledge and skill and social effectiveness level of participants. The questionnaire were composed both closed- and open-ended questions. The questionnaire form was filled in with the face-to-face communication method. The data of survey were compared with the core curriculum of European Society of Cardiology for general cardiology training period. Chi-square or Fischer exact test was used for statistical analysis. Results: The participants were working in various university hospitals (70.3%) and training-research (state) hospitals in 31 different provinces in Turkey (40.8% in Marmara region). They visited 40±10 outpatients and 10±5 hospitalized pts daily in the clinics. The 3-5 residents worked at the clinic on night shifts and mostly (89%) 8 or more night shifts per month were held in their first training years. During first three-years of training 76% of residents have performed echocardiography, 40.8%-transesophageal echocardiography and 10%-intraoperative echocardiography. The 84.3% of them evaluated exercise tests, 76.4%-Holter electrocardiography and 53.3%-tilt-table tests. The rate of residents working in coronary angiography laboratories was 54.3%. The 53.7% of residents performed coronary angiography and 64%-only in the 4th year of their training. The number of coronary angiography performance was under expected when compared with European Society of Cardiology curriculum. The 18.5% of residents were participated as assistant researcher in an international multi-center study and only 10% had an article published in national journals (4.3% published in Science Citation Index). The 30.6% considered the cardiology training period in their centers to be insufficient, whereas 37.4% found it partially sufficient and 31.9% sufficient. Only 32.9% of participants could dedicate time for social activities. Conclusion: According to the referred core curriculum of ESC for general cardiology the training of cardiology residents in non-invasive applications is adequate however coronary angiography applications are slightly insufficient in Turkey. In addition, the number of publications per capita is quiet low. [ABSTRACT FROM AUTHOR]

Pulmonary arterial hypertension (PAH) is an uncommon disorder that may be hereditable, idiopathic or associated with conditions like drug exposure, connective tissue disease, HIV infection or congenital heart disease. Familial disease are usually due to mutations in the bone morphogenic protein receptor type 2 (BMPR2), activin-like kinase-type 1 (ALK1) and endoglin (ENG). Functional and structural changes in the pulmonary vasculature lead to increased pulmonary vascular resistance. Vascular remodeling involves endothelial dysfunction, activation of fibroblasts and smooth muscle cells and recruitment of circulating progenitor cells. Vasoconstriction has also been shown to affect the remodeling process. Genetics, cellular and molecular basis of PAH are discussed in the paper. [ABSTRACT FROM AUTHOR]

Acute heart failure syndrome is a heterogeneous group, which requires distinct therapeutic approaches. Diuretics and/or vasodilators for patients with high blood pressure and inotropic agents for patients with low left ventricular ejection fraction and evidence of hypoperfusion are kept in the forefront. Early initiation of therapy along with accurate and early diagnosis in acute heart failure reduces mortality and morbidity significantly. Therefore, it is critically important to develop algorithms, which could easily be followed by all physicians. In this paper, in parallelism with heart failure guidelines and utilizing data of the clinical studies, performed by colleagues of our country in this area, therapeutic algorithms compatible with the conditions of our country are suggested for distinct clinical presentations of acute heart failure. [ABSTRACT FROM AUTHOR]

Objective: Despite impressive advances in therapeutics in the last years, acute heart failure (AHF) remains a major cause of cardiovascular morbidity and mortality. Patients hospitalized because of heart failure (HF), irrespective of left ventricular systolic function, represent a high-risk population with limited short-term prognosis. A substantial component of HF-related mortality occurs during a hospital stay. In this study, we aimed to determine the factors impacting on in-hospital mortality in patients with AHF.Methods: During a 15-month period (December 2005-March 2007), 85 consecutive patients with (mean age: 64±8 years, male: 54%) an episode of AHF were included in this study. The effect of demographic, clinical, electrocardiographic, and echocardiographic characteristics, laboratory findings on in-hospital mortality were evaluated retrospectively. Results: Of 85 patients 24.7% of patients had new-onset HF. Coronary artery disease (61%) was the most common underlying disease. The 44.7% of patients had hypertension, 37.6% had diabetes mellitus, 21% had chronic renal failure and 16.4% had chronic obstructive pulmonary disease. Left ventricular ejection fraction was 35±7%. In-hospital mortality rate was found as 11.7% (10 patients).The major cause of mortality was the progression of HF to cardiogenic shock in 60% of deaths. In comparison with surviving patients in terms of the clinical, demographic, electrocardiographic, and laboratory characteristics and left and right ventricular functions, patients died during hospitalization had higher blood urea nitrogen (45±20 mg/dl vs. 36±12 mg/dl, p=0.04), higher creatinine level (2.2±0.8 mg/dl vs. 1.1±0.5 mg/dl, p=0.001), and wider QRS duration (130±13 ms vs. 116±18 ms, p=0.04) whereas they had lower plasma sodium level (128±5 mmol/l vs. 135±9 mmol/l, p=0.02) and systolic blood pressure (p=0.01). Logistic regression analysis revealed that plasma creatinine level (OR 1.5, 95% CI 1.2 to 2.1, p=0.01), blood urea nitrogen (OR 2.1, 95% CI 1.8 to 3.1, p=0.001), plasma sodium level (OR 1.3, 95% CI 1.1 to 1.7, p=0.02), and systolic blood pressure (OR 2.2, 95% CI 1.9 to 2.8, p=0.01) were the independent predictors of in-hospital mortality. Conclusion: In-hospital mortality increases in patients who had lower systolic blood pressure, lower plasma sodium level, and renal dysfunction on admission. [ABSTRACT FROM AUTHOR]

Objective: Myocardial bridge (MB) is a congenital anomaly characterized by narrowing during systole of some of the epicardial coronary arterial segments running in the myocardium. Although, it is considered as a benign anomaly, it may lead to such complications as acute myocardial infarction, ventricular tachycardia, syncope, atrioventricular block and sudden cardiac death. In this study, we aimed to investigate demographic, clinical and angiographic characteristics of the patients with MB found on coronary angiography. Methods: The present study included 71 patients with MB found on coronary angiographies performed in our institution between January 1999 and September 2003. Based on the findings on angiography, the patients were subdivided into group A (n=41) and group B (n=30). The patients in the group A had no atherosclerotic lesion and the patients in the group B had coronary artery disease in addition to MB. Angiographic, demographic and clinical characteristics of both groups were compared. Results: There were no differences between two groups in distribution of gender and risk factors of coronary artery disease whereas mean age of the patients in the group A was lower (47+/-5 years vs 55+/-11 years, p=0.01). Frequency of two or more risk factors for coronary artery disease in a particular patient was significantly higher in the group B (55% vs 30%, p=0.03). Myocardial bridge was located at proximal or mid segments of left anterior descending artery (LAD) in 40 patients whereas its presence in both LAD and right coronary artery was found only in one patient in group A. Mean bridging percent was 43+/-27% in group A. Localization of MB was LAD in 29 patients of group B. One patient with severe aortic valve stenosis in this group had MB at first septal branch. Mean bridging percent was 70+/-25% in group B, which was significantly higher than in group A (p<0.05). Atherosclerotic narrowing developed in only LAD in 14 patients, LAD and other vessels in 7 patients and in the vessels without MB in 9 patients. In patients with MB in LAD atherosclerotic narrowing of vessel developed proximally to the MB. Clinically, stable angina pectoris was seen more frequently in group A than group B (70% vs 35%, p=0.01), whereas the frequency of acute coronary syndrome was higher in group B (65% vs 30%, p=0.04). In regard to therapeutic approach, more patients in the group A received medical management (80% vs 50%, p=0.01), while more patients in the group B underwent surgical and percutaneous interventions (50% vs 18%, p=0.04). Conclusion: Myocardial bridge probability should be considered in young patients presenting with angina or if the same symptoms are persistent in the patients without more than one risk factor for coronary artery disease. Myocardial bridge may initiate the development of atherosclerotic lesion or may facilitate progression of atherosclerosis in the proximal segment of the vessel. The risk of acute coronary syndrome rises when atherosclerosis is superimposed on MB. Myocardial bridge should be considered in the young patients, presenting with angina or its equivalents without atherosclerotic lesions on coronary angiography. [ABSTRACT FROM AUTHOR]

The article presents a case study of a 18-year-old male patient who underwent heart transplantation for dilated cardiomyopathy. It informs that fistula between right coronary artery (RCA) and pulmonary artery was detected by coronary angiography (CAG). It informs that , treatment was done to prevent complications and to treat present complications.