Familial Mediterranean fever gene mutations as a risk factor for early coronary artery disease.

OBJECTIVE: Cardiovascular diseases (CVD) are very common in the general population. Atherosclerosis is the main pathogenesis. Familial Mediterranean fever (FMF) is an autosomal recessive disease. The gene causing FMF, designated MEFV, encodes a protein called pyrin or marenostrin that is expressed mainly in myeloid bone marrow precursors, neutrophils and monocytes. We herein aimed to determine the prevalence of MEFV mutations (all exon 2, 10 mutations) in patients with early coronary heart disease (early CHD) and coronary heart disease (CHD) with multiple risk factors and among the healthy subjects as controls. METHODS: A total of 197 patients and 119 healthy subjects were recruited and enrolled into three groups in terms of inclusion criteria. Ninety-one patients diagnosed with early CHD enrolled into group one (men < 45 years of age, women < 40 years of age), 106 patients with CHD (men > 50 years of age) to group two and 119 healthy controls enrolled into group three. None of patients was diagnosed with FMF. The diagnosis of CHD was established on electrocardiographic changes, echocardiography and coronary angiography. RESULTS: Thirty-eight patients (41.8%) with early CHD, 17 patients (16%) with CHD and 24 healthy controls (20.2%) carried at least one mutated MEFV allele. Young patients with CHD have different risk factor profiles, clinical presentations and prognoses than older patients. Young patients with CHD usually have multiple risk factors. CONCLUSION: This study suggests that MEFV mutations in early CHD patients had significantly increased in contrast to CHD patients and healthy controls.

Novel Ventricular Repolarization Indices in Patients with Coronary Slow Flow.

Background: Coronary slow flow (CSF) phenomenon is described angiographically as delayed progression of the injected contrast agents through the coronary arteries. Aim of this study was to analyze ventricular repolarization in CSF patients by using Tpeak-Tend interval, Tpeak-Tend/QT ratio, Tpeak-Tend/QTc ratio and other repolarization parameters since these parameters are used as predictors for ventricular arrhythmogenesis. Materials and Methods: We have retrospectively analyzed diagnostic coronary angiography results of 160 patients between 2010 and 2014. Patients were divided into two groups according to coronary flow results. CSF group consisted of 33 female, 82 male patients with mean age 51,9±11,5 years. Control group included patients with normal coronary flow; 13 female, 32 male with mean age 50,8±11,7 years. In all patients, ventricular repolarization parameters as well as other associated electrocardiographic intervals were measured on the twelve-lead surface electrocardiogram. Results: The ventricular repolarization parameters: QTmax interval, QTmin interval, QTc, QTI, QTcI, JTmax interval, JTmin interval, JTdispersion and JTIndex were not significantly different between the groups. However followings parameters differed significantly between patients and controls; QRS (92,8±11,5 msn versus 78,3±16,713,40 msn, respectively; p=0.001), T wave (89±20,2 msn vs. 73,3±13,3 msn respectively, p=0.001), QT dispersion (26,8±17,5 msn vs. 13,5±20,4 msn respectively, p=0.002), JTcorrected (331,6±39,8%; vs. 350,1±39,7% respectively; p=0.01). Furthermore; Tpeak-Tend duration (89±20,2 msn vs. 73,3±13,9 msn respectively; p=0.001), T wave (204±34,9 msn vs. 189,2±24,8 msn respectively; p=0.003), Tpeak-Tend/QT ratio (0,22±0,05 msn vs. 0,19±0,03 msn respectively, p=0.001) were significantly higher in patients compared to controls. Tpeak-Tend/QTc ratio was also significantly higher in the CSF group compared to the controls. (0,21±0,05 msn vs. 0,17±0,03 msn respectively, p =0.001). Conclusion: Ventricular repolarization parameters are prolonged in patients with CSF.

Relationship between Neurocardiogenic Syncope and Ventricular Repolarization.

BACKGROUND: We aimed to analyze ventricular repolarization in neurocardiogenic syncope (NCS) patients by using T-peak-T-end interval, T-peak-T-end/QT ratio, T-peak-T-end/QTc ratio and corrected QT interval (QTc), QT dispersion (QTd), QT index (QTI) and corrected QT interval index (QTcI) comparing with the corrected JT dispersion (JTcd), and corrected JT (JTc) and the corrected JT interval index (JTcI), by inspecting ventricular activation until termination of repolarization. These parameters are used as an index of potential ventricular arrhythmogenesis. METHODS: We have studied patients with head-up tilt table test (HUTT) (+) (33 patients; mean age: 28 ± 11 years) and HUTT (-) as control group (33 patients; mean age: 30 ± 11 years). In all patients, T-peak-T-end interval, T-peak-T-end/QT ratio, T-peak-T-end/QTc ratio, QT, QTd, QTI, QTc, QTcI, JTd, JTc, and JTcI were measured on electrocardiogram. RESULTS: The following parameters were statistically significant between groups: QTd (22.72 ± 17.54 msn; 11.21 ± 13.40 msn; P = 0.004), QTc (424.27 ± 33.75 msn; 403.66 ± 38.08 msn; P = 0.023), QTcI (114.09 ± 14, 29%; 106.71 ± 15.33%, P = 0.047), and QTI (100.72 ± 7.19%; 97.14 ± 7.13%, P = 0.046). Furthermore, T-peak-T-end interval was significantly prolonged in the study group (93.78 ± 20.27 msn; 81.21 ± 11.66 msn; P = 0.003). T-peak-T-end/QT ratio was significantly higher in the study group (0.24 ± 0.04 msn; 0.22 ± 0.04 msn; P = 0.030). T-peak-T-end/QTc ratio was significantly higher in the study group (0.22 ± 0.04 msn; 0.20 ± 0.03 msn; P = 0.015). The JTc and JTd were also significantly higher in the study group ([103.00 ± 9, 72%; 95.44 ± 10.26%, P = 0.003], [27.57 ± 16.01 msn; 10.45 ± 16.08 msn; P < 0.001], respectively). CONCLUSIONS: Electrocardiographic ventricular repolarization parameters including T-peak-T-end interval, T-peak-T-end/QT ratio, T-peak-T-end/QTc ratio, QTc, QTd, QTI, QTcI, JTc, JTd, and JTI are prolonged in NCS.

Extensive vascular and valvular involvement in Gaucher disease.

A 17-year-old patient with type III Gaucher disease was hospitalized for recurrent syncope. Echocardiography showed calcified aortic and mitral stenosis. Three-dimensional computed tomography showed severely calcified plaques on the ascending aorta, arcus, and abdominal aorta. On follow-up, palpitations and syncope were triggered by emotional stress, followed by severe bradycardia that was resulted in cardiogenic shock; the patient died after 8 h despite all efforts. Gaucher disease should be kept in mind in the differential diagnosis of a young patient with extensive vascular and valvular calcification. Patients with symptomatic severe valvular disease must be referred for early surgery.

Effects of lying position on P-wave dispersion in patients with heart failure.

OBJECTIVE: It was the aim of this study to investigate the effects of the right lateral decubitus, left lateral decubitus and supine lying position on P-wave dispersion (PWD) in patients with heart failure (HF). SUBJECTS AND METHODS: Seventeen patients with HF whose ejection fraction was <35% were included in the study. Right lateral decubitus, left lateral decubitus and supine electrocardiogram (ECG) recordings were obtained. The recordings for each of the three positions were taken after the patients had maintained each position for 30 min to ensure a stabilized hemodynamic position. For the baseline recording, in supine position, there was no 30-min waiting period before the ECG. RESULTS: After the right lateral decubitus position, there was a statistically significant reduction in the longest P-wave duration (100.0 ± 14.5 and 84.7 ± 16.2 ms; p = 0.001) and a significant decrease in PWD (41.7 ± 8.0 and 24.1 ± 7.1 ms; p < 0.0001). After the left lateral decubitus position, there was no significant change between the baseline PWD values (41.7 ± 8.0 and 40.2 ± 9.7 ms; p = 0.606). After the supine position, there was no significant change between the baseline PWD values (41.7 ± 8.0 and 39.7 ± 9.4 ms; p = 0.427). CONCLUSION: Our study revealed that patients' PWDs and maximum P-wave durations were lower in the right lateral decubitus lying position than in other positions. The clinical implication of this study needs to be further explored.

Prognostic role of echocardiographic and hematologic parameters in heart failure patients complicated with incidental pleural effusion diagnosed during echocardiographic evaluation.

We aimed to determine long-term prognostic role of echocardiographic and hematologic parameters in heart failure patients complicated with incidental pleural effusion (PE) diagnosed during echocardiographic evaluation. The study was performed by evaluating patient records in which PE was incidentally detected during echocardiographic examination in a tertiary teaching hospital between Jan 2002 and Dec 2012. Total 151 patients with heart failure complicated with PE were analyzed. All patients' mortality data were collected from registry center of Social Insurance Institution which is officially responsible for recording all mortality data in Turkey. Detailed echocardiographic and hematologic parameters including creatinine, uric acid, albumin, sodium, potassium, hemoglobin at the time of hospital admission were analyzed. Data from 151 eligible patients were analyzed. We compared patients who died (n = 51) during follow-up with the survivors (n = 100) in terms of patients' echocardiographic and hematological features. Mean duration of follow-up was 71.5 ± 45.6 months. Fifty-one patients (33.8 %) died during this follow-up period. From all echocardiographic parameters only dilated left atrium (LA) diameter was found to be associated with a poor prognosis (p = 0.034). Low albumin, sodium and hemoglobin levels were associated with poor prognosis (p < 0.001, p = 0.002 and p = 0.007, respectively). We showed that dilated LA, as well as low albumin, low sodium and low hemoglobin levels had a worse prognostic significance than patients with normal LA size, within normal limits of albumin, sodium and hemoglobin levels in patients with heart failure complicated with PE determined incidentally by echocardiography.

Prognostic role of incidental pleural effusion diagnosed during echocardiographic evaluation.

BACKGROUND: The purpose of this study was to determine the long-term prognostic implications of incidental pleural effusion (PE) detected during echocardiographic examination and its relationship with concomitant diseases. HYPOTHESIS: The study hypothesis is to test whether incidental pleural PE detected during echocardiographic examination be used as a prognostic marker. METHODS: The study was performed by evaluating patient records (N = 251) in whom PE was incidentally detected during echocardiographic examination in a tertiary hospital between 1999 and 2012. The patients were classified into 4 major groups according to the concomitant primary disease: malignancy, and cardiovascular, renal, and pulmonary diseases. The total survival time was obtained from hospital records for patients who died during the hospital stay and social security institution records for patients with out-of-hospital death. RESULTS: One-year and 5-year life expectancies of PE cases concomitant with different disorders were as follows; heart failure (n = 151), 81% and 70%; malignancies (n = 45), 53% and 44%; pulmonary diseases (n = 37), 89% and 78%; renal diseases (n = 18), 100% and 83%; respectively. PE associated with heart failure, renal disease, and pulmonary disease had similar (P > 0.05 for all) and favorable outcomes compared to PE associated with malignancies (P < 0.001). CONCLUSIONS: The prognosis of incidental PE was the worst in patients with concomitant malignancies; however, PE associated with nonmalignant diseases including heart failure, pulmonary disease, and renal disease have similar and favorable outcomes.

Anxiety score as a risk factor for radial artery vasospasm during radial interventions: a pilot study.

We determined the role of anxiety level on radial artery spasm during transradial coronary angiography. Eighty-one patients who had an indication for coronary angiography were enrolled. Radial artery vasospasm was determined by addressing 5 signs: persistent forearm pain, pain response to catheter manipulation, pain response to sheath withdrawal, difficult catheter manipulation after being "trapped" by the radial artery, and considerable resistance on withdrawal of sheath. Radial spasm defined as at least 2 of the 5 signs. The Hamilton Anxiety scale questionnaire was used to determine the level of anxiety. Vasospasm was observed in 19.1% of the patients. Anxiety score was significantly higher in women (11.1 ± 7.2 vs 17.6 ± 7.3; P < .001). Vasospasm was significantly correlated with female sex (P < .001, r = .43) and anxiety score (P = .007, r = .29). Female sex was associated with higher anxiety scores (P < .001, r = .43). In conclusion, higher anxiety scores and female sex are important risk factors for radial artery vasospasm.

Myocardial Performance Index in Neurocardiogenic Syncope Patients.

BACKGROUND: Many syncopes resulting from neural reflexes in various conditions are called neurocardiogenic syncope (NCS). We aimed to investigate the presence of left ventricular (LV) myocardial performance index (MPI) in patients with NCS, which was diagnosed with head-up tilt table test (HUTT), and the accurateness of the test in order to use it as a method in patients with NCS. Assuming the MPI as a potential cause of syncope, we assessed the Tei index with non-invasive tissue Doppler echocardiography method. METHODS: Consecutive outpatients with a history of recurrent unexplained syncope underwent HUTT. Twenty-nine HUTT (+) patients (24 female and five male, mean age: 30 ± 15 years) as the study group and HUTT (-) 23 healthy patients (six female and 17 male, mean age: 34 ± 16 years) as the control group were included into the study. Conventional and tissue Doppler echocardiography was performed to both groups. The MPI was determined by using PW Doppler. Measurements of Doppler time intervals, according to Tei index ((isovolumic contraction time + isovolumic relaxation time)/ejection time) is calculated as (a - b/b), where "a" is the interval between cessation and onset of the mitral inflow, and "b" is the ejection time (ET) at the LV outflow. RESULTS: When comparing the groups in terms of MPI and ET, there was significant difference between groups. Patients with NCS had significantly longer ET and lower MPI value than control group (284 ± 24 ms vs. 260 ± 24 ms, P < 0.001, respectively and 0.44 ± 0.7 vs. 0.52 ± 0.8, P < 0.001, respectively). There was no significant difference in ejection fraction between groups. CONCLUSION: In the present study, LV MPI value decreases in patients with NCS.

Relation of mitral annular dilation with dynamic mitral regurgitation in patients with rheumatic mitral regurgitation.

INTRODUCTION: Dynamic mitral regurgitation (MR) is frequently investigated in patients with left ventricular systolic dysfunction (LVSD). Data about the dynamic MR in patients with organic valve disease are limited. The aim of this study was to evaluate the alteration of MR by exercise in patients with rheumatic valve disease (RVD). METHODS: Asymptomatic patients with rheumatic MR and normal left ventricular function had been included in our study. Transthoracic echocardiography and Doppler measurements were performed at rest and just after submaximal exercise test performed with treadmill. Severity of MR was evaluated quantitatively by measuring effective regurgitant orifice area (EROA) with flow convergence method. RESULTS: A total of 34 patients with rheumatic MR had been included. Severity of MR increased in 10 patients with exercise (Group 1) and decreased in 24 of them (Group 2). When the variables of two groups were compared; diastolic blood pressure after exercise, EROA, left atrial volume, left ventricular diastolic volume and mitral annular area values were significantly higher in Group 1 patients. A linear regression model was constructed by considering change of EROA by exercise the dependent, and the variables showing significant differences as the independents. Mitral annular area was found to be independently associated with EROA increase with exercise (R(2) = 0.499; P < 0.001). CONCLUSION: Mitral annular dilation is independently associated with increase of MR with submaximal exercise in asymptomatic patients with MR due to RVD with normal left ventricular function.

[Intracardiac echocardiography-guided device closure of atrial septal defects: our initial experience]. / Intrakardiyak ekokardiyografi esliginde sekundum atriyal septal defekt kapatilmasi: Klinigimizin baslangiç tecrübesi.

OBJECTIVES: Although intracardiac echocardiography (ICE) has long been used for various cardiologic interventions, its utilization has been quite limited in Turkey. We assessed our experience with the use of ICE during transcatheter closure of secundum atrial septal defects (ASD). STUDY DESIGN: Fourteen patients (8 females, 6 males; mean age 34 years; range 15 to 62 years) underwent transcatheter device closure of ASD with ICE guidance. Before the procedure, 13 patients were examined by transesophageal echocardiography (TEE). Intracardiac echocardiography was used to evaluate the interatrial septum, defect size, the relationship of the septal occluder with neighboring structures before its release, and residual shunts after device release. RESULTS: Using short- and long-axis ICE images, the anteroposterior and superoinferior rims of the ASD, coronary sinus, and pulmonary vein openings were successfully visualized in all the patients. Defect diameters measured by ICE were closely correlated with those measured by TEE (97%) and balloon sizing (95%). The defects were closed successfully in 13 patients; the procedure was terminated in one patient due to the prolapse of both discs into the left atrium. There was no procedural complication. One patient experienced gastrointestinal hemorrhage that required blood transfusion two days after the procedure. No residual shunts were observed on follow-up transthoracic echocardiographic examinations one and six months after the procedure. CONCLUSION: Having high image quality and color Doppler features, ICE is quite functional in determining defect size, position of the septal occluder and its relationship with neighboring structures; thus, it is a reliable alternative to TEE which is used routinely in transcatheter closure of ASDs.

[Closure of secundum atrial septal defects by the Amplatzer occluder device]. / Sekundum atriyal septal defektlerin Amplatzer cihazi ile kapatilmasi

OBJECTIVES: Percutaneous closure of secundum atrial septal defects (ASD) has become an important alternative treatment to surgery. We evaluated our clinical experience with, and short-term results of transcatheter closure of ASDs with the Amplatzer septal occluder in adult patients. STUDY DESIGN: The study included 52 patients (36 women, 16 men; mean age 33±14 years; range 14 to 69 years) who underwent transcatheter ASD closure with the Amplatzer occluder device. The mean ASD diameter measured by transesophageal echocardiography was 19.5±5.7 mm and the mean device diameter was 24.5±5.7 mm. All the patients were assessed clinically and echocardiographically one month after the procedure. RESULTS: Transcatheter ASD closure was successfully performed in 48 patients (92.3%) and failed in four patients (7.7%). Echocardiographic controls showed significant decreases in tricuspid regurgitation, right ventricular dilatation, and pulmonary artery pressure (p=0.003, p=0.026, and p=0.0001, respectively). Functional capacity of the patients also showed significant improvements (p=0.0001). After implantation, residual shunts were detected in four patients, all of which disappeared one month after the procedure. Major complications were seen in two patients. One patient developed ventricular fibrillation immediately after the procedure due to device embolization. One patient with left ventricular dysfunction developed device thrombosis due to cessation of dual antiplatelet therapy, which was successfully treated by anticoagulation therapy. Arrhythmia was not observed in any patient. CONCLUSION: Percutaneous closure of secundum ASDs with the Amplatzer occluder device is a safe and effective procedure with a high success rate.

Embolized Amplatzer septal occluder device presenting with ventricular fibrillation.

The Amplatzer septal occluder device is used with high success and low-complication rate. Device embolization occurs in around 0.3-0.55% of cases. We report the case of a 44-year-old man with ventricular fibrillation (VF) due to embolization of Amplatzer septal occluder into the right ventricle, 1 h after the successful closure of secundum ASD with a 26-mm Amplatzer septal occluder device. The patient was immediately defibrillated. Bedside echocardiography revealed a free floating device in transit in the right chambers. Percutaneous retrieval attempt was unsuccessful. He was referred to surgery immediately. To our knowledge, VF has not been reported yet.

Relationship between mean platelet volume and atrial thrombus in patients with atrial fibrillation.

Platelets and clotting cascade play a major role in development of atrial thrombus in patients with atrial fibrillation. The mean platelet volume (MPV) reflects platelet size and is considered a marker and determinant of platelet function because larger platelets are hemostatically more reactive than platelets of normal size, increasing the propensity to thrombosis. We have investigated the relationship between MPV and left atrial thrombus in patients with persistent atrial fibrillation. A total of 205 consecutive patients (men: 67.3%, women: 32.7%; mean age: 62.3 ± 12.8) who had persistent atrial fibrillation, undergone transesophageal and transthoracic echocardiography. Study individuals were divided into two groups. Group 1 (n: 96, 46.8%): atrial fibrillation complicated with atrial thrombus and group 2 (n: 109, 53.2%): atrial fibrillation free of thrombus, which was identified by means of transesophageal echocardiogram. The MPV, platelet distribution weight, and platelet count were measured. There was no difference in terms of MPV, platelet distribution weight, and platelet count in two groups. MPV was not correlated with thrombus and spontaneous echo contrast. Left atrial thrombus was included in multivariate logistic regression analysis and only low ejection fraction was a predictor of left atrial thrombus (P = 0.04). This is first report showing that MPV is not related with left atrial thrombus in patients with atrial fibrillation. According to our result, MPV cannot be considered as an index of left atrial thrombus in patients with atrial fibrillation.

Relationship between renal stone formation, mitral annular calcification and bone resorption markers.

BACKGROUND AND OBJECTIVES: Mitral annular calcification (MAC) is associated with osteoporosis and there is evidence of reduced bone mineral density (BMD) in patients with renal stone formation (RSF). Therefore, we designed this study to test if RSF was associated with MAC and if this association could be linked to bone resorption. METHODS: Fifty-nine patients (mean age, 41.5 years) with RSF and 40 healthy subjects (mean age, 44.2 years) underwent screening for MAC and BMD, and measurements were taken of serum and urine electrolytes, parathyroid hormone, alkaline phosphatase and urine dypyridoline. RESULTS: MAC was diagnosed in 11 (18%) patients with RSF compared with 1 (2.5%) control (P=.01). Urine phosphorus, magnesium, sodium, potassium and chloride levels were lower (P<.001, P=.02, P<.001, P<.001 and P<.001, respectively), but serum alkaline phosphatase, calcium and potassium levels were higher (P=.008, P=.007 and P=.001, respectively) in patients with RSF versus those without RSF. None of these abnormalities were found in patients or subjects with MAC. Urine pyridoline levels were higher and T-scores were more negative (more osteopenic) in patients and subjects with MAC than in those without MAC (P=.01 and P=.004, respectively). In a multivariate analysis, only T-scores and urine dipyridoline level were predictive of MAC (P=.03 and P=.04, respectively). CONCLUSIONS: Screening for MAC and bone resorption markers in patients with RSF demonstrated a high incidence of MAC in these patients. The presence of MAC in patients with RSF was associated with bone resorption markers. This seemingly complex interrelationship between RSF, MAC and bone loss needs to be clarified in further studies.