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Öğe Right ventricular free-wall longitudinal speckle tracking strain in patients with pulmonary arterial hypertension under specific treatment(Wiley, 2017) Kemal, Hatice S.; Kayikcioglu, Meral; Kultursay, Hakan; Vuran, Ozcan; Nalbantgil, Sanem; Mogulkoc, Nesrin; Can, LeventBackground Right ventricular (RV) dysfunction is a major determinant of outcomes in patients with pulmonary arterial hypertension (PAH), although the optimal measure of RV function is poorly defined. We evaluated the utility of RV free-wall speckle tracking strain as an assessment tool for RV function in patients with PAH who are already under specific treatment compared with conventional echocardiographic parameters and investigated the relationship of RV free-wall strain with clinical hemodynamic parameters of RV performance. Methods Right ventricular free-wall strain was evaluated in 92 patients (Group-1 and Group-4 pulmonary hypertension) who were on PAH-specific treatment for at least 3 months. Right atrial (RA) area, RV FAC, TAPSE, tricuspid S, functional class, 6-minute walking distance, and NT-proBNP were studied. The mean duration of follow-up was 222 +/- 133 days. Results All patients were under PAH-specific treatment, and mean RV free-wall strain was -13.16 +/- 6.3%. RV free-wall strain correlated well with functional class (r=.312, P=.01), NT-proBNP (r=.423, P=.0001), RA area (r=.427, P=.0001), FAC (r=-.637, P=.0001), TAPSE (r=-.524, P=.0001), tricuspid S (r=-.450, P=.0001), 6-minute walking distance (r=-.333, P=.002). RV free-wall strain significantly correlated with all follow-up adverse events, death, and clinical right heart failure (RHF) (P=.04, P=.03, P=.02, respectively). According to the receiver operator characteristic analysis, the cutoff value for RV free-wall strain for the development of clinical RHF was -12.5% (sensitivity: 71%, specificity: 67%) and for all cardiovascular adverse events (death included) was -12.5% (sensitivity: 54%, specificity: 64%). Conclusion Assessment of RV free-wall strain is a feasible, easy-to-perform method and may be used as a predictor of RHF, clinical deterioration, and mortality in patients already under PAH-specific treatment.Öğe Sequencing of mutations in the serine/threonine kinase domain of the bone morphogenetic protein receptor type 2 gene causing pulmonary arterial hypertension(Turkish Soc Cardiology, 2016) Mutlu, Zeynep; Kayikcioglu, Meral; Nalbantgil, Sanem; Vuran, Ozcan; Kemal, Hatice; Mogulkoc, Nesrin; Erturk, Biray; Onay, Huseyin; Eroglu, Zuhal; Kultursay, HakanObjective: 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.Öğe Stress Cardiomyopathy (Tako-Tsubo) Associated with Sustained Polymorphic Ventricular Tachycardia(Wiley-Blackwell, 2013) Hasdemir, Can; Vuran, Ozcan; Yuksel, Alper; Yavuzgil, OguzWe present a case of 38-year-old woman with stress cardiomyopathy presenting to the emergency department with a 1-week history of recurrent syncope due to sustained polymorphic ventricular tachycardia. (PACE 2013; 36:e111-e114)Öğe VALUE OF SPECKLE TRACKING FOR THE ASSESSMENT OF RIGHT VENTRICULAR FUNCTION IN PATIENTS WITH CONGENITAL HEART DISEASE ASSOCIATED PULMONARY HYPERTENSION: CORRELATION WITH CONSERVATIVE ECHOCARDIOGRAPHY AND CLINICAL PARAMETERS(Elsevier Science Inc, 2016) Kemal, Hatice S.; Kayikcioglu, Meral; Can, Levent H.; Nalbantgil, Sanem; Vuran, Ozcan; Mogolkoc, Nesrin; Kultursay, Hakan
