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Öğe Assessment of Pulmonary Arterial Hypertension and Vascular Resistance by Measurements of the Pulmonary Arterial Flow Velocity Curve in the Absence of a Measurable Tricuspid Regurgitant Velocity in Childhood Congenital Heart Disease(Springer, 2013) Çevik, Ayhan; Kula, Serdar; Olguntürk, Rana; Tunaoğlu, F. Sedef; Oğuz, A. Deniz; Saylan, Berna; Şanlı, CihatThis study aimed to determine mean pulmonary arterial pressure (PAPmean) and pulmonary vascular resistance (PVR) using transthoracic echocardiography (TTE) measurements of the pulmonary artery flow velocity curve in children with pulmonary arterial hypertension (PAH) and congenital heart disease when the tricuspid regurgitant velocity (TRV) is not sufficient. This study enrolled 29 congenital heart disease cases with pulmonary arterial hypertension and 40 healthy subjects followed at our center. The mean age was 66.9 +/- A 77.9 months in the patient group and 76.3 +/- A 62.1 months in the control group. A positive correlation was found between TRV and systolic pulmonary arterial pressure (r = 0.394, p = 0.035, 95 % confidence interval [CI] = 0.032-0.665), whereas a negative correlation was found between corrected acceleration time (AcTc) and PAPmean (r = -0.559, p = 0.002, 95 % CI = -0.768 to -0.242). Furthermore, a negative correlation was found between parameters TRV and AcTc (r = -0.383, p = 0.001, 95 % CI = -0.657 to -0.019). Based on the cutoff criterion of 124 ms for AcTc, sensitivity was found to be 79.3 % and specificity to be 77.5 % in distinguishing between the PAH patients and the healthy control patients (receiver operating characteristic [ROC] area under the curve [AUC] = 0.804, 95 % CI = 0.691-0.890, p < 0.0001). The sensitivity and specificity of the concomitant use of AcTc and/or TRV were found to be 90 and 73 %, respectively, in distinguishing between the PAH patients and the the healthy control patients. The data obtained by TTE also can be appropriate for measuring PAPmean, PVR, and the vasoreactivity test and for determining the priority of implementing cardiac catheterization even if there is no measurable TRV value.Öğe Elevated Homocysteine and Asymmetric Dimethyl Arginine Levels in Pulmonary Hypertension Associated With Congenital Heart Disease(Springer, 2012) Sanli, Cihat; Oguz, Deniz; Olgunturk, Rana; Tunaoglu, Fatma Sedef; Kula, Serdar; Pasaoglu, Hatice; Cevik, AyhanPulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality among patients with congenital heart disease (CHD). This study was designed to determine biomarker levels in patients with PAH associated with CHD (PAH-CHD) and CHD patients without PAH and to investigate the relationship of these potential biomarkers with hemodynamic findings. In this prospective single-center study, patients with CHD were analyzed according to the presence or absence of PAH and compared with healthy control subjects. Cardiac catheterization and echocardiographs were performed. Plasma homocysteine, asymmetric dimethyl arginine (ADMA), and nitric oxide (NO) levels were determined by enzyme-linked immunosorbent assay. Homocysteine and ADMA levels were higher in the PAH-CHD group (n = 30) than among CHD patients with left-to-right shunting but no PAH (n = 20; P < 0.001) and healthy control subjects (n = 20; P < 0.001). There was no difference in NO levels. Cyanotic PAH-CHD patients had significantly higher homocysteine than acyanotic patients in the same group. No correlation was shown between echocardiographic/hemodynamic parameters and homocysteine, ADMA, and NO levels. Homocysteine and ADMA levels are increased in patients with PAH-CHD. These parameters have the potential to be used as biomarkers in the diagnosis and follow-up evaluation of patients with PAH-CHD. However, large, multicentered prospective studies are required to facilitate routine use of these biologic markers in the clinical setting.Öğe Evaluation of pulmonary vascular resistance and vasoreactivity testing with oxygen in children with congenital heart disease and pulmonary arterial hypertension(Turkish Soc Cardiology, 2014) Cevik, Ayhan; Kula, Serdar; Olgunturk, Rana; Tunaoglu, Sedef; Oguz, Deniz; Saylan, Berna; Sanli, Cihat…Öğe Health-related quality of life and parental depression in children with pulmonary arterial hypertension(Wiley, 2022) Canbeyli, Fatma H.; Atasayan, Vildan; Tokgoz, Semiha; Tunaoglu, Fatma S.; Oguz, Ayse D.; Celik, Bulent; Kula, SerdarBackground Impaired health-related quality of life (HRQoL) is a common problem in pulmonary arterial hypertension (PAH), but there is limited data on HRQoL in children with PAH. We aimed to investigate the QoL, determine the potential risk factors for poor HRQoL in children with PAH, and assess the depression and anxiety of their families. Methods We performed a prospective cross-sectional study of children with PAH, healthy peers, and their parents. HRQoL was measured by the self-reported and age-adapted KINDL questionnaire. Beck Depression Inventory (BDI) and hospital anxiety and depression scale (HADS) were used to assess the depression and anxiety of parents. Results Children with PAH had statistically lower total HRQoL scores than healthy peers (p < 0.001). There was no correlation between HRQoL and duration of disease, World Health Organization functional class, pro-B-type natriuretic peptide, 6-min walk test, and combined or single treatment. BDI and HADS scores were significantly higher in the parents of patients (p < 0.001, p = 0.023, p < 0.001, respectively). There was a negative correlation between HRQoL and BDI in patients (p = 0.016), while there was no significant correlation between HRQoL and HADS (p > 0.05). Conclusion We demonstrated impairment of HRQoL of children with PAH. In addition, there was a correlation between the depression of the families and the QoL of the children.Öğe Persistent left superior vena cava: Experience of a tertiary health-care center(Wiley-Blackwell, 2011) Kula, Serdar; Cevik, Ayhan; Sanli, Cihat; Pektas, Ayhan; Tunaoglu, Fatma Sedef; Oguz, Ayse Deniz; Olgunturk, RanaBackground: The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC. Methods: The present study is based on a retrospective review of 1205 children who consecutively underwent cardiac catheterization from 2000 to 2007. In order to determine the existence of PLSVC, all the subjects routinely underwent superior vena cava (SVC) injection during angiography at the catheter laboratory of the study center. Results: The prevalence of PLSVC was computed to be 6.1% for the present study population. Transthoracic echocardiography was able to detect PLSVC in 32 children (2.6%) whereas angiography diagnosed PLSVC in 74 children (6.1%). The mean age of the patients with PLSVC was 40.09 +/- 50.21 months. A communication between the right and left SVC was determined in 27% of the children who were diagnosed with PLSVC after angiography was performed (20 out of 74). A statistically significant association was present between PLSVC and other congenital cardiac anomalies, including ventricular septal defect (n = 42, 56.8%), atrial septal defect (n = 31, 41.9%), pulmonary stenosis (n = 19, 25.7%), atrioventricular septal defect (n = 10, 13.5%), patent ductus arteriosus (n = 6, 8.1%) and cor triatriatum (n = 3, 4.1%). Conclusion: Transthoracic echocardiography usually visualizes dilated coronary sinus in association with PLSVC. However, SVC injection should be performed in patients undergoing angiography so that morbidity and mortality related with persistent left superior vena cava can be avoided during cardiovascular surgery.Öğe Prognostic value of oxygen saturation and heart rate during a six-minute walk test in pediatric pulmonary hypertension(Tubitak Scientific & Technological Research Council Turkey, 2021) Atasayan, Vildan; Canbeyli, Fatma; Tunaoglu, Fatma Sedef; Oğuz, Ayşe Deniz; Çelik, Bülent; Kula, SerdarBackground/aim: Th evaluate the pre and post-treatment heart rate and oxygen (O-2) saturation values measured before, during and after 6MWT in children who received PAH-specific treatment and to determine the effect of these variables on prognosis. Materials and methods: This retrospective study included 29 patients who were diagnosed as PAH and treated. The transcutaneous O-2 saturation and heart rate levels were recorded before the test: baseline (Sat0, HR0), at the end of the test: exercise (Sal1, HR1) and 5 min after the test: recovery (Sat2, HR2). The increase in heart rate was defined as HR1-HR0 and the decrease in saturation as Sat0-Sat1. The results obtained before and after the PAH-specific treatment were analyzed and their effect on survival was assessed. Results: Fifteen of 29 patients were female (51.7%). The mean age was 127.2 +/- 44.8 months and the median follow-up time was 60 (12-156) months. Only pre-treatment HR1 was found associated with survival. The mean HR1 value of survivors was 122.8 +/- 18.4 and that of deceased 94.3 +/- 19.1 (p = 0.03). Post-treatment 6MWT was associated with survival. It was 453.3 +/- 96.5 in for survivors and 250 +/- 135.2 m for deceased (p = 0.02). Conclusion: Post-treatment 6MWT, FC and proBNP had prognostic value in pediatric patients with PAH. The decrease in saturation and increase in heart rate were not found to have a prognostic value. Pre-treatment HR1 was associated with survival. Identification of these prognostic factors at the beginning and throughout the treatment may be a guide for detecting the severity of the disease and follow-up.
