Evren Semizel - Academia.edu (original) (raw)

Papers by Evren Semizel

Research paper thumbnail of Epileptik çocuklarda QT dispersiyonu ve Antiepileptik İlaçların QT dispersiyonu üzerine etkisi

Erciyes Medical Journal - Erciyes Tıp Dergisi.

Research paper thumbnail of The Turkish Journal of Pediatrics 2009; 51: 22-27 Original Carvedilol therapy in pediatric patients with dilated cardiomyopathy

Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited... more Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. The objective of this study was to determine the dosing, efficacy and side effects of carvedilol for the management of dilated cardiomyopathy in children. Sixteen children with idiopathic dilated cardiomyopathy, aged 7 months to 138 months and with an ejection fraction less than 40%, were treated with carvedilol. The average initial dose was 0.1 mg/kg/day and it was uptitrated to 0.4 mg/kg/day. After six months on carvedilol, there were improvements in clinical scoring system from an average of 2.94 to 2.50 (p<0.05), in mean fractional shortening from 17.2±6.1 % to 22.7±5.1 % (p<0.05), and in ejection fraction from 35.2±6.8 % to 43.1±11.2 % (p<0.05). No side effect was observed during the study period. Two patients died due to serious infection. Carvedilol in addition to standard therapy for dilated cardiomyopathy in children ...

Research paper thumbnail of Kronik Böbrek Yetersizliği Olan Hastalarda Kardiyak Tutulum

Güncel Pediatri, 2004

Kardiyak problemler bircok cocuk ve adolesan kronik bobrek yetersizlikli KBY , son donem bobrek y... more Kardiyak problemler bircok cocuk ve adolesan kronik bobrek yetersizlikli KBY , son donem bobrek yetersizlikli SDBY , ve bobrek transplantasyonu yapilmis olan hastada gozlenmekte olup 1 ; 1987-1990 yillari arasinda Avrupa’da renal replasman tedavisi altinda olan cocuk hastalarda, dializde olanlarin %51 ve transplant yapilanlarin %37’sinin mortalitesinde kardiyak nedenlerin sorumlu oldugu gosterilmistir 2 .

Research paper thumbnail of Konjestif Kalp Yetmezliği

Güncel Pediatri, 2006

Konjestif kalp yetmezligi KKY , kalbin dokularin metabolik gereksinimlerini karsilayacak miktarda... more Konjestif kalp yetmezligi KKY , kalbin dokularin metabolik gereksinimlerini karsilayacak miktarda kani perifere pompalayamamasi sonucu olusan sistemik ve pulmoner konjesyon ile karakterize klinik bir durumdur.

Research paper thumbnail of Akut Romatizmal Ateş

Güncel Pediatri, 2005

Akut romatizmal ates ARA , grup A beta hemolitik streptokokal GAS farenjit sonrasi ortaya cikan s... more Akut romatizmal ates ARA , grup A beta hemolitik streptokokal GAS farenjit sonrasi ortaya cikan sistemik bir hastaliktir. ARA, kalp ve kalp kapakciklari uzerinde kronik ilerleyici hasara neden olabilir. Akut romatizmal ates, 1960’lara kadar cocukluk cagi olumlerinin ve yapisal kalp hastaliklarinin baslica nedeni olarak gosterilmekteydi.

Research paper thumbnail of Left Ventricular Aneurysm ina 4-Year-Old Boy

Texas Heart Institute Journal from the Texas Heart Institute of St Luke S Episcopal Hospital Texas Children S Hospital, 2005

Research paper thumbnail of Carvedilol therapy in pediatric patients with dilated cardiomyopathy

The Turkish journal of pediatrics

Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited... more Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. The objective of this study was to determine the dosing, efficacy and side effects of carvedilol for the management of dilated cardiomyopathy in children. Sixteen children with idiopathic dilated cardiomyopathy, aged 7 months to 138 months and with an ejection fraction less than 40%, were treated with carvedilol. The average initial dose was 0.1 mg/kg/day and it was uptitrated to 0.4 mg/kg/day. After six months on carvedilol, there were improvements in clinical scoring system from an average of 2.94 to 2.50 (p<0.05), in mean fractional shortening from 17.2 +/- 6.1% to 22.7 +/- 5.1% (p<0.05), and in ejection fraction from 35.2 +/- 6.8% to 43.1 +/- 11.2% (p<0.05). No side effect was observed during the study period. Two patients died due to serious infection. Carvedilol in addition to standard therapy for dilated cardiomyopathy ...

Research paper thumbnail of Eisenmenger syndrome: identifying the clues for arrhythmia

Anadolu kardiyoloji dergisi : AKD = the Anatolian journal of cardiology, 2008

The aim of this case-controlled, cross-sectional study is to investigate the tendency towards arr... more The aim of this case-controlled, cross-sectional study is to investigate the tendency towards arrhythmia using noninvasive arrhythmia markers (QT dispersion and heart rate variability) in children with Eisenmenger syndrome. We studied 23 patients, whose pulmonary-to-systemic resistance ratio was calculated to be greater than 0.75, and who were diagnosed as Eisenmenger syndrome between 1990 and 2001. Twenty healthy children were studied as the control group. Electrocardiographic recordings with calculation of QT dispersion, Holter monitoring, echocardiographic studies and heart rate variability (HRV) analysis were performed in both groups. Catheterization records were analyzed in all the patients. QT and QTc dispersion were higher (p=0.007 and p=0.006, respectively) and PR interval was longer (p=0.009) in the patients with Eisenmenger syndrome, than those in the control group. In addition, low frequency component, high frequency component, very low frequency component, and total powe...

Research paper thumbnail of Left ventricular aneurysm in a 4-year-old boy

Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2005

Left Ventricular Aneurysm in a 4-Year-Old Boy Images in Cardiovascular Medicine Left Ventricular ... more Left Ventricular Aneurysm in a 4-Year-Old Boy Images in Cardiovascular Medicine Left Ventricular Aneurysm in a 4-Year-Old Boy 4-year-old boy with a history of acute lymphoblastic leukemia was referred for evaluation of cardiac function due to progressive decline in exercise tolerance and to cardiomegaly on chest radiography. Six months earlier, there had been no such finding on the chest film. Echocardiography, also performed at the earlier visit, had yielded a normal result. Echocardiography now showed severe left ventricular dysfunction. In addition, a parasternal long-axis view revealed a large aneurysmal sac, estimated to be 8 × 6 cm, in the apical wall of the left ventricle (Fig. 1). The aneurysm's wall was much thinner than a normal left ventricular wall. Because the thin tissue posed a risk of perforation, and because echocardiography was enough for diagnosis, we did not perform angiography. The patient was started on anticoagulation therapy due to the risk of thromboemboli and was given medication for left ventricular systolic dysfunction, in preparation for elective surgery. The operation was performed through a median sternotomy with use of cardiopulmonary bypass and aortic cross-clamping. At operation, the left ventricle was seen to be dilated and the left ventricular wall to have a thin myocardial outpouching (Fig. 2) near the apex. Aneurysmectomy was performed, after which the ventriculotomy was closed with a knitted Dacron patch using running suture (Fig. 3). The morphology of the aneurysm's sac was elastic, its thickness was 2 mm, and its internal surface was covered by thrombus. The patient made an uneventful recovery and was discharged from the hospital on postoperative day 7. Histologic examination of the material obtained during operation showed nonspecific inflammation. Echocardiography 1 month after the operation showed no residual aneurysm and partial improvement in left ventricular function (Fig 4). The patient was observed to be symptom-free during the next 12 months of follow-up.

Research paper thumbnail of Permanent form of junctional reciprocating tachycardia and tachycardia-induced cardiomyopathy treated by catheter ablation: a case report

The Turkish journal of pediatrics

The permanent form of junctional reciprocating tachycardia (PJRT) is usually refractory to drug t... more The permanent form of junctional reciprocating tachycardia (PJRT) is usually refractory to drug therapy, and these patients are at risk of developing tachycardia-induced cardiomyopathy. The electrocardiogram insribes inverted P waves in leads 2, 3, aVF as well as left lateral leads, along with a P-R interval shorter than R-P interval during the tachycardia. This report describes a three-year-old male patient with PJRT who underwent successful radiofrequency catheter ablation (RFA) of accessory pathway. On transthoracic echocardiography of patient, decreased ventricular systolic function was observed. RFA was performed by applying radiofrequency pulses. Echocardiograms of the patient, two months after catheter ablation, demonstrated progressive improvement of ventricular function. Transcatheter radiofrequency ablation of accessory pathways in patients with PJRT is an effective, and possibly preferable, form of treatment, especially in cases of tachycardia refractory to multiple pharm...

Research paper thumbnail of Triosephosphate isomerase deficiency with elevated sweat chloride test: report of a case

The Turkish journal of pediatrics

A 15-month-old girl with severe hemolytic anemia and progressive respiratory failure is presented... more A 15-month-old girl with severe hemolytic anemia and progressive respiratory failure is presented. She was well until the age of six months when she developed a pulmonary infection. During the next six months, she had frequent respiratory infections and her paleness became evident. At the age of 12 months, she was observed to have easy fatigability and muscle weakness, and she received her first blood transfusion. She was referred to our hospital at the age of 15 months. The physical examination revealed a malnourished girl with hypotonia, nystagmus, generalized muscle weakness and severe breathing difficulty requiring ventilatory support The hemoglobin (Hb) was 9.7 g/dl; hematocrit (Hct) 29%, mean corpuscular volume (MCV) 101 fl and reticulocyte count 15%. Peripheral blood smear revealed macrocytosis and stomatocytosis (30% of the red cells) and polychromasia. Sweat chloride test was 90 and 94 mEq/L on two separate occasions. The serum vitamin E level was 0.26 mg/dl (N: 0.44-0.68)....

Research paper thumbnail of Evaluation of electrocardiographic parameters for early diagnosis of autonomic dysfunction in children and adolescents with type-1 diabetes mellitus

Pediatrics International, 2014

Background Cardiovascular complications are continuing to be a major cause of morbidity and morta... more Background Cardiovascular complications are continuing to be a major cause of morbidity and mortality in diabetes mellitus type I (DMTI). The study aimed to evaluate the rate of changes in ECG parameters in children with DMTI compared with healthy children. Materials and Methods This case-control study was performed on 140 participants which consisted of 70 patients with DMTI (case group) matched in sex and age with 70 healthy ones (control group) in two centers in collaboration with Ali Asghar Hospital, Zahedan, Iran, between March 2017 and April 2018. Electrocardiography was performed in all participants (patients and healthy), and heart rate, QT (start of the Q wave and the end of the T wave) QTc interval (QT/ √ RR), QTd (dispersion between maximum and minimum of QT), and QTcd (dispersion between maximum and minimum of QTc) were measured. Duration of diabetic and level of HbA1c were obtained for patients and the effects of these parameters on ECGs were investigated. Results Height, weight and heart rate were higher in the patients (P<0.001) when S in V1 was higher in case (6.16±3.23) compared to the control group (4.33± 2.22) (P<0.001). QT (356.71±27.28 compared to 347.00±23.55), QTd (49.00±14.66 compared to 41.21±8.32), and QTcd (60.47±17.32 compared to 49.93±10.44) were higher in case group (p<0.05). Hemoglobin A1C (p=0.043) was higher in boys and heart rate was higher in girls (p<0.001). Diabetic time duration and HbA1c normality states did not change the length of these parameters in diabetic patients. Conclusion Based on the results, QT, QTd, QTc and QTcd were higher in patients with DMTI. Diabetic time duration and HbA1c states did not change the ECG parameters in diabetic patients.

Research paper thumbnail of Costello Sendromunda Kardiyak Tutulum: Altı Olgunun Değerlendirilmesi

Güncel Pediatri, 2013

Cardiac Involvement in Costello Syndrome: Evaluation of Six Cases ÖZET Gi rifl: Costello sendromu... more Cardiac Involvement in Costello Syndrome: Evaluation of Six Cases ÖZET Gi rifl: Costello sendromu postnatal büyüme ve gelişme geriliği, kaba yüz, gevşek deri, ilerleyici olmayan kardiyomiyopati ve sempatik kişilik ile karakterize otozomal dominant geçiş gösterdiği düşünülen bir sendromdur. Bu çalışmanın amacı Costello sendromu tanısı konulan ve konjenital kalp hastalığı olan altı olgunun klinik bulgularının değerlendirilmesidir. Gereç ve Yöntem: Çalışmada üfürüm nedeni ile çocuk kardiyoloji polikliniğine 2002-2013 yılları arasında başvuran ve Costello sendromunun fenotipik özellikleri olan 6 olgu değerlendirildi. Olguların yakınmaları, klinik bulguları, tedavi ve klinik gidişi incelendi. Bulgular: Olguların 3'ü kız, 3'ü erkekti ve ortalama yaşları 34±12 ay (7 ay-11 yaş) idi. Tüm hastalarda kaba yüz görünümü, el içi ve ayak tabanı çizgilerinde belirginlik, hipertelorizm, epikantus, basık burun kökü ve kardiyak muayenelerinde üfürüm mevcut idi. Hastaların hepsinde konjenital kalp hastalığı saptandı. Hastaların 5'inde değişik derecelerde pulmoner stenoz saptandı ve bu hastaların 3'üne balon pulmoner valvüloplasti yapıldı. 3 hastada atriyal septal defekt tespit edilirken 1 hastada hipertrofik kardiyomiyopati saptandı. 2 hastada mental retardasyon mevcut idi. Sonuç: Psikomotor gelişme geriliği, kaba yüz görünümü, seyrek saçları, derin avuç içi ve ayak tabanı çizgileri olan ve depo hastalığı düşünülen hastalarda özellikle pulmoner stenoz veya hipertrofik kardiyomiyopati varlığında Costello sendromu mutlaka akla gelmelidir.(Gün cel Pe di at ri 2013; 11: 63-7) Anah tar ke li me ler: Costello sendromu, kardiyomiyopati, pulmoner kapak stenozu, kaba yüz, depo hastalığı SUM MARY In tro duc ti on: Costello syndrome is probably an autosomal dominant inherited disorder that is characterized by postnatal growth retardation, developmental delay, coarse facies, loose skin, nonprogressive cardiomyopathy and friendly behavior. The aim of this study was to evaluate a variety of clinical findings and cardiac involvement of six patients diagnosed as Costello syndrome. Materials and Methods: Six cases that had a typical features of Costello syndrome admitted to pediatric cardiology department due to cardiac murmur between 2002-2013 years were evaluated. Their complaints, clinical findings, treatments and clinical courses were examined. Results: Three of the patients were male and the mean age of patients was 34±12 months (7 months to 11 years). All of the cases had coarse faces, deep palmar and plantar creases, hypertelorism, epicanthal folds, depressed nasal bridges and cardiac murmur at auscultation. Congenital heart disease was found in all patients. Five patients had pulmonary stenosis and balloon pulmonary valvuloplasty was performed in 3 of these patients. Atrial septal defect was detected in 3 patients and 1 of the patient had hypertrophic cardiomyopathy. Mental retardation was found in 2 patients. Conclusions: Costello syndrome must be keep in mind in all patients with psychomotor retardation, coarse faces, sparse hairs, deep palmar and plantar creases, phenotypical features resembling a storage disease especially in association with pulmonary stenosis and hypertrophic cardiomyopathy.

Research paper thumbnail of Congenital Anomalies of Coronary Arteries in Children: The Evaluation of 22 Patients

Pediatric Cardiology, 2013

Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing co... more Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing coronary angiography, the data for the pediatric population are few. This study of 22 children with coronary artery anomalies evaluated them in terms of demographic and clinical features and analyzed their angiographic findings and surgical results. Databases in the Department of Pediatric Cardiology at the University of Uludag were searched for all the patients with a diagnosis of congenital coronary artery anomaly who underwent coronary angiography between 1993 and 2013. Patients with coexistent congenital heart disease were excluded from the study. The study noted 22 patients (0.9 %; 10 boys and 11 girls) with coronary artery anomalies. The mean age of these patients was 58.77 ± 52.04 months (range, 1 month-16 years). Coronary arteriovenous fistula (50 %) and anomalous left coronary artery from the pulmonary artery (ALCAPA) (36 %) were the most common anomalies. In addition, the study included one patient with diffuse coronary artery hypoplasia, one patient with muscular bridge, and one patient with left main coronary artery originating from the right aortic sinus valsalva. Of the 11 patients who had coronary atrioventricular fistula, 7 were asymptomatic, whereas 75 % of the patients with ALCAPA syndrome were admitted because of heart failure. Although 13 patients had an exact diagnosis by echocardiography, 50 % of the patients with ALCAPA syndrome had their diagnosis determined by catheter angiography performed because of severe mitral regurgitation or dilated cardiomyopathy. The mortality rate for all the patients was found to be 18.1 %. Eight patients with coronary arteriovenous fistula have been followed up without surgery to the present. In contrast, seven patients with ALCAPA syndrome have undergone surgery, and three have died. Two of these patients died during the postoperative period, and the remaining patient died suddenly during the preoperative period at home. Isolated congenital coronary artery anomalies are very rare in the pediatric population. Although most congenital coronary artery anomalies are clinically silent, they may be associated with severe symptoms in children. Recognition of potentially serious anomalies such as ALCAPA syndrome is mandatory so that early surgical treatment can be prescribed.

Research paper thumbnail of Treatment of infective endocarditis with recombinant tissue plasminogen activator

Pediatric Blood & Cancer, 2008

Infective endocarditis (IE) caused by microbial infection is virtually always fatal if untreated.... more Infective endocarditis (IE) caused by microbial infection is virtually always fatal if untreated. High-dose and long-term antibiotic treatment is required to eradicate microorganisms. If increased risk of embolic events, persistent infection, and progressive cardiac failure are present, surgery is indicated. However, surgery can carry an increased risk of mortality and morbidity in critically ill children of whom other treatment options such as administering, a thrombolytic agent; recombinant tissue plasminogen activator (r-tPA) could be an alternative choice. Here, we report a 14-year-old male with Down syndrome and acute myeloblastic leukemia, diagnosed with IE characterized by two large vegetations on aortic and mitral valves, who was successfully treated with r-tPA.

Research paper thumbnail of Early left ventricular diastolic dysfunction in premature infants born to preeclamptic mothers

Journal of Perinatal Medicine, 2011

Aim: To evaluate the cardiac function in premature infants born to preeclamptic mothers and its c... more Aim: To evaluate the cardiac function in premature infants born to preeclamptic mothers and its clinical consequences. Methods: This was a prospective observational cohort study performed in a tertiary neonatal intensive care unit. Fiftythree premature infants born to preeclamptic mothers comprising the study group were evaluated and compared with 42 premature infants born to normotensive mothers (control group). Relationship between echocardiographic measures and neonatal morbidity were assessed as the main outcome measures. Results: Left ventricle end-diastolic dimension (LVEDD), peak flow velocities during early diastole (peak E wave), peak flow velocities during atrial contraction (peak A wave), and peak E/A ratio were significantly lower in the study group. Within the study group, these parameters were also significantly lower in infants with respiratory problems. LVEDD was significantly smaller in preeclamptic infants with intrauterine growth retardation (IUGR). Conclusion: Left ventricle diastolic dysfunction (LVDD) was detected in premature infants born to preeclamptic mothers in the first week after delivery. LVDD was associated with higher incidence of respiratory problems, transient tachypnea of the newborn, longer duration of oxygen requirement, and IUGR.

Research paper thumbnail of Suspicion, diagnosis, and successful surgical treatment of anomalous origin of the anterior descending coronary artery from the pulmonary trunk

Cardiology in the Young, 2009

Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare... more Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare. We suspected such anomalous origin in a boy aged 2.5 years owing to the echocardiographic demonstration of mitral regurgitation and abnormal diastolic flow in the ventricular septum. The diagnosis was confirmed by angiography. The anomalous artery was successfully reimplanted into the aorta.

Research paper thumbnail of Bilateral multiple pulmonary arteriovenous fistulas and duplicated renal collecting system in a child with Noonan's syndrome

Cardiology in the Young, 2007

Noonan's syndrome involves the association of multiple congenital abnormalities, with a varie... more Noonan's syndrome involves the association of multiple congenital abnormalities, with a variety of cardiac defects. We describe here the association of Noonan's syndrome with multiple pulmonary arteriovenous fistulas and bilateral duplicated renal collecting systems. To the best of our knowledge, this is the first reported case of an association of the Noonan phenotype with pulmonary arteriovenous fistulas.

Research paper thumbnail of The effect of age and gender on the electrocardiogram in children

Cardiology in the Young, 2007

Our aim was to determine, using a computer program for measurement and analysis, the effects, if ... more Our aim was to determine, using a computer program for measurement and analysis, the effects, if any, of age and gender on the electrocardiographic measurements in a large cohort of Turkish children. We analyzed standard simultaneous 12-lead electrocardiograms from 2241 healthy Turkish children, aged from 1 day to 16 years, to calculate limits of normality of a variety of electrocardiographic measurements for 12 age groups. Cinically significant differences were shown to exist, and the results compared with previously established normal limits. Differences were demonstrated for gender in measurements of both amplitude and duration, particularly in the adolescent period. We have provided tables and figures showing age and genderdependent means and upper limits of normal electrocardiographic parameters for the important leads.

Research paper thumbnail of Eisenmenger Sendromlu Çocuk Hastalar› n Telekardiyografi ve Elektrokardiyografi Bulgular›

guncelpediatri.com

... NSR: normal sinüs ritmi; N AKS: normal aks; KB AKS: kuzey bat› aks; KTI: kardiyotorasik indek... more ... NSR: normal sinüs ritmi; N AKS: normal aks; KB AKS: kuzey bat› aks; KTI: kardiyotorasik indeks; N/P: normal/patolojik EKG bulgular› ... kard›n elektriksel aktivitesinde meydana gelen de¤ifliklikler so-nucu, kalbin normal aks›nda ES'li hastalarda sa¤a do¤ru yer de¤ifltirme olur. ...

Research paper thumbnail of Epileptik çocuklarda QT dispersiyonu ve Antiepileptik İlaçların QT dispersiyonu üzerine etkisi

Erciyes Medical Journal - Erciyes Tıp Dergisi.

Research paper thumbnail of The Turkish Journal of Pediatrics 2009; 51: 22-27 Original Carvedilol therapy in pediatric patients with dilated cardiomyopathy

Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited... more Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. The objective of this study was to determine the dosing, efficacy and side effects of carvedilol for the management of dilated cardiomyopathy in children. Sixteen children with idiopathic dilated cardiomyopathy, aged 7 months to 138 months and with an ejection fraction less than 40%, were treated with carvedilol. The average initial dose was 0.1 mg/kg/day and it was uptitrated to 0.4 mg/kg/day. After six months on carvedilol, there were improvements in clinical scoring system from an average of 2.94 to 2.50 (p<0.05), in mean fractional shortening from 17.2±6.1 % to 22.7±5.1 % (p<0.05), and in ejection fraction from 35.2±6.8 % to 43.1±11.2 % (p<0.05). No side effect was observed during the study period. Two patients died due to serious infection. Carvedilol in addition to standard therapy for dilated cardiomyopathy in children ...

Research paper thumbnail of Kronik Böbrek Yetersizliği Olan Hastalarda Kardiyak Tutulum

Güncel Pediatri, 2004

Kardiyak problemler bircok cocuk ve adolesan kronik bobrek yetersizlikli KBY , son donem bobrek y... more Kardiyak problemler bircok cocuk ve adolesan kronik bobrek yetersizlikli KBY , son donem bobrek yetersizlikli SDBY , ve bobrek transplantasyonu yapilmis olan hastada gozlenmekte olup 1 ; 1987-1990 yillari arasinda Avrupa’da renal replasman tedavisi altinda olan cocuk hastalarda, dializde olanlarin %51 ve transplant yapilanlarin %37’sinin mortalitesinde kardiyak nedenlerin sorumlu oldugu gosterilmistir 2 .

Research paper thumbnail of Konjestif Kalp Yetmezliği

Güncel Pediatri, 2006

Konjestif kalp yetmezligi KKY , kalbin dokularin metabolik gereksinimlerini karsilayacak miktarda... more Konjestif kalp yetmezligi KKY , kalbin dokularin metabolik gereksinimlerini karsilayacak miktarda kani perifere pompalayamamasi sonucu olusan sistemik ve pulmoner konjesyon ile karakterize klinik bir durumdur.

Research paper thumbnail of Akut Romatizmal Ateş

Güncel Pediatri, 2005

Akut romatizmal ates ARA , grup A beta hemolitik streptokokal GAS farenjit sonrasi ortaya cikan s... more Akut romatizmal ates ARA , grup A beta hemolitik streptokokal GAS farenjit sonrasi ortaya cikan sistemik bir hastaliktir. ARA, kalp ve kalp kapakciklari uzerinde kronik ilerleyici hasara neden olabilir. Akut romatizmal ates, 1960’lara kadar cocukluk cagi olumlerinin ve yapisal kalp hastaliklarinin baslica nedeni olarak gosterilmekteydi.

Research paper thumbnail of Left Ventricular Aneurysm ina 4-Year-Old Boy

Texas Heart Institute Journal from the Texas Heart Institute of St Luke S Episcopal Hospital Texas Children S Hospital, 2005

Research paper thumbnail of Carvedilol therapy in pediatric patients with dilated cardiomyopathy

The Turkish journal of pediatrics

Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited... more Carvedilol reduces mortality and hospitalization in adults with congestive heart failure. Limited information is available about its use in children. The objective of this study was to determine the dosing, efficacy and side effects of carvedilol for the management of dilated cardiomyopathy in children. Sixteen children with idiopathic dilated cardiomyopathy, aged 7 months to 138 months and with an ejection fraction less than 40%, were treated with carvedilol. The average initial dose was 0.1 mg/kg/day and it was uptitrated to 0.4 mg/kg/day. After six months on carvedilol, there were improvements in clinical scoring system from an average of 2.94 to 2.50 (p<0.05), in mean fractional shortening from 17.2 +/- 6.1% to 22.7 +/- 5.1% (p<0.05), and in ejection fraction from 35.2 +/- 6.8% to 43.1 +/- 11.2% (p<0.05). No side effect was observed during the study period. Two patients died due to serious infection. Carvedilol in addition to standard therapy for dilated cardiomyopathy ...

Research paper thumbnail of Eisenmenger syndrome: identifying the clues for arrhythmia

Anadolu kardiyoloji dergisi : AKD = the Anatolian journal of cardiology, 2008

The aim of this case-controlled, cross-sectional study is to investigate the tendency towards arr... more The aim of this case-controlled, cross-sectional study is to investigate the tendency towards arrhythmia using noninvasive arrhythmia markers (QT dispersion and heart rate variability) in children with Eisenmenger syndrome. We studied 23 patients, whose pulmonary-to-systemic resistance ratio was calculated to be greater than 0.75, and who were diagnosed as Eisenmenger syndrome between 1990 and 2001. Twenty healthy children were studied as the control group. Electrocardiographic recordings with calculation of QT dispersion, Holter monitoring, echocardiographic studies and heart rate variability (HRV) analysis were performed in both groups. Catheterization records were analyzed in all the patients. QT and QTc dispersion were higher (p=0.007 and p=0.006, respectively) and PR interval was longer (p=0.009) in the patients with Eisenmenger syndrome, than those in the control group. In addition, low frequency component, high frequency component, very low frequency component, and total powe...

Research paper thumbnail of Left ventricular aneurysm in a 4-year-old boy

Texas Heart Institute journal / from the Texas Heart Institute of St. Luke's Episcopal Hospital, Texas Children's Hospital, 2005

Left Ventricular Aneurysm in a 4-Year-Old Boy Images in Cardiovascular Medicine Left Ventricular ... more Left Ventricular Aneurysm in a 4-Year-Old Boy Images in Cardiovascular Medicine Left Ventricular Aneurysm in a 4-Year-Old Boy 4-year-old boy with a history of acute lymphoblastic leukemia was referred for evaluation of cardiac function due to progressive decline in exercise tolerance and to cardiomegaly on chest radiography. Six months earlier, there had been no such finding on the chest film. Echocardiography, also performed at the earlier visit, had yielded a normal result. Echocardiography now showed severe left ventricular dysfunction. In addition, a parasternal long-axis view revealed a large aneurysmal sac, estimated to be 8 × 6 cm, in the apical wall of the left ventricle (Fig. 1). The aneurysm's wall was much thinner than a normal left ventricular wall. Because the thin tissue posed a risk of perforation, and because echocardiography was enough for diagnosis, we did not perform angiography. The patient was started on anticoagulation therapy due to the risk of thromboemboli and was given medication for left ventricular systolic dysfunction, in preparation for elective surgery. The operation was performed through a median sternotomy with use of cardiopulmonary bypass and aortic cross-clamping. At operation, the left ventricle was seen to be dilated and the left ventricular wall to have a thin myocardial outpouching (Fig. 2) near the apex. Aneurysmectomy was performed, after which the ventriculotomy was closed with a knitted Dacron patch using running suture (Fig. 3). The morphology of the aneurysm's sac was elastic, its thickness was 2 mm, and its internal surface was covered by thrombus. The patient made an uneventful recovery and was discharged from the hospital on postoperative day 7. Histologic examination of the material obtained during operation showed nonspecific inflammation. Echocardiography 1 month after the operation showed no residual aneurysm and partial improvement in left ventricular function (Fig 4). The patient was observed to be symptom-free during the next 12 months of follow-up.

Research paper thumbnail of Permanent form of junctional reciprocating tachycardia and tachycardia-induced cardiomyopathy treated by catheter ablation: a case report

The Turkish journal of pediatrics

The permanent form of junctional reciprocating tachycardia (PJRT) is usually refractory to drug t... more The permanent form of junctional reciprocating tachycardia (PJRT) is usually refractory to drug therapy, and these patients are at risk of developing tachycardia-induced cardiomyopathy. The electrocardiogram insribes inverted P waves in leads 2, 3, aVF as well as left lateral leads, along with a P-R interval shorter than R-P interval during the tachycardia. This report describes a three-year-old male patient with PJRT who underwent successful radiofrequency catheter ablation (RFA) of accessory pathway. On transthoracic echocardiography of patient, decreased ventricular systolic function was observed. RFA was performed by applying radiofrequency pulses. Echocardiograms of the patient, two months after catheter ablation, demonstrated progressive improvement of ventricular function. Transcatheter radiofrequency ablation of accessory pathways in patients with PJRT is an effective, and possibly preferable, form of treatment, especially in cases of tachycardia refractory to multiple pharm...

Research paper thumbnail of Triosephosphate isomerase deficiency with elevated sweat chloride test: report of a case

The Turkish journal of pediatrics

A 15-month-old girl with severe hemolytic anemia and progressive respiratory failure is presented... more A 15-month-old girl with severe hemolytic anemia and progressive respiratory failure is presented. She was well until the age of six months when she developed a pulmonary infection. During the next six months, she had frequent respiratory infections and her paleness became evident. At the age of 12 months, she was observed to have easy fatigability and muscle weakness, and she received her first blood transfusion. She was referred to our hospital at the age of 15 months. The physical examination revealed a malnourished girl with hypotonia, nystagmus, generalized muscle weakness and severe breathing difficulty requiring ventilatory support The hemoglobin (Hb) was 9.7 g/dl; hematocrit (Hct) 29%, mean corpuscular volume (MCV) 101 fl and reticulocyte count 15%. Peripheral blood smear revealed macrocytosis and stomatocytosis (30% of the red cells) and polychromasia. Sweat chloride test was 90 and 94 mEq/L on two separate occasions. The serum vitamin E level was 0.26 mg/dl (N: 0.44-0.68)....

Research paper thumbnail of Evaluation of electrocardiographic parameters for early diagnosis of autonomic dysfunction in children and adolescents with type-1 diabetes mellitus

Pediatrics International, 2014

Background Cardiovascular complications are continuing to be a major cause of morbidity and morta... more Background Cardiovascular complications are continuing to be a major cause of morbidity and mortality in diabetes mellitus type I (DMTI). The study aimed to evaluate the rate of changes in ECG parameters in children with DMTI compared with healthy children. Materials and Methods This case-control study was performed on 140 participants which consisted of 70 patients with DMTI (case group) matched in sex and age with 70 healthy ones (control group) in two centers in collaboration with Ali Asghar Hospital, Zahedan, Iran, between March 2017 and April 2018. Electrocardiography was performed in all participants (patients and healthy), and heart rate, QT (start of the Q wave and the end of the T wave) QTc interval (QT/ √ RR), QTd (dispersion between maximum and minimum of QT), and QTcd (dispersion between maximum and minimum of QTc) were measured. Duration of diabetic and level of HbA1c were obtained for patients and the effects of these parameters on ECGs were investigated. Results Height, weight and heart rate were higher in the patients (P<0.001) when S in V1 was higher in case (6.16±3.23) compared to the control group (4.33± 2.22) (P<0.001). QT (356.71±27.28 compared to 347.00±23.55), QTd (49.00±14.66 compared to 41.21±8.32), and QTcd (60.47±17.32 compared to 49.93±10.44) were higher in case group (p<0.05). Hemoglobin A1C (p=0.043) was higher in boys and heart rate was higher in girls (p<0.001). Diabetic time duration and HbA1c normality states did not change the length of these parameters in diabetic patients. Conclusion Based on the results, QT, QTd, QTc and QTcd were higher in patients with DMTI. Diabetic time duration and HbA1c states did not change the ECG parameters in diabetic patients.

Research paper thumbnail of Costello Sendromunda Kardiyak Tutulum: Altı Olgunun Değerlendirilmesi

Güncel Pediatri, 2013

Cardiac Involvement in Costello Syndrome: Evaluation of Six Cases ÖZET Gi rifl: Costello sendromu... more Cardiac Involvement in Costello Syndrome: Evaluation of Six Cases ÖZET Gi rifl: Costello sendromu postnatal büyüme ve gelişme geriliği, kaba yüz, gevşek deri, ilerleyici olmayan kardiyomiyopati ve sempatik kişilik ile karakterize otozomal dominant geçiş gösterdiği düşünülen bir sendromdur. Bu çalışmanın amacı Costello sendromu tanısı konulan ve konjenital kalp hastalığı olan altı olgunun klinik bulgularının değerlendirilmesidir. Gereç ve Yöntem: Çalışmada üfürüm nedeni ile çocuk kardiyoloji polikliniğine 2002-2013 yılları arasında başvuran ve Costello sendromunun fenotipik özellikleri olan 6 olgu değerlendirildi. Olguların yakınmaları, klinik bulguları, tedavi ve klinik gidişi incelendi. Bulgular: Olguların 3'ü kız, 3'ü erkekti ve ortalama yaşları 34±12 ay (7 ay-11 yaş) idi. Tüm hastalarda kaba yüz görünümü, el içi ve ayak tabanı çizgilerinde belirginlik, hipertelorizm, epikantus, basık burun kökü ve kardiyak muayenelerinde üfürüm mevcut idi. Hastaların hepsinde konjenital kalp hastalığı saptandı. Hastaların 5'inde değişik derecelerde pulmoner stenoz saptandı ve bu hastaların 3'üne balon pulmoner valvüloplasti yapıldı. 3 hastada atriyal septal defekt tespit edilirken 1 hastada hipertrofik kardiyomiyopati saptandı. 2 hastada mental retardasyon mevcut idi. Sonuç: Psikomotor gelişme geriliği, kaba yüz görünümü, seyrek saçları, derin avuç içi ve ayak tabanı çizgileri olan ve depo hastalığı düşünülen hastalarda özellikle pulmoner stenoz veya hipertrofik kardiyomiyopati varlığında Costello sendromu mutlaka akla gelmelidir.(Gün cel Pe di at ri 2013; 11: 63-7) Anah tar ke li me ler: Costello sendromu, kardiyomiyopati, pulmoner kapak stenozu, kaba yüz, depo hastalığı SUM MARY In tro duc ti on: Costello syndrome is probably an autosomal dominant inherited disorder that is characterized by postnatal growth retardation, developmental delay, coarse facies, loose skin, nonprogressive cardiomyopathy and friendly behavior. The aim of this study was to evaluate a variety of clinical findings and cardiac involvement of six patients diagnosed as Costello syndrome. Materials and Methods: Six cases that had a typical features of Costello syndrome admitted to pediatric cardiology department due to cardiac murmur between 2002-2013 years were evaluated. Their complaints, clinical findings, treatments and clinical courses were examined. Results: Three of the patients were male and the mean age of patients was 34±12 months (7 months to 11 years). All of the cases had coarse faces, deep palmar and plantar creases, hypertelorism, epicanthal folds, depressed nasal bridges and cardiac murmur at auscultation. Congenital heart disease was found in all patients. Five patients had pulmonary stenosis and balloon pulmonary valvuloplasty was performed in 3 of these patients. Atrial septal defect was detected in 3 patients and 1 of the patient had hypertrophic cardiomyopathy. Mental retardation was found in 2 patients. Conclusions: Costello syndrome must be keep in mind in all patients with psychomotor retardation, coarse faces, sparse hairs, deep palmar and plantar creases, phenotypical features resembling a storage disease especially in association with pulmonary stenosis and hypertrophic cardiomyopathy.

Research paper thumbnail of Congenital Anomalies of Coronary Arteries in Children: The Evaluation of 22 Patients

Pediatric Cardiology, 2013

Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing co... more Although congenital coronary artery anomalies are seen in 0.6-1 % of adult patients undergoing coronary angiography, the data for the pediatric population are few. This study of 22 children with coronary artery anomalies evaluated them in terms of demographic and clinical features and analyzed their angiographic findings and surgical results. Databases in the Department of Pediatric Cardiology at the University of Uludag were searched for all the patients with a diagnosis of congenital coronary artery anomaly who underwent coronary angiography between 1993 and 2013. Patients with coexistent congenital heart disease were excluded from the study. The study noted 22 patients (0.9 %; 10 boys and 11 girls) with coronary artery anomalies. The mean age of these patients was 58.77 ± 52.04 months (range, 1 month-16 years). Coronary arteriovenous fistula (50 %) and anomalous left coronary artery from the pulmonary artery (ALCAPA) (36 %) were the most common anomalies. In addition, the study included one patient with diffuse coronary artery hypoplasia, one patient with muscular bridge, and one patient with left main coronary artery originating from the right aortic sinus valsalva. Of the 11 patients who had coronary atrioventricular fistula, 7 were asymptomatic, whereas 75 % of the patients with ALCAPA syndrome were admitted because of heart failure. Although 13 patients had an exact diagnosis by echocardiography, 50 % of the patients with ALCAPA syndrome had their diagnosis determined by catheter angiography performed because of severe mitral regurgitation or dilated cardiomyopathy. The mortality rate for all the patients was found to be 18.1 %. Eight patients with coronary arteriovenous fistula have been followed up without surgery to the present. In contrast, seven patients with ALCAPA syndrome have undergone surgery, and three have died. Two of these patients died during the postoperative period, and the remaining patient died suddenly during the preoperative period at home. Isolated congenital coronary artery anomalies are very rare in the pediatric population. Although most congenital coronary artery anomalies are clinically silent, they may be associated with severe symptoms in children. Recognition of potentially serious anomalies such as ALCAPA syndrome is mandatory so that early surgical treatment can be prescribed.

Research paper thumbnail of Treatment of infective endocarditis with recombinant tissue plasminogen activator

Pediatric Blood & Cancer, 2008

Infective endocarditis (IE) caused by microbial infection is virtually always fatal if untreated.... more Infective endocarditis (IE) caused by microbial infection is virtually always fatal if untreated. High-dose and long-term antibiotic treatment is required to eradicate microorganisms. If increased risk of embolic events, persistent infection, and progressive cardiac failure are present, surgery is indicated. However, surgery can carry an increased risk of mortality and morbidity in critically ill children of whom other treatment options such as administering, a thrombolytic agent; recombinant tissue plasminogen activator (r-tPA) could be an alternative choice. Here, we report a 14-year-old male with Down syndrome and acute myeloblastic leukemia, diagnosed with IE characterized by two large vegetations on aortic and mitral valves, who was successfully treated with r-tPA.

Research paper thumbnail of Early left ventricular diastolic dysfunction in premature infants born to preeclamptic mothers

Journal of Perinatal Medicine, 2011

Aim: To evaluate the cardiac function in premature infants born to preeclamptic mothers and its c... more Aim: To evaluate the cardiac function in premature infants born to preeclamptic mothers and its clinical consequences. Methods: This was a prospective observational cohort study performed in a tertiary neonatal intensive care unit. Fiftythree premature infants born to preeclamptic mothers comprising the study group were evaluated and compared with 42 premature infants born to normotensive mothers (control group). Relationship between echocardiographic measures and neonatal morbidity were assessed as the main outcome measures. Results: Left ventricle end-diastolic dimension (LVEDD), peak flow velocities during early diastole (peak E wave), peak flow velocities during atrial contraction (peak A wave), and peak E/A ratio were significantly lower in the study group. Within the study group, these parameters were also significantly lower in infants with respiratory problems. LVEDD was significantly smaller in preeclamptic infants with intrauterine growth retardation (IUGR). Conclusion: Left ventricle diastolic dysfunction (LVDD) was detected in premature infants born to preeclamptic mothers in the first week after delivery. LVDD was associated with higher incidence of respiratory problems, transient tachypnea of the newborn, longer duration of oxygen requirement, and IUGR.

Research paper thumbnail of Suspicion, diagnosis, and successful surgical treatment of anomalous origin of the anterior descending coronary artery from the pulmonary trunk

Cardiology in the Young, 2009

Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare... more Anomalous origin of the anterior descending coronary artery from the pulmonary trunk is very rare. We suspected such anomalous origin in a boy aged 2.5 years owing to the echocardiographic demonstration of mitral regurgitation and abnormal diastolic flow in the ventricular septum. The diagnosis was confirmed by angiography. The anomalous artery was successfully reimplanted into the aorta.

Research paper thumbnail of Bilateral multiple pulmonary arteriovenous fistulas and duplicated renal collecting system in a child with Noonan's syndrome

Cardiology in the Young, 2007

Noonan's syndrome involves the association of multiple congenital abnormalities, with a varie... more Noonan's syndrome involves the association of multiple congenital abnormalities, with a variety of cardiac defects. We describe here the association of Noonan's syndrome with multiple pulmonary arteriovenous fistulas and bilateral duplicated renal collecting systems. To the best of our knowledge, this is the first reported case of an association of the Noonan phenotype with pulmonary arteriovenous fistulas.

Research paper thumbnail of The effect of age and gender on the electrocardiogram in children

Cardiology in the Young, 2007

Our aim was to determine, using a computer program for measurement and analysis, the effects, if ... more Our aim was to determine, using a computer program for measurement and analysis, the effects, if any, of age and gender on the electrocardiographic measurements in a large cohort of Turkish children. We analyzed standard simultaneous 12-lead electrocardiograms from 2241 healthy Turkish children, aged from 1 day to 16 years, to calculate limits of normality of a variety of electrocardiographic measurements for 12 age groups. Cinically significant differences were shown to exist, and the results compared with previously established normal limits. Differences were demonstrated for gender in measurements of both amplitude and duration, particularly in the adolescent period. We have provided tables and figures showing age and genderdependent means and upper limits of normal electrocardiographic parameters for the important leads.

Research paper thumbnail of Eisenmenger Sendromlu Çocuk Hastalar› n Telekardiyografi ve Elektrokardiyografi Bulgular›

guncelpediatri.com

... NSR: normal sinüs ritmi; N AKS: normal aks; KB AKS: kuzey bat› aks; KTI: kardiyotorasik indek... more ... NSR: normal sinüs ritmi; N AKS: normal aks; KB AKS: kuzey bat› aks; KTI: kardiyotorasik indeks; N/P: normal/patolojik EKG bulgular› ... kard›n elektriksel aktivitesinde meydana gelen de¤ifliklikler so-nucu, kalbin normal aks›nda ES'li hastalarda sa¤a do¤ru yer de¤ifltirme olur. ...