Ercan Tutar - Academia.edu (original) (raw)

Papers by Ercan Tutar

Research paper thumbnail of Severe myocardial injury in an infant with hemorrhagic shock and encephalopathy syndrome

Journal of Pediatric Neurology, 2015

Hemorrhagic shock and encephalopathy syndrome is a severe disease that affects previously healthy... more Hemorrhagic shock and encephalopathy syndrome is a severe disease that affects previously healthy infants and associated with significant mortality and neurologic morbidity. It is characterized by sudden onset of shock, convulsion and coma, bleeding due to severe coagulopathy, fever, diarrhea, metabolic acidosis, and hepatorenal dysfunction. We report a 3-month-old boy with hemorrhagic shock and encephalopathy syndrome, who had clinical and laboratory findings consistent with myocardial injury. Severe myocardial injury was demonstrated by elevation of cardaic troponin I and MB fraction of total kinase in this patient.

Research paper thumbnail of Cardiovascular findings in a boy with arterial tortuosity syndrome: case report and review of the literature

The Turkish journal of pediatrics

Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue d... more Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue disorder. Herein, we describe a five-year-old boy. He had hyperextensible skin, atypical facial features and inguinal hernia. We present his vascular imaging studies of kinking and tortuosity of the aorta and bilateral multiple peripheral pulmonary artery stenoses determined by conventional and magnetic resonance angiographic (MRA) examination. ATS must be considered in patients with connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints and other organs. Vascular imaging studies, especially MRA, are useful in the screening of these vascular abnormalities.

Research paper thumbnail of Percutaneous transcatheter retrieval of intracardiac central venous catheter fragments in two infants using Amplatz Goose Neck snare

The Turkish journal of pediatrics

Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and ... more Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and few cases have been reported in children. Catheter fragments may cause cardiac perforation, arrythmias, pulmonary embolism, and sepsis. We report the successful retrieval of intracardiac CVC fragments by percutaneous transcatheter technique in two infants, aged 8 and 15 months. Double-lumen 7 French Hickman CVCs were accidentally fractured during their removal. Chest radiographs of the two patients revealed migrated intracardiac catheter fragments extending from the superior vena cava (SVC) to the right atrium and from the SVC to the right ventricle, respectively. The procedure was performed under ketamine anesthesia and fluoroscopic guidance using a percutaneous femoral vein approach. Nitinol Amplatz Goose Neck snares (10 mm in diameter) were used to successfully retrieve the catheter fragments without any complication. Percutaneous transcatheter retrieval of catheter fragments using Go...

Research paper thumbnail of Spontaneous closure of small apical muscular ventricular septal defects

The Turkish journal of pediatrics

Between 1993 and 2002, apical muscular ventricular septal defects were identified in 99 cases by ... more Between 1993 and 2002, apical muscular ventricular septal defects were identified in 99 cases by echocardiographic examination. Spontaneous closure time was analyzed in 42 cases followed up at least two years, retrospectively. The ages of 42 cases ranged between 1 day and 13 years old. Initial examination was performed in 22 cases within the newborn period, in 8 cases between 1 and 6 months of age and in 12 cases at more than 6 months of age. Spontaneous closure was seen in 24 of 42 cases (57.1%) between 1 and 36 months of age, and it was most commonly recorded during the first 6 months. It was remarkable that spontaneous closure occurred in 20 of 22 cases (90%) diagnosed in the neonatal period. Closure was seen in 4 of 8 cases (50%) whose initial examination was performed between 1 and 6 months and in none of the patients diagnosed at more than 6 months of age. In conclusion, the frequency of spontaneous closure in cases diagnosed in the neonatal period is higher than previously be...

Research paper thumbnail of Characteristics of hyperthermia and its complications in patients with Prader Willi syndrome

Pediatrics International, 2005

Background : Thermoregulation problems, resulting in hypo-or hyperthermia, have been infrequently... more Background : Thermoregulation problems, resulting in hypo-or hyperthermia, have been infrequently reported in children with Prader Willi syndrome (PWS), yet their clinical details remained unknown. Methods : The clinical characteristics of three infants with PWS are reported. Results : Etiologies of high fever could not be identified in three children with PWS. One of these children was also admitted to the intensive care unit with extremely high body temperature in a life-threatening condition, similar to septic shock, without a plausible explanation. Conclusion : Hyperthermia may be a part of the clinical spectrum in young infants with PWS and should be carefully monitored, since it may cause life-threatening complications.

Research paper thumbnail of Vecuronium induced prolonged paralysis in two pediatric intensive care patients

The Canadian Journal of Neurological Sciences, 2005

Research paper thumbnail of Intrapleural streptokinase treatment for postoperative coagulated hemothorax in a newborn

Pediatrics International, 2009

results in diminished GTP sensitivity is not known. The possibility of racial specificity needs f... more results in diminished GTP sensitivity is not known. The possibility of racial specificity needs further study. The present study makes the following important suggestions for the differential diagnosis of HHS. First, plasma ammonia and sugar levels should be ascertained for patients exhibiting seizures of unknown cause. Second, elevated urinary orotic acid level might be present in HHS. Third, when hyperammonemia is identified, it is important to determine the blood ammonia level in all family members. Finally, variant phenotypes might be present in HHS. In conclusion, although HHS is a rare metabolic disease, accurate diagnosis is achievable. When patients are found to have hypoglycemic seizure accompanied with hyperammonemia, HHS should be considered as a differential diagnosis. Acknowledgment We thank James Steed for his help in professional English editing. References 1 Zammarchi E, Filippi L, Novembre E, Donati MA. Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia. Metabolism 1996; 45: 957-60. 2 Stanley CA, Lieu YK, Hsu BY et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.

Research paper thumbnail of Does Surgically Induced Right Bundle Branch Block Really Effect Ventricular Function in Children After Ventricular Septal Defect Closure?

Pediatric Cardiology, 2014

In this prospective study, we aimed to assess left and right ventricular function in terms of the... more In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p \ 0.05). When compared to controls, operated group had statistically higher tricuspid annular plane systolic excursion (p \ 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values \0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

Research paper thumbnail of Pericardial tamponade in a child with nephrotic syndrome

Pediatric Nephrology, 2011

Sirs, As pericardial effusion and tamponade is an extremely rare but serious complication of neph... more Sirs, As pericardial effusion and tamponade is an extremely rare but serious complication of nephrotic syndrome we would like to report on case that we believe will be informative to the readers of Pediatric Nephrology. A previously healthy 8-year-old girl was admitted to a local hospital with generalized edema with massive proteinuria and diagnosed as having idiopathic nephrotic syndrome (NS). She was treated with prednisolone 1 mg/kg/ day for 4 weeks, but did not have a remission. Oral cyclophosphamide 2 mg/kg/day was added to the treatment, and because of hypertension she was also started on amlodipine and enalapril. She did not show any improvement after 3 months of oral prednisolone and 4 weeks of oral cyclophospamide. A kidney biopsy revealed focal segmental glomerulosclerosis. Immunsupression treatment was switched to cyclosporine 4 mg/kg/day after a 3-day course of methylprednisolone pulse therapy. However, her condition worsened despite these therapies, and she was admitted to our hospital with complaints of vomiting and diarrhea. On admission, she presented with pallor, blurred consciousness, and tachycardia; her blood pressure was 50/20 mmHg, and she weighed 24.5 kg (weight prior to diagnosis of NS: 20.5 kg). She was edematous with extensive periorbital puffiness, bilateral pitting peripheral edema, and ascites. The laboratory findings were as follows: white blood cell count 9.1 × 10/L, platelet count 918 × 10/L, hemoglobin 8.8 g/dL, blood urea nitrogen 30.5 mg/dL, creatinine 1.2 mg/dL, creatinine clearance (CrCl) 44 mL/min/ 1.73 m, total protein 3.3 g/dL, albumin 1.5 g/dL, total cholesterol 560 mg/dL, triglyceride 970 mg/dL. Urinalysis revealed a specific gravity of 1020, pH 5.5, protein 3+(440 mg/m/h). The chest X-ray was normal. She was treated with repeated doses of albumin infusions (1 gm/kg/ day, 3 times/week) and furosemide (1 mg/kg/day). The steroid dose was increased to 2 mg/kg/day, and cyclosporine was stopped. On the 30th day of hospitalization, she developed a cough. The chest X-ray revealed left pleural effusion. The furosemide (2 mg/kg/dose × 2) and albumin (1 gm/kg daily) doses were increased, and aldactazide (2 mg/kg/day) was added to the treatment regimen. This therapy was continued for approximately 10 days. The pleural effusion disappeared within 2 weeks, and the albumin infusions were stopped. However, after 8 days she developed chest pain, respiratory distress, tacyhcardia, and tachypne. The patient presented with pallor and was uncomfortable, tachypneic, and orthopneic. Her pulse rate was 118 beats/min, blood pressure 110/60 mmHg, respiratory rate 48/min, and weight 26.8 kg. Jugular venous distention and large hepatomegaly were detected. Laboratory findings were as follows: blood urea nitrogen 72 mg/dL, creatinine 1.8 mg/dL, CrCl 29 mL/min/1.73 m, total protein 4.2 g/dL, albumin 2.9 g/dL. The chest X-ray revealed enlarged cardiac shadow, a low QRS voltage was noted on electrocardiography, and a massive pericardial effusion and collapse of the right atrium and ventricule was found on echocardiography (Fig. 1). She was diagnosed with pericardial tamponade and an urgent pericardiocenthesis was performed, resulting in the discharge of 450 cc fluid. A. Kavaz : Z. B. Özçakar : F. Yalçınkaya Department of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey

Research paper thumbnail of Predictors of left ventricular hypertrophy in children on chronic peritoneal dialysis

Pediatric Nephrology, 2010

Conflicting results have been reported in small non-homogenous groups of children with chronic re... more Conflicting results have been reported in small non-homogenous groups of children with chronic renal failure in terms of casual blood pressure and ambulatory blood pressure monitoring (ABPM) parameters and left ventricular hypertrophy (LVH). The aim of our study was to assess the value of ABPM and hematological and biochemical parameters in predicting LVH in children on chronic peritoneal dialysis (CPD). Echocardiography and 24-h ABPM were performed in addition to routine biochem-p<0.01) to be independent predictors of LVH. The sensitivity, specificity, positive predictive value, and negative predictive values for the combination of the SBP load >15% and hematocrit value <31% for predicting LVH were 95 [95% confidence interval (CI) 76-99], 78 (95%CI 45-94), 91 (95%CI 73-98), and 88% (95%CI 69-96%), respectively. We conclude that: (1) LVH is prevalent in children on CPD, and (2) a target hematocrit level >31% and daytime SBP load <15% may be preventive for the progression of LVH in the follow-up of children on CPD.

Research paper thumbnail of Unusually Redundant Chiari Network

Pediatric Cardiology, 2006

Research paper thumbnail of Acremonium spp. peritonitis in an infant

Mycoses, 2008

Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis. Clinica... more Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis. Clinical presentation of fungal peritonitis is similar to bacterial peritonitis and Candida spp. are the most common agent. Fungal peritonitis has been usually associated with high morbidity, mortality and its treatment is difficult. In this report, we present an infant with Acremonium spp. peritonitis. A 7-month-old boy with Down syndrome, congenital heart disease, pulmonary hypertension and congestive heart failure required peritoneal dialysis for his persistent pulmonary oedema and symptomatic hyponatremia. Acremonium spp. peritonitis developed while he was on extended spectrum antibiotics and fluconazole. The patient was successfully treated with peritoneal dialysis catheter removal and liposomal amphotericin B. The case was presented to draw attention to a rare cause of peritonitis -Acremonium spp. - in a paediatric patient.

Research paper thumbnail of Early detection of myocardial dysfunction in children with beta-thalassaemia major

The International Journal of Cardiovascular Imaging, 2008

To evaluate left and right ventricular myocardial performance using pulsed-tissue Doppler imaging... more To evaluate left and right ventricular myocardial performance using pulsed-tissue Doppler imaging (TDI) and its relation to BNP levels in patients with beta-thalassaemia major (beta-TM). We enrolled 36 thalassaemic patients (21 male, 15 female; mean age: 14.2 +/- 4.1 years) with normal left ventricular systolic and diastolic functions with conventional echocardiography and 30 healthy control subjects (18 male, 12 female, and 12.5 +/- 4.2 years). Myocardial performance indexes (MPI) of left ventricular (LV) lateral wall, interventricular septum (IVS) and right ventricular (RV) lateral wall were calculated with TDI. Plasma BNP levels were measured in all patients. MPIs and other echocardiographic parameters of patients with beta-TM were compared with control group. All the patients&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; plasma BNP levels were within normal limits. There were no differences between conventional echocardiographic parameters of patients and control group. MPI of LV, IVS, and RV of patients were significantly higher than control group (P = 0.01, and P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01, and P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001, respectively). Our study confirms that MPI obtained by TDI seems to be an early sensitive parameter of cardiac dysfunction in beta-TM. We concluded that MPI obtained by TDI may be an adjunctive parameter to conventional echocardiography for detecting early myocardial damage.

Research paper thumbnail of Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections

The International Journal of Cardiovascular Imaging, 2007

Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by... more Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by cardiac catheterization. Although angiography has been the gold standard for evaluation it carries certain inherent risks, especially in small and sick infants. In this study we retrospectively assessed the utility of magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the evaluation of pulmonary venous abnormalities in pediatric patients. The results were compared with operative findings. We reviewed nine patients with total APVC and four with partial APVC. Twelve patients were infant (age range: 1 day to 8 months) and the other was 16 years old. MRA and/or CTA clearly visualized the anomalous drainage of pulmonary veins in all patients. Cardiac catheterization was performed five patients with one total anomalous pulmonary venous connection and four with partial anomalous pulmonary venous connection. Ten patients were operated on. Diagnoses of patients were confirmed by operative findings. In Conclusions, MR and CT angiography allow detailed and comprehensive evaluation of the APVC and are good diagnostic modalities for use in the preoperative assessment of the anomaly in pediatric patients.

Research paper thumbnail of PP-003 Fatal Fungal Endocarditis Due to Trichosporon Asahii in an Adolescent Boy

International Journal of Cardiology, 2010

Objective: Transcatheter aortic valve implantation (TAVI) is a rapidly evolving technique with en... more Objective: Transcatheter aortic valve implantation (TAVI) is a rapidly evolving technique with encouraging early results. We report two patients with critical aortic stenosis and severe comorbidities treated with TAVI. Methods: Both patients had severe calcified aortic stenosis with clinical symptoms (NYHA Class 4) and comorbities including diabetes mellitus, hyperlipidemia and hypertension. First patient was 80 years old, who had undergone coronary surgery previously and had myelodysplastic syndrome and orthopedic problems leading to mobility defects. The second patient had cirrhosis and hematologic problems with platelet count of 25.000. Surgical risk was deemed excessive in both patients due to these risk factors. Transcatheter valve implantation was planned. Results: Transcatheter Edwards-SAPIEN valve implantation with a transfemoral approach was peformed in two patients. No perioperative complications was observed. Successful valve implantation was associated with an increase in echocardiographic valve area and significant early improvement in clinical condition. The patients were discharged in postoperative 3rd and 10 th day. Conclusions: Transcatheter aortic valve implantation in patients with severe comorbidities is related with early hemodynamic and clinical improvement.

Research paper thumbnail of Quantitation of Doppler color flow jet areas for mitral regurgitation in children: angiographic correlation

International Journal of Cardiology, 1999

Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 year... more Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 years (mean age +/-SD, 12.69+/-3.47 years) were analyzed with color Doppler imaging. Sixteen patients were performed cardiac catheterization within 24 h. Jets were classified as eccentric and central. Regurgitant jet area and its ratio to left atrial area and body surface area were measured by Doppler color flow imaging. Regurgitant volume and regurgitant fractions were calculated with angiography. There was a good correlation between regurgitant jet area and angiographic grade of mitral regurgitation (P&lt;0.01). The correlation between regurgitant jet area/left atrial area ratios and angiographic grade of mitral regurgitation was limited (P&lt;0.01). There was excellent correlation between regurgitant jet area/body surface area and angiographic regurgitant fraction (r = 0.85; P&lt;0.001). There was also a good correlation between regurgitant jet area and regurgitant fraction (r = 0.82; P&lt;0.001). However, the relation of regurgitant jet area/left atrial area to regurgitant fraction was weak (r = 0.72; P&lt;0.01). In conclusion, the measurement of regurgitant fraction and its ratios to left atrial area and body surface area by color Doppler flow imaging can predict the angiographic severity in children who have even eccentric regurgitant jets.

Research paper thumbnail of Plasma endothelin-1 levels in patients with left-to-right shunt with or without pulmonary hypertension

International Journal of Cardiology, 1999

The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmo... more The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmonary hypertension (PH) secondary to congenital heart disease with left-to-right shunt. Twenty-three children (12 male, 11 female) aged 0.58-13 years were enrolled the study. Blood samples were drawn from superior vena cava, right atrium, right ventricle, pulmonary artery and pulmonary wedge or pulmonary vein during cardiac catheterization. Plasma ET-1 levels were assayed by ELISA. Patients were divided into two groups according to the presence or absence of PH. Plasma ET-1 levels of the study group were compared to the peripheral venous and arterial ET-1 levels of 11 healthy infants and children (aged 0.75-13 years). Plasma ET-1 levels in patients with left-to-right shunt were found significantly higher than those of controls. However, plasma ET-1 levels were similar between the two groups of the patients. Pulmonary venous ET-1 levels were higher than the levels of superior vena cava, this suggested an increased production of ET-1 in pulmonary vascular bed in patients with PH. No correlations were found between plasma ET-1 levels and pulmonary arterial pressure, pulmonary vascular resistance and pulmonary blood flow in the patients. Plasma ET-1 levels of the patients with left-to-right shunt were increased independently from pulmonary arterial pressure and pulmonary vascular resistance. This increase was related to the production of ET-1 in pulmonary vascular bed in patients with PH. ET-1 could not be found to be directly related to the development of PH in the patients with left-to-right shunt.

Research paper thumbnail of Unusual Cardiac Phenotype in a Newborn with Noonan Syndrome

Congenital Heart Disease, 2010

Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hyperteloris... more Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hypertelorism, downward slanting of the palpebral fissures, ptosis, low-set posteriorly angulated ears, short stature, and congenital heart disease, most commonly pulmonary valve stenosis, hypertrophic cardiomyopathy (HCM), and atrial septal defects (ASDs). We report a 6-day-old girl who had an unusual combination of supravalvular pulmonary stenosis (PS), obstructive HCM, secundum ASD, and abnormal mitral chordae. Diagnosis was based on characteristic dysmorphic features and presence of HCM. Subsequent cardiac catheterization confirmed the presence of hemodynamically significant PS, and surgical correction was planned. Supravalvular PS is an unusual cardiac phenotype in NS and its association with HCM and ASD has not been reported previously. A detailed echocardiographic examination is mandatory in NS patients for accurate diagnosis of cardiac phenotype and for further treatment plans.c hd_343 178..181

Research paper thumbnail of Absent ductus venosus associated with persistent truncus arteriosus: prenatal diagnosis

Cardiology in the Young, 2010

An absent ductus venosus is a rare anomaly which results aberrant umbilical venous return. The fe... more An absent ductus venosus is a rare anomaly which results aberrant umbilical venous return. The fetus which is defined here referred to our clinic at 30th gestational week because of cardiomegaly. The diagnosis of ductus venosus agenesis and anomalous umbilical venous return was done by fetal echocardiography. The fetus has two unique features. The drainage of anomalous umbilical vein into the superior vena cava and associated persistent truncus arteriosus have not been reported yet in fetuses with absent ductus venosus.

Research paper thumbnail of A germlinePTEN mutation with manifestations of prenatal onset and verrucous epidermal nevus

American Journal of Medical Genetics Part A, 2006

Research paper thumbnail of Severe myocardial injury in an infant with hemorrhagic shock and encephalopathy syndrome

Journal of Pediatric Neurology, 2015

Hemorrhagic shock and encephalopathy syndrome is a severe disease that affects previously healthy... more Hemorrhagic shock and encephalopathy syndrome is a severe disease that affects previously healthy infants and associated with significant mortality and neurologic morbidity. It is characterized by sudden onset of shock, convulsion and coma, bleeding due to severe coagulopathy, fever, diarrhea, metabolic acidosis, and hepatorenal dysfunction. We report a 3-month-old boy with hemorrhagic shock and encephalopathy syndrome, who had clinical and laboratory findings consistent with myocardial injury. Severe myocardial injury was demonstrated by elevation of cardaic troponin I and MB fraction of total kinase in this patient.

Research paper thumbnail of Cardiovascular findings in a boy with arterial tortuosity syndrome: case report and review of the literature

The Turkish journal of pediatrics

Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue d... more Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue disorder. Herein, we describe a five-year-old boy. He had hyperextensible skin, atypical facial features and inguinal hernia. We present his vascular imaging studies of kinking and tortuosity of the aorta and bilateral multiple peripheral pulmonary artery stenoses determined by conventional and magnetic resonance angiographic (MRA) examination. ATS must be considered in patients with connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints and other organs. Vascular imaging studies, especially MRA, are useful in the screening of these vascular abnormalities.

Research paper thumbnail of Percutaneous transcatheter retrieval of intracardiac central venous catheter fragments in two infants using Amplatz Goose Neck snare

The Turkish journal of pediatrics

Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and ... more Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and few cases have been reported in children. Catheter fragments may cause cardiac perforation, arrythmias, pulmonary embolism, and sepsis. We report the successful retrieval of intracardiac CVC fragments by percutaneous transcatheter technique in two infants, aged 8 and 15 months. Double-lumen 7 French Hickman CVCs were accidentally fractured during their removal. Chest radiographs of the two patients revealed migrated intracardiac catheter fragments extending from the superior vena cava (SVC) to the right atrium and from the SVC to the right ventricle, respectively. The procedure was performed under ketamine anesthesia and fluoroscopic guidance using a percutaneous femoral vein approach. Nitinol Amplatz Goose Neck snares (10 mm in diameter) were used to successfully retrieve the catheter fragments without any complication. Percutaneous transcatheter retrieval of catheter fragments using Go...

Research paper thumbnail of Spontaneous closure of small apical muscular ventricular septal defects

The Turkish journal of pediatrics

Between 1993 and 2002, apical muscular ventricular septal defects were identified in 99 cases by ... more Between 1993 and 2002, apical muscular ventricular septal defects were identified in 99 cases by echocardiographic examination. Spontaneous closure time was analyzed in 42 cases followed up at least two years, retrospectively. The ages of 42 cases ranged between 1 day and 13 years old. Initial examination was performed in 22 cases within the newborn period, in 8 cases between 1 and 6 months of age and in 12 cases at more than 6 months of age. Spontaneous closure was seen in 24 of 42 cases (57.1%) between 1 and 36 months of age, and it was most commonly recorded during the first 6 months. It was remarkable that spontaneous closure occurred in 20 of 22 cases (90%) diagnosed in the neonatal period. Closure was seen in 4 of 8 cases (50%) whose initial examination was performed between 1 and 6 months and in none of the patients diagnosed at more than 6 months of age. In conclusion, the frequency of spontaneous closure in cases diagnosed in the neonatal period is higher than previously be...

Research paper thumbnail of Characteristics of hyperthermia and its complications in patients with Prader Willi syndrome

Pediatrics International, 2005

Background : Thermoregulation problems, resulting in hypo-or hyperthermia, have been infrequently... more Background : Thermoregulation problems, resulting in hypo-or hyperthermia, have been infrequently reported in children with Prader Willi syndrome (PWS), yet their clinical details remained unknown. Methods : The clinical characteristics of three infants with PWS are reported. Results : Etiologies of high fever could not be identified in three children with PWS. One of these children was also admitted to the intensive care unit with extremely high body temperature in a life-threatening condition, similar to septic shock, without a plausible explanation. Conclusion : Hyperthermia may be a part of the clinical spectrum in young infants with PWS and should be carefully monitored, since it may cause life-threatening complications.

Research paper thumbnail of Vecuronium induced prolonged paralysis in two pediatric intensive care patients

The Canadian Journal of Neurological Sciences, 2005

Research paper thumbnail of Intrapleural streptokinase treatment for postoperative coagulated hemothorax in a newborn

Pediatrics International, 2009

results in diminished GTP sensitivity is not known. The possibility of racial specificity needs f... more results in diminished GTP sensitivity is not known. The possibility of racial specificity needs further study. The present study makes the following important suggestions for the differential diagnosis of HHS. First, plasma ammonia and sugar levels should be ascertained for patients exhibiting seizures of unknown cause. Second, elevated urinary orotic acid level might be present in HHS. Third, when hyperammonemia is identified, it is important to determine the blood ammonia level in all family members. Finally, variant phenotypes might be present in HHS. In conclusion, although HHS is a rare metabolic disease, accurate diagnosis is achievable. When patients are found to have hypoglycemic seizure accompanied with hyperammonemia, HHS should be considered as a differential diagnosis. Acknowledgment We thank James Steed for his help in professional English editing. References 1 Zammarchi E, Filippi L, Novembre E, Donati MA. Biochemical evaluation of a patient with a familial form of leucine-sensitive hypoglycemia and concomitant hyperammonemia. Metabolism 1996; 45: 957-60. 2 Stanley CA, Lieu YK, Hsu BY et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene.

Research paper thumbnail of Does Surgically Induced Right Bundle Branch Block Really Effect Ventricular Function in Children After Ventricular Septal Defect Closure?

Pediatric Cardiology, 2014

In this prospective study, we aimed to assess left and right ventricular function in terms of the... more In this prospective study, we aimed to assess left and right ventricular function in terms of the presence of right bundle branch block (RBBB) in the cases with repaired ventricular septal defect (VSD). Fifty-three patients who had VSD surgery at least 1-year preceding admission and 52 healthy controls were enrolled into the study. All the participants underwent electrocardiographic and echocardiographic examination. The cases with RBBB were determined. The conventional and tissue Doppler echocardiographic measurements of the patients with and without RBBB were compared with each other and healthy controls. Twenty-eight of VSD repair groups were male and 25 were female. Control group consisted of 30 males and 22 females. The mean age of the study and control groups was 7.5 ± 5.0 and 6.9 ± 4.3 years, respectively. RBBB was detected in 20 of 53 (37.7 %) operated patients. The only significant difference between the cases with and without RBBB was decreased right ventricular fractional area change (%) in the former group (33 ± 7 vs. 39 ± 5 p \ 0.05). When compared to controls, operated group had statistically higher tricuspid annular plane systolic excursion (p \ 0.05), lower systolic, early diastolic, and late diastolic myocardial velocities, higher left and right ventricular myocardial performance indices, irrespective of the presence of RBBB. The ratios of mitral or tricuspid inflow to left or right ventricular myocardial in early diastolic velocities measured from lateral annular levels were increased in operated group (all p values \0.05). In conclusion, RBBB in the cases with surgical VSD repair might be associated with right ventricular dysfunction. Biventricular systolic and diastolic dysfunction may develop following VSD repair irrespective of the presence of RBBB. Tissue Doppler-derived myocardial performance indices are useful in detection of those subclinical dysfunctions.

Research paper thumbnail of Pericardial tamponade in a child with nephrotic syndrome

Pediatric Nephrology, 2011

Sirs, As pericardial effusion and tamponade is an extremely rare but serious complication of neph... more Sirs, As pericardial effusion and tamponade is an extremely rare but serious complication of nephrotic syndrome we would like to report on case that we believe will be informative to the readers of Pediatric Nephrology. A previously healthy 8-year-old girl was admitted to a local hospital with generalized edema with massive proteinuria and diagnosed as having idiopathic nephrotic syndrome (NS). She was treated with prednisolone 1 mg/kg/ day for 4 weeks, but did not have a remission. Oral cyclophosphamide 2 mg/kg/day was added to the treatment, and because of hypertension she was also started on amlodipine and enalapril. She did not show any improvement after 3 months of oral prednisolone and 4 weeks of oral cyclophospamide. A kidney biopsy revealed focal segmental glomerulosclerosis. Immunsupression treatment was switched to cyclosporine 4 mg/kg/day after a 3-day course of methylprednisolone pulse therapy. However, her condition worsened despite these therapies, and she was admitted to our hospital with complaints of vomiting and diarrhea. On admission, she presented with pallor, blurred consciousness, and tachycardia; her blood pressure was 50/20 mmHg, and she weighed 24.5 kg (weight prior to diagnosis of NS: 20.5 kg). She was edematous with extensive periorbital puffiness, bilateral pitting peripheral edema, and ascites. The laboratory findings were as follows: white blood cell count 9.1 × 10/L, platelet count 918 × 10/L, hemoglobin 8.8 g/dL, blood urea nitrogen 30.5 mg/dL, creatinine 1.2 mg/dL, creatinine clearance (CrCl) 44 mL/min/ 1.73 m, total protein 3.3 g/dL, albumin 1.5 g/dL, total cholesterol 560 mg/dL, triglyceride 970 mg/dL. Urinalysis revealed a specific gravity of 1020, pH 5.5, protein 3+(440 mg/m/h). The chest X-ray was normal. She was treated with repeated doses of albumin infusions (1 gm/kg/ day, 3 times/week) and furosemide (1 mg/kg/day). The steroid dose was increased to 2 mg/kg/day, and cyclosporine was stopped. On the 30th day of hospitalization, she developed a cough. The chest X-ray revealed left pleural effusion. The furosemide (2 mg/kg/dose × 2) and albumin (1 gm/kg daily) doses were increased, and aldactazide (2 mg/kg/day) was added to the treatment regimen. This therapy was continued for approximately 10 days. The pleural effusion disappeared within 2 weeks, and the albumin infusions were stopped. However, after 8 days she developed chest pain, respiratory distress, tacyhcardia, and tachypne. The patient presented with pallor and was uncomfortable, tachypneic, and orthopneic. Her pulse rate was 118 beats/min, blood pressure 110/60 mmHg, respiratory rate 48/min, and weight 26.8 kg. Jugular venous distention and large hepatomegaly were detected. Laboratory findings were as follows: blood urea nitrogen 72 mg/dL, creatinine 1.8 mg/dL, CrCl 29 mL/min/1.73 m, total protein 4.2 g/dL, albumin 2.9 g/dL. The chest X-ray revealed enlarged cardiac shadow, a low QRS voltage was noted on electrocardiography, and a massive pericardial effusion and collapse of the right atrium and ventricule was found on echocardiography (Fig. 1). She was diagnosed with pericardial tamponade and an urgent pericardiocenthesis was performed, resulting in the discharge of 450 cc fluid. A. Kavaz : Z. B. Özçakar : F. Yalçınkaya Department of Pediatric Nephrology, Ankara University School of Medicine, Ankara, Turkey

Research paper thumbnail of Predictors of left ventricular hypertrophy in children on chronic peritoneal dialysis

Pediatric Nephrology, 2010

Conflicting results have been reported in small non-homogenous groups of children with chronic re... more Conflicting results have been reported in small non-homogenous groups of children with chronic renal failure in terms of casual blood pressure and ambulatory blood pressure monitoring (ABPM) parameters and left ventricular hypertrophy (LVH). The aim of our study was to assess the value of ABPM and hematological and biochemical parameters in predicting LVH in children on chronic peritoneal dialysis (CPD). Echocardiography and 24-h ABPM were performed in addition to routine biochem-p<0.01) to be independent predictors of LVH. The sensitivity, specificity, positive predictive value, and negative predictive values for the combination of the SBP load >15% and hematocrit value <31% for predicting LVH were 95 [95% confidence interval (CI) 76-99], 78 (95%CI 45-94), 91 (95%CI 73-98), and 88% (95%CI 69-96%), respectively. We conclude that: (1) LVH is prevalent in children on CPD, and (2) a target hematocrit level >31% and daytime SBP load <15% may be preventive for the progression of LVH in the follow-up of children on CPD.

Research paper thumbnail of Unusually Redundant Chiari Network

Pediatric Cardiology, 2006

Research paper thumbnail of Acremonium spp. peritonitis in an infant

Mycoses, 2008

Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis. Clinica... more Fungal peritonitis is a rare but serious complication in children on peritoneal dialysis. Clinical presentation of fungal peritonitis is similar to bacterial peritonitis and Candida spp. are the most common agent. Fungal peritonitis has been usually associated with high morbidity, mortality and its treatment is difficult. In this report, we present an infant with Acremonium spp. peritonitis. A 7-month-old boy with Down syndrome, congenital heart disease, pulmonary hypertension and congestive heart failure required peritoneal dialysis for his persistent pulmonary oedema and symptomatic hyponatremia. Acremonium spp. peritonitis developed while he was on extended spectrum antibiotics and fluconazole. The patient was successfully treated with peritoneal dialysis catheter removal and liposomal amphotericin B. The case was presented to draw attention to a rare cause of peritonitis -Acremonium spp. - in a paediatric patient.

Research paper thumbnail of Early detection of myocardial dysfunction in children with beta-thalassaemia major

The International Journal of Cardiovascular Imaging, 2008

To evaluate left and right ventricular myocardial performance using pulsed-tissue Doppler imaging... more To evaluate left and right ventricular myocardial performance using pulsed-tissue Doppler imaging (TDI) and its relation to BNP levels in patients with beta-thalassaemia major (beta-TM). We enrolled 36 thalassaemic patients (21 male, 15 female; mean age: 14.2 +/- 4.1 years) with normal left ventricular systolic and diastolic functions with conventional echocardiography and 30 healthy control subjects (18 male, 12 female, and 12.5 +/- 4.2 years). Myocardial performance indexes (MPI) of left ventricular (LV) lateral wall, interventricular septum (IVS) and right ventricular (RV) lateral wall were calculated with TDI. Plasma BNP levels were measured in all patients. MPIs and other echocardiographic parameters of patients with beta-TM were compared with control group. All the patients&amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;#39; plasma BNP levels were within normal limits. There were no differences between conventional echocardiographic parameters of patients and control group. MPI of LV, IVS, and RV of patients were significantly higher than control group (P = 0.01, and P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.01, and P &amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;amp;lt; 0.001, respectively). Our study confirms that MPI obtained by TDI seems to be an early sensitive parameter of cardiac dysfunction in beta-TM. We concluded that MPI obtained by TDI may be an adjunctive parameter to conventional echocardiography for detecting early myocardial damage.

Research paper thumbnail of Diagnostic tools in the preoperative evaluation of children with anomalous pulmonary venous connections

The International Journal of Cardiovascular Imaging, 2007

Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by... more Pulmonary venous abnormalities are generally diagnosed by echocardiography and often confirmed by cardiac catheterization. Although angiography has been the gold standard for evaluation it carries certain inherent risks, especially in small and sick infants. In this study we retrospectively assessed the utility of magnetic resonance angiography (MRA) and computed tomography angiography (CTA) in the evaluation of pulmonary venous abnormalities in pediatric patients. The results were compared with operative findings. We reviewed nine patients with total APVC and four with partial APVC. Twelve patients were infant (age range: 1 day to 8 months) and the other was 16 years old. MRA and/or CTA clearly visualized the anomalous drainage of pulmonary veins in all patients. Cardiac catheterization was performed five patients with one total anomalous pulmonary venous connection and four with partial anomalous pulmonary venous connection. Ten patients were operated on. Diagnoses of patients were confirmed by operative findings. In Conclusions, MR and CT angiography allow detailed and comprehensive evaluation of the APVC and are good diagnostic modalities for use in the preoperative assessment of the anomaly in pediatric patients.

Research paper thumbnail of PP-003 Fatal Fungal Endocarditis Due to Trichosporon Asahii in an Adolescent Boy

International Journal of Cardiology, 2010

Objective: Transcatheter aortic valve implantation (TAVI) is a rapidly evolving technique with en... more Objective: Transcatheter aortic valve implantation (TAVI) is a rapidly evolving technique with encouraging early results. We report two patients with critical aortic stenosis and severe comorbidities treated with TAVI. Methods: Both patients had severe calcified aortic stenosis with clinical symptoms (NYHA Class 4) and comorbities including diabetes mellitus, hyperlipidemia and hypertension. First patient was 80 years old, who had undergone coronary surgery previously and had myelodysplastic syndrome and orthopedic problems leading to mobility defects. The second patient had cirrhosis and hematologic problems with platelet count of 25.000. Surgical risk was deemed excessive in both patients due to these risk factors. Transcatheter valve implantation was planned. Results: Transcatheter Edwards-SAPIEN valve implantation with a transfemoral approach was peformed in two patients. No perioperative complications was observed. Successful valve implantation was associated with an increase in echocardiographic valve area and significant early improvement in clinical condition. The patients were discharged in postoperative 3rd and 10 th day. Conclusions: Transcatheter aortic valve implantation in patients with severe comorbidities is related with early hemodynamic and clinical improvement.

Research paper thumbnail of Quantitation of Doppler color flow jet areas for mitral regurgitation in children: angiographic correlation

International Journal of Cardiology, 1999

Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 year... more Eighteen patients with chronic isolated rheumatic mitral regurgitation aged between 7 and 19 years (mean age +/-SD, 12.69+/-3.47 years) were analyzed with color Doppler imaging. Sixteen patients were performed cardiac catheterization within 24 h. Jets were classified as eccentric and central. Regurgitant jet area and its ratio to left atrial area and body surface area were measured by Doppler color flow imaging. Regurgitant volume and regurgitant fractions were calculated with angiography. There was a good correlation between regurgitant jet area and angiographic grade of mitral regurgitation (P&lt;0.01). The correlation between regurgitant jet area/left atrial area ratios and angiographic grade of mitral regurgitation was limited (P&lt;0.01). There was excellent correlation between regurgitant jet area/body surface area and angiographic regurgitant fraction (r = 0.85; P&lt;0.001). There was also a good correlation between regurgitant jet area and regurgitant fraction (r = 0.82; P&lt;0.001). However, the relation of regurgitant jet area/left atrial area to regurgitant fraction was weak (r = 0.72; P&lt;0.01). In conclusion, the measurement of regurgitant fraction and its ratios to left atrial area and body surface area by color Doppler flow imaging can predict the angiographic severity in children who have even eccentric regurgitant jets.

Research paper thumbnail of Plasma endothelin-1 levels in patients with left-to-right shunt with or without pulmonary hypertension

International Journal of Cardiology, 1999

The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmo... more The aim of this study was to evaluate the role of endothelin-1 (ET-1) in pathophysiology of pulmonary hypertension (PH) secondary to congenital heart disease with left-to-right shunt. Twenty-three children (12 male, 11 female) aged 0.58-13 years were enrolled the study. Blood samples were drawn from superior vena cava, right atrium, right ventricle, pulmonary artery and pulmonary wedge or pulmonary vein during cardiac catheterization. Plasma ET-1 levels were assayed by ELISA. Patients were divided into two groups according to the presence or absence of PH. Plasma ET-1 levels of the study group were compared to the peripheral venous and arterial ET-1 levels of 11 healthy infants and children (aged 0.75-13 years). Plasma ET-1 levels in patients with left-to-right shunt were found significantly higher than those of controls. However, plasma ET-1 levels were similar between the two groups of the patients. Pulmonary venous ET-1 levels were higher than the levels of superior vena cava, this suggested an increased production of ET-1 in pulmonary vascular bed in patients with PH. No correlations were found between plasma ET-1 levels and pulmonary arterial pressure, pulmonary vascular resistance and pulmonary blood flow in the patients. Plasma ET-1 levels of the patients with left-to-right shunt were increased independently from pulmonary arterial pressure and pulmonary vascular resistance. This increase was related to the production of ET-1 in pulmonary vascular bed in patients with PH. ET-1 could not be found to be directly related to the development of PH in the patients with left-to-right shunt.

Research paper thumbnail of Unusual Cardiac Phenotype in a Newborn with Noonan Syndrome

Congenital Heart Disease, 2010

Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hyperteloris... more Noonan syndrome (NS; MIM #163950) is an autosomal dominant syndrome characterized by hypertelorism, downward slanting of the palpebral fissures, ptosis, low-set posteriorly angulated ears, short stature, and congenital heart disease, most commonly pulmonary valve stenosis, hypertrophic cardiomyopathy (HCM), and atrial septal defects (ASDs). We report a 6-day-old girl who had an unusual combination of supravalvular pulmonary stenosis (PS), obstructive HCM, secundum ASD, and abnormal mitral chordae. Diagnosis was based on characteristic dysmorphic features and presence of HCM. Subsequent cardiac catheterization confirmed the presence of hemodynamically significant PS, and surgical correction was planned. Supravalvular PS is an unusual cardiac phenotype in NS and its association with HCM and ASD has not been reported previously. A detailed echocardiographic examination is mandatory in NS patients for accurate diagnosis of cardiac phenotype and for further treatment plans.c hd_343 178..181

Research paper thumbnail of Absent ductus venosus associated with persistent truncus arteriosus: prenatal diagnosis

Cardiology in the Young, 2010

An absent ductus venosus is a rare anomaly which results aberrant umbilical venous return. The fe... more An absent ductus venosus is a rare anomaly which results aberrant umbilical venous return. The fetus which is defined here referred to our clinic at 30th gestational week because of cardiomegaly. The diagnosis of ductus venosus agenesis and anomalous umbilical venous return was done by fetal echocardiography. The fetus has two unique features. The drainage of anomalous umbilical vein into the superior vena cava and associated persistent truncus arteriosus have not been reported yet in fetuses with absent ductus venosus.

Research paper thumbnail of A germlinePTEN mutation with manifestations of prenatal onset and verrucous epidermal nevus

American Journal of Medical Genetics Part A, 2006