[Co-occurrence of Carpenter syndrome and double outlet right ventricle]. / Carpenter sendromu ve çift çikisli sag ventrikül birlikteligi.

Carpenter syndrome (Acrocephalopolysyndactyly type 2, OMIM 201000) is a rarely seen autosomal recessive disorder. In addition to abnormalities such as acrocephaly, craniosynostosis, facial asymmetry, polydactyly and syndactyly, obesity, hypogonadism, mental retardation, and corneal opacity, it may frequently be accompanied by congenital heart diseases such as ventricular septal defect, patent ductus arteriosus and pulmonary stenosis. Double outlet right ventricle is a defect in which both major arteries originate in the morphological right ventricle. To the best of our knowledge, this is the first report in the literature of double outlet right ventricle disease in combination with Carpenter syndrome.

Heart-Type Fatty Acid Binding Protein Level as a Tool in Identification of Early Cardiac Effects of Diabetic Ketoacidosis.

OBJECTIVE: This study aimed to measure the serum levels of heart-type fatty acid binding protein (H-FABP) in patients presenting with diabetic ketoacidosis (DKA) and diabetic ketosis (DK) and to determine its role in identifying early-period cardiac ischemia. METHODS: This prospective study included 35 patients diagnosed with DKA, 20 patients diagnosed with DK, and 20 control subjects. H-FABP, creatine kinase-MB (CK-MB), and troponin I levels were investigated at presentation in patients with DKA and DK and in the control group. H-FABP values were measured again after acidosis correction in the DKA patients. RESULTS: No statistically significant differences were found with respect to troponin I and CK-MB within the groups. The H-FABP values of DKA patients at presentation were found to be significantly higher than those of DK patients and the control group (p=0.015). The H-FABP value of the DKA group was also found to be significantly higher than the value at hour 36 after acidosis correction (p=0.0001). CONCLUSION: We would like to propose H-FABP as a potential marker for indicating myocardial ischemia.

A rare situation in acute rheumatic carditis: Involvement of all four valves.

Güvenç O, Çimen D. A rare situation in acute rheumatic carditis: Involvement of all four valves. Turk J Pediatr 2017; 59: 497-500. Acute rheumatic fever continues to be an important health problem, especially in countries that are socioeconomically underdeveloped. Carditis, which develops in approximately half of the patients, is responsible for both early-stage mortality as well as late-stage surgical treatment due to heart valve insufficiency or stenosis. The most frequent and severe valve involvement is with the mitral valve, while the aortic valve has the second highest incidence of involvement. Pulmonary and tricuspid valves are rarely involved. The literature cites a few adult cases in which all four valves are affected by rheumatic carditis; however, to the best of our knowledge, there have been no acute-stage rheumatic carditis pediatric cases reported. This article presents a 13-year-old male patient of Syrian origin who escaped to Turkey from the war in his country, and who was in the acute stage of rheumatic carditis in which all four valves were involved.

Hypertrophic cardiomyopathy with Jeune syndrome: The first reported case.

Jeune syndrome (Asphyxiating thoracic dysplasia) is a rare dystrophy of the skeleton, inherited as an autosomal recessive condition. Patients develop a narrowed thorax, rhizomelic dwarfism, and hepatic, renal, and pancreatic abnormalities. High rates of pulmonary hypoplasia and pulmonary hypertension have been reported. Some patients die in early stages of life due to respiratory failure. The case of a patient referred with a history of severe asphyxiating birth, who had been diagnosed with Jeune syndrome and later hypertrophic cardiomyopathy (HCM) upon echocardiographic examination is described in the present report. This rare disease is discussed with respect to the current literature, as the present is the first reported case to be accompanied by HCM.

N-terminal-pro brain natriuretic peptide levels in children with allergic rhinitis.

BACKGROUND/AIM: Persistent upper airway obstruction may lead to increased pulmonary arterial pressure (PAP). The aim of this study was to evaluate N-terminal pro brain natriuretic peptide (NT-proBNP) concentrations and PAP values in children with allergic rhinitis. MATERIALS AND METHODS: Sixty-six patients with allergic rhinitis and 22 healthy children were prospectively enrolled in this study. Plasma NT-proBNP levels were measured at first admission and after treatment. Simultaneously, echocardiography was done to assess pulmonary arterial hypertension, and rhinitis symptom scores were recorded. RESULTS: The median age of the study group was 9.0 (5.0‒17.0) years; 26 were female. PAP was found to be normal in all the patients. There was a negative correlation between age and NT-proBNP levels (r = -0.452, P < 0.01). Nasal blockage levels affected NT-proBNP levels mildly (P = 0.067). No significant difference between before and after nasal steroid treatment was observed in NT-proBNP levels. CONCLUSION: These results suggest that NT-proBNP level and PAP may not be affected in children with allergic rhinitis, and primarily this influence may be associated with the severity of nasal obstruction.

Abducens nerve palsy in a girl with incomplete Kawasaki disease.

Kawasaki disease (KD) is a systemic vasculitis that can involve the nervous system, including the cranial nerves. Central nervous system findings, especially irritability, lethargy, and aseptic meningitis, occur in 1-30 % of KD patients (1). Cranial nerve palsies are seen rarely, and abducens nerve palsy has been reported in only three children. We describe a 2.5-year-old girl with incomplete KD who developed transient abducens nerve palsy after intravenous immunoglobulin (IVIG) treatment.

Short-Term Outcomes of Patent Ductus Arteriosus Closure With New Occlutech® Duct Occluder: A Multicenter Study.

AIM: Over the past 2 decades, transcatheter occlusion of patent ductus arteriosus (PDA) with coils and the duct occluders evolved to be the procedure of choice. A new device, the Occlutech PDA® occluder (ODO) device has been designed. Herein, we aimed to evaluate the characteristics and short-term results of patients who underwent transcatheter closure of PDA using the ODO. METHODS: We reviewed the clinical records of 60 patients from different centers in Turkey between December 2013 and January 2016. The medical records were reviewed for demographic characteristics and echocardiographic findings. Device size was selected on the narrowest diameter of PDA. RESULTS: The median patient age was 2.5 years (6 months-35 years), and median PDA diameter was 2.5 mm (1.2-11 mm). Fifty-eight of 60 patients (96.6%) had successful ODO implantation. The occlusion rates were 37/58 (63.7%) at the end of the procedure, 51/58 (87.9%) at 24-48 hours post-procedure, and 57/58 (98.2%) on echocardiography at a median follow-up of 7.6 months. CONCLUSION: Our results indicate that transcatheter closure of PDA using the ODO is effective. Larger studies and longer follow-up are required to assess whether its shape and longer length make it superior to other duct occluders in large, tubular, or window-type ducts. (J Interven Cardiol 2016;29:325-331).

Growth-differentiation Factor-15 and Tissue Doppler Imaging in Detection of Anthracycline-induced Cardiomyopathy During Therapy of Childhood Cancers.

OBJECTIVES: The aim of this study was to evaluate the importance of growth-differentiation factor-15 (GDF-15) levels and tissue Doppler imaging (TDI) in the early detection of anthracycline-induced cardiomyopathy during the treatment of childhood cancers. PATIENTS AND METHODS: Twenty patients (13 males and 7 females) newly diagnosed with childhood cancer whose treatment protocol included anthracycline were included in the study. Echocardiography, including M-mode, pulse Doppler, and TDI, was performed after the first anthracycline treatment at cumulative doses of 100, 200, and 300 mg/m and at least 6 months after the last treatment. GDF-15 and troponin-I were also measured at these time points. RESULTS: The median age of the patients was 14 years (range, 3 to 18 y). The median cumulative anthracycline dose was 220 mg/m (range, 60 to 400 mg/m). Conventional pulse wave and pulse wave tissue Doppler methods revealed significant differences in the right ventricular myocardial performance indices of the patients who received cumulative anthracycline doses of 300 mg/m compared with their indices at least 6 months after the last treatment. The serum GDF-15 levels after the cumulative anthracycline dose of 200 mg/m were also higher than the patients' pretreatment levels. CONCLUSIONS: Doppler/TDI and GDF-15 levels may be used in the early determination of anthracycline-induced cardiomyopathy during the treatment of childhood cancers.

Normal M mode values in healthy Turkish children.

AIM: Evaluation of cardiac structures requires a large study group for accurate data on normal values. The aim of the study was to obtain normal M mode echocardiographic values in a substantial sample of healthy term neonates and children to develop centile charts. MATERIALS AND METHODS: Data were obtained over 2 years from a single center in Turkey, from 1200 healthy infants and children aged 1 day to 17 years. Using echocardiographic investigation, measurements were obtained of the following: left ventricular dimension at end diastole and end systole; thickness of interventricular septum and posterior wall of the left ventricle; aortic and pulmonary root diameter; and left atrial dimension. The influence of systematic errors as statistical noise in this large sample was decreased using third- degree polynomial curves. RESULTS: Measurements are presented graphically as curved lines of centiles with respect to body weight for healthy term neonates and children. The values showed a good correlation with body weight and allowed the construction of percentile curves (5%, 25%, 50%, 75%, and 95%). Higher values were observed in boys during adolescence. CONCLUSION: The presented charts and tables make it possible to judge the echocardiographic measurements of a particular patient as normal or abnormal.

Truncus arteriosus: Diagnosis with dual-source computed tomography angiography and low radiation dose.

Truncus arteriosus is an uncommon congenital cardiac abnormality which is characterized by a single arterial trunk origin from the heart that supplies both the systemic, pulmonary and coronary circulation. We present a preterm newborn female patient with type 2 truncusarteriosus, left superior vena cava and aberrant subclavian artery diagnosed with low dose dual-source cardiac computed tomography (CT). We discuss that low dose dual-source cardiac CT has more advantages than other imaging methods and it is an important modality for assessment of patients with conotruncal anomalies such as truncusarteriosus.

Prolonged QT dispersion in the infants of diabetic mothers.

Myocardial hypertrophy and cardiac dysfunction frequently occur in newborns of diabetic mothers. The authors hypothesized that wall hypertrophy or disproportionate left ventricular wall thickness in newborns of diabetic mothers may affect both QT and QTc dispersion. This study aimed to assess whether left ventricular hypertrophy affects the QT variables of infants born to diabetic mothers. This prospective cross-sectional study was conducted with 47 consecutively selected neonates of gestational diabetic mothers and 30 healthy neonates born to healthy mothers. All the subjects were evaluated during the neonatal period. Electrocardiography with echocardiography was performed for the patients and the control subjects. The newborns of the diabetic mothers were classified according to septal thickness as group 1 (16 newborns with septal hypertrophy) or group 2 (31 newborns without septal hypertrophy). The study group consisted of three cohorts: groups 1, 2, and 3 (control group). Both QT and QTc dispersion were computed from a randomly selected beat as well as from an average beat derived from 12 beats included in a 10-s electrocardiography. A total of 16 infants (34%) had a septal thickness of 6 mm or greater. The left ventricular end-systolic diameter in group 1 was smaller (p = 0.0029) than in groups 2 and 3 (p = 0.003). The interventricular septal thickness at end diastole (IVSTd) and the left ventricular posterior wall thickness at end diastole in group 1 were higher than in of groups 2 and 3. The QT and QTc dispersion intervals were longer in group 1 than in groups 2 and 3 (p < 0.001), and a highly significant positive correlation was detected between IVSTd and QT dispersion (r = 0.514, p = 0.042). Elevated QT and QTc dispersions may be risk factors for the development of arrhythmias in newborns of diabetic mothers. These patients may critically need systematic cardiac screening.

Assessment of P-wave dispersion in children with atrial septal aneurysm.

BACKGROUND: This was a prospective controlled study to determine the P-wave duration and P-wave dispersion in patients with atrial septal aneurysm. METHODS: A total of 41 children with atrial septal aneurysm, including 21 boys and 20 girls (mean age 11.85 ± 3.8 years), and 32 controls, including 17 boys and 15 girls (mean age 12.3 ± 2.9 years), were included. P-wave dispersion was calculated from the 12-lead electrocardiogram. Cardiac functions, morphology of the aneurysm, and left atrial diameter were measured using conventional echocardiography. The diagnosis of atrial septal aneurysm was made when the base of the aneurysms with an excursion ratio ≥25% was found on echocardiography. RESULTS: There was no significant difference between the patient and control groups in demographic, clinical findings, and M-mode echocardiographic parameters. The P-wave dispersion in patients with atrial septal aneurysm was significantly longer compared with the control group (64.4 ± 13.4 ms; p < 0.0001). Similarly, the the maximum duration of the P wave in the patient group was significantly longer compared with the control group (106.1 ± 13.3 ms; p < 0.001). The P-wave duration and dispersion were not correlated with age, gender, systolic and diastolic blood pressure, or m-mode echocardiographic parameters. CONCLUSIONS: This study shows that P-wave dispersion is delayed in atrial septal aneurysm patients. Prolonged P-wave dispersion was determined to indicate electrical disturbance, and therefore it has an increased electrocardiographic risk of atrial arrhythmia in children with atrial septal aneurysm.

Evaluation of cardiac functions of infants of diabetic mothers using tissue Doppler echocardiography.

AIM: Transient cardiac hypertrophy occurs in infants of diabetic mothers. The effect of this state on cardiac functions was investigated with a case-control study using tissue Doppler technique. MATERIAL AND METHODS: In this study, right and left ventricle systolic and diastolic functions of 45 term babies of diabetic mothers and 50 healthy term newborns were examined using tissue Doppler echocardiography. RESULTS: The septum was found to be thick in 16 (36%) of the babies of diabetic mothers. Both the left and right ventricle myocardial velocities were found to be lower in the babies of diabetic mothers compared to the control group. In our study, the Em/Am ratio was found to be below one only in the babies of diabetic mothers in the left ventricle in contrast to the control group. In addition, the Em/Am ratio in the septum and right ventricle was found to be below one both in the babies of diabetic mothers (group 1, 2) and control group. The calculated Tei index was found to be higher in the babies of diabetic mothers who had a thicker interventricular septum compared to the control group. CONCLUSION: Interventricular septal thickening in babies of diabetic mothers disrupt the diastolic function of both ventricles. This can be demonstrated by tissue Doppler echocardiography. These results show that diastolic function is disrupted in both ventricles in babies of diabetic mothers and only in the right ventricle in healthy babies. It was thought that this could be explained by right ventricular dysfunction arising from physiological pulmonary hypertension in the neonatal period. Subclinical right and left ventricular diastolic dysfunctions which we found by tissue Doppler indicate that babies of diabetic mothers especially with a thick septum should be closely monitored.

Does oxybutynin hydrochloride cause arrhythmia in children with bladder dysfunction?

AIM: Pediatric surgeons frequently encounter children presenting with voiding dysfunction symptoms, including urgency, frequency, and incontinence. Antimuscarinic agents Oxybutynin) are the main drugs used to treat patients with overactive bladder (OAB) syndrome, defined as urgency, with or without urgency incontinence, usually with increased daytime frequency and nocturia. Increased QT dispersion is known to be the cause of ventricular arrhythmia in various systemic diseases and leads to increased mortality and morbidity. METHOD: This study represents a subset of a complete data set, considering only those children aged admitted to the Pediatric Surgery and Pediatric Nephrology Clinics during the period January 2011 to July 2012. RESULT: In this study, we have determined that the QT interval changes significantly depending on the use of oxybutynin. The QT changes increased cardiac arrhythmia in children. CONCLUSION: For this reason, children using such drugs should be closely monitored for cardiac arrhythmia.

Growth-differentiation factor-15 and tissue doppler imaging in detection of asymptomatic anthracycline cardiomyopathy in childhood cancer survivors.

OBJECTIVES: Anthracyclines have led to an increased risk of cardiac morbidity and mortality. Late cardiac complications in cancer survivors may develop from subclinical myocardial damage. Tissue Doppler imaging (TDI) also has potential as a clinically useful technique for the assessment of myocardial function. Biochemical markers may be used to detect cardiac damage growth-differentiation factor-15 (GDF-15) and are emerging as a biomarker of cardiac dysfunction. The aim of this study is to assess the value of the plasma levels of GDF-15 and TDI in detecting late myocardial dysfunction in childhood cancer survivors (CCS) who were treated with anthracyclines. DESIGN AND METHODS: Thirty-eight CCS who had completed chemotherapy treatment with anthracyclines were included in this study. Control group consisted of 32 age- and gender-matched healthy volunteers. All children underwent a detailed echocardiography, which contained an M-mode, pulse Doppler and tissue Doppler imaging. However, GDF-15 and cardiac troponin-I (cTnI) were measured. RESULTS: Although, systolic function of the left ventricular was similar in all groups, there were significant differences between parameters of diastolic function of the heart. The mitral valve E wave, E/A ratio, left ventricular E'm wave, and E'm/A'm ratio were different in the patients than in the controls (p = 0.049, p = 0.037, p < 0.0001, p = 0.001, respectively). The tricuspid valve E/A ratio, right ventricular E't wave, and E't/A't ratio in the patients were also different from those of the controls (p = 0.031, p < 0.0001, p < 0.0001, respectively). Mean plasma GDF-15 was significantly higher in patients than healthy controls (p = 0.027). There were no significant differences in cTnI between both groups. CONCLUSIONS: Growth-differentiation factor-15 level may be used as a biomarker of anthracycline-induced cardiovascular disease severity in the CCS.

The usefulness of plasma asymmetric dimethylarginine (ADMA) levels and tissue doppler echocardiography for heart function in term infants born to mothers with gestational diabetes mellitus.

OBJECTIVE: The aim of this study was to examine whether asymmetric dimethylarginine (ADMA) concentrations are associated with ventricular function in the infants of mothers with gestational diabetes. METHOD: Twenty-five term newborns of mothers with gestational diabetes and term newborns as the control group (n = 25) with normal general health status were evaluated at two time points, on the 3rd postnatal day, at the 3th months. Echocardiographic evaluations of all participants were performed and ADMA level was measured. RESULTS: In the first analysis, 10 patients (40%) had a septal thickness of 6 mm or more, indicating septal hypertrophy. In the first and second analysis, interventricular septum end-diastolic thickness (IVSTd) and the left ventricular posterior wall end-diastolic thickness (LVPWTd) in the patient group were higher than the control group. ADMA level measurement was not significantly different between the groups the first and second analysis. There was no difference in ADMA levels of the group with septal thickness ≥6 mm and the group with <6 mm. CONCLUSION: Newborn cardiac wall thickness was increased in pregnancies complicated by Gestational diabetes mellitus (GDM), and the increase was independent of glycemic control. Diastolic newborn cardiac function was impaired in GDM, and this effect was independent of septal thickness. We found no association between ADMA levels and cardiac systolic, diastolic functions or septum thickness in the GDM newborn.

Release of NT-pro brain natriuretic peptide in children before and after adenotonsillectomy.

OBJECTIVES: To measure serum B-type natriuretic peptide, especially N-terminal segment of its prohormone (NT-proBNP) and pulmonary arterial pressure levels and to determine whether NT-proBNP concentrations correlate with pulmonary arterial pressure levels in children before and after adenotonsillectomy. METHODS: Twenty children with adenoid and tonsil hypertrophy and 20 healthy subjects were included in the study. Transthoracic echocardiography was performed in all patients to assess pulmonary artery systolic and diastolic pressure with cardiac dysfunction. The NT-proBNP was analyzed for correlation with pulmonary artery pressure. RESULTS: Our results showed that prohormone serum concentrations and pulmonary arterial pressures were significantly higher in the study group than in the control group at the preoperative evaluation. A significant decrease was detected among patients after surgery (p<0.05). There was no significant difference between the study and control groups for prohormone serum concentration and pulmonary arterial pressure at the postoperative evaluation (p>0.05). CONCLUSION: We found increased serum prohormone levels and height pulmonary artery pressures in the children with adenoid and tonsil hypertrophy when comparing with healthy subjects. Our study supported that increased serum prohormone levels and pulmonary artery pressures as a result of adenoid and tonsil hypertrophy are reversible. Pediatric cardiologs and otolaryngologs should keep in mind an increased pulmonary artery pressure during management of children with adenoid and tonsil hypertrophy. Furthermore, Doppler echocardiography may be useful in the monitoring of pulmonary arterial pressure and in the follow-up of surgical outcome of children with adenoid and tonsil hypertrophy.

P-wave duration and dispersion in children with uncomplicated familial Mediterranean fever.

OBJECTIVES: This was a prospective controlled study to determine the P-wave duration (Pdu) and P-wave dispersion (Pd) in patients with familial Mediterranean fever (FMF). METHODS: The study group consisted of 26 children with uncomplicated FMF and 25 age- and sex-matched healthy controls. We performed electrocardiography (ECG) with Doppler echocardiography on patients and controls. All participants underwent 12-lead electrocardiography under strict standards. Pdu and Pd were computed from a randomly selected beat and from an averaged beat constructed from 12 beats, included in a 10-s ECG. RESULTS: The left ventricle (LV) dimensions, LV ejection fraction (LVEF), and LV fractional shortening (LVFS) values, left atrium dimension, and aortic dimension were in normal range in both groups. There were significant differences between the groups regarding LV-isovolumic relaxation time (IRT), LV-isovolumic contraction time (ICT), right ventricle (RV)-ICT, RV-IRT, and Pd (all p < 0.0001). However, highly significant positive correlation was detected between LV-ICT, LV-IRT, RV-ICT, RV-IVT, C-reactive protein (CRP), and Pd (r = 0.505, p < 0.0001; r = 0.483, p < 0.0001; r = 0.433, p = 0.001; r = 0.421, p = 0.001; r = 0.452, p = 0.001; r = 0.478, p < 0.0001, respectively). CONCLUSIONS: Uncomplicated FMF children who are continuously treated with colchicine and do not develop amyloidosis have abnormal atrial dispersion and therefore seemingly have an increased electrocardiographic risk of atrial fibrillation.

Platelet distribution width and mean platelet volume in children with pulmonary arterial hypertension secondary to congenital heart disease with left-to-right shunt: new indices of severity?

The majority of patients with congenital heart disease (CHD), particularly those with relevant systemic-to-pulmonary shunts, if left untreated, will experience the development of pulmonary arterial hypertension (PAH). Previous studies have shown that platelet activation occurs in patients with PAH. In response, this study aimed to investigate the platelet indices, including platelet distribution width (PDW) and mean platelet volume (MPV), in patients with PAH. The study was conducted in the pediatrics cardiology unit of Selcuk University Medical Faculty between July 2010 and January 2012. The patients' clinical and laboratory data were obtained retrospectively from hospital recordings. The study enrolled 57 children with CHD (all with left-to-right shunting). The patients who had undergone diagnostic cardiac catheterization were analyzed according to the presence or absence of PAH. Group 1 had CHD with PAH, and group 2 had CHD without PAH. Compared with the group 2 patients, a significant decrease in PDW (p < 0.0001) was noted in the group 1 patients (with PAH). Likewise, a significant difference was found in the MPV of group 1 (p < 0.0001). Statistically, groups 1 and 2 did not differ in terms of platelet count (p = 0.3). Patients who had pulmonary hypertension secondary to CHD with left-to-right shunting exhibited a lower PDW and MPV.