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.

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.

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.

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.

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.

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.

Is myocardial performance index useful in differential diagnosis of moderate and severe hypoxic-ischaemic encephalopathy? A serial Doppler echocardiographic evaluation.

INTRODUCTION: The aim of this study was to investigate the importance of myocardial performance index as an additive criterion to Sarnat criteria in differential diagnosis of newborn babies with moderate and severe hypoxic-ischaemic encephalopathy. METHODS: Our study group included 50 healthy term newborn babies and 20 newborn babies with hypoxic-ischaemic encephalopathy. The 20 newborn babies with hypoxic-ischaemic encephalopathy were scored using Sarnat grades. Left and right ventricular functions were determined on the first day and thereafter in the 1, 3-4, 6-7, and 11-12 months of life by M-Mode and pulsed Doppler. RESULTS: Myocardial performance indexes of the left ventricle were significantly higher in the severe hypoxic-ischaemic encephalopathy group than in the control group during the first, second, and third analyses (p = 0.01, p = 0.02, p = 0.02, respectively) and only during the first analysis (p = 0.01) in the moderate hypoxic-ischaemic encephalopathy group. In addition, the myocardial performance indexes of the right ventricle were significantly higher during the first, second, and third analyses in both severe and moderate hypoxic-ischaemic encephalopathy groups than in the control group (p = 0.01, all). Hypoxia-induced alterations last longer in the right ventricle than in the left ventricle in the moderate group, as during the second and third analyses myocardial performance index continues to be higher than the control group. CONCLUSION: Myocardial performance indexes for the left and right ventricles were significantly higher in both severe and moderate hypoxic-ischaemic encephalopathy groups than in the control group during the first analysis, and myocardial performance index greater than or equal to 0.5 can be used in order to distinguish moderate and severe hypoxic-ischaemic encephalopathy babies according to Sarnat grades as a discriminative additive criterion.

Successful treatment with propranolol in a patient with a segmental hemangioma: a case report.

The treatment of hemangiomas in infancy may be associated with significant morbidity. In addition to morbidity, an objective response cannot be obtained because of the absence of targeted therapeutic options. Herein, we present an infant with a segmental hemangioma and marked glucocorticoid toxicity due to prior steroid therapy that was successfully treated with propranolol. Propranolol was tolerated well and no side effects were observed during the treatment. The only problem to occur was disease recurrence following the withdrawal of propranolol at age 13 months, which was not within the age of spontaneous regression (generally considered as >18 months).

Release of N-terminal pro-brain natriuretic peptide in children with acute rheumatic carditis.

BACKGROUND: Acute rheumatic carditis is still an important cause of cardiac failure in developing countries. B-type natriuretic peptides, especially N-terminal segment of its prohormone are now recognised as essential parts of cardiologic evaluation. Increased plasma concentrations of B-type natriuretic peptide and its prohormone are markers of cardiac failure and hypoxia in adults. AIM: To measure the prohormone levels in children with acute rheumatic carditis and to determine whether its concentrations correlate with clinical and laboratory findings. METHODS: A total of 24 children with acute rheumatic carditis and 23 age and sex-matched healthy subjects were entered in the study. Transthoracic echocardiography was performed in all patients to assess the severity of the valve insufficiency and cardiac dysfunction. The prohormone plasma levels were tested for correlation with cardiac dysfunction and other biochemical markers, such as C-reactive protein, erythrocyte sedimentation rate, and anti-streptolysin-O titter. RESULTS: The prohormone plasma concentrations were significantly higher in children with acute rheumatic carditis than in control subjects at the time of diagnosis. A significant decrease of the plasma level was detected among patients after treatments (6-8 weeks). CONCLUSION: We found increased plasma prohormone levels in children with acute rheumatic carditis in the acute stage of illness compared with healthy subjects. Another result is increased plasma prohormone levels as acute rheumatic carditis are reversible.

Cardiac functions evaluated with tissue Doppler imaging in childhood cancers treated with anthracyclines.

AIM: The aim of this study was to assess the cardiac functions using conventional echocardiography and tissue Doppler imaging in childhood cancers treated with anthracyclines. METHODS: The study group was selected from the patients admitted to the pediatric oncology department for a treatment protocol that included doxorubicin. Body surface area was calculated and complete 2-dimensional, M-mode, pulse wave Doppler and pulse wave tissue Doppler echocardiographic examinations were performed just before the first treatment and at least 6 months after the last treatment. RESULTS: This study included 20 patients (12 males and 8 females). Mean cumulative antracycline dose was 189 +/- 102.90 mg/m(2). There were no significant differences between the pre- and post-treatment groups regarding systolic and diastolic blood pressures, heart rates, left ventricular ejection fraction and fractional shortening, right and left ventricular conventional and tissue Doppler diastolic parameters (E and A waves, E/A ratio, E' and A' waves, E'/A' ratio), but there were significant differences between the pre- and post-treatment groups regarding body surface area, right and left ventricular myocardial performance index observed by conventional pulse wave and pulse wave tissue Doppler methods. CONCLUSION: Tissue Doppler imaging provided additional information on cardiac functions. While systolic and diastolic functions were in normal range, myocardial performance index observed by tissue Doppler method was impaired in children who were treated with anthracyclines.

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.

[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.

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.

The myocardial performance index in children with isolated left-to-right shunt lesions.

OBJECTIVE: The myocardial performance index (MPI) measures the ratio of isovolumic time intervals to ventricular ejection time. The effects of altered ventricular preload or afterload on MPI have yet to be determined. This study was designed to determine the impact of altered preload on left and right ventricular myocardial performance index in the clinical setting of left-to-right lesions. METHODS: The left and right ventricular myocardial performance indexes were measured in 17 patients with atrial septal defect (ages 6 to 148 months), 23 patients with ventricular septal defect (ages 2 to 160 months), and 24 healthy children (ages 3 to 160 months). A complete 2- dimensional and Doppler echocardiographic examination was performed in all study groups. RESULTS: In patients with atrial septal defect, ventricular septal defect, and control group subjects, the left ventricular MPI was 0.38, 0.37 and 0.32, respectively, and the right ventricular MPI was 0.24, 0.21, and 0.20, respectively. No significant differences in the left and right ventricular myocardial performance indexes were seen between patients with left-to-right shunt lesions and control subjects. CONCLUSION: This study documents that the myocardial performance index is a quantitative measure of ventricular function that appears to be relatively independent of changes in preload.

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.