Serdar Kula

Peripheric stem cell transplantation in children with dilated cardiomyopathy: Preliminary report of first two cases

Olguntürk R, Kula S, Sucak GT, Özdoğan ME, Erer D, Saygili A. Peripheric stem cell transplantation in children with dilated cardiomyopathy: Preliminary report of first two cases.
Pediatr Transplantation 2010:14:257–260.

Persistent left superior vena cava: Experience of a tertiary health-care center

Background:  The aim of this study was to assess the prevalence of persistent left superior vena cava (PLSVC) in patients with all types of congenital heart defects and to determine the congenital heart anomalies accompanying PLSVC.

Mayer-Rokitansky-Küster-Hauser syndrome associated with pulmonary stenosis.

Two siblings with Mayer‐Rokitansky‐Küster‐Hauser (MRKH) syndrome associated with pulmonary valvular stenosis are reported. Although the syndrome is well documented, the genetic background and familial occurrence is not known and the association with cardiac anomalies has not previously been reported. This report is the first report which describes the combination of cardiac anomaly with MRKH syndrome.

Early diagnosis of anthracycline toxicity in asymptomatic long-term survivors: dobutamine stress echocardiography and tissue Doppler velocities in normal and abnormal myocardial wall motion

AIMS Asymptomatic long-term cancer survivors treated with anthracycline were investigated for late anthracycline cardiotoxicity using dobutamine stress echocardiography (DSE) and tissue Doppler (TD) velocities. METHODS AND RESULTS The study comprised 20 asymptomatic patient and 18 healthy children as the control group. Twenty patients were divided into two groups according to the myocardial wall motion during DSE: Group 1 (normal myocardial wall motion; six girls and five boys) and Group 2 (abnormal myocardial wall motion: nine boys). Intravenous dobutamine infusion was started at a dose of 5 µg/kg/min (D5) and gradually increased to 10 (D10), 15 (D15) and 20 µg/kg/min (D20). Echocardiographic assessment was performed at rest and after each dose of dobutamine infusion. Abnormal myocardial wall motion was observed at rest in 3 patients and during DSE in six patients. There were no significant differences between the patients and control groups at rest except the end systolic wall stress and mitral deceleration time measured by conventional methods; however, both patients group showed significant differences of systolic and diastolic functions at D20. In patients groups, systolic and diastolic functions of interventricular septum (IVS) and systolic function of left ventricle (LV) and right ventricle (RV) TD velocities showed significant changes compared with control group at rest. Significant differences of diastolic functions of IVS and RV were noted during dobutamine infusion in abnormal myocardial wall motion compared with other groups. CONCLUSION LV, RV and IVS TD velocities systolic function at rest and during DSE can provide valuable information for early detection of subclinical cardiac toxicity. TD velocities of diastolic functions during DSE are a valuable parameter for assessment of subclinical cardiac toxicity in patient with abnormal wall motion.

Haemodynamic characterisation and heart catheterisation complications in children with pulmonary hypertension: Insights from the Global TOPP Registry (tracking outcomes and practice in paediatric pulmonary hypertension)

BACKGROUND The TOPP Registry has been designed to provide epidemiologic, diagnostic, clinical, and outcome data on children with pulmonary hypertension (PH) confirmed by heart catheterisation (HC). This study aims to identify important characteristics of the haemodynamic profile at diagnosis and HC complications of paediatric patients presenting with PH. METHODS AND RESULTS HC data sets underwent a blinded review for confirmation of PH (defined as mean pulmonary arterial pressure ≥ 25 mmHg, pulmonary capillary wedge pressure ≤ 12 mmHg and pulmonary vascular resistance index [PVRI] of >3 WU × m(2)). Of 568 patients enrolled, 472 who fulfilled the inclusion criteria and had sufficient data from HC were analysed. A total of 908 diagnostic and follow-up HCs were performed and complications occurred in 5.9% of all HCs including five (0.6%) deaths. General anaesthesia (GA) was used in 53%, and conscious sedation in 47%. Complications at diagnosis were more likely to occur if GA was used (p=0.04) and with higher functional class (p=0.02). Mean cardiac index (CI) was within normal limits at diagnosis when analysed for the entire group (3.7 L/min/m(2); 95% confidence interval 3.4-4.1), as was right atrial pressure despite a severely increased PVRI (16.6 WU × m(2,) 95% confidence interval 15.6-17.76). However, 24% of the patients had a CI of <2.5L/min/m(2) at diagnosis. A progressive increase in PVRI and decrease in CI was observed with age (p<0.001). CONCLUSION In TOPP, haemodynamic assessment was remarkable for preserved CI in the majority of patients despite severely elevated PVRI. HC-related complication incidence was 5.9%, and was associated with GA and higher functional class.

Abnormality of the Left Ventricular Sympathetic Nervous Function Assessed by I‐123 Metaiodobenzylguanidine Imaging in Pediatric Patients with Neurocardiogenic Syncope

The purpose of this study was to assess the left ventricular sympathetic nervous system function in the patients with neurocardiogenic syncope (NCS) using I‐123 metaiodobenzylguanidine (MIBG) imaging of the heart, and to compare the plasma noradrenaline (NA) and MIBG results of tilt positive and tilt negative patients following a head‐up tilt test (HUT). The study included 30 patients. Their physical and laboratory examinations did not show a pathology that may be the cause of their syncope. HUT test was positive in 13 patients and negative in 17 patients. Plasma NA concentrations were higher in the HUT positive than the HUT negative group at the beginning and at the 10th minute of the test. Specific I‐123 MIBG uptake assessed as the cardiac to mediastinal activity ratio in the delayed image was significantly higher in HUT positive group. The higher levels of MIBG uptake and plasma NA observed in HUT positive patients may reflect the greater capacity of NA storage in cardiac adrenergic neuronal tissue in patients with NCS. The results of this study support the critical role of autonomic nervous system in the pathophysiology of NCS and the excessive sympathetic nervous stimulation as the trigger of paradox reflex. (PACE 2003; 26:1926–1930)

Can heart rate variability in children with epilepsy be used to predict seizures

PURPOSE The aim of this study was to examine interictal, pre-ictal and ictal autonomic system disturbance by comparing heart rate variability in children with uncontrolled epilepsy with that seen in healthy controls and children with controlled epilepsy. METHODS Our study group included 20 children with refractory epilepsy, our control groups were composed of 20 children with well-controlled epilepsy and 20 healthy children. All subjects were evaluated by Holter ECG monitoring and 12-lead ECG to assess heart rate variability and QTc dispersion. The study group was also evaluated by Holter ECG during seizures. RESULTS The study group exhibited significantly more pathological QTc dispersion than did the control groups. Heart rate variability was significantly suppressed: reduced parasympathetic activity with lower low frequency (LF) and high frequency (HF) band values were observed in the study group. Findings were similar in the well-controlled epilepsy group and the healthy group but differed from the uncontrolled epilepsy group. The examination of heart rate variability parameters during and before seizures revealed higher nLF and LF/HF ratio and lower nHF values demonstrating increased sympathetic activity. CONCLUSION We suggest that children with refractory epilepsy have abnormalities of autonomic nervous system functioning which could be linked to the increased risk of sudden unexpected death seen in the patient group. It is possible that a chronically reduced vagal tone predisposes patients to a more dramatic stress response during their seizures. It is possible that heart rate variability parameter arising prior to seizures could be used to predict future seizures.

Comparison of initial evaluation of neonatal heart murmurs by pediatrician and pediatric cardiologist.

The objective of this study was to determine differences between pediatrician and pediatric cardiologists regarding initial assessment of neonatal heart murmur and to evaluate the role of echocardiography in this group of patients. During a 6-month period, all term births in the obstetric unit at Faculty of Medicine, Gazi University, Ankara, Turkey, were initially evaluated clinically by a pediatrician and the most likely clinical diagnosis was recorded. Neonates with a heart murmur were evaluated by the pediatric cardiologist who was unaware of the previous diagnosis. A similar number of neonates without a heart murmurs was also evaluated by a pediatric cardiologist as a control group. Echocardiography was performed in both groups. For pediatrician and pediatric cardiologists, accuracy of the clinical examination demonstrated a sensitivity of 33.3% and 40% in detecting a pathological murmur and a specificity of 95.5% and 98.8%, respectively. No statistically significant differences existed between the two groups. Pediatricians can assess the significance of neonatal heart murmurs as well as pediatric cardiologists.

Acute Vasodilator Response in Pediatric Pulmonary Arterial Hypertension: Current Clinical Practice From the TOPP Registry

BACKGROUND In pulmonary arterial hypertension (PAH), acute vasodilator response testing (AVT) is considered important to identify adult patients with favorable prognosis using calcium-channel blocker (CCB) therapy. However, in pediatric PAH, criteria used to identify acute responders and CCB use are insufficiently studied. OBJECTIVES This study sought to describe current clinical practice of AVT and subsequent treatment decisions in pediatric PAH. METHODS From January 2008 to May 2013, 529 consecutive children with confirmed pulmonary hypertension were enrolled in an international registry. We analyzed those children with evaluable AVT. RESULTS Of 382 children with evaluable AVT, 212 had idiopathic/familial PAH (IPAH/FPAH) and 105 had PAH associated with congenital heart disease (PAH-CHD). In 70% of the patients, AVT was performed using inhaled nitric oxide; other agents were used in the remaining patients. In IPAH/FPAH patients, 78 (37%) patients were acute responders according to their physician, 62 (30%) according to REVEAL (Registry-to-Evaluate-Early-And-Long-term PAH disease management)-pediatric criteria, and 32 (15%) according to Sitbon criteria. For PAH-CHD patients, the numbers of AVT responders were 38 (36%), 14 (13%), and 7 (7%) respectively. Correlation between AVT responder status as judged by the treating physician and by published response criteria was poor. Moreover, of the IPAH/FPAH patients judged by the treating physician as acute responders, only 23% were treated with CCB without additional PAH-targeted therapy. The Sitbon criteria selected patients with better prognosis who had excellent outcome when treated with CCB. CONCLUSIONS The current practice of identifying responders to AVT and subsequent treatment with CCB therapy demonstrated large discrepancies with current international guidelines. Also, in pediatric IPAH, the Sitbon criteria are the criteria of choice to identify patients with excellent survival when treated with CCB therapy.

Two unusual presentations of acute rheumatic fever

Patients with acute rheumatic fever sometimes present with atypical signs and symptoms. In these circumstances, the Jones criterions may not be sufficient to make a clinical diagnosis. We describe here two patients with unusual presentations, highlighting that, both in regions where the disease is endemic, or where it is seen only sporadically, physicians should be more alert and careful in making the diagnosis.