Rana Olguntürk

Chest pain in children referred to a cardiology clinic

One hundred consecutive patients (54 girls, 46 boys) referred to a pediatric cardiology department with the primary complaint of chest pain were evaluated. The age distribution was 2.5–16.0 years (mean 11.3 years for girls and 9.9 years for boys). The history showed 17% of patients with chest pain, 22% with heart disease, and 19% with recent death in the family. The time course of the pain was longer than 1 week in 92 patients. Localization was on the left precordium in 60 patients, and there was no radiation from the original site in 66 cases. Ninety-two percent of cases were idiopathic in origin. Of the 74 patients who had a psychiatric interview, 55 (74%) had psychiatric symptoms and 5 required psychiatric care. Anxiety, conversion disorder, and depression were the main psychiatric symptoms.

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.

Cardiac Troponin T in Children With Acute Rheumatic Carditis

The existence of cardiac damage in active rheumatic carditis patients is unknown, especially in those without pericarditis. The aim of this study was to determine cardiac myocyte damage using cardiac troponin T (cTnT) measurements in active rheumatic carditis. The levels of creatine kinase MB isoenzyme (CK-MB), cTnT, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), cardiothoracic ratio (CTR), and fractional shortening (FS) were compared using Mann–Whitney U test between 28 patients with active rheumatic carditis and 32 controls (healthy children). Association of cTnT levels with ESR, CRP, and CTR was evaluated with Spearman correlation analysis. ESR, CRP, cTnT levels, and CTR were statistically higher in the patients than in the controls. There were no differences between the groups for CK-MB levels and FS. No relationships were found between cTnT levels and ESR, CRP, and CTR in the patient group. The results of the study suggest that when cTnT levels are within non-pathological range, there is no serious cardiac damage; however, statistically significant increases in cTnT levels may indicate minor damages in patients with active carditis due to acute rheumatic fever.

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.

A Comparison Between MUGA and Echocardiography in Patients with Muscular Dystrophy in the Early Detection of Cardiac Involvement

Abstract. Muscular dystrophies are a group of sex-linked diseases with frequent myocardial involvement. In this study 14 patients with Duchenne Muscular Dystrophy (DMD), 7 with Becker Muscular Dystrophy (BMD), and 8 female carriers who were asymptomatic were evaluated with echocardiography and multigated radionuclide ventriculography (MUGA). All showed predominant systolic and minor diastolic left ventricular dysfunction determined both by echocardiography and MUGA when compared with healthy controls. In conclusion, it is recommended that DMD, BMD, and female carriers be evaluated and closely monitored for cardiac functions. Though MUGA and echocardiography are both effective and sensitive techniques, echocardiography is more convenient for availability and serial evaluation.

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.