Abstract: OBJECTIVE: Assessment of spatial and temporal performance of fetal myocardial speckle tracking, using high frame rate storing and Lagrangian strain analysis. METHODS: Dummy ECG signalling permitted DICOM high frame rate storing (HFR) in 124 normal fetuses and paired low frame rate video storing (LFR) at 25Hz in 93 of them. Vector velocity imaging (VVI) tracking co-ordinates were used to compare time and spatial domain measures. We compared tracking success, Lagrangian strain, peak diastolic velocity and positive strain rate values in HFR vs LFR. Further comparisons within a HFR subset included Lagrangian vs Natural strain, VVI vs M-Mode annular displacement, VVI vs pulsed wave tissue Doppler imaging (TDI) peak velocities RESULTS: HFR (average 79.4 Hz) tracking was more successful than LFR (86% vs. 76%, p=0.024). Lagrangian and Natural HFR strain correlated highly (LV: r(S)=.883, p<0.001 RV: r(S)=0.792, p<0.001) but Natural strain gave 20% lower values suggesting reduced reliability of measurement. Lagrangian HFR strain was similar in left (LV) and right (RV) ventricles and decreased with gestation (p<0.001, p=0.026). LV Lagrangian LFR strain was significantly lower than RV (p<0.001) and than paired HFR (p=0.007). Annular displacement methods correlated highly (LV = 1.046, r = 0.90, p<0.001, RV = 1.171, r=0.88, p<0.001). Early diastolic waves were visible in 95% TDI, 26% HFR but 0% LFR, and HFR VVI velocities were significantly lower than TDI (p <0.001). CONCLUSIONS: Doppler estimation of velocities remains superior to VVI but image gating and use of original co-ordinates should improve off-line VVI assessment of fetal myocardial function. Copyright (c) 2010 ISUOG. Published by John Wiley & Sons, Ltd.
Abstract: AIM: To evaluate the performance of experienced cardiologists by use of digital phonocardiography for the correct identification of heart disease and innocent murmurs in children. METHODS: Two independent paediatric cardiologists blindly evaluated 83 digital phonocardiograms obtained from consecutive paediatric cardiology outpatients. Each observer had to document the presence and characteristics of murmurs (intensity, quality, location), the presence of additional abnormal auscultatory findings (systolic click, second heart tone abnormalities) and whether he would recommend echocardiography (ECHO). The accuracy of their diagnoses was tested against the ECG diagnosis within two severity levels of heart disease. RESULTS: Twenty-three out of 24 cases (95.8%) with moderate-to-severe heart defects and 12 to 13 out of 19 cases (63.2%-68.4%) with trivial-to-mild heart defects were correctly identified by phonocardiography. Additionally, 37 to 38 out of 40 cases (92.5%-95.0%) without ECHO evidence of heart disease were correctly interpreted as having innocent murmurs. The level of agreement between the two observers was substantial regarding their recommendations for ECHO (kappa, kappa=0.83) and in identifying abnormal murmurs (kappa=0.78). CONCLUSION: Evaluation of digital phonocardiography by experienced cardiologists could allow for correct identification of moderate-to-severe forms of heart disease while preventing the majority of children with innocent murmurs from unnecessary referral.
Abstract: Anthracycline-related cardiotoxicity has a substantial negative impact on long-term survivors of childhood cancer. The detection of cardiotoxicity is currently based on echocardiography or radionuclide angiography. However, as they depict only the final outcome of myocardial injury in terms of reduced heart contractility, heart specific biomarkers of myocardial destruction or dysfunction could be advantageous by allowing for an earlier detection of cardiotoxicity. In the present study, the usefulness of cardiac troponins and natriuretic peptides, the most commonly used biomarkers of myocardial destruction and ventricular dysfunction respectively, to detect and to predict the development of anthracycline cardiotoxicity has been reviewed.
Abstract: We describe a new family with Adams-Oliver syndrome (AOS). The propositus is a 14-month-old boy presenting with aplasia cutis congenita, distal limb transverse defects, growth retardation, and a wide atrial septal defect. Central nervous system abnormalities included central hypotonia, and magnetic resonance imaging (MRI) findings consistent with periventricular leukomalacia (PVL). Fetal MRI at 26 weeks' gestation had shown bilateral dilatation of lateral ventricles and periventricular cysts at the site of postnatal lesions. The patient's father and paternal grandfather also had manifestations indicative of AOS. Antenatal and postnatal MRI findings suggest that our patient's PVL represents an unusual congenital feature of AOS, possibly due to vascular disruption and decreased perfusion during critical periods of fetal brain development.
Abstract: The optimal anticoagulation following Fontan operation and its modifications remain controversial and it is even less well defined as regards patients with inherited thrombophilia. We present a case of a child with bidirectional Glenn anastomosis for double inlet left ventricle that suffered a stroke despite aspirin prophylaxis; the patient was combined homozygous for prothrombin G20210A mutation and for methylenetetrahydrofolate reductase C677T mutation as well. The family history was positive for fetal loss and premature cardiovascular disease. Large-scale studies are needed to evaluate whether carriers of thrombophilia mutations need more intense thromboprophylaxis.
Abstract: BACKGROUND: The incidence of subclinical cardiotoxicity following anthracycline treatment for childhood cancer varies according to the method used for its detection. The aim of the study was to document the prevalence of left ventricular myocardial mass (LVM) reduction and its possible association with plasma N-terminal pro-brain natriuretic peptide (NT-proBNP) levels in asymptomatic children treated with anthracyclines. PATIENTS AND METHODS: Nineteen asymptomatic children who had received anthracyclines during their treatment for cancer were evaluated. They had received an equivalent of doxorubicin dose 240 mg/m2 (22-1200 mg/m2) on average 3.9 years (0.6-8.3) before (median age at diagnosis 3.8 years). The LVM was determined by M-Mode echocardiography and compared to the expected value, obtained from the regression equation of LVM on height of a group of 160 healthy children. Additionally the patients' plasma NT-pro BNP levels were determined. RESULTS: A high prevalence of reduced LVM associated with increased NT-proBNP levels was found. The average LVM value was -14.4% (+/-4.9) lower than expected whereas fourteen patients (73%) had a lower LVM than predicted. The NT-pro BNP levels in patients with reduced LVM were significantly higher than those measured in patients without LVM reduction (0.316+/-0.02 versus 0.17+/-0.01 pmol/ml respectively, p=0.009). A cut off NT-pro BNP level of 0.2 pmol/ml could differentiate patients with LVM reduction from those with normal or greater than expected LVM. CONCLUSION: The association of higher NT-proBNP levels with reduced LVM in asymptomatic children after anthracycline administration could be an early indication of subclinical cardiotoxicity.
Abstract: Fetal echocardiography allows for early detection of congenital heart disease, and pregnancy termination may be an option in cases of complex defects. In the current study, the most important factors contributing to the diagnosis and termination of affected pregnancies are reviewed and their combined effect on the future prevalence of liveborn congenital heart disease is evaluated. The relative reduction of the prevalence of the most severe forms of congenital heart disease is estimated as the product of the probability that (1) a fetal cardiac screening is performed (p (evaluation)), (2) an affected pregnancy is detected (P (detection)), (3) pregnancy termination is decided following antenatal diagnosis (P (decision)). In areas where termination of pregnancy is a realistic and supported option, a universal sonographic screening of all pregnancies (P (evaluation) = 1), with an average reported sensitivity of 35% and a termination rate of 43% following antenatal diagnosis, would result in a 15% overall reduction of the prevalence of the most severe forms of congenital heart disease. However, wide variability exists regarding the defect-specific estimates (2-50% prevalence relative reduction) due to considerable differences in the reported diagnostic sensitivity and termination rates associated with each heart defect. If an earlier diagnosis could be achieved, which is reported to be associated with an average 1.4-fold increased probability of termination, the overall reduction of the prevalence of congenital heart disease could approach 21%. As the skills of obstetric and pediatric cardiology sonographers improve, fetal echocardiography is expected to have a substantial impact on the future epidemiology of liveborn congenital heart disease.
Abstract: In the present study the authors evaluated the status of spinal bone mineral density (BMD) using dual-energy X-ray absorptiometry in a group of 31 children with acute lymphoblastic leukemia, treated by ALL-BFM protocols without irradiation, at different phases of their treatment. These patients were studied (1) within 2 months of diagnosis (group 1, n = 17, median time postdiagnosis 17 days), (2) during chemotherapy (group 2, n = 16, median time on treatment 22 months), and (3) shortly after completion of therapy (group 3, n = 10, median time postchemotherapy 13 months). Twelve patients underwent serial measurements in either of the groups they entered (6 patients of group 1 and 6 patients of group 2). The mean cumulative prednisone dose was 0.8, 4.3, and 5 g/m2 in groups 1, 2, and 3, respectively. A gradual decline of BMD from group 1 through group 3 was observed. The mean value of age- and sex-specific BMD z-score was -0.74 (+/-0.32) in group 1, -1.59 (+/-0.24) in group 2, and -2.03 (+/-0.27) in group 3; there was a statistically significant difference between groups 1 and 3 (p = .022). A BMD z-score < -2 was found in 3 (17%), 4 (25%), and 5 (50%) patients of groups 1, 2, and 3, respectively. In conclusion, a high incidence of reduced bone mass was observed during and shortly after treatment. These findings could have significant consequences in long-term survivors.
Abstract: The effect of clarithromycin on the QT interval was studied in a group of 28 children treated for respiratory tract infections. QTc was measured before and following 24 h of treatment. A modest (average 22 ms, 95% CI 14-30 ms) but significant QTc prolongation (p<0.001) was observed, with seven cases having a QTc >440 ms during treatment (including a single case with QTc >460 ms). CONCLUSION: Serial QTc measurements are necessary for early detection of children at risk for drug-induced arrhythmias.
Abstract: BACKGROUND: The prevalence of thrombophilic traits, which might further enhance the risk of thrombotic complications in children treated for cancer, varies significantly among different populations. OBJECTIVE: To evaluate the prevalence of common thrombophilic traits of the East Mediterranean Region, among native Cretan children treated for malignancy. METHODS: Blood samples were consecutively collected from 31 native Cretan children treated for acute lymphoblastic leukaemia (n = 19) or other malignancies (n = 12) over 3 years. A molecular diagnosis based on the presence of Factor V Leiden (FVL), as well as on PT G20210A and MTHFR C677T mutation (in 14 patients) using PCR was applied. Patients who had central venous catheters (n = 29) were treated with an intensified thromboprophylaxis protocol that had been previously established in our institution. RESULTS: The prevalence of the FVL mutation was 19.4% (95% CI = 5-32). The allele frequency is estimated at 11.3% (95% CI: 3.5-19.1) which is higher than that reported for the population of the mainland of Greece. The prevalence of the PT G20210A and MTHFR C677T mutation was 14.3 and 71.4%, respectively (corresponding allele frequencies 7.1 and 50%, respectively). Only one patient developed thrombosis, having although no thrombophilic trait. CONCLUSIONS: Thrombophilic traits were relatively common in this group of native Cretan children treated for malignancy. Thromboprophylaxis should be considered in Cretan children in the presence of known acquired risk factors for thrombosis, but a larger prospective to study is first needed.
Abstract: BACKGROUND: Paired quantitative and qualitative blood cultures have been introduced for the diagnosis of catheter-related bloodstream infections (CRBI) with the catheter in situ. The aim of the study was to compare the diagnostic performance and the prognostic value of the two methods in the evaluation of febrile episodes without an apparent source in children with cancer. PROCEDURE: During a 4-year period, in every febrile episode without an apparent focus, blood was drawn simultaneously from the catheter lumen and a peripheral vein in order to perform paired quantitative (Isolator) as well as qualitative (BacT/Alert) blood cultures. The diagnosis of a CRBI was defined as either a case of greater (at least 10 fold) or earlier (differential time to positivity >2 h) bacterial growth from the catheter compared to the peripheral blood sample, respectively. RESULTS: Nineteen febrile episodes manifested in 16 children (total period of observation 11,150 catheter-days) were evaluated with both methods. A concordant diagnosis of CRBI was stated with both methods in six episodes; one episode was diagnosed as CRBI only with qualitative culture criteria. Treatment failure resulted in catheter removal in five out of the seven episodes defined as CRBI with either method. Episodes where a CRBI was ruled out with both methods had a favorable outcome. CONCLUSIONS: In this study the two methods showed comparable results in the diagnosis of CRBI and both were of prognostic significance, regarding the outcome of the treatment. However, large scale studies are required in order to evaluate the clinical relevance and the cost effectiveness of performing routinely paired blood cultures with either method.
Abstract: Catecholamine treatment for hypotension is common practice in neonatal intensive care units. In the presence of left ventricular hypertrophy, the positive inotropic action of catecholamines with mainly beta-receptor specificity can lead to excessive hypercontractility and paradoxical hypotension. This reports presents two cases of hypercontractile heart failure during beta-agonist treatment in very low birthweight (VLBW) infants. Both patients (27 wk, 5 d; and 26 wk, 6 d of gestation) underwent surgical arterial duct ligation and coarctation repair. After operation they developed arterial hypotension that failed to respond to epinephrine (adrenaline) or dobutamine treatment. Echocardiography demonstrated a complete systolic obliteration of the left ventricular cavity. Epinephrine and dobutamine were stopped, and norepinephrine (noradrenaline) was successfully used to control the hypotension. CONCLUSION: Treatment of hypotension with beta-agonists in VLBW infants with left ventricular hypertrophy can lead to hypercontractility and left ventricular obliteration with paradoxical hypotension. Careful echocardiographic monitoring is indicated in such cases.
