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Background: Our knowledge regarding the epidemiology of pediatric cardiomyopathy is based on large national population studies reporting an annual incidence of 1 case per 100,000 children, with a higher incidence observed in infancy and among selected populations. The aim here is to document the epidemiology of pediatric cardiomyopathy in a Mediterranean population., Methods: Children younger than 18 years of age living on the Mediterranean island of Crete, Greece, who have been evaluated since the establishment of tertiary pediatric cardiology services (2002-2022) were included in this retrospective study., Results: A total of 40 children were included, corresponding to an average annual incidence of pediatric cardiomyopathy of 1.59 cases (95% CI: 1.4-2.3) and a prevalence of 26 cases per 100,000 children. In decreasing order of frequency, most cases corresponded to dilated (50%), followed by hypertrophic (42.5%), arrhythmogenic (5%), and restrictive (2.5%) cardiomyopathy. An etiology was identified in 40%, including a genetic diagnosis in 22.5%., Conclusions: The incidence of pediatric cardiomyopathy in the Mediterranean island of Crete is higher compared with that reported previously for other Caucasian populations. Further study is needed to investigate the exact prevalence and specific genetic factors associated with the epidemiology of pediatric cardiomyopathy in Mediterranean populations.

Paediatric cardiomyopathies form a heterogeneous group of disorders characterized by structural and electrical abnormalities of the heart muscle, commonly due to a gene variant of the myocardial cell structure. Mostly inherited as a dominant or occasionally recessive trait, they might be part of a syndromic disorder of underlying metabolic or neuromuscular defects or combine early developing extracardiac abnormalities (i.e., Naxos disease). The annual incidence of 1 per 100,000 children appears higher during the first two years of life. Dilated and hypertrophic cardiomyopathy phenotypes share an incidence of 60% and 25%, respectively. Arrhythmogenic right ventricular cardiomyopathy (ARVC), restrictive cardiomyopathy, and left ventricular noncompaction are less commonly diagnosed. Adverse events such as severe heart failure, heart transplantation, or death usually appear early after the initial presentation. In ARVC patients, high-intensity aerobic exercise has been associated with worse clinical outcomes and increased penetrance in at-risk genotype-positive relatives. Acute myocarditis in children has an incidence of 1.4-2.1 cases/per 100,000 children per year, with a 6-14% mortality rate during the acute phase. A genetic defect is considered responsible for the progression to dilated cardiomyopathy phenotype. Similarly, a dilated or arrhythmogenic cardiomyopathy phenotype might emerge with an episode of acute myocarditis in childhood or adolescence. This review provides an overview of childhood cardiomyopathies focusing on clinical presentation, outcome, and pathology., (Copyright © 2023 Hellenic Society of Cardiology. Published by Elsevier B.V. All rights reserved.)

Aim: We studied the incidence and time course of any coronary artery changes in children up to 2 years of age who were hospitalised with mild COVID-19., Methods: This was a single-centre prospective study of 29 children (19 males) with a median age of 3 months and interquartile range (IQR) of 1.6-4.3 months. They were admitted to a Greek University hospital for mild COVID-19 from 1 March to 30 December 2021. Three echocardiographic evaluations were performed at a median (IQR) of 19 (16-24) days, 82 (75-89) days and 172 (163-197) after the first symptoms. The prevalence of coronary artery dilation, regression, and changes was documented., Results: Coronary artery dilation was present in 3 (10.3%) cases at the first evaluation, with complete regression at the second. Regression was observed in 18/24 (75%) cases with follow-up data and 9 (31%) demonstrated significant z-score changes of >2. Coronary artery changes in any segment at any time were documented in 18/29 (62%) of the patients., Conclusion: Cases of transient and very mild coronary artery dilatation following mild COVID-19 completely regressed within 3 months. Large-scale studies are needed to document the extent and time course of coronary artery dilation following paediatric COVID-19., (© 2022 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Objectives: Recent advances in Spatial Temporal Image Correlation (STIC) 4 D fetal echocardiography include the application of eSTIC based on electronic probe image acquisition. We aimed to directly compare the performance of conventional STIC versus eSTIC technique (B-Mode and color Doppler imaging) during off-line reconstruction of STIC/eSTIC fetal heart volume pairs., Methods: Pairs of B-Mode and Color Doppler STIC volumes were acquired sequentially by firstly conventional (STIC) followed by electronic (eSTIC) probes during 33 consecutive obstetric scans at median 23 (range 13-31) gestational weeks. The resulting 66 fetal heart volume pairs were assessed blindly off-line by a fetal cardiologist who documented feasibility of reconstruction, presence of motion artifacts, subjective image quality on a 4-level scale: 1-best to 4-non-diagnostic and morphological diagnosis, to enable a paired comparison of STIC and eSTIC in the same fetus under similar scanning conditions., Results: eSTIC volumes had higher temporal resolution (37 vs. 24 frames per second, p  < .001), less motion during acquisition (12 vs. 20 cases, O.R. 7.0, p  = .002) and better average image quality (1.9 vs. 2.2, p  = .006) compared to STIC volumes. More diagnostic reconstructions were achieved by eSTIC ( n  = 55, 86%) than STIC ( n  = 52, 78.8%), p  = .001), in a comparable analysis time (mean 4.96 vs. 4.94 min). During a comparison of image quality of the original acquisition (A) and reconstructed planes (B and C planes) e STIC was superior in 22 (33%), 39 (59%) and 21 (38%) volumes, respectively, with the remaining cases being of similar quality (<10% in each plane in favor of STIC). Imaging mode and gestational age had a similar impact on both eSTIC and STIC performance: diagnostically acceptable studies in 49 (75.8%) vs. 48 (72.2%) by B-Mode, 60 (90.9%) vs. 56 (84.8%) by Color Doppler Mode, 8 (62.5%) vs. 10 (50%) in early scans, 38 (95%) vs. 38 (95%) in mid-gestation scans, and 7 (70%) vs. 6 (60%) in third trimester scans. Eight obstetric scans identified a fetus with a cardiac variant or structural abnormality. Diagnostic concordance of the two STIC approaches was comparable (40/48 concordant interpretations, kappa 0.657) all confirmed by fetal and/or postnatal echocardiography., Conclusions: eSTIC was associated with more effective 4 D fetal heart reconstruction due to reduced motion artifacts and superior image quality in all planes, when compared to STIC. Early gestation reconstructions were not generally successful using either technology. Further study is needed to define the cost-effectiveness and diagnostic impact of eSTIC over conventional STIC and their role over, or in addition to, screening 2 D fetal echocardiography by appropriately trained sonographers.

Acute Q fever is usually asymptomatic or is associated with a mild self-limited course and a favorable outcome. The occurrence of endocarditis during acute infection by Coxiella burnetii is an emerging clinical entity observed in adults that has been attributed to an autoimmune complication of early infection. Herein, we report the first case of a previously healthy 2-year-old child with endocarditis complicated by septic embolic stroke, in which the identified microbiological evidence was suggestive of acute rather than chronic C. burnetii infection. The development of endocarditis in this case occurred in the absence of any autoimmune reaction, but in the context of a very mild form of congenital heart disease, a small ventricular septal defect, which might serve as a predisposing factor for endocarditis. This case suggests that acute Q fever endocarditis may affect children as well and can be attributed not only to autoimmune mechanisms but also to a potential effect of the infectious agent per se on the cardiac endothelium in patients with underlying heart defects, regardless of their severity.

This study concerns the task of automatic structural heart abnormality risk detection from digital phonocardiogram (PCG) signals aiming at pediatric heart disease screening applications. Recently, various systems based on convolutional neural networks trained on time-frequency representations of segmental PCG frames have been presented that outperform systems using hand-crafted features. This study focuses on the segmentation and time-frequency representation components of the CNN-based designs. We consider the most commonly used features (MFCC and Mel-Spectrogram) used in state-of-the-art systems and a time-frequency representation influenced by domain-knowledge, namely sub-band envelopes as an alternative feature. Via tests carried on two high quality databases with a large set of possible settings, we show that sub-band envelopes are preferable to the most commonly used features and period synchronous windowing is preferable over asynchronous windowing., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Cardiovascular diseases are among the most significant causes of mortality in humans. Pesticides toxicity and risk for human health are controlled at a European level through a well-developed regulatory network, but cardiotoxicity is not described as a separate hazard class. Specific classification criteria should be developed within the frame of Regulation (EC) No 1272/2008 in order to classify chemicals as cardiotoxic, if applicable to avoid long-term cardiovascular complications. The aim of this study was to review the cardiac pathology and function impairment due to exposure to pesticides (i.e. organophosphates, organothiophisphates, organochlorines, carbamates, pyrethroids, dipyridyl herbicides, triazoles, triazines) based on both animal and human data. The majority of human data on cardiotoxicity of pesticides come from poisoning cases and epidemiological data. Several cardiovascular complications have been reported in animal models including electrocardiogram abnormalities, myocardial infarction, impaired systolic and diastolic performance, functional remodeling and histopathological findings, such as haemorrhage, vacuolisation, signs of apoptosis and degeneration., (Copyright © 2018. Published by Elsevier Inc.)

Background and Objective: Early detection of cardiovascular (CV) disease or associated risk factors during childhood is of paramount importance, allowing for early treatment or lifestyle modifications, respectively. The objective of this study was to describe the development of an electronic health record (EHR), with integrated computerized decision support system (CDSS), specifically designed for supporting the needs of a pilot pediatric CV disease screening program applied on primary school students of a Mediterranean island., Methods: Evidence-based knowledge, national and international practice guidelines regarding sport preparticipation CV screening of children and young athletes has been used for the design of the designated EHR. A CDSS, capable for providing alerts for further cardiology evaluation need, has been incorporated into the EHR, based on normative anthropometric and electrocardiographic data as well as predefined positive history responses., Results: We developed a designated EHR with integrated CDSS supporting pediatric CV disease screening, capable for documenting CV-related personal and family history responses, physical evaluation data (weight, height, blood pressure), allowing for entering electrocardiogam (ECG) measurements and for uploading of multimedia files (including ECG images and digital phonocardiogram audio files). The EHR incorporates clinical calculators and referral alerts for the presence (and degree) of adiposity, hypertension, ECG abnormalities and positive history responses indicative of high CV disease risk. In a preliminary EHR validation, performed by entering data from 53 previously available paper-based health records, the EHR was proven to be fully functional., Conclusions: The pediatric cardiology EHR with CDSS features which we developed might serve as a model for EHR for primary health care purposes, capable to document and early detect CV disease and associated risk factors in pediatric populations., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Despite its robust diagnostic capabilities in adolescents and adult patients after the arterial switch operation, little information is available on the cardiovascular magnetic resonance findings in this population., Methods and Results: The cardiovascular magnetic resonance findings of 220 consecutive patients evaluated in our center were retrospectively reviewed (median age at cardiovascular magnetic resonance, 15.4 years; 66.8% male sex). Compared with published normal values, left and right ventricular end-diastolic volume z scores were mildly enlarged (0.48±1.76 and 0.33±1.5; P=0.0003 and 0.0038, respectively), with 26% of patients having left ventricular dilatation and 20% having right ventricular dilatation. Left ventricular dysfunction was present in 21.5% of patients (mild in most), and only 5.1% of patients had mild right ventricular dysfunction. Myocardial scar was found in 1.8% of patients. Dilatation of the neoaortic root was common (76%), and root z score increased at an average rate of 0.03 points per year. By multivariable analysis, neoaortic root dilatation was associated with worse neoaortic valve regurgitation (OR, 5.29; P=0.0016). The diameters of the thoracic aorta distal to the root were near-normal in most patients, whereas the neomain pulmonary artery was typically oval shaped with decreased anteroposterior and normal lateral diameters., Conclusions: Although the majority of arterial switch operation patients have normal ventricular size and function and myocardial scar is rare, an important minority exhibits ventricular enlargement or dysfunction. Neoaortic root dilatation, which is present in most patients and progresses over time, is strongly associated with significant neoaortic valve regurgitation. The findings of this study provide reference values against which arterial switch operation patients can be compared with their peers., (© 2016 American Heart Association, Inc.)

Abuse of anabolic androgenic steroids is linked to a variety of cardiovascular complications. The aim of our study was to investigate the possible cardiovascular effects of nandrolone decanoate on young rabbits using echocardiography, histology and monitoring of telomerase activity, oxidative stress and biochemical markers. Fourteen rabbits were divided into three administration groups and the control group. Doses of 4mg/kg and 10mg/kg of nandrolone decanoate, given intramuscularly and subcutaneously, two days per week for six months were applied. A 4-months wash-out period followed. Focal fibrosis and inflammatory infiltrations of cardiac tissue were observed in the high dose groups. Thiobarbituric acid-reactive species (TBARS) levels were significantly increased in the high dose groups, while catalase activity decreased. Myocardial Performance Index (MPI) is the main echocardiographic index primarily affected by nandrolone administration in rabbits. Despite the preserved systolic performance, histological lesions observed associated with distorted MPI values, point to diastolic impairment of the thickened myocardium due to nandrolone treatment. Oxidative stress accumulates and telomerase activity in cardiac tissue rises. Subcutaneous administration seems to be more deleterious to the cardiovascular system, as oxidative stress, telomerase activity and biochemical markers do not appear to return into normal values in the wash-out period., (Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.)

Background: Eisenmenger syndrome (ES) is the most advanced form of pulmonary arterial hypertension (PAH) related to congenital heart disease. Several studies have suggested that the presence and location of the shunt defines the natural history of these patients by influencing right ventricular adaptation to PAH. We aimed to echocardiographically assess differences in cardiac physiology and outcome between various types of ES., Methods and Results: In this longitudinal cohort study, 191 patients with ES and non-complex congenital heart disease were recruited, 36 with pre-tricuspid and 155 with post-tricuspid shunts. Patients with pre-tricuspid shunts were older, had higher BNP concentrations and lower exercise tolerance compared to patients with post-tricuspid shunts. Right ventricular (RV) function was impaired in patients with atrial septal defects, with larger right ventricles, impaired systolic function and adaptation. The left ventricular eccentricity index was significantly higher in pre-tricuspid defects. Within post-tricuspid shunts, patients with atrio-ventricular septal defects had better right ventricular function compared to ventricular septal defects, while in those with a patent ductus arteriosus this was worse. There was a trend towards lower mortality in patients with post versus pre-tricuspid shunts, which was significant for patients above the age of 48 years., Conclusion: The presence of a post-tricuspid shunt appears to carry physiological and possibly prognostic benefits in ES compared to patients with pre-tricuspid shunts. This should be borne in mind when management decisions and advanced therapies are considered., (Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.)

Lethal cardiac complications leading to death and various arrhythmias have been reported after organophosphate and/or carbamate poisonings. The present study focuses on the long-term effects of repeated low-level exposure to diazinon, propoxur, and chlorpyrifos (CPF) on cardiac function in rabbits. The yearly based experimental scheme of exposure consisted of two oral administration periods, lasting 3 months and 1 month each, interrupted by an 8-month washout period (total duration 12 months). At the end of the experimental scheme, the rabbits underwent an echocardiographic evaluation under sedation, after which they were killed and the tissue and serum samples were collected. A mild localized cardiotoxic effect was established by echocardiography for the three pesticides tested. Severe histological alterations were identified, especially in the diazinon-treated animals in agreement with increased persistence of this pesticide established in the cardiac tissue. In addition, all pesticides tested increased the oxidative stress and oxidative modifications in the genomic DNA content of the cardiac tissues, each one following a distinct mechanism., (© The Author(s) 2014.)

The present study focuses on the short term effects of repeated low level administration of turinabol and methanabol on cardiac function in young rabbits (4 months-old). The experimental scheme consisted of two oral administration periods, lasting 1 month each, interrupted by 1-month wash-out period. Serial echocardiographic evaluation at the end of all three experimental periods was performed in all animals. Oxidative stress markers have also been monitored at the end of each administration period. Treated animals originally showed significantly increased myocardial mass and systolic cardiac output, which normalized at the end of the wash out period. Re-administration led to increased cardiac output, at the cost though of a progressive myocardial mass reduction. A dose-dependent trend towards impaired longitudinal systolic, diastolic and global myocardial function was also observed. The adverse effects were more pronounced in the methanabol group. For both anabolic steroids studied, the low dose had no significant effects on oxidative stress markers monitored, while the high dose created a hostile oxidative environment. In conclusion, anabolic administration has been found to create a possible deleterious long term effect on the growth of the immature heart and should be strongly discouraged especially in young human subjects., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

Aim: To evaluate the performance of primary healthcare physicians in paediatric cardiac auscultation and the impact of a multimedia-based teaching intervention., Methods: A total of 106 primary healthcare physicians (77 paediatricians, 14 general practitioners and 15 medical graduates) attended four paediatric cardiac auscultation teaching courses based on virtual patients' presentation (digital phonocardiography). Their auscultatory performance was documented at the beginning of each course and at the end of two of the courses., Results: Participants initially detected 73% of abnormal murmurs and 17% of additional sounds, while 22% of innocent murmurs were interpreted as abnormal. Overall cardiac auscultation performance, assessed by a combined auscultation score, was low and independent of training level (graduates: 39.5/trainees: 42.8/board certified: 42.6, p = 0.89) or specialty (paediatricians: 42.7/general practitioners: 43.1, p = 0.89). Multimedia-based teaching was associated with a significant improvement in abnormal murmur (92.5%) and additional sound (40%) detection (p < 0.001), while 25% of innocent murmurs were still interpreted as abnormal (p = 0.127)., Conclusion: Clinical skills of primary healthcare physicians in paediatric cardiac auscultation, independent of training level or specialty, still leave potential for improvement. Multimedia-based teaching interventions represent an effective means of improving paediatric cardiac auscultatory skills., (©2012 The Author(s)/Acta Paediatrica ©2012 Foundation Acta Paediatrica.)

Background: Eisenmenger syndrome differs significantly from other types of pulmonary arterial hypertension in its physiology and prognosis. We sought to assess the relationship between the echocardiographic characteristics of patients with Eisenmenger syndrome and mortality., Methods and Results: Clinical and echocardiographic variables were assessed in 181 consecutive patients with Eisenmenger syndrome, excluding those with complex congenital heart disease. Patients' mean age was 39.1 ± 12.8 years, 59 (32.6%) were male, 122 (67.4%) were in functional class III or higher, and 74 (40.9%) were on advanced therapies. Mean oxygen saturation at rest was 85.1 ± 7.8%, and median B-type natriuretic peptide was 55.4 ng/L. Over a median follow-up of 16.4 months, 19 patients died; the strongest predictors of mortality were tricuspid annular plane systolic excursion and peak systolic velocity, myocardial performance (expressed as total isovolumic time and ratio of systolic to diastolic duration), and elevated central venous pressure (expressed as right atrial [RA] area, RA pressure, and ratio of RA to left atrial area), even after we accounted for advanced therapies. A composite score based on the strongest echocardiographic predictors of outcome, including 1 point for each of the following: tricuspid annular plane systolic excursion <15 mm, ratio of right ventricular effective systolic to diastolic duration ≥ 1.5, RA area ≥ 25 cm², ratio of RA to left atrial area ≥ 1.5, was highly predictive of clinical outcome (area under the curve 0.90 ± 0.01), with no improvement when B-type natriuretic peptide and resting saturations were added into the model., Conclusions: Echocardiographic parameters of right ventricular function and RA area predict mortality in Eisenmenger patients. A new composite echocardiographic score, described herewith, may be incorporated into the noninvasive, periodic assessment of these patients.

Aim: To evaluate the feasibility of offline anatomic M-mode (AMM) to study fetal atrioventricular annulus long-axis displacement (LAD) and compare its performance against real-time conventional M-mode (MM)., Material and Methods: Paired AMM and MM LAD studies were recorded prospectively in 54 fetuses, and performance was compared. Insonation angles were less than 30° in all but 4 cases. The overall feasibility of AMM was tested in a composite total sample of 91 normal singleton pregnancies (median gestational age 23⁺⁶ weeks, range 12-36). AMM LAD was measured by placement of a virtual M-mode line on digitally stored raw data of fetal 4-chamber video loops. We studied annulus LAD at the lateral mitral (left ventricle; LV), proximal mitral (intraventricular septum; IVS), and lateral tricuspid (right ventricle; RV) myocardial segments. We compared LAD and its regression with gestational age measured using both methods in paired studies and AMM in the whole cohort., Results: Annulus LAD was measured using AMM, in all cases and segments, irrespective of cardiac axis alignment to the ultrasound beam. Good correlation existed between AMM and MM (RV r = 0.901, LV r = 0.899, IVS r = 0.815, p < 0.001). AMM recorded higher LAD values than MM in RV [mean 6.17 (SD 1.46) vs. 5.82 (SD 1.74) mm, p = 0.002] and LV [mean 4.18 (SD 1.11) vs. 3.98 (SD 1.12), p = 0.007]. Both methods showed LAD in RV > LV > IVS and a significant gestational increase in LAD values in all segments (p < 0.001), Conclusions: AMM permits offline evaluation of fetal longitudinal myocardial function in routinely obtained 2D fetal heart images with similar values to conventional MM in paired studies recorded <30°., (Copyright © 2012 S. Karger AG, Basel.)

Objectives: To compare myocardial deformation patterns in fetuses with congenital heart disease (CHD) with our reference range using speckle tracking echocardiography., Methods: We prospectively stored and analyzed 4-chamber loops of 28 fetuses with CHD (median gestation 27 weeks, range 20.9-37.0). The peak longitudinal left (LVs) and right (RVs) ventricular free wall Lagrangian strain and LV/RV strain ratio were measured from Syngo VVI software- (Siemens) derived original coordinates. Strain values from the first examination were compared with normative data from the same population using ANOVA with post hoc tests and serial examinations described in 14 fetuses., Results: Simple shunt lesions (0.82) and shunts with pulmonary stenosis or atresia (0.93) had reduced mean LV/RV strain ratios compared to normal fetuses (1.01; 95% CI 0.97-1.05). Fetuses with hypoplastic left heart had the lowest (0.29), and those with Ebstein the highest (1.55), LV:RV ratio. Serial measurements showed increased LVs in aortic coarctation and aortic stenosis, but not in one developing important mitral regurgitation. Increased right ventricular loading in a fetus developing pulmonary regurgitation was associated with increasing RVs., Conclusions: Myocardial strain reflects the changing physiology of fetal CHD. Speckle tracking might be a useful tool to study the progress of myocardial function in affected fetuses., (Copyright © 2012 S. Karger AG, Basel.)

Objective: To report the current status of speckle tracking techniques in evaluation of fetal myocardial deformation., Methods: A variety of non-Doppler ultrasound methods are available using offline analysis of standard four-chamber and short axis views of the heart., Results: Most reports have used techniques developed for the measurement of strain and strain rate in the adult heart and produced conflicting descriptions of gestational changes in strain. Myocardial velocities usually reflect mean modal velocities and are lower than the peak velocities obtained using Doppler techniques., Conclusions: In the fetus, most current methods of acquisition result in frame rates that are too low, and the fetal heart size is too small to achieve reliable measures of fetal myocardial deformation., (Copyright © 2012 S. Karger AG, Basel.)

Objectives: To assess the spatial and temporal performance of fetal myocardial speckle tracking, using high-frame-rate (HFR) storing and Lagrangian strain analysis., Methods: Dummy electrocardiographic signaling permitted DICOM HFR in 124 normal fetuses and paired low-frame-rate (LFR) video storing at 25 Hz 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 video storing. Further comparisons within an HFR subset included Lagrangian vs. natural strain, VVI vs. M-mode annular displacement, and 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 (left ventricle (LV): r = 0.883, P < 0.001; right ventricle (RV): r = 0.792, P < 0.001) but natural strain gave 20% lower values, suggesting reduced reliability of measurement. Lagrangian HFR strain was similar in LV and RV and decreased with gestation (P = 0.015 and P < 0.001, respectively). LV Lagrangian LFR strain was significantly lower than the values for the RV (P < 0.001) and those using paired LV-HFR recordings (P = 0.007). Annular displacement methods correlated highly (LV = 1.046, r = 0.90, P < 0.001; RV = 1.170, r = 0.88, P < 0.001). Early diastolic waves were visible in 95% of TDI, but in only 26% of HFR and 0% of LFR recordings, and HFR-VVI velocities were significantly lower than those for TDI (P < 0.001)., Conclusions: Doppler estimation of velocities remains superior to VVI but image gating and use of original co-ordinates should improve offline VVI assessment of fetal myocardial function., (Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.)

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., (Copyright 2008 Wiley-Liss, Inc.)

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., ((c) 2008 Wiley-Liss, Inc.)

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.

Unlabelled: 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.

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.

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.

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.

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., (2005 Wiley-Liss, Inc.)

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.

Unlabelled: 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.