Behavioural interventions to increase adherence to palivizumab prophylaxis in children with CHD.

OBJECTIVES: Adherence to palivizumab prophylaxis programmes is crucial to protect infants with CHD against respiratory syncytial virus infections. We analysed the effectiveness of two nudge interventions in increasing adherence. METHODS: Our study included 229 infants, and their caregivers, from five centers in Turkey in the 2020-2021 respiratory syncytial virus season. We randomly allocated caregivers to a control and two intervention groups. Caregivers in all groups were informed about the prophylaxis programme and provided a schedule. Additionally, caregivers in Intervention 1 were called two days before appointments (default bias) and were asked to plan the appointment day (implementation intention), whereas caregivers in Intervention 2 received biweekly text messages informing them about the programme's benefits (availability bias) and current adherence rate (social norm). RESULTS: Caregivers in Intervention 1 had a significantly higher adherence rate than Control (97.3% versus 90.9%) (p = 0.014). Both interventions had a significant effect on participants in their first prophylaxis season (p = 0.031, p = 0.037). Families where the father was employed had a 14.2% higher adherence rate (p = 0.001). Every additional child was associated with a 2.2% decrease in adherence rate (p = 0.02). In control, ICU admission history was associated with an 18.8% lower adherence rate (p = 0.0001), but this association disappeared in intervention groups. CONCLUSION: This is the first prospective interventional study which, in the context of palivizumab prophylaxis, analyses the effectiveness of nudge interventions based on established cognitive biases by comparing randomly generated intervention and control groups. We found that default bias and implementation intention have significant effects on adherence.Clinical trial, in the name and number "Adherence of palivizumab prophylaxis, NCT05778240" registered retrospectively. https://clinicaltrials.gov/ct2/show/NCT05778240.

Retrospective evaluation of 130 cases with kawasaki disease follow-up in a tertiary care center in Turkey between 1999 and 2019: a 20-year experience.

OBJECTIVES: Kawasaki disease (KD), which is a medium vessel vasculitis, is common in Asian countries and is the most common cause of childhood-acquired heart diseases in developed countries. However, disease course and epidemiological data are limited in non-Asian developing countries like ours. We aimed to evaluate the clinical features and prognosis of patients with KD in our country and ethnicity, one of the referee centers of our country. METHODS: Patients with KD in our center for the last 20 years in the pre-COVID-19 pandemic era were included in the study. The clinical and laboratory findings, treatments, and follow-up findings were reviewed retrospectively in different age groups. RESULTS: Of the 130 patients, 82 (63%) were male. The median age at diagnosis was 2.97 years (2 months-11.5 years). Thirty-six (27.7%) patients were diagnosed with incomplete KD, and there was no significant laboratory difference between incomplete KD and complete KD patients. Thirty-three (25.3%) patients had coronary artery lesions (CAL), and it persisted in only 3 of 33 patients. One of 15 patients with IVIG resistance had CAL. The independent risk factors were days of illness at initial IVIG administration for CAL (p = 0.013, OR [95%CI] = 1.20 [1.04-1.38]) and low hemoglobin (p = 0.003, OR [95%CI] = 0.51 [0.33-0.79]) and low sodium for IVIG resistance (p = 0.012, OR [95%CI] = 0.81[0.69-0.95]). CONCLUSIONS: The rate of CAL is approximately three times higher in our results than in the Japanese data in recent years. We showed that the time of IVIG administration is the most critical factor for preventing CAL. Wide-ranging studies are needed to decently predict the disease process according to the age and region of patients.

Evaluation of the Left Ventricular Myocardium Using Layer-Specific Strain Analysis in Adolescent Athletes Performing High-Intensity Interval Training.

High-intensity interval training (HIIT) has been demonstrated to be an efficient way of improving physical performance in adolescent athletes compared to conventional training modalities. The objective of this study was to evaluate the impact of HIIT on the myocardial function of adolescent athletes, specifically focusing on left ventricular (LV) function, using conventional echocardiography and layer-specific strain (LSS) analysis. A total of 19 male adolescent athletes (with mean age of 16.83 ± 1.29 years) participating in various football clubs were recruited for this study. During the course of 8 weeks, these adolescent male athletes engaged in HIIT program centered around running. Upon completion of HIIT program, a treadmill exercise test was conducted. Subsequently, conventional and LSS echocardiography were conducted to acquire the evaluation of LV myocardial function. Interventricular septum thickness and ventricular mass index were significantly increased post high-intensity interval training (p < 0.005). After the HIIT, the treadmill exercise test demonstrated a significant increase in test duration and metabolic equivalent compared to the pre-training values (p < 0.005). Post high-intensity interval training, LSS analysis revealed significantly improved LV circumferential strain values in the basal and mid-segments of the left ventricle when compared to the pre-training measurements (p < 0.005). The implementation of high-intensity interval training led to an enhancement of circumferential LSS in the LV, indicating a favorable physiological adaptation and improved efficiency of the myocardium.

Evaluation of endothelial dysfunction in hypertensive children and adolescents.

BACKGROUND: Atherosclerotic changes can be attributed to early endothelial damage in individuals with hypertension. We aimed to explore the relationship between endothelial dysfunction and hypertension in newly diagnosed children without end-organ damage, considering carotid intima-media thickness (CIMT), flow-mediated dilatation (FMD), and functional capillaroscopy parameters. We also analyzed the differences between dipper and non-dipper patients. METHODS: In this cross-sectional study, 20 patients diagnosed with essential hypertension with no target organ damage, and 20 age and sex-matched healthy volunteers were enrolled. The patient group comprised newly diagnosed individuals not receiving antihypertensive treatment. Hypertensive patients were divided into two groups (dipper and non-dipper patients). The measurements of CIMT, brachial FMD, and functional capillaroscopy were performed before starting treatment. RESULTS: Among the patients, 11 were boys, and 9 were girls, with a median age of 16.0 (2.13) years. Of 20 hypertensive patients, 10 were dipper and 10 were non-dipper. Significant differences were observed between the hypertensive patients and controls in terms of CIMT (p = 0.04), brachial artery FMD (p = 0.02), and functional capillary density (p < 0.001). Hypertensive patients exhibited increased CIMT, reduced brachial artery FMD, and lower capillary density. However, there were no differences between dippers and non-dippers regarding age, sex, height SDS, weight SDS, CIMT SDS, brachial artery FMD, and capillary density. CONCLUSIONS: Understanding the vascular consequences associated with essential hypertension emphasizes the importance of early detection and management of hypertension. Herein, we have effectively highlighted significant endothelial changes through the analysis of three parameters in newly diagnosed children without apparent target organ damage.

Mediastinal Radiotherapy-induced Early-onset Valvulopathy in a 6-Year-old Boy With Hodgkin Lymphoma.

Mediastinal radiotherapy for childhood cancers, particularly Hodgkin disease, has numerous potential adverse effects, including coronary artery disease, pericarditis, cardiomyopathy, valvular disease, and conduction abnormalities. The prevalence of valvular stenosis is relatively low, and regurgitation is more common. Mediastinal radiotherapy-induced valvular disease develops more than 10 years after radiotherapy. Here, we present a case of a 6-year-old boy with moderate to significant mitral stenosis + moderate mitral regurgitation and mild aortic regurgitation that appeared 1.5 months after radiotherapy and showed a progressive course.

Effect of different dose regimens of everolimus in a series of neonates with giant cardiac rhabdomyomas.

Everolimus is a mTOR inhibitor that has been increasingly used in high-risk cardiac rhabdomyomas in recent years. There are questions regarding the optimal dose and duration of therapy with everolimus for cardiac rhabdomyomas. The purpose of this study was to examine retrospectively the dosage-efficacy relationship in seven babies diagnosed with rhabdomyoma treated with different everolimus dose regimens retrospectively. Cardiac rhabdomyoma diagnosis was made in six of seven babies during the prenatal period. Indication of everolimus was an obstruction in six patients and supraventricular tachycardia which is resistant to antiarrhythmic drugs in the remaining one patient. The median age was 8 days (range; 2-105 days) at the time of starting everolimus. It was administered at a dose of 0.25 mg twice a day for two days a week in four patients; 0.1 mg/day in two and 0.4 mg/day in one patient. Serum everolimus level was kept between 5 and 15 ng/ml. All seven cases showed significant regression of cardiac rhabdomyoma within four weeks, and supraventricular tachycardia was controlled in two weeks after everolimus administration.This study demonstrates that everolimus was effective in accelerating regression of the cardiac rhabdomyoma. Dose with 2 × 0,25 mg/day, 2 days a week, seems appropriate. However, lower doses such as 0.1 mg/day are also effective. But dose adjustment should be made according to serum level monitoring.

Evaluation of clinical features and outcome of eight fetuses with ectopia cordis; A study from a fetal cardiology center.

We aim to evaluate the clinical course and outcome of cases with a prenatal diagnosis of ectopia cordis in our center. In this retrospective study, we analyzed clinical variables including gestational age at diagnosis, maternal age, associated cardiac, extracardiac, genetic anomalies and, outcome in prenatally diagnosed ectopia cordis cases in our tertiary referral center. Eight ectopia cordis cases from seven pregnancies were included in the study. All fetuses had complete type of ectopia cordis and pentalogy of Cantrell. Five multiple pregnancies were found, four were twin pregnancies (three dichorionic diamniotic, one monochorionic monoamniotic) and one was triplet (trichorionic triamniotic). In the monochorionic monoamniotic twin pregnancy, both fetuses have pentalogy of Cantrell. Two cases had intracardiac structural defects including Tetralogy of Fallot and hypoplastic right heart syndrome. Three pregnancies were terminated, four cases delivered alive could not survive beyond the neonatal period. The striking feature in our study is its association with multiple pregnancies.

Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period. Herein, we report a 20-week-old fetus with a right aortic arch and an aberrant left subclavian artery with this rare combination. We report a case of a 20-week fetus diagnosed having this rare combination of right aortic arch and aberrant left subclavian artery. The APVS with complete atrioventricular septal defects may represent another type of APVS.

Prenatal Diagnosis, Associations and Outcome for Fetuses with Congenital Absence of the Pulmonary Valve Syndrome.

BACKGROUND: The aim of this study is to review the spectrum of the prenatally detected absent pulmonary valve syndrome and its outcome after diagnosis. METHODS: Clinical data and echocardiographic findings of 37 cases with a fetal diagnosis of absent pulmonary valve syndrome between 2008 and 2020 were analyzed in this retrospective multicenter study. RESULTS: Median gestational age at diagnosis was 25 weeks. Three subtypes of absent pulmonary valve syndrome were observed: (1) with tetralogy of Fallot (n=30; 81.0%); (2) absent pulmonary valve syndrome with intact ventricular septum (n=5; 13.5%); (3) with complete atrioventricular septal defect (n=2; 5.4%). In contrast to 7/25 fetuses (28%) with tetralogy of Fallot-absent pulmonary valve syndrome who had a patent ductus arteriosus, all 5 fetuses with absent pulmonary valve syndrome-intact ventricular septum had a patent ductus arteriosus (P < .001). No significant difference was found between the z-scores of pulmonary artery branches in fetuses with or without patent ductus arteriosus (P > .05). The analysis did not reveal any correlation between gestational week and z-scores of pulmonary artery, pulmonary artery branches (right pulmonary artery, left pulmonary artery), and ratio of aorta/pulmonary artery ratio. The echocardiographic measurements of survivors did not differ significantly from non-survivors (P > .05). Extracardiac anomalies were observed in 8/37 fetuses (21.6%). The incidence of extracardiac anomaly was significantly higher in cases of tetralogy of Fallot-absent pulmonary valve syndrome (P < .05). Overall, 9 fetuses (24%) had genetic anomalies. All 6 fetuses (20%) with 22q11.2 microdeletion were within the tetralogy of Fallot-absent pulmonary valve syndrome group. Overall survival after initial diagnosis in the total cases was 36.6% (11/30), with 9 of 30 (30%) tetralogy of Fallot-absent pulmonary valve syndrome cases and 2 of 5 (40%) absent pulmonary valve syndrome-intact ventricular septum cases. CONCLUSIONS: In this largest series of absent pulmonary valve syndrome, extracardiac, and chromosomal anomalies were found to be a common occurrence. The risk of 22q11.2 microdeletion was higher in tetralogy of Fallot cases at 40%. The sizes of the pulmonary artery and its branches and the aorta had no correlation of high mortality antenatally or after birth, which were 63.4% and 47.7%, respectively.

Prenatal Diagnosis of the Right Aortic Arch: Change in Detection Rate, the Status of Associated Anomalies, and Perinatal Outcomes in 137 Fetuses.

To evaluate prenatal findings of the right aortic arch (RAA), associated cardiac, extracardiac, and genetic anomalies, its perinatal outcomes and the need for postnatal interventions in cases of isolated RAA with a view to facilitating appropriate counseling. This was a multicenter, cohort study, that was undertaken in two international major cardiac centers between 2009 and 2020. The study subjects were prenatally diagnosed RAA cases with and without other structural cardiac defects. A RAA was identified in 137 fetuses. There were 84 cases of isolated RAA. Associations with additional intracardiac malformations were found in 53 (38.7%) cases. An extracardiac anomaly was observed in 26/137 (18.9%) fetuses, 11/84 (13.0%) fetuses with isolated RAA, and 15/53 (28.3%) fetuses with an additional intracardiac anomaly. The incidence of extracardiac and chromosomal anomalies was significantly higher in cases of RAA with abnormal intracardiac anatomy (28.3-18.8%, respectively), compared with RAA with normal intracardiac anatomy (13.0-5.9%, respectively) (p < 0.05). 22q11.2 microdeletion was found higher in RAA with CHD (4/18 fetuses) than isolated RAA (2/24 fetuses) (22.2% vs. 8.3% respectively). ALSA was present in 19.3% of cases. ALSA was more frequently observed in cases of isolated RAA (23.6%), than in RAA with structural CHD (7.6%) (p < 0.05). The pregnancy was interrupted in six fetuses, and one died in utero. The mortality rate was higher in fetuses with intracardiac anomaly than RAA without cardiac anomaly (11/49 (22.4%) vs. 2/81 (2.4%). Vascular ring formation was revealed in 21/98 cases. The RAA caused symptoms of a vascular ring in only one patient (0.7%) requiring surgery in the follow-up. Overall survival after initial diagnosis in the total cohort was 85.4% with 38 of 53 (71%) RAA with CHD cases and 79 of 84 (94.0%) isolated RAA cases. Chromosomal and extracardiac anomalies are lower in isolated RAA but not negligible hence amniocentesis should be routinely offered in all cases. The requirement for postnatal intervention in the immediate neonatal period is remote, therefore delivery of these fetuses need not be undertaken at a cardiac or surgical center.

Is strain echocardiography a more sensitive indicator of myocardial involvement in patients with multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2?

OBJECTIVE: The aim of the study was to evaluate the early myocardial dysfunction detected by strain echocardiography in children with multisystem inflammatory syndrome related to SARS-CoV-2 infection. METHODS: This cross-sectional study was conducted with 47 patients diagnosed with MIS-C and 32 healthy age- and gender-matched children. All patients underwent two-dimensional, colour, pulsed, and tissue Doppler, and 2D speckle tracking echocardiography examination at admission, 2 weeks, and 2 months after discharge. The MIS-C patient group was compared with the control group. Echocardiographic changes in MIS-C patients during follow-up were evaluated. RESULTS: Of 47 patients, 30 (63.8%) were male and 17 (36.2%) were female. The mean age at diagnosis was 9.1 ± 4.3 (1.25-17) years. At admission, 25 patients had abnormal findings on conventional echocardiography. Among them, eight patients had left ventricular systolic dysfunction. Ejection fraction and fractional shortening were significantly lower in the patient group at admission compared to controls (p = 0.013, p = 0.010, respectively). While the ejection fraction was <55% in eight patients, and global longitudinal strain was lower than -2SD in 29 patients at admission. Global longitudinal strain z-score <-2SD persisted in 13 patients at 2-month follow-up. Ejection fraction increased above 55% in 3.42 ± 0.53 days in 7 of 8 patients with left ventricular systolic dysfunction, ejection fraction was 51% at discharge in one patient, and left ventricular systolic dysfunction persisted at the 6-month of follow-up. CONCLUSION: These results confirmed that speckle tracking echocardiography is more likely to detect subclinical myocardial damage compared to conventional echocardiography. In addition, it is a valuable method for follow-up in this patient group.

CXCL10/IP10 as a Biomarker Linking Multisystem Inflammatory Syndrome and Left Ventricular Dysfunction in Children with SARS-CoV-2.

BACKGROUND: To investigate the diagnostic accuracy of CXCL10/IP10 for left ventricular (LV) dysfunction in multisystemic inflammatory syndrome (MIS-C). METHODS: This cross-sectional, longitudinal study included 36 patients with MIS-C. Patients were classified as follows: (1) patients presenting with Kawasaki-like features (group I = 11); (2) patients presenting with LV systolic dysfunction (group II = 9); and (3) other presentations (group III = 3). CXCL10/IP10 levels were measured upon admission and on days 3 and 7 of treatment. RESULTS: Twenty patients were male and 16 were female. The median age of patients at diagnosis was 7.5 (1.5-17) years. All patients had a fever lasting for a median of 4 (2-7) days. Ten patients had LV systolic dysfunction. The duration of hospitalization was longer in group II. Lymphocyte and platelet counts were lower, whereas NT-pro-BNP, troponin-I, D-dimer, and CXCL10/IP10 levels were higher in group II. Baseline levels of CXCL10/IP10 were weakly negatively correlated with ejection fraction (r = -0.387, p = 0.022). Receiver operator characteristic curve analysis yielded a cutoff value of CXCL10/IP10 to discriminate patients with LV dysfunction was 1839 pg/mL with sensitivity 88% and specificity 68% (Area under curve (AUC) = 0.827, 95% CI 0.682-0.972, p = 0.003). CONCLUSION: Having a good correlation with cardiac function, CXCL10/IP10 is a potential biomarker to predict LV dysfunction in MIS-C patients.

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.

OBJECTIVES: To describe fetal spectrum and echocardiographic characteristics of interrupted inferior vena cava (IIVC) with azygos/hemiazygous continuation without other structural heart defects and to evaluate its association with visceral heterotaxy and isomerism, extracardiac and genetic anomalies, and to review neonatal outcome. METHODS: This was a retrospective study of 14 fetuses with a confirmed diagnosis of IIVC with normal intracardiac anatomy. The following variables were collected; indication for referral, gestational age at diagnosis; associated isomerism and visceral heterotaxy, heart rhythm, genetic and extracardiac abnormalities, and fetal/neonatal outcome. RESULTS: Among 36 fetuses with IIVC, 14 cases (38.8%) had normal intracardiac anatomy. These IIVC cases correspond to 0.19% (14/7250) of all fetal cardiac examinations, and to 1.5% (14/922) of all cardiac abnormalities. Six patients had visceral abnormalities. Atrial appendage morphology was clearly depicted in three fetuses, both appendages were left. One fetus had bradyarrhythmia revealing atrial ectopic rhythm. Six fetuses did not have any concomitant cardiac or visceral abnormalities, therefore regarded as isolated. All babies were delivered at term with a good prognosis. CONCLUSION: Our study has shown that almost half of the IIVC cases without intracardiac structural anomalies displayed other findings of isomerism while the other half was isolated benign vascular variant. Therefore, prenatal diagnosis of IIVC should prompt a comprehensive evaluation for cardiac, situs, and visceral anomalies. The outcome is favorable.

Transplacental treatment of foetal supraventricular tachycardia with triple antiarrhythmic combination to avoid intra-foetal intervention.

Although a high percentage of foetuses with supraventricular tachycardia respond to single or dual antiarrhythmic therapy, on occasion when there is no response to these combination regimens, direct intra-foetal therapy remains the only choice, albeit such an approach carries a potential risk to the foetus.Data with regard to the safety and efficacy of triple antiarrhythmic combination have not been reported before. Here, we present a foetus with intractable tachycardia in whom arrhythmia termination was successfully achieved with triple oral antiarrhythmic therapy.

Multisystemic inflammatory syndrome in children associated with COVID-19: a single center experience in Turkey.

OBJECTIVE: Multisystem inflammatory syndrome in children (MIS-C) associated with the coronavirus disease 2019 (COVID-19) is a new concern emerging as a severe presentation of COVID-19 in children. We aimed to describe the characteristics and short-term outcomes of children diagnosed with MIS-C. MATERIAL AND METHODS: A retrospective study was conducted on 24 patients who were diagnosed with MIS-C between June 1, 2020 and December 1, 2020. A total of 24 (14 male and 10 female) patients were included in the study. RESULTS: The median age at the diagnosis was 111 (10-180) months. A total of 17 patients had a history of contact with a patient with COVID-19. Among the 24 patients, the most common findings were gastrointestinal involvement (n=20), followed by conjunctivitis (n=12), erythematous rash (n=11), and oral changes (n=10). Cardiovascular involvement was detected in 12 patients, of whom six had systolic dysfunction, four had mild coronary artery involvement, four had pericardial effusion, and three had mitral insufficiency. All patients received intravenous immunoglobulin, and 14 patients were treated with methylprednisolone in addition. Anti-interleukin-1 was given to two patients. The median duration of hospitalization was 8 (5-15) days. A total of 23 patients were discharged and evaluated on the median of 68.5 (52-140) days after discharge. The remaining one patient with dilated cardiomyopathy died after 2 months in the intensive care unit. CONCLUSION: Increasing the knowledge on MIS-C will provide clinicians with information on early recognition, evaluation, and management of these patients.

A prenatal diagnosis of unusual aortopulmonary communication: tubular aortopulmonary window.

We described a very rare case of aorto-pulmonary communication with right aortic arch and crossed pulmonary artery that cannot be placed in the typical anatomic classification of aortopulmonary window. At 23 weeks gestation, fetal echocardiography revealed a large tunnel-like communication connecting the great vessels proximal to the main pulmonary artery bifurcation, rather than a classic aortopulmonary window between the ascending aorta and the main pulmonary artery.

Is there myocardial involvement in children with long-term follow-up for Kawasaki disease? A study based on two-dimensional speckle tracking echocardiography.

OBJECTIVE: This study aimed to determine the possibility of subclinical myocardial dysfunction detected by strain echocardiography in the late period of children with Kawasaki disease. MATERIAL AND METHODS: The study enrolled 30 patients with Kawasaki disease with a follow-up period of at least 12 months and 30 healthy age- and gender-matched children. During the follow-up period, standard echocardiography, pulsed and tissue Doppler, and strain echocardiography were recorded for both groups. RESULTS: The mean age at the time of the diagnosis was 2.6±2.3 years (2 months-11 years). The mean follow-up period after the diagnosis was 3.55±2.20 years. Conventional echocardiography, M mode, pulsed and tissue Doppler values, and myocard performance index did not reveal significant differences. Left ventricle strain and strain rate parameters obtained by apical four-, three-, and two-chamber views did not show statistical differences between patients and controls. There was a positive correlation between the duration of follow-up and global four- and three-chamber longitudinal strain and global longitudinal strain values (r=0.465, p=0.010; r=0.414, p=0.023; r=0.492, p=0.006, respectively), whereas global radial strain showed negative correlation (r=-0.517, p=0.003). CONCLUSION: The analysis of systolic strain and strain rate did not detect a subclinical myocardial dysfunction in the long-term follow-up of Kawasaki disease. However, strain values showed variability with the follow-up periods, which indicates that Kawasaki disease might cause left ventricular dysfunction in the later phases. Therefore, a follow-up of children with a diagnosis of Kawasaki disease is of capital importance.

Myeloid sarcoma of the heart: Extramedullary relapse of acute myeloblastic leukemia, presenting with complete heart block and atrial flutter after second allogeneic stem cell transplantation.

We report isolated extramedullary relapse in a 14-year-old boy, sequentially presenting with intestinal and cardiac myeloid sarcoma (MS). Acute myeloblastic leukemia M5 was diagnosed 41 months ago. On the 14th month of the second HSCT, he presented with ileus and underwent surgical treatment. After 2 weeks, arrhythmia, bradycardia, complete heart block, and atrial flutter developed and echocardiography revealed multiple cardiac masses. There was no bone marrow relapse but pathology of the intestinal biopsy showed leukemic infiltration. Patient was successfully treated with a permanent pacemaker and salvage chemotherapy. To the best of our knowledge, this is the first pediatric cardiac MS developed after HSCT.

Evaluation of Early Repolarization Pattern in Male Teenage Competitive Athletes and Association With Left Ventricular Remodeling.

OBJECTIVE: Early repolarization pattern (ERP) on electrocardiogram is more common among young athletes than in the general population, and has been considered a benign finding. However, ERP has been associated with increased risk of sudden cardiac death. The objectives of this study were to evaluate ERP in teenage athletes; investigate associations between ERP and echocardiographic findings of the left ventricle (LV); and to describe the impact of different sports disciplines on ERP. METHODS: ERP was assessed in male teenage athletes from sports institutions for 5 different types of sport--basketball, swimming, football, wrestling, and tennis. All had been training for at least 3 hours per week for over at least 2 years. ERP was defined as J-point elevation ≥ 1 mV in 2 contiguous and/or lateral leads. A conventional echocardiography was performed in all athletes. RESULTS: ERP was evaluated in 159 athletes with a mean age of 14 (range 10-18 years). It was more common in those training with combined exercise. There was no association between ERP and echocardiographic findings of left ventricular remodeling and geometric pattern. CONCLUSION: ERP is a frequent finding among teenage athletes. However, frequency varies by sports type, being more common in those training with combined exercise. It is not associated with structural echocardiographic alterations and is primarily seen as an electrophysiological change.

A Study of Mortality in Cardiac Patients in a Pediatric Intensive Care Unit.

OBJECTIVE: One of the major causes of mortality in the pediatric intensive care unit (PICU) is heart disease. This study aimed to determine the causes of mortality in children with pre-existing cardiac abnormalities who were admitted into the PICU. METHODS: Data were collected through patient profile assessment and outcome and heart diseases affecting prognosis were analyzed. Medical records of children were reviewed retrospectively. The updated Pediatric Index of Mortality 2 (PIM2) scores were used. Exploratory data analysis was performed using descriptive measures. Kolmogorov-Smirnov tests were used to test the normality of data distribution. RESULTS: Out of 566 admissions into PICU, 76 (13.4%) had cardiac abnormalities. Median and range of PICU stay were 5.50 and 417.88 days. The mean PIM2 score on admission was found to be 31.05. The most common admission was due to atrioventricular septal defect (AVSD) (15.7%), cardiomyopathy (13.1%), ventricular septal defect (VSD) (11.8%), tetralogy of Fallot (10.5%) and others (48.9%). There were multiple cardiac anomalies in 3.9% of patients. The most important cause of cardiac mortality in PICU was septic shock (26.0%) followed by cardiogenic shock (20.6%), and cardiac failure (13.7%). The nosocomial infection rate of cardiac patients in PICU was 10.5%. CONCLUSIONS: Our study reconfirmed that the PIM2 score is a good indicator of cardiac diseases. Infections, nosocomial infections, pneumonia, and septic shock were the leading causes of mortality in cardiac patients. Better infection control in the PICU may have a significant impact on decreasing mortality rates.