Cardiac involvement and its clinical significance in patients with anorexia nervosa.

Cardiac complications are a major concern in patients with anorexia nervosa (AN) which contribute to morbidity and mortality. However, limited information exists regarding risk factors for the development of these complications. Our objective was to investigate the prevalence and associated risk factors of cardiac involvement among children and adolescents with AN admitted to a tertiary pediatric hospital. We collected demographic, clinical, and laboratory data from individuals with AN hospitalized between 2011 and 2020 in Schneider Children's Medical Center in Israel. Diagnosis was based on established criteria (DSM-5). Patients with other co-morbidities were excluded. Cardiac investigations included electrocardiograms (ECG) and echocardiograms. We conducted correlation tests between cardiac findings and clinical and laboratory indicators. A total of 403 AN patients (81.4% were females) with a median age of 15 ± 2 years were included in the study. Sinus bradycardia was the most common abnormality, observed in 155 (38%) participants. Echocardiogram was performed in 170 (42.2%) patients, of whom 37 (22%) demonstrated mild cardiac aberrations. Among those aberrations, 94.6% could be attributed to the current metabolic state, including pericardial effusion (15.3%) and valve dysfunction (8.8%). Systolic or diastolic cardiac dysfunction, tachyarrhythmias, or conduction disorders were not observed. Patients with new echocardiographic aberration had significantly lower body mass index (BMI) at admission, and the prevalence of amenorrhea and hypotension was higher in this group. CONCLUSIONS: The prevalence of cardiac involvement, except for sinus bradycardia, was notably low in our cohort. The presence of cardiac aberrations is correlated with several clinical variables: lower body mass index (BMI) and the presence of amenorrhea and hypotension at admission. Patients presenting with these variables may be at high risk for cardiac findings per echocardiography. Dividing the patients into high and low risk groups may enable targeted evaluation, while avoiding unnecessary cardiac investigations in low-risk patients. WHAT IS KNOWN: • Cardiac involvement in anorexia nervosa (AN) patients is a major concern, which contributes to morbidity and mortality. • It is unknown which patients are prone to develop this complication. WHAT IS NEW: • Cardiac complications in our cohort are less frequent compared to previous studies, and it is correlated with lower body mass index (BMI) at admission, and the prevalence of amenorrhea and hypotension.

Three-Patch Reconstruction of Hourglass Supravalvar Pulmonary Artery Stenosis Mid-Term Results.

BACKGROUND: Some patients with pulmonary stenosis present with a complex multilevel disease that involves the valve, the leaflets, and the sinotubular junction (STJ) forming an hourglass appearance. We herein report the mid-term results of our experience with the reconstruction of the supravalvar narrowing using three pericardial patches. MATERIAL AND METHODS: Retrospective analysis of patient charts and echocardiography studies of patients who underwent three-patch reconstruction of the pulmonary valve (PV) from 2013 to 2022. After PV transection distal to STJ, vertical incisions into the sinuses were performed, and leaflets were trimmed and thinned. The three sinuses were augmented using three pericardial patches. RESULTS: Nineteen patients underwent repair of hourglass supravalvar pulmonary stenosis. Mean weight at surgery was 9.4 kg (median 7.2, range 4.7-35); 16 patients underwent previous catheterization with unsuccessful balloon dilatation of the PV (13 pts.). Preoperative aortic/pulmonary annulus ratio was 1.02 (median 1, range 0.89-1.25). After surgery, gradients across the PV were significantly reduced (94 ± 26 vs 29 ± 9 mm Hg, P = .02). Postoperatively, 14 patients had mild or no pulmonary insufficiency (PI) and five had mild to moderate PI. At a mean follow-up of 71 months (median 78 months, range 8-137), gradients continued to decrease (29 ± 9 vs 15 ± 5 mm Hg, P < .001). CONCLUSION: The three-patch technique for the repair of supravalvar pulmonary stenosis is simple, reproducible, and achieves excellent and long-standing relief of the right ventricular outflow tract gradient.

The Amplatzer duct occluder (ADOII) and Piccolo devices for patent ductus arteriosus closure: a large single institution series.

Purpose: Evaluate Piccolo and ADOII devices for transcatheter patent ductus arteriosus (PDA) closure. Piccolo has smaller retention discs reducing risk of flow disturbance but residual leak and embolization risk may increase. Methods: Retrospective review of all patients undergoing PDA closure with an Amplatzer device between January 2008 and April 2022 in our institution. Data from the procedure and 6 months follow-up were collected. Results: 762 patients, median age 2.6 years (range 0-46.7) years and median weight 13 kg (range 3.5-92) were referred for PDA closure. Overall, 758 (99.5%) had successful implantation: 296 (38.8%) with ADOII, 418 (54.8%) with Piccolo, and 44 (5.8%) with AVPII. The ADOII patients were smaller than the Piccolo patients (15.8 vs. 20.5 kg, p < 0.001) and with larger PDA diameters (2.3 vs. 1.9 mm, p < 0.001). Mean device diameter was similar for both groups. Closure rate at follow-up was similar for all devices ADOII 295/296 (99.6%), Piccolo 417/418 (99.7%), and AVPII 44/44 (100%). Four intraprocedural embolizations occurred during the study time period: two ADOII and two Piccolo. Following retrieval the PDA was closed with an AVPII in two cases, ADOI in one case and with surgery in the fourth case. Mild stenosis of the left pulmonary artery (LPA) occurred in three patients with ADOII devices (1%) and one patient with Piccolo device (0.2%). Severe LPA stenosis occurred in one patient with ADOII (0.3%) and one with AVPII device (2.2%). Conclusions: ADOII and Piccolo are safe and effective for PDA closure with a tendency to less LPA stenosis with Piccolo. There were no cases of aortic coarctation related to a PDA device in this study.

Long COVID in Children: Observations From a Designated Pediatric Clinic.

Systematic data are lacking on pediatric long COVID. This study prospectively assessed 90 children with persistent symptoms who presented to a designated multidisciplinary clinic for long COVID. In nearly 60%, symptoms were associated with functional impairment at 1-7 months after the onset of infection. A comprehensive structured evaluation revealed mild abnormal findings in approximately half the patients, mainly in the respiratory aspect.

Peripheral Venous Catheter-related Bloodstream Infections in Hospitalized Children: The Role of Gram-negative Bacteria.

BACKGROUND: Peripheral venous catheter (PVC) is the most used vascular access device in medicine, allowing administration of intravenous fluids and medications. Known complications associated with PVC include extravasation, phlebitis and rarely bloodstream infection (BSI). Data regarding PVC-related BSI in children are lacking. Our aim was to evaluate the epidemiology, clinical and microbiologic characteristics of pediatric inpatients with PVC-related BSI. METHODS: A retrospective study was conducted in a pediatric tertiary care center. Children with BSI, admitted to general pediatric departments during 2010-2019, were identified and their medical records examined. Patients with BSI and phlebitis were further characterized and included in the analysis. We excluded patients with central venous catheters, other identified source of infection and with BSI upon admission. Data collected included patients' demographics and clinical and microbiologic characteristics. RESULTS: Twenty-seven children with PVC-related BSI were identified and included in the study, consisting of 0.2% of the total BSI cases. Patient's median age was 24 (range, 1.5-213) months, 14/27 (52%) were female and 6 (22%) were previously healthy while 21 (78%) had prior medical conditions. Sixteen (59.3%) patients had Gram-negative BSI and 6 (22.2%) Gram-positive bacteria. Polymicrobial infection occurred in 4 (14.8%) patients and Candida albicans in 1 (3.7%) patient. The most common isolated bacteria were Klebsiella spp and Staphylococcus aureus. Longer dwell-time was a predictor of Gram-negative bacteria. CONCLUSIONS: PVC-related BSI due to Gram-negative bacteria was more common than to Gram-positive bacteria. Clinicians should consider an initial broad-spectrum antibiotic coverage for PVC-related BSI in hospitalized pediatric patients.

Transient Cardiac Injury in Adolescents Receiving the BNT162b2 mRNA COVID-19 Vaccine.

BACKGROUND: Vaccines are paramount in the effort to end the coronavirus disease 2019 global epidemic. BNT162b2 is approved for the vaccination of adolescents over 16 years of age. Systemic adverse events were scarce though the pretested cohort of this age group was relatively small. The aim of the current study is to raise awareness for potential adverse reactions. METHODS: This is a case series of patients diagnosed with perimyocarditis following vaccination. Patients were compiled from 3 pediatric medical centers in Israel through a network of pediatricians and data regarding those cases was collected. In addition, incidence of perimyocarditis during the vaccination period was compared with previous years. RESULTS: All patients were males 16-18 years old, of Jewish descent, who presented with chest pain that began 1-3 days following vaccination (mean, 2.1 days). In 6 of the 7 patients, symptoms began following the 2nd dose and in 1 patient following the 1st dose. All cases were mild and none required cardiovascular or respiratory support. The incidence of perimyocarditis during the vaccination period was elevated in comparison to previous years. CONCLUSIONS: This case series describes a time association between coronavirus disease 2019 vaccine and perimyocarditis in adolescents. All cases were mild, although only long-term follow-up can reveal the true impact of this cardiac injury. While it seems that the incidence of perimyocarditis during the vaccination campaign period is increased, a more comprehensive data collection on a wider scale should be done. We hope this report will serve as a reminder to report events and allow for analysis of potential adverse reactions.

Clinical Features and Comparison of Kingella and Non-Kingella Endocarditis in Children, Israel.

Kingella spp. have emerged as an important cause of invasive pediatric diseases. Data on Kingella infective endocarditis (KIE) in children are scarce. We compared the clinical features of pediatric KIE cases with those of Streptococcus species IE (StIE) and Staphylococcus aureus IE (SaIE). A total of 60 patients were included in the study. Throughout the study period, a rise in incidence of KIE was noted. KIE patients were significantly younger than those with StIE and SaIE, were predominately boys, and had higher temperature at admission, history of oral aphthae before IE diagnosis, and higher lymphocyte count (p<0.05). Pediatric KIE exhibits unique features compared with StIE and SaIE. Therefore, in young healthy children <36 months of age, especially boys, with or without a congenital heart defect, with a recent history of oral aphthae, and experiencing signs and symptoms compatible with endocarditis, Kingella should be suspected as the causative pathogen.

Hospitalization for Respiratory Syncytial Virus Bronchiolitis in the Palivizumab Prophylaxis Era: Need for Reconsideration of Preventive Timing and Eligibility.

BACKGROUND: Respiratory syncytial virus (RSV)-related bronchiolitis is a common cause of morbidity in young infants. The recommendations for its passive prevention by palivizumab are currently under intensive debate. OBJECTIVES: To elucidate the optimal prevention strategy by studying the morbidity of RSV disease under the current recommendations for palivizumab prophylaxis in Israel. METHODS: We collected demographic and clinical data of all children hospitalized with microbiologically confirmed RSV bronchiolitis during 2015-2016 at Schneider Children's Medical Center. The seasonality of RSV disease was also studied for the period 2010-2017 in sentinel clinics scattered throughout Israel. RESULTS: Of the 426 hospitalized children, 106 (25%) had underlying diseases but were not eligible for palivizumab prophylaxis according to the current criteria in Israel. Their course was severe, with a mean hospital stay of 6.7 days and a 12% admission rate to the pediatric intensive care unit (PICU). Palivizumab-eligible children who did not receive the prophylaxis before hospitalization had the most severe course, with 22% admitted to the PICU. More children were diagnosed with RSV disease in October than in March among both hospitalized and ambulatory children; 44% of the palivizumab-eligible hospitalized children were admitted in the last 2 weeks of October, before 1 November which is the recommended date for starting palivizumab administration in Israel. CONCLUSIONS: According to the results of the present study we suggest advancing RSV prophylaxis in Israel from 1 November to mid-October. The precise palivizumab-eligible categories should be reconsidered.

Urgent Surgical Treatment of Aortic Endocarditis in Infants and Children.

Infective endocarditis (IE) in the pediatric population can present as a life-threatening condition. Optimal timing for surgical intervention should consider surgical risks versus the risk of neurologic complications. We herein report our experience with this group of critically ill children. Retrospective analysis of patient charts of all patients who underwent urgent surgical treatment of aortic IE from 1994 to 2014 was performed. Nine patients with acute storming IE of the aortic valve or the ascending aorta were urgently operated (eight normal heart, one congenital aortic stenosis), age ranged from 8 weeks to 4.2 years. Causative organisms were Staphylococcus aureus (2), Staphylococcus coagulase negative (1), Kingella kingae (2), Streptococcus pneumoniae (2), or culture negative (2). Presenting symptoms other than hemodynamic instability were neurologic decompensation (5) coronary embolization (1) and cardiogenic shock due to scalded skin syndrome (1). CT and MRI demonstrated significant brain infarcts in four patients. Operations performed were the Ross operation (7) and ascending aortic reconstruction (2). There were no operative deaths. At a median follow-up of 6 years (range 2-196 months), all patients are alive and well. Re-intervention included homograft replacement (2) and transcatheter Melody valve implantation (1). At their last follow-up, the neo-aortic valve was functional in all patients with minimal regurgitation and all had full resolution of the neurological deficits. Urgent surgical treatment for aortic valve IE in infants is challenging. Although surgery is complex and pre-disposing conditions such as sepsis, neurologic and cardiac decompensations are prevalent, operative results are excellent and gradual and significant neurologic improvement was noted over time.

Plasma microvesicle analysis identifies microRNA 129-5p as a biomarker of heart failure in univentricular heart disease.

Biomarkers of heart failure in adults have been extensively studied. However, biomarkers to monitor the progression of heart failure in children with univentricular physiology are less well understood. We proposed that as mediators of diverse pathophysiology, miRNAs contained within circulating microvesicles could serve as biomarkers for the presence and progression of heart failure in univentricular patients. To test this, we studied the association of heart failure with elevations in specific miRNAs isolated from circulating microvesicles in a cohort of children with univentricular heart disease and heart failure. We conducted a single site cross-sectional observational study of 71 children aged 1 month-7 years with univentricular heart disease and heart failure. We demonstrated that levels of miR129-5p isolated from plasma microvesicles were inversely related to the degree of clinical heart failure as assessed by Ross score. We then showed that miR129-5p levels are downregulated in HL1 cells and human embryonic stem cell-derived cardiomyocytes exposed to oxidative stress. We demonstrated that bone morphogenetic protein receptor 2, which has been implicated in the development of pulmonary vascular disease, is a target of miR129-5p, and conversely regulated in response to oxidative stress in cell culture. Levels of miR129-5p were inversely related to the degree of clinical heart failure in patients with univentricular heart disease. This study demonstrates that miR129-5p is a sensitive and specific biomarker for heart failure in univentricular heart disease independent of ventricular morphology or stage of palliation. Further study is warranted to understand the targets affected by miR129-5p with the development of heart failure in patients with univentricular physiology.

Neonatal Cardiac Surgery in the New Era: Lessons learned from 1000 Consecutive Cases.

BACKGROUND: neonatal cardiac surgery has evolved over the last 50 years with a large percentage of the patients achieving complete physiological repair in the neonatal period. The remaining patients achieve staged palliation with an increasing amount of success. OBJECTIVES: To report our experience with 1000 neonatal cardiac surgical procedures performed in the last 10 years. METHODS: We conducted a retrospective analysis of surgical outcome in all neonatal patients who underwent cardiac surgery between January 2007 and July 2016 at Schneider Children's Medical Center of Israel. RESULTS: A total of 1003 neonates aged < 30 days underwent surgery for congenital heart defects at our center. Neonatal surgery accounted for 22.5% of all cardiac surgeries. Neonatal operative mortality was 7.3%, Operative mortality for individual lesions were: simple aortic coarctation (CoA) (198 patients, 2.5%), CoA with hypoplastic arch (24, 4%), CoA with ventricular septal defect (VSD) (84, 2.3%), transposition of the great arteries (TGA, simple and complex, 185, 6.3%), TGA with VSD (37, 0%), truncus arteriosus (26, 3.8%), interrupted aortic arch (25, 4%), Norwood Sano (71, 19.7%), neonatal tetralogy of Fallot (41, 0%), and shunt (131 patients, 12%). CONCLUSIONS: Neonatal surgical capabilities have improved substantially over the last decades. Excellent results can be expected for lesions that can be repaired to create biventricular circulation. Improved results can be attributed in part to the evolution of surgical strategies and assistive technologies, but essential is the collaborative effort of surgeons, cardiologists, anesthesiologists, and intensive care specialists acting as a cohesive team whose performance far exceeds the sum of its individual members' contributions.

Extremely elevated C-reactive protein levels are associated with unfavourable outcomes, including death, in paediatric patients.

AIM: The aim of this study was to investigate the clinical significance of extremely elevated C-reactive protein (CRP) levels in hospitalised children. METHODS: We searched the electronic database of a tertiary paediatric medical centre for all patients admitted in 2010-2013 with a CRP of ≥ 30 mg/dL, and these comprised the study group. The controls were the other admissions. Data were collected on demographics, admission details, pre-existing conditions, discharge diagnosis, laboratory results and clinical outcomes. RESULTS: Our study group comprised 435 (0.72%) of the 59,997 patients hospitalised during the study period. The mortality rate and mean hospital stays were significantly higher in the study group, and infectious diseases were the most common diagnoses, affecting 389 patients (89.4%), particularly pneumonia (47.1%). Higher CRP was correlated with low albumin levels (p < 0.01). Bacteraemia was the most prevalent diagnosis (38%) in the 84 oncology patients, with a crude mortality rate of 17.6%. CONCLUSION: Infectious diseases, mainly bacterial, were the most common diagnoses in previously healthy children with CRP ≥ 30 mg/dL. Extremely elevated CRP levels were associated with an unfavourable clinical outcome, including high mortality, particularly in oncology patients. Paediatricians should be aware of the significance of extremely elevated CRP levels.

Feasibility of neonatal pulse wave velocity and association with maternal hemoglobin A1c.

BACKGROUND: Use of non-invasive peripheral arterial tonometry to assess arterial stiffness has not been studied in neonates. Perinatal factors impact childhood vascular health, but the effect in neonates remains to be examined. OBJECTIVES: To examine the feasibility of pulse wave velocity (PWV) among healthy term neonates, and to evaluate the effects of perinatal factors on neonatal PWV. METHODS: Pregnant women with singleton gestation presenting for routine care were enrolled. Postnatally, PWV measurements of their neonates were obtained using an arterial tonometer. A variability index was calculated for each PWV measurement. Intra- and inter-observer reproducibility were illustrated with Bland-Altman plots. Medical records were reviewed. Relationships between neonatal PWV and perinatal factors were examined. RESULTS: PWV measurements were attempted in 76 neonates and successfully obtained in 67 (88%). Using PWV measurements with a variability index ≤ 0.25 (48 neonates), the intra-class coefficient was 0.69. The mean differences (limits of agreement) for intra- and inter-rater reproducibility were 0.02 (-3.64 to 3.60) and 0.34 (-2.23 to 2.39), respectively. Median neonatal PWV was 2.80 m/s (range 0.60-8.40). Neonates of mothers with HgbA1c ≥6% had significantly higher PWV than neonates of mothers with HgbA1c <6% (4.12 m/s, 95% CI 3.22-5.02, vs. 2.78 m/s, 95% CI 2.28-3.28, p = 0.02). CONCLUSIONS: Neonatal PWV using peripheral arterial tonometry is feasible and reproducible when using measurements with a variability index ≤ 0.25. Neonates of mothers with increased HgbA1c had higher PWV, suggesting an effect of maternal hyperglycemia on neonatal vasculature. The long-term implications of this finding warrant further investigation.

Ebstein anomaly and Trisomy 21: A rare association.

This is a case report of a patient with Trisomy 21 with Ebstein anomaly, a ventricular septal defect, and acquired pulmonary vein stenosis; a rare combination, diagnosed during a routine neonatal examination.

Arterial applanation tonometry: feasibility and reproducibility in children and adolescents.

BACKGROUND: Aortic pulse wave velocity (PWV) and augmentation index (AIx) are markers of vascular health and have recently been used in pediatric clinical trials. However, there are limited data on standardization of these measurements in pediatrics. The objective of this study was to prospectively test the feasibility and reproducibility of PWV and AIx in children and adolescents. METHODS: We performed arterial tonometry on 2 different days within 2 weeks in 40 healthy subjects aged 10-19 years. PWV and AIx were measured in triplicate on each visit. RESULTS: The visits were separated by a mean of 3.08±3.7 days. We obtained PWV in 77 of 80 (96%) visits and AIx in 76 of 80 (95%) visits in triplicate. Intraclass correlation coefficients (ICCs) for PWV were 0.61 (95% confidence interval (CI) = 0-0.86) when at least 2 measurements and 0.92 (95% CI = 0-1) when 3 measurements were obtained at each visit that met the quality criteria established for adults by the manufacturer (n = 17 and 3 paired visits, respectively). For AIx, ICCs were 0.78 (95% CI = 0.58-0.88) and 0.81 (95% CI = 0.63-0.90) when measurements with an operator index ≥80, a measure of the quality of the waveform, were included (n = 39 and 36 paired visits, respectively). CONCLUSIONS: Arterial applanation tonometry is feasible and reproducible in healthy children and adolescents. AIx has excellent intervisit reproducibility, whereas the intervisit reproducibility of PWV relies on acquisition of multiple measurements that meet quality criteria established for adults. These results have implications for the methodology of future pediatric clinical trials in a population at increasingly higher risk for premature atherosclerosis.

The value of noninvasive measurement of the compensatory reserve index in monitoring and triage of patients experiencing minimal blood loss.

Currently available triage and monitoring tools are often late to detect life-threatening clinically significant physiological aberrations and provide limited data in prioritizing bleeding patients for treatment and evacuation. The Compensatory Reserve Index (CRI) is a novel means of assessing physiologic reserve, shown to correlate with central blood volume loss under laboratory conditions. The purpose of this study was to compare the noninvasive CRI device with currently available vital signs in detecting blood loss. Study subjects were soldiers volunteering for blood donation (n = 230), and the control group was composed of soldiers who did not donate blood (n = 34). Data collected before and after blood donation were compared, receiver operator characteristic curves were generated after either donation or the appropriate time interval, and areas under the curves (AUCs) were compared. Compared with pre-blood loss, blood donation resulted in a mean reduction of systolic blood pressure by 3% (before, 123 mmHg; after, 119 mmHg; P < 0.01). The CRI demonstrated a 16% reduction (before, 0.74; after, 0.62; P < 0.01). Heart rate, diastolic blood pressure, and oxygen saturation remained unchanged. The AUC for change in CRI was 0.81, 0.56 for change in heart rate, 0.53 for change in systolic blood pressure, 0.55 and 0.58 for pulse pressure and shock index, respectively. The AUCs for detecting mild blood loss at a single measurement were 0.73 for heart rate, 0.60 for systolic blood pressure, 0.62 for diastolic blood pressure, 0.45 for pulse oximetry, and 0.84 for CRI. The CRI was better than standard indices in detecting mild blood loss. Single measurement of CRI may enable a more accurate triage, and CRI monitoring may allow for earlier detection of casualty deterioration.

Prenatal tricuspid valve size as a predictor of postnatal outcome in patients with severe pulmonary stenosis or pulmonary atresia with intact ventricular septum.

INTRODUCTION: Tricuspid valve (TV) size at birth correlates with intervention strategy in patients with severe pulmonary stenosis (SPS) or pulmonary atresia/intact ventricular septum (PA/IVS). Prenatal features that might predict postnatal TV size have not been well studied. We hypothesized that prenatal echocardiographic measurements predict the postnatal TV Z-score in fetuses with SPS and PA/IVS. MATERIALS AND METHODS: We identified 16 neonates (gestational age 28 ± 4.8 weeks) with a fetal diagnosis of SPS or PA/IVS from 2001 to 2010. Measurements were performed offline. ROC (receiver operating characteristic) analysis was used to generate AUC (areas under the curve) for each of the variables. RESULTS: AUC was 0.94 for tricuspid to mitral valve (TV/MV) ratio, 0.88 for TV Z-score, and 0.85 for TV inflow duration. A cut-off value of >0.63 for TV/MV yielded a sensitivity of 78%, specificity of 100% for predicting postnatal TV Z-score >-3. Neonates with TV Z-score ≥-3 and all fetuses with antegrade flow across the pulmonary valve or more than moderate tricuspid regurgitation had biventricular circulation in follow-up. CONCLUSION: Fetal TV/MV >0.63 predicts favorable TV Z-score at birth in patients with SPS and PA/IVS. Antegrade pulmonary valve flow and more than moderate tricuspid regurgitation also conferred a favorable outcome.

Expanding the phenotype of cardiovascular malformations in Adams-Oliver syndrome.

We describe a newborn with a phenotype consistent with Adams-Oliver syndrome and truncus arteriosus. Although cardiovascular malformations associated with this syndrome have been previously published in the literature, this is the first description of truncus arteriosus in a patient with Adams-Oliver syndrome. We review other reports of Adams-Oliver syndrome previously described with cardiovascular malformations, consider possible genetic and embryologic mechanisms, and emphasize the need for cardiology consultation when a diagnosis of Adams-Oliver syndrome is suspected in the differential diagnosis.

Echocardiographic predictors of early postsurgical myocardial dysfunction in pediatric patients with aortic valve insufficiency.

In chronic aortic insufficiency (AI), left-ventricular (LV) dysfunction must be detected early to allow timely surgery. Strain and strain rate have been used for this purpose in adults, but the value of this method in pediatric AI has not been established. Forty patients with moderate to severe AI were included in this retrospective study. LV function was assessed by strain analysis and conventional echocardiography both before and after surgery. Of the 32 patients with preserved preoperative ejection fraction (EF; >50 %), 8 had postoperative dysfunction (<50 %). Mean conventional indices of global LV systolic performance for the entire cohort of patients with AI were predominantly in the normal range before surgery. Preoperative values for LV global longitudinal strain (GLS) and strain rate (GLSr) were normal. After surgery, there was a significant decrease in shortening and EF. There was a significant decrease from preoperative to postoperative values for both GLS (-16.07 ± 3.82 vs. -11.06 ± 3.88; p < 0.0001) and GLSr (-0.89 ± 0.24 vs. -0.72 ± 0.27; p = 0.0021). A preoperative GLS of -15.3 (AUC = 0.83, CI = 0.69-0.98, p < 0.0001) and a GLSr of -0.79/s (AUC = 0.86, CI = 0.73-0.98, p < 0.0001) were determined to be predictors of early postoperative dysfunction after surgical repair of moderate to severe AI. A preoperative GLS value of ≤-15.3 and GLSr value of -0.79/s or less are predictors of postoperative ventricular dysfunction, which is defined by EF <50 %. GLS and GLSr value determination may be useful as part of the echocardiographic assessment AI and may help determine the optimal timing of surgery in pediatric patient with at least moderate AI.