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We report a series of 13 consecutive patients diagnosed in utero with heterotaxy syndrome in which we found a slight predominance (8 of 13) of fetuses with right atrial isomerism. In previous studies in which diagnosis of left or right isomerism was made based upon findings at fetal echocardiography, there has been a preponderance of infants (95%) diagnosed with left isomerism. [ABSTRACT FROM AUTHOR]

Influenza can lead to significant morbidity and mortality in children. Aortopathy tends to occur in adults during influenza season; however, aortic pathology in children with influenza is exceedingly rare. This report describes a child who experienced an aortic rupture during an influenza infection without evidence of secondary bacterial infection or connective tissue disorder., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

In post-operative Tetralogy of Fallot (TOF) patients cMRI data are used for determining right ventricle (RV) dilation to justify the pulmonary valve placement. Echocardiogram analysis of RV volumes has inherent accuracy issues. There are no data exploring the relationship of transthoracic echocardiography tricuspid valve (TV) annulus size and cMRI RV dilation. Retrospective study in post-operative TOF patients. Patients less than 21 years old and with high-quality transthoracic echocardiogram and MRI within 6 months of each other were included. We collected demographic data, type of initial repair, echocardiographic, and cMRI data. A single reviewer measured echocardiogram TV size. Boston z-scores were used. 77 echocardiogram and cMRI pairs were analyzed. Average age was 14 years old. There is not a strong linear relationship between TV z-score and indexed right ventricular end diastolic volume (RVEDVi) (R 2  = 0.31). Twenty patients had RVEDVi's of greater than 150 ml/m 2 . From those 20 patients, 9 patients had a TV z-score of > 2. No patient had a TV z-score > 2 and a normal RVEDVi. For a TV z-score < 2, there was a positive predictive value of 100%, a negative predictive value of 83%, specificity of 100%, and sensitivity of 45%. In our study population, the TV z-score is not sufficient to predict RV dilation. However, TV z-score > 2 has a high positive predictive value for an RVEDVi of > 150 ml/m 2 . This would suggest that a cMRI should be done if the TV z-score is > 2 on echocardiography., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Heritable connective tissue disorders are a group of diseases, each rare, characterized by various combinations of skin, joint, musculoskeletal, organ, and vascular involvement. Although kidney abnormalities have been reported in some connective tissue disorders, they are rarely a presenting feature. Here we present three patients with prominent kidney phenotypes who were found by whole exome sequencing to have variants in established connective tissue genes associated with Loeys-Dietz syndrome and congenital contractural arachnodactyly. These cases highlight the importance of considering connective tissue disease in children presenting with structural kidney disease and also serves to expand the phenotype of Loeys-Dietz syndrome and possibly congenital contractural arachnodactyly to include cystic kidney disease and cystic kidney dysplasia, respectively., (© 2021 Wiley Periodicals LLC.)

Background: Echo assessment of right ventricular function is difficult due to its complex geometry and regional differences in wall motion. In patients with repaired tetralogy of Fallot, cardiac MRI is the gold standard for assessment of right ventricular function. There is scant data regarding the correlation of echo measures of right ventricular function with cardiac MRI right ventricular function in the paediatric population., Methods: Echocardiographic measures of regional and global right ventricular function were reviewed in 56 patients with repaired tetralogy of Fallot and 27 patients with structurally normal hearts. These measures were compared to global right ventricular ejection fraction by cardiac MRI., Results: Despite having the same right ventricular ejection fraction by cardiac MRI as normal controls, patients with repaired tetralogy of Fallot had significantly worse global and regional echocardiographic measures of right ventricular function. Right ventricular fractional area change and fractional shortening of the right ventricular outflow tract correlated best with global right ventricular function by MRI. Incorporation of these measures into a regression model allowed accurate prediction of global right ventricular function by cardiac MRI., Conclusion: Children with repaired tetralogy of Fallot have abnormal regional and global right ventricular function compared to patients with normal hearts. Despite the presence of patch material, the right ventricular outflow tract significantly contributes to global right ventricular function by cardiac MRI. When incorporated into a model that includes regional and global echocardiographic measures of right ventricular function, right ventricular outflow tract fractional shortening helps predict global right ventricular function by MRI.

Objective: Echocardiographic parameters assessing left and right heart function were evaluated in children with established pulmonary hypertension (PH) from bronchopulmonary dysplasia (BPD) to look for correlations with each other, and pulmonary artery pressure (PAPs) from right heart catheterizations (RHC)., Study Design: Data were retrospectively collected on patients with BPD and PH and correlations were performed between various objective echocardiographic and RHC measurements., Results: A total of 31 patients with BPD were found to have PH by echocardiogram and RHC after chart review. Median age of evaluation was 0.58 years. Correlations were noted between measurements of right heart function, indirect measures of pulmonary artery pressures and left ventricular dimensions. A trend was noted between the tricuspid annular plane systolic excursion obtained at echocardiography and systolic pulmonary artery pressure, obtained during RHC., Conclusion: Significant correlations were found between objective echocardiographic measurements of left and right heart function, in patients with PH from BPD.

A cardiovascular comorbidity in obese adolescents is increased aortic pulse wave velocity (aPWV), carotid intima-media thickness (cIMT) and left ventricular mass (LVM). We investigated in obese adolescents 1) the risk factors associated with aPWV, cIMT and LVM, and 2) the effects of aerobic (AE) versus resistance (RE) exercise alone (without calorie restriction) on aPWV, cIMT, LVM index (LVMI) and cardiometabolic risk factors. Eighty-one obese adolescents (12-18 yrs, BMI ≥95th percentile) were randomized to 3 months of AE (n = 30), RE (n = 27) or a control group (n = 24). Outcome measures included aPWV, cIMT, LVMI, body composition, cardiorespiratory fitness (CRF), blood pressure (BP) and lipids. At baseline, the strongest correlates of aPWV were body weight (r = .31) and diastolic BP (r = .28); of cIMT were body weight (r=0.26) and CRF (r=-0.25); and of LVMI was CRF (r=0.32) after adjusting for sex and race (p < .05 for all). Despite significant reductions in total fat and improvements in CRF in the AE and RE groups, aPWV, cIMT, LVMI, BP, lipids and body weight did not change as compared with controls (p > .05 for all). Interventions of longer duration or together with weight loss may be required to improve these early biomarkers of CVD in obese adolescents.

EMB, the gold standard for diagnosis of ACR, poses unique risks in children. Limited cross-sectional data have associated LV MPI with ACR. We hypothesize that a relative change in MPI from baseline without ACR to the time of ACR will better detect ACR than an absolute threshold LV MPI value. We identified 40 children with ACR ≥60 days post-transplant matching them by age and time from transplantation to 40 children without ACR. There was a significant increase in LV MPI at time of ACR vs. baseline (0.59 ± 0.17 vs. 0.41 ± 0.11; p < 0.001). There was no difference in LV MPI between baseline and follow-up (0.41 ± 0.11 vs. 0.42 ± 0.11; p = 0.65). An absolute increase in LV MPI of ≥0.47 had 82.5% sensitivity and 85% specificity for ACR, whereas an increase in LV MPI from baseline of ≥20.4% was 90% sensitive and 100% specific. Serial measurement of LV MPI appears to be a sensitive and specific marker of ACR. LV MPI shows good interobserver agreement and increases at the time of EMB-proven ACR with subsequent resolution to baseline measurements upon EMB-proven resolution of ACR. Future studies in larger, prospective cohorts should be undertaken to validate these findings., (© 2013 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

DTI indices have been associated with cellular rejection in adult heart transplant recipients, but their predictive value in pediatric recipients is unknown. The purpose of this study was to evaluate DTI measures in the detection of cellular and AMR in pediatric heart transplant recipients. One hundred and forty-eight pediatric heart transplant recipients who had 267 cardiac catheterization procedures with EMB, echocardiogram with DTI, and BNP level performed on the same day were included in the study. For the mitral and tricuspid valves, the ratios (E/E') between the early diastolic inflow velocity by pulsed Doppler (E, m/s) and the early diastolic annular velocity by DTI (E', m/s) were obtained and compared between subjects with and without rejection. Of the 148 recipients, 30 subjects had a total of 37 episodes of rejection: 10 cellular (>or=1B), 17 AMR, and 10 biopsy-negative clinical rejection. Mitral and tricuspid valve E/E' ratios were significantly higher in rejectors than in non-rejectors (5.5 +/- 1.3 vs. 4.4 +/- 1.4, p < 0.001 and 4.9 +/- 2.1 vs. 4.1 +/- 1.5, p < 0.01, respectively). By multivariate linear regression, mitral valve E/E' was an independent predictor of rejection. Mitral and tricuspid valve E/E' <5.0 had 93% and 89% NPV, respectively, for rejection. Mitral and tricuspid valve E/E' ratios <5.0 may be useful non-invasive screening measures to exclude rejection in pediatric heart transplant recipients.

Background: Cardiac repair for congenital heart disease in children awaiting liver transplantation presents unique therapeutic challenges and dilemmas. We tested the hypothesis that operations in these children requiring cardiopulmonary bypass (CPB) were not associated with prohibitive morbidity and mortality., Methods: Over the last 10 years (1994-2004), five infants were identified in our database with end-stage liver disease and awaiting liver transplantation that required cardiac surgery. Primary end point for the study was mortality. Secondary end points included morbidity and time to liver transplantation. The new pediatric end-stage liver disease (PELD) model was used to score liver disease severity., Results: Three boys and two girls with mean age of 8.6 months (range, 1.5-21 months) and mean PELD of 18.0 (range, 10-29) required CPB for repair. The only early mortality in the series occurred after cardiac arrest during creation of a central shunt. The child expired two days later despite extracorporeal membrane oxygenation support. The patient had important myocardial hypertrophy. All other patients survived and underwent successful liver transplantation., Conclusions: Children with significant congenital heart disease awaiting liver transplantation can undergo safe cardiac repair with judicious perioperative support thereby reducing the risks of subsequent liver transplantation.

Bicuspid aortic valve (BAV) is associated with aortic root dilation and dissection in adults, but the age and conditions when dilation begins are unknown. Using echocardiographic data, we compared the aortic root dimensions and valve hemodynamics of 76 children with BAV with valves derived from 41 normal children. The children with BAV had significantly larger aortic roots (p <0.001) regardless of the presence of aortic stenosis or regurgitation.

Objective: Ketorolac is a potent nonsteroidal analgesic agent used to treat postoperative pain. It produces excellent analgesia without the sedating side effects of opioid analgesics. Routine use of ketorolac after cardiac surgery is limited by concerns of bleeding complications. The purpose of this study was to evaluate the risk of bleeding complications of ketorolac for treatment of pain after congenital heart surgery in infants and children., Design: Prospective randomized, controlled trial., Setting: Pediatric cardiac intensive care unit in tertiary teaching hospital., Participants: Seventy infants and children, median age 10 months (range 2.5-174), who underwent congenital heart surgery requiring cardiopulmonary bypass were randomized in the trial., Intervention: Pain control was performed with ketorolac and opioid analgesics in one arm of the study and opioid analgesics alone in the other arm., Outcome Measures: The main outcome evaluated was bleeding complications measured by chest-tube drainage and wound and gastrointestinal bleeding., Results: Thirty-five patients were randomized to each treatment arm. In the ketorolac group, the median chest-tube drainage was 13.3 (range 4-22) mL/kg/d, no patient had significant wound bleeding, and 1 (0.03%) patient had gastrointestinal bleeding. In the control group, the median chest-tube drainage was 16.5 (range 3-24) mL/kg/d, 1 (0.03%) patient had wound bleeding, and no patient had gastrointestinal bleeding., Conclusion: Ketorolac can be used to treat pain after congenital heart surgery without an increased risk of bleeding complications.

Neonates with double-inlet left ventricle or tricuspid atresia with transposed great arteries and a bulboventricular foramen (BVF) area <2 cm(2)/m(2) develop BVF obstruction. This study examined the outcome of neonates with BVF area between 1 and 2 cm(2)/m(2) whose BVF was bypassed after the neonatal period. We reviewed 29 neonates with double-inlet left ventricles (n = 18) or tricuspid atresia (n = 11) and transposed great arteries. The study group consisted of 9 patients with neonatal BVF areas of 1 to 2 cm(2)/m(2) who did not undergo repair of the BVF obstruction as a neonate. The comparison group consisted of 8 "ideal" patients without BVF obstruction. Precavopulmonary shunt data from cardiac catheterization and echocardiogram and outcomes of the cavopulmonary shunt were compared. Study group patients developed a mild BVF gradient (18 +/- 10 mm Hg by cardiac catheterization) by a mean of 7 months. Left ventricular wall thickness, however, remained in the normal range (4.2 +/- 0.3 mm) and was not statistically different from the comparison group (4.1 +/- 0.4 mm). No difference was found in the precavopulmonary mean pulmonary artery pressure (15 +/- 5 vs 15 +/- 6 mm Hg), transpulmonary gradient (8 +/- 4 vs 8 +/- 5 mm Hg), and left ventricular end-diastolic pressure (7 +/- 2 vs 8 +/- 3 mm Hg). One patient in the study group died from respiratory syncytial virus pneumonia while awaiting cavopulmonary shunt. Neither group had mortality from the cavopulmonary shunt. The lengths of hospital stay were comparable (8.3 +/- 3.7 vs 8.9 +/- 6.0 days). Thus, neonates with BVF area between 1 and 2 cm(2)/m(2) develop mild but hemodynamically insignificant BVF gradient by 7 months of age. This group of patients can be managed safely with relief of BVF obstruction later in infancy.

This study was carried out to compare echocardiographic findings of children taking tacrolimus and cyclosporin A (CsA) after orthotopic liver transplantation (OLT). Echocardiograms of 19 children were reviewed during hospitalizations after OLT, and echocardiograms were performed on 23 children who returned to the clinic for a routine follow-up visit after OLT. Measurements were made of the left ventricle (LV) end-diastolic dimension, and of the thickness of the LV free wall (LVFW) and the inter-ventricular septum (IVS). From these measurements, the LV mass was calculated. LV outflow gradient was measured by using Doppler interrogation. Comparisons were made between patients on CsA and patients on tacrolimus. Children with hypertrophic cardiomyopathy (HCM) were identified. Two patients from the in-patient tacrolimus group were found to have HCM. These two patients had asymmetric septal hypertrophy with dynamic LV outflow obstruction and were successfully treated with propranolol, with or without discontinuing tacrolimus. In the out-patient studies, there was no difference in LVFW and IVS thickness, or LV mass index, between children on CsA and children on tacrolimus. Hence, tacrolimus is associated with the development of HCM in children. The effect of tacrolimus on HCM development may be acute and temporary. More data are needed to determine the incidence of HCM in children on tacrolimus therapy and to establish guidelines for clinicians who follow-up these children.

Objectives: We sought to compare bubble contrast echocardiography and pulmonary angiography in detecting pulmonary arteriovenous malformation (PAVM) in children with cavopulmonary anastomosis (CPA), and to examine anatomic and physiologic variables associated with the development of PAVM., Background: Development of PAVM in patients with CPA may cause profound cyanosis. Pulmonary arteriovenous malformation has been traditionally diagnosed by pulmonary angiography with reported incidence of 20% to 25% in patients with CPA., Methods: Fourteen patients (age 1.1 to 12.6 years) with any forms of CPA and normal pulmonary venous drainage formed the study population. All patients underwent cardiac catheterization and pulmonary angiography. Bubble contrast echocardiographic studies were performed with injection of 10 ml of agitated saline solution into branch pulmonary arteries. Transthoracic echocardiograms using an apical view were performed to assess the appearance of bubble contrast in the systemic ventricles. We compared the results of pulmonary angiograms and contrast echocardiograms, and findings of contrast echocardiograms between lungs with hepatic venous blood flow and lungs without hepatic venous blood., Results: Ten of the 14 patients (71%) had positive contrast echocardiographic studies, compared with three (21%) detected by pulmonary angiograms (p = 0.01). No difference was found in pulmonary artery pressure, transpulmonary gradient or presence of heterotaxy syndrome between patients with positive contrast echocardiographic studies and patients with negative studies. However, patients with positive contrast echocardiograms tended to have lower oxygen saturation (81%) and higher hemoglobin (16.4 g/dl) compared with patients with negative studies (88% and 14.7 g/dl, p = 0.10 and p = 0.18 respectively). Patients with Glenn shunt or unidirectional Fontan had higher incidence of PAVM (10/11) compared with patients with classic or lateral tunnel Fontan (0/3, p = 0.01). All 12 lungs with no perfusion of hepatic venous blood had positive contrast echocardiographic studies. Lungs with no hepatic venous blood flow were more likely to develop PAVM compared with lungs with hepatic venous blood flow (12/12 and 3/16 respectively, p < 0.01)., Conclusions: Bubble contrast echocardiography is more sensitive in detecting PAVM compared with pulmonary angiography. The prevalence of PAVM in patients with CPA may be much higher than what had been reported previously. Lungs with no hepatic venous blood flow are more likely to develop PAVM than lungs with hepatic venous blood flow.

Objectives: The purpose of the study was to assess the effects of maternal HIV-1 (human immunodeficiency virus) infection and vertically transmitted HIV-1 infection on the prevalence of congenital cardiovascular malformations in children., Background: In the United States, an estimated 7000 children are born to HIV-infected women annually. Previous limited reports have suggested an increase in the prevalence of congenital cardiovascular malformations in vertically transmitted HIV-infected children., Methods: In a prospective longitudinal multicenter study, diagnostic echocardiograms were performed at 4-6-month intervals on two cohorts of children exposed to maternal HIV-1 infection: 1) a Neonatal Cohort of 90 HIV-infected, 449 HIV-uninfected and 19 HIV-indeterminate children; and 2) an Older HIV-Infected Cohort of 201 children with vertically transmitted HIV-1 infection recruited after 28 days of age., Results: In the Neonatal Cohort, 36 lesions were seen in 36 patients, yielding an overall congenital cardiovascular malformation prevalence of 6.5% (36/558), with a 8.9% (8/90) prevalence in HIV-infected children and a 5.6% (25/449) prevalence in HIV-uninfected children. Two children (2/558, 0.4%) had cyanotic lesions. In the Older HIV-Infected Cohort, there was a congenital cardiovascular malformation prevalence of 7.5% (15/201). The distribution of lesions did not differ significantly between the groups., Conclusions: There was no statistically significant difference in congenital cardiovascular malformation prevalence in HIV-infected versus HIV-uninfected children born to HIV-infected women. With the use of early screening echocardiography, rates of congenital cardiovascular malformations in both the HIV-infected and HIV-uninfected children were five- to ten-fold higher than rates reported in population-based epidemiologic studies but not higher than in normal populations similarly screened. Potentially important subclinical congenital cardiovascular malformations were detected.

Acute or chronic myocardial ischemia may develop in patients with pulmonary atresia with intact ventricular septum and right ventricular-dependent coronary circulation. In such cases an aorta to right ventricle shunt may be used to reverse this ischemia. This report summarizes our experience with the placement of an aortic to right ventricular shunt in 5 patients. The shunts were made of Gore-Tex and ranged from 4 mm to 8 mm. Associated procedures were bidirectional Glenn (n = 2) and Fontan (n = 2). All 5 patients survived the procedure with documented early graft patency and no evidence of ischemia.

A conventional method for microinjection, using erythrocyte ghosts as the injection vector, has been modified to provide a protocol for the highly efficient delivery of small quantities of material into the cytoplasm of target cells. The technique is applicable for use with a variety of proteins, sugars, nucleotides and dyes. When the intercalating dye propidium iodide is included within the sealed ghosts their subsequent fusion with target cells can be continuously monitored by fluorescence spectroscopy, providing a convenient and sensitive parameter of cell-cell fusion. The protocol can be adapted for use with both adherent and non-adherent target cells, and can be used to monitor the relative effectiveness of a variety of fusogenic agents.