Background Assessment of fetal ventricular function is mostly subjective, and currently, for the objective assessment left ventricular shortening fraction is obtained. However, this by itself is not very reliable. Hence, more tools that can provide an objective assessment are needed to increase the confidence of functional assessment. Speckle tracking imaging can provide one such tool. Goal : In this study we sought to establish the normative value of global longitudinal and circumferential strain for our fetal patients and for two major forms of congenital heart diseases, namely atrioventricular canal defects (AVC) and uncorrected dextro-transposition of the great arteries (dTGA) to act as a benchmark. Methods The study was completed via a single center retrospective analysis on 72 fetal echocardiograms (26 normal, 15 dTGA, and 31 AVC). Tomtec Arena™ echocardiography analysis software was used for analysis. Results In normal fetuses, mean left ventricular (LV) global longitudinal strain (GLS) was − 22.6% (95% CI -24, -21.1) and mean right ventricular (RV) GLS was − 22.1% (95% CI -23.6, -20.6). In AVC patients LV GLS was-26.6% (95% CI -28,-25.3) and mean RV GLS was − 26.5% (95% CI -27.9,-25.2). In dTGA patients LV GLS was − 22.9% (95% CI of -24.8, -21) and RV GLS was − 21.3% (95% CI was − 23.4, -20.8). There was good intra-rater reliability though poor to fair inter-rater reliability. Conclusion Notwithstanding its current limitations, strain imaging can provide useful information that can increase confidence of cardiac functional assessment in fetal patients. However, to be reliable across the board, further automation and standardization is required.

The occurrence of acute kidney injury (AKI) among patients with acute coronary syndrome (ACS) undergoing invasive management is associated with worse outcomes. However, the prognostic implications of transient or in-hospital persistent AKI may differ.The aim of this study was to evaluate the prognostic implications of transient or in-hospital persistent AKI in patients with ACS.In the MATRIX (Minimizing Adverse Haemorrhagic Events by Transradial Access Site and Systemic Implementation of Angiox) trial, 203 subjects were excluded because of incomplete information or end-stage renal disease, with a study population of 8,201 patients. Transient and persistent AKI were defined as renal dysfunction no longer or still fulfilling the AKI criteria (>0.5 mg/dL or a relative >25% increase in creatinine) at discharge, respectively. Thirty-day coprimary outcomes were the out-of-hospital composite of death, myocardial infarction, or stroke (major adverse cardiovascular events [MACE]) and net adverse cardiovascular events (NACE), defined as the composite of MACE or Bleeding Academic Research Consortium type 3 or 5 bleeding.Persistent and transient AKI occurred in 750 (9.1%) and 587 (7.2%) subjects, respectively. After multivariable adjustment, compared with patients without AKI, the risk for 30-day coprimary outcomes was higher in patients with persistent AKI (MACE: adjusted HR: 2.32; 95% CI: 1.48-3.64; P < 0.001; NACE: adjusted HR: 2.29; 95% CI: 1.48-3.52; P < 0.001), driven mainly by all-cause mortality (adjusted HR: 3.43; 95% CI: 2.03-5.82; P < 0.001), whereas transient AKI was not associated with higher rates of MACE or NACE. Results remained consistent when implementing the KDIGO (Kidney Disease Improving Global Outcomes) criteria.Among patients with ACS undergoing invasive management, in-hospital persistent but not transient AKI was associated with higher risk for 30-day MACE and NACE. (Minimizing Adverse Haemorrhagic Events by Transradial Access Site and Systemic Implementation of Angiox [MATRIX]; NCT01433627)

Left atrial appendage occlusion in young children has not been reported before. Herein, we describe a successful occlusion using hydrogel coils in a toddler. The decision to occlude the appendage was made to mitigate the potential risk of systemic thromboembolism, given the child's unusual anatomy.

Background: Somatic growth delays are common in infants with single ventricle heart disease. Health care is largely centralized to higher volume centers located predominantly in urban environments. No studies to date have assessed how home location in an urban or rural environment impacts outcomes in single ventricle infants. Methods: Data was reviewed from 67 centers using the National Pediatric Cardiology – Quality Improvement Collaborative (NPC-QIC) database. Urban or rural home location was determined using the home zip code’s rural urban commuting area (RUCA) code. One year weight and height z-scores and one year transplant-free survival were compared between urban and rural groups. Analysis was performed both unadjusted and with adjustments for co-variates. Sub-group analysis was performed for race/ethnicity, sex, and insurance type. Multiple imputation was performed to account for missing data. Results: Data from 1403 urban and 351 rural patients was analyzed. Urban infants had higher weight and height z-scores (-0.95 and -1.65, respectively) at one year of age when compared to rural infants (-1.16 and -2.01, respectively, p <0.01). When adjustments were made for covariates, there was no significant difference (p ≥0.06). Rural infants were more likely to survive, transplant-free, to their first birthday (odds ratio 0.72, p <0.02). Conclusion: Rural single ventricle infants are more likely to survive, transplant-free, to their first birthday but their growth lags behind urban peers. However, this difference in growth is attributable to demographic and disease severity factors.

INTRODUCTION:Transesophageal echocardiography (TEE) use continues to expand to include extracardiac applications. However, there is limited research investigating the use of TEE as a tool to confirm the position of the epidural catheter. This prospective observational study aimed to evaluate whether TEE could be used to visualize the anatomy of the thoracic spinal canal in pediatrics. A subsequent prospective case series was conducted to evaluate whether TEE could be used to assist in the placement of epidural catheters in pediatric surgical patients. METHODS:Seventy-five patients (50 pediatric and 25 adult subjects) were enrolled. The operators attempted to identify four structures (spinal cord, cerebrospinal fluid, dura mater, epidural space) within the thoracic spinal canal with TEE. After demonstrating the feasibility of this technique for observing the spinal anatomy, 20 pediatric surgical patients were enrolled in a case series. These patients had epidural catheter placement, and the final catheter position was confirmed with TEE. RESULTS:The total number of thoracic spinal segment visualized in pediatric patients was 542 out of 550 (99%) segments, and 191 out of 275 (70%) segments in adult subjects (difference, 29% [95% confidence interval, 23-34]; p < 0.001). Additionally, a case series of 20 pediatric surgical patients demonstrated successful caudal or epidural catheter placement at target spinal level in 17 cases. CONCLUSIONS:This observational study demonstrated the successful visualization of the thoracic spinal cord at virtually every level in pediatric patients. A subsequent case series demonstrated that TEE could be used to successfully confirm the position of the epidural catheter in the targeted thoracic spinal segment for pediatric surgical patients.

Buysse, Janelle1; Wu, Chaorong2; Pierick, Trudy3; Reinking, Benjamin3 Author Information

BACKGROUND:Hypoplastic left heart syndrome and single ventricle variants with aortic hypoplasia are commonly classified as severe forms of CHD. We hypothesised patients with these severe defects and reported genetic abnormalities have increased morbidity and mortality during the interstage period. METHODS AND RESULTS:This was a retrospective review of the National Pediatric Cardiology Quality Improvement Collaborative Phase I registry. Three patient groups were identified: major syndromes, other genetic abnormalities, and no reported genetic abnormality. Tukey post hoc test was applied for pairwise group comparisons of length of stay, death, and combined outcome of death, not a candidate for stage 2 palliation, and heart transplant. Participating centres received a survey to establish genetic testing and reporting practices. Of the 2182 patients, 110 (5%) had major genetic syndromes, 126 (6%) had other genetic abnormalities, and 1946 (89%) had no genetic abnormality. Those with major genetic syndromes weighed less at birth and stage 1 palliation. Patients with no reported genetic abnormalities reached full oral feeds sooner and discharged earlier. The combined outcome of death, not a candidate for stage 2 palliation, and heart transplant was more common in those with major syndromes. Survey response was low (n = 23, 38%) with only 14 (61%) routinely performing and reporting genetic testing. CONCLUSIONS:Patients with genetic abnormalities experienced greater morbidity and mortality during the interstage period than those with no reported genetic abnormalities. Genetic testing and reporting practices vary significantly between participating centres.

Introduction: Hypoplastic left heart syndrome (HLHS) is a genetically heterogenous, severe form of congenital heart disease. Hypothesis: Patients with reported genetic abnormalities will have worse outcomes than those without reported genetic abnormalities. Methods: This was a retrospective review of patients enrolled in the National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) phase I registry. Registry enrollment occurred at discharge following stage 1 palliation. Patients were placed into one of three groups: major syndromes (Turner, Down, CHARGE, DiGeorge, Jacobsen, VATER, heterotaxy), other genetic abnormality, or no reported genetic abnormality. Demographic and clinical variables were compared using Pearson Chi-square, one-way ANOVA, or Kruskal-Wallis test. Tukey post-hoc test was applied to adjust p-values for pairwise group comparisons. Outcomes of length of stay, death, and combined outcomes of death, not a candidate for stage 2 palliation and transplant were compared among the groups. Results: Of the 2182 patients, major syndromes were reported in 110 (5%), other genetic abnormalities in 126 (5.8%) and no abnormalities in 1946 (89.2%). Those with major syndromes weighed less at birth and the time of stage I palliation, were more likely to be female and have a primary cardiac diagnosis of unbalanced AVC. Those with major syndromes or other genetic abnormalities were more likely to have moderate to severe AV valve regurgitation, moderate to severe ventricular dysfunction, arrhythmia requiring therapy, and major anomalies of other organ systems compared to those with no abnormalities. Patients with no reported genetic abnormalities reached full oral feeds sooner after the Norwood (19 vs 24 days), were more likely to be discharged on all oral feeds (75% vs 59% and 62%) and were discharged earlier (35 vs 45 and 43 days). For the outcome of death, there was no significant difference among the groups. The combined outcome of death, not a candidate for stage 2 palliation, and heart transplant was more likely in those with a major syndrome after adjusting for covariates. Conclusions: Patients with HLHS and major syndromes or other genetic abnormalities experience greater morbidity and mortality during the interstage period.

A 4-day-old male with prenatal diagnosis of absent pulmonary valve (APV) syndrome underwent a CT angiogram of the chest for detailed assessment of the thoracic great vessels and airway. The CT angiogram confirmed the echocardiographic diagnosis of APV with intact ventricular septum (IVS) and unobstructed right ventricular outflow tract (RVOT; Fig 1A,C). His main pulmonary artery (MPA; Fig. 1A,B,C) measured 2.5 cm in diameter (Z-score 6.61). The aneurysmal dilation of MPA displaced his trachea (Fig. 1A,B,C), ascending (AAo; Fig. 1A) and thoracic descending aorta (DAo; Fig. 1B and C) to the right, however with no significant compression of the large airways. He had a left-sided aortic arch and small patent ductus arteriosus (PDA; Fig. 1B). This PDA was abnormally aligned in right-to-left axis instead of usual anteroposterior axis as it extended from aorta to proximal left pulmonary artery due to rightward displacement of the thoracic aorta by dilated pulmonary arteries.

INTRODUCTION:Reported frequencies of cardiomyopathy in limb girdle muscular dystrophy R9 (LGMDR9) vary. We describe the frequency and age at onset of cardiomyopathy in an LDMDR9 cohort. METHODS:Echocardiograms from 56 subjects (157 echocardiograms) with LGMDR9 were retrospectively reviewed. The cumulative probability of having an abnormal echocardiogram as a function of age was assessed by survival analysis for interval-censored data by genotype. Correlations between cardiac and clinical function were evaluated. RESULTS:Twenty-five (45%) participants had cardiomyopathy. The median age at first abnormal echocardiogram for subjects homozygous for the c.826C>A variant was 54.2 y compared to 18.1 y for all other fukutin-related protein (FKRP) genotypes (P < .0001). There was a weak correlation between ejection fraction and 10-Meter Walk Test speed (r = 0.25), but no correlation with forced vital capacity (r = 0.08). DISCUSSION:Cardiomyopathy is prevalent among those with LGMDR9 and occurs later in subjects homozygous for the c.826C>A mutation. These data will help to guide surveillance and management.

Background: Echocardiography is utilized to assess cardiac structure and function. In infants, children, and adolescents, a normative data set can be utilized to evaluate cardiac size relative to patient body size and provides the clinician with a quantified z-score based on body surface area. A complete normative data set for premature infants is not yet available. The aim of this study was to develop a normative data set for commonly measured cardiac structures in a cohort of premature infants. Methods and Results: Single center retrospective echocardiographic study in a cohort of premature infants without congenital heart disease. A total of 184 infants admitted at the University of Iowa’s Neonatal Intensive Care Unit between 2009-2019 were included in the study. All infants were between 23 to 26- and 6/7-weeks gestational age. We considered patients with a patent ductus arteriosus and/or a patent foramen ovale as having normal intra-cardiac anatomy given their prematurity. Some infants had numerous echocardiograms during this time interval which resulted in 439 utilized echocardiogram. Each echocardiogram used was interpreted individually regardless if the patient had multiple between the gestational ages of 23 and 26- and 6/7-weeks. Commonly examined cardiac structures were assessed via 2-dimensional and M-mode echocardiography. The mean and standard deviations were obtained for each cardiac structure and grouped by week of gestational age ( Table 1 ). Conclusions: We present normative data for commonly measured cardiac structures based on gestational age in a premature patient population. This information is valuable for clinicians to assess for appropriate cardiac structural development and may be utilized to guide clinical management and need for possible intervention as the child continues to develop and grow.

Background As a result of medical and surgical advancements in the management of congenital heart disease (CHD), survival rates have improved substantially, which has allowed the focus of CHD management to shift toward neurodevelopmental outcomes. Previous studies of the neuropathology occurring in CHD focused on cases preceding 1995 and reported high rates of white matter injury and intracranial hemorrhage, but do not reflect improvements in management of CHD in the past 2 decades. The purpose of this study is therefore to characterize the neuropathological lesions identified in subjects dying from CHD in a more‐recent cohort from 2 institutions. Methods and Results We searched the autopsy archives at 2 major children's hospitals for patients with cyanotic congenital cardiac malformations who underwent autopsy. We identified 50 cases ranging in age from 20 gestational weeks to 46 years. Acquired neuropathological lesions were identified in 60% (30 of 50) of subjects upon postmortem examination. The most common lesions were intracranial hemorrhage, most commonly subarachnoid (12 of 50; 24%) or germinal matrix (10 of 50; 20%), hippocampal injuries (10 of 50; 20%), and diffuse white matter gliosis (8 of 50; 16%). Periventricular leukomalacia was rare (3 of 50). Twenty‐six subjects underwent repair or palliation of their lesions. Of the 50 subjects, 60% (30 of 50) had isolated CHD, whereas 24% (12 of 50) were diagnosed with chromosomal abnormalities (trisomy 13, 18, chromosomal deletions, and duplications) and 16% (8/50) had multiple congenital anomalies. Conclusions In the modern era of pediatric cardiology and cardiac surgery, intracranial hemorrhage and microscopic gray matter hypoxic‐ischemic lesions are the dominant neuropathological lesions identified in patients coming to autopsy. Rates of more severe focal lesions, particularly periventricular leukomalacia, have decreased compared with historical controls.

INTRODUCTION:Congenital heart disease (CHD) is multifactorial in origin, resulting from an interaction between environmental and genetic factors. Multifactorial growth delay is common in infants with CHD. The impact of a genetic abnormality and CHD on the growth of an infant is lacking in the literature. The aim of this study is to compare the growth and method of feeding following neonatal cardiac surgery in infants with normal versus abnormal genetic testing.METHODS:A retrospective chart review of neonates who underwent a Risk Adjustment in Congenital Heart Surgery IV-VI procedure between 1 January, 2006 and 22 September, 2016 was performed at our institution. Weight, length, head circumference measurements, and feeding method were collected at birth, time of neonatal surgery, and monthly up to 6 months of age.RESULTS:A total of 53 infants met inclusion criteria, of which 22 had abnormal genetic testing. Approximately 90% of infants were discharged following neonatal cardiac surgery with supplemental tube feeds. At each monthly follow-up visit, more infants were exclusively fed orally: 80% of infants with normal genetics at 5 months post-operative follow-up versus 60% of infants with abnormal genetic testing, although statistically insignificant. Growth was not different among the two groups.CONCLUSIONS:Infants with critical CHD with or without genetic abnormalities are at risk for growth delays and many need supplemental tube feeds post-operatively and throughout follow-up. Infants with genetic abnormalities are slower to achieve oral feeds and more likely to require tube feedings. It is important to have a systematic protocol for managing these high-risk infants.

Background: Selective serotonin reuptake inhibitors (SSRIs) are antidepressants prescribed in 10% of pregnancies in the USA. We have previously shown in preclinical studies that sertraline exposure impacts cardiomyocyte development, leading to reductions in left ventricular size and cardiac function. Objectives: We hypothesized that in utero SSRI exposure will lead to reduced left ventricular dimensions and cardiac function on echocardiography immediately after birth. Methods: Twenty term infants with and 21 term infants without in utero exposure to SSRIs underwent echocardiograms to assess cardiac size and function. The exclusion criteria for infants were prematurity, small or large for gestational age, any respiratory or cardiovascular support needed after birth, and any major congenital malformation. Results: Infants exposed to in utero SSRIs had significantly reduced right ventricular dimensions in the diastole (controls 1.0 cm [0.86, 1.20], SSRI 0.89 cm [0.730, 1.05], p = 0.03), and left ventricular lengths in the diastole and systole (diastole: controls 3.4 cm [3.25, 3.65], SSRI 3.25 cm [3.10, 3.45], p = 0.03; systole: controls 2.9 cm [2.65, 3.05], SSRI 2.6 cm [2.50, 2.85], p = 0.01). No differences were observed in cardiac function. Importantly, there were no differences in maternal conditions or infant birth weight, body surface area, or gestational age. Conclusions: Our findings suggest an association between in utero exposure to SSRIs and ventricular size in infants. Given the increasing use of SSRIs during pregnancy and the importance of early life programming on future cardiovascular health, larger studies need to be completed to determine if in utero SSRI exposure impacts ventricular size.

Acquired intracardiac left-to-right shunts are rare occurrences. Chest trauma and myocardial infection are well-known causes of acquired ventricular septal defect (VSD). There have been several case reports describing left ventricle to right atrium shunt after infective endocarditis (IE). We present here a patient found to have an acquired VSD secondary to IE of the aortic and tricuspid valves in the setting of a known bicuspid aortic valve. This is the first case reported of acquired VSD in a pediatric patient in the setting of IE along with literature review of acquired left-to-right shunts.

Objectives: Arch branching has never been shown to influence recoarctation after extended end-to-end anastomosis via thoracotomy, yet in each study bovine arch identification is grossly underreported. This study aims to (1) assess chart review reliability in bovine arch identification; (2) determine recoarctation risk with a bovine arch; and (3) explore an anatomic explanation for recurrent arch obstruction based on arch anatomy. Patients: A total of 49 consecutive patients underwent thoracotomy with extended end-to-end aortic coarctation repair at a single institution (2007-2012). Methods: Echocardiograms from these patients were reviewed for arch anatomy and compared with the echocardiographic reports. Recurrent arch obstruction was defined as an echocardiographic gradient across the repair of 20mmHg or greater. For cases with angiographic images (n = 17), a scaled clamping distance between the left subclavian artery and the maximal proximal clamp location on orthogonal projections was then calculated across arch anatomies. Results: Chart review identified 6.1% (3/49) of patients with a bovine arch compared with 28.6% (14/49) on targeted image review. A total of 28.6% (4/14) of patients with a bovine arch had a follow-up gradient of 20 mm Hg or greater. Only 5.7% (2/35) of patients with normal arch branching had a followup gradient of 20 mm Hg or greater. The mean clamping index was significantly diminished in patients with bovine arch anatomy. Conclusions: Arch anatomy often goes undocumented on preoperative imaging, yet children undergoing extended end-to-end repair with bovine arch anatomy are at a significantly increased risk of recoarctation. This may be due to a reduced clampable distance to facilitate repair. These results should be considered in the preoperative assessment, parental counseling, and surgical approach for children with discrete aortic coarctation.

Prematurity is associated with reduced cardiac dimensions and an increased risk of cardiovascular disease. While prematurity is typically associated with ex utero neonatal growth restriction (GR), the independent effect of neonatal GR on cardiac development has not been established. We tested the hypothesis that isolated neonatal GR decreases cardiomyocyte growth and proliferation, leading to long-term alterations in cardiac morphology. C57BL/6 mice were fostered in litters ranging in size from 6 to 12 pups to accentuate normal variation in neonatal growth. Regardless of litter size, GR was defined by a weight below the 10th percentile. On postnatal day 8, Ki67 immunoreactivity, cardiomyocyte nucleation status and cardiomyocyte profile area were assessed. For adult mice, cardiomyocyte area was determined, along with cardiac dimensions by echocardiography and cardiac fibrosis by Masson's trichrome stain. On day 8, cardiomyocytes from GR versus control mice were significantly smaller and less likely to be binucleated with evidence of persistent cell cycle activity. As adults, GR mice continued to have smaller cardiomyocytes, as well as decreased left ventricular volumes without signs of fibrosis. Neonatal GR reduces cardiomyocyte size, delays the completion of binucleation, and leads to long-term alterations in cardiac morphology. Clinical studies are needed to ascertain whether these results translate to preterm infants that must continue to grow and mature in the midst of the increased circulatory demands that accompany their premature transition to an ex utero existence. Anat Rec, 2018. © 2018 Wiley Periodicals, Inc.

To describe frequency and age at onset of abnormal echocardiograms in a cohort of patients with dystroglycanopathies.

•Entrustable professional activities define the tasks, knowledge and skills expected of a physician.•II The training guidelines define the curriculum and training environment.•Curricula components define knowledge, skills and scope of practice.