OBJECTIVE: Current risk adjustment models for congenital heart surgery do not fully incorporate multiple factors unique to neonates such as granular gestational age (GA) and birth weight (BW) z score data. This study sought to develop a Neonatal Risk Adjustment Model for congenital heart surgery to address these deficiencies.

METHODS: Cohort study of neonates undergoing cardiothoracic surgery during the neonatal period captured in the Pediatric Cardiac Critical Care Consortium database between 2014 and 2020. Candidate predictors were included in the model if they were associated with mortality in the univariate analyses. GA and BW z score were both added as multicategory variables. Mortality probabilities were predicted for different GA and BW z scores while keeping all other variables at their mean value.

RESULTS: The C statistic for the mortality model was 0.8097 (95% confidence interval, 0.7942-0.8255) with excellent calibration. Mortality prediction for a neonate at 40 weeks GA and a BW z score 0 to 1 was 3.5% versus 9.8% for the same neonate at 37 weeks GA and a BW z score -2 to -1. For preterm infants the mortality prediction at 34 to 36 weeks with a BW z score 0 to 1 was 10.6%, whereas it was 36.1% for the same infant at <32 weeks with a BW z score of -2 to -1.

CONCLUSIONS: This Neonatal Risk Adjustment Model incorporates more granular data on GA and adds the novel risk factor BW z score. These 2 factors refine mortality predictions compared with traditional risk models. It may be used to compare outcomes across centers for the neonatal population.

BACKGROUND: There is ongoing debate about the best strategy to treat patients with tetralogy of Fallot who are symptomatic in the neonatal period.

OBJECTIVES: The aim of this study was to compare the outcomes of complete versus staged surgery (i.e., initial palliative procedure for possible later complete repair).

METHODS: A retrospective cohort study was performed using the Pediatric Health Information System database, including patients who underwent complete or staged tetralogy of Fallot repair prior to 30 days of age. The primary outcome was death during 2-year follow-up after the initial procedure. Inverse probability-weighted Cox and logistic regression models were used to examine the association between surgical approach group and mortality while accounting for patient- and hospital-level factors. Causal mediation analyses examined the role of intermediate variables.

RESULTS: A total of 2,363 patients were included (1,032 complete and 1,331 staged). There were 239 deaths. Complete neonatal repair was associated with a significantly higher risk for mortality during the 2-year follow-up period (hazard ratio: 1.51; 95% confidence interval: 1.05 to 2.06), between 7 and 30 days after the initial procedure (hazard ratio: 2.29; 95% confidence interval: 1.18 to 4.41), and during the initial hospital admission (odds ratio: 1.72; 95% confidence interval: 1.15 to 2.62). Post-operative cardiac complications were more common in the complete repair group and mediated the differences in 30-day and 2-year mortality.

CONCLUSIONS: Complete surgical repair for neonates with tetralogy of Fallot is associated with a significantly higher risk for early and 2-year mortality compared with the staged approach, after accounting for patient and hospital characteristics. Post-operative cardiac complications mediated these findings.

<p>Atrial septal defects are one of the most common forms of congenital heart disease, however sinus venosus communications, particularly pulmonary vein-type defects, are rare and are easily misdiagnosed. Patients with pulmonary vein-type sinus venosus defects often present earlier than those with ostium secundum defects with significant right heart dilation. Correct diagnosis has important implications for management. We discuss the clinical courses and review multimodality imaging of three patients correctly diagnosed with pulmonary vein-type defects after an initial diagnosis of an ostium secundum atrial septal defect, in order to promote understanding of the unique anatomy of this entity.</p>

<p>BACKGROUND Children with single ventricle heart disease have significant morbidity and mortality. The maternal-fetal environment (MFE) may adversely impact outcomes after neonatal cardiac surgery. We hypothesized that impaired MFE would be associated with an increased risk of death after stage 1 Norwood reconstruction. METHODS AND RESULTS We performed a retrospective cohort study of children with hypoplastic left heart syndrome (and anatomic variants) who underwent stage 1 Norwood reconstruction between 2008 and 2018. Impaired MFE was defined as maternal gestational hypertension, preeclampsia, gestational diabetes, and/or smoking during pregnancy. Cox proportional hazards regression models were used to investigate the association between impaired MFE and death while adjusting for confounders. Hospital length of stay was assessed with the competing risk of in-hospital death. In 273 children, the median age at stage 1 Norwood reconstruction was 4&nbsp;days (interquartile range [IQR], 3-6 days). A total of 72 children (26%) were exposed to an impaired MFE; they had more preterm births (18% versus 7%) and a greater percentage with low birth weights &lt;2.5&nbsp;kg (18% versus 4%) than those without impaired MFE. Impaired MFE was associated with a higher risk of death (hazard ratio [HR], 6.05; 95% CI, 3.59-10.21; &lt;0.001) after adjusting for age at surgery, Hispanic ethnicity, genetic syndrome, cardiac diagnosis, surgeon, and birth era. Children with impaired MFE had almost double the risk of prolonged hospital stay (HR, 1.95; 95% CI, 1.41-2.70; &lt;0.001). CONCLUSIONS Children exposed to an impaired MFE had a higher risk of death following stage 1 Norwood reconstruction. Prenatal exposures are potentially modifiable factors that can be targeted to improve outcomes after pediatric cardiac surgery.</p>

<p><strong>PURPOSE OF REVIEW: </strong>There is an increasing recognition that structural abnormalities and functional changes in the placenta can have deleterious effects on the development of the fetal heart. This article reviews the role of the placenta and the potential impact of placental insufficiency on fetuses with congenital heart disease.</p>

<p><strong>RECENT FINDINGS: </strong>The fetal heart and the placenta are directly linked because they develop concurrently with shared regulatory and signaling pathways. Placental disease is more common in pregnancies carrying a fetus with congenital heart disease and the fetal response to placental insufficiency may lead to the postnatal persistence of cardiac remodeling. The mechanisms underlying this placental-fetal axis of interaction potentially include genetic factors, oxidative stress, chronic hypoxia, and/or angiogenic imbalance.</p>

<p><strong>SUMMARY: </strong>The maternal-placental-fetal circulation is critical to advancing our understanding of congenital heart disease. We must first expand our ability to detect, image, and quantify placental insufficiency and dysfunction in utero. Elucidating the modifiable factors involved in these pathways is an exciting opportunity for future research, which may enable us to improve outcomes in patients with congenital heart disease.</p>

<p>Interventricular septal hematoma is a rare complication of pediatric cardiac surgery, usually in the context of ventricular septal defect closure.&nbsp;We present intraoperative echocardiography of a large interventricular septal hematoma secondary to the placement of a left ventricular assist device (VAD) in an infant who required conversion to biventricular support with bi-atrial cannulation. As the patient later died of neurologic complications, we present correlation with pathologic findings.</p>

<p><strong>Background</strong> Despite excellent operative survival, correction of tetralogy of Fallot frequently is accompanied by residual lesions that may affect health beyond the incident hospitalization. Measuring resource utilization, specifically cost and length of stay, provides an integrated measure of morbidity not appreciable in traditional outcomes.</p>

<p><strong>Methods and Results</strong> We conducted a retrospective cohort study, using de-identified commercial insurance claims data, of 269 children who underwent operative correction of tetralogy of Fallot from January 2004 to September 2015 with ≥2&nbsp;years of continuous follow-up (1) to describe resource utilization for the incident hospitalization and subsequent 2&nbsp;years, (2) to determine whether prolonged length of stay (&gt;7&nbsp;days) in the incident hospitalization was associated with increased subsequent resource utilization, and (3) to explore whether there was regional variation in resource utilization with both direct comparisons and multivariable models adjusting for known covariates. Subjects with prolonged incident hospitalization length of stay demonstrated greater resource utilization (total cost as well as counts of outpatient visits, hospitalizations, and catheterizations) after hospital discharge (&lt;0.0001 for each), though the number of subsequent operative and transcatheter interventions were not significantly different. Regional differences were observed in the cost of incident hospitalization as well as subsequent hospitalizations, outpatient visits, and the costs associated with each.</p>

<p><strong>Conclusions</strong> This study is the first to report short- and medium-term resource utilization following tetralogy of Fallot operative correction. It also demonstrates that prolonged length of stay in the initial hospitalization is associated with increased subsequent resource utilization. This should motivate research to determine whether these differences are because of modifiable factors.</p>

<p><strong>BACKGROUND: </strong>The surgical strategy for neonates with tetralogy of Fallot (TOF) consists of complete or staged repair. Assessing the comparative effectiveness of these approaches is facilitated by a large multicenter cohort. We propose a novel process for cohort assembly using the Pediatric Health Information System (PHIS), an administrative database that contains clinical and billing data for inpatient and emergency department stays from tertiary children's hospitals.</p>

<p><strong>METHODS: </strong>A 4-step process was used to identify neonates with TOF: (1) screen neonates in PHIS with International Classification of Diseases-9 (ICD-9) diagnosis or procedure codes for TOF; (2) include patients with TOF procedures before 30 days of age; (3) exclude patients with missing 2-year follow-up data; (4) analyze patients' 2-year surgery sequence patterns, exclude patients inconsistent with a treatment strategy for TOF, and designate patients as complete or staged repair. Manual chart review at 1 PHIS center was performed to validate this process.</p>

<p><strong>RESULTS: </strong>Between January 2004 and March 2015, 5862 patients were identified in step 1. Step 2 of cohort assembly excluded 3425 patients (58%); step 3 excluded 148 patients (3%); and step 4 excluded 54 patients (1%). The final cohort consisted of 2235 neonates with TOF from 45 hospitals. Manual chart review of 336 patients showed a positive predictive value for accurate PHIS identification of 44% after step 1 and 97% after step 4.</p>

<p><strong>CONCLUSIONS: </strong>This systematic cohort identification algorithm resulted in a high positive predictive value to appropriately categorize patients. This carefully assembled cohort offers a unique opportunity for future studies in neonatal TOF outcomes.</p>

<p><strong>BACKGROUND: </strong>Post-operative chylothorax in patients with congenital heart disease is a challenging problem with substantial morbidity and mortality. Currently, the etiology of chylothorax is poorly understood and treatment options are limited.</p>

<p><strong>OBJECTIVES: </strong>This study aimed to report lymphatic imaging findings, determine the mechanism of chylothorax after cardiac surgery, and analyze the outcomes of lymphatic embolization.</p>

<p><strong>METHODS: </strong>We conducted a retrospective review of 25 patients with congenital heart disease and post-operative chylothorax who presented for lymphatic imaging and intervention between July 2012 and August&nbsp;2016.</p>

<p><strong>RESULTS: </strong>Based on dynamic contrast-enhanced magnetic resonance lymphangiography and intranodal lymphangiography, we identified 3 distinct etiologies of chylothorax: 2 patients (8%) with traumatic leak from a thoracic duct (TD) branch, 14 patients (56%) with pulmonary lymphatic perfusion syndrome (PLPS), and 9 patients (36%) with central lymphatic flow disorder (CLFD), the latter defined as abnormal central lymphatic flow, effusions in more than 1&nbsp;compartment, and dermal backflow. Patients with traumatic leak and PLPS were combined into 1 group of 16 patients without CLFD, of whom 14 (88%) had an intact TD. Sixteen patients underwent lymphatic intervention, including complete TD embolization. All 16 patients had resolution of chylothorax, with a median of 7.5 days from intervention to chest tube removal and 15 days from intervention to discharge. The 9 patients with CLFD were considered a separate group, of whom 3 (33%) had an intact TD. Seven patients underwent lymphatic intervention but none survived.</p>

<p><strong>CONCLUSIONS: </strong>Most patients in this study had nontraumatic chylothorax and dynamic contrast-enhanced magnetic resonance lymphangiography was essential to determine etiology. Lymphatic embolization was successful in patients with traumatic leak and PLPS and, thus, should be considered first-line treatment. Interventions in patients with CLFD&nbsp;were not successful to resolve chylothorax and alternate approaches need to be&nbsp;developed.</p>