Clinical Variables Associated with Pre-Fontan Aortopulmonary Collateral Burden.

Aortopulmonary collaterals (APCs) develop universally, but to varying degrees, in patients with single ventricle congenital heart disease (CHD). Despite their ubiquitous presence, APCs remain poorly understood. We sought to evaluate the association between APC burden and common non-invasive clinical variables. We conducted a single center, retrospective study of patients with single ventricle CHD and previous Glenn palliation who underwent pre-Fontan cardiac magnetic resonance (CMR) imaging from 3/2018 to 3/2021. CMR was used to quantify APC flow, which was normalized to aortic (APC/QAo) and pulmonary vein (APC/QPV) blood flow. Univariate, multivariable, and classification and regression tree (CART) analyses were done to investigate the potential relationship between CMR-quantified APC burden and clinical variables. A total of 29 patients were included, all of whom had increased APC flow (APC/QAo: 26.9, [22.0, 39.1]%; APC/QPV: 39.4 [33.3, 46.9]%), but to varying degrees (APC/QAo: range 11.9-44.4%; APC/QPV: range 17.7-60.0%). Pulmonary artery size (Nakata index, at pre-Fontan CMR) was the only variable associated with APC flow on multivariable analysis (APC/QAo: p = 0.020, R2 = 0.19; APC/QPV: p = 0.0006, R2 = 0.36) and was the most important variable associated with APC burden identified by CART analysis (size inversely related to APC flow). APC flow is universally increased but highly variable in patients with single ventricle CHD and Glenn circulation. Small branch pulmonary artery size is a key factor associated with increased APC burden; however, the pathogenesis of APCs is likely multifactorial. Further research is needed to better understand APC pathogenesis, including predisposing and mitigating factors.

The Relationship Between Placental Pathology and Neurodevelopmental Outcomes in Complex Congenital Heart Disease.

Complex congenital heart disease (CCHD) is associated with impaired neurodevelopmental outcomes. Peri- and post-operative factors are known contributors while the impact of the prenatal environment is not yet delineated. Variations in fetal circulation, seen in transposition of the great arteries (TGA) and single ventricular physiology (SVP), are associated with placenta abnormalities. These abnormalities may be associated with placental insufficiency, a risk factor for poor neurodevelopmental outcomes. We hypothesized there is a correlation between placental pathology and impaired neurodevelopmental outcomes in patients with CCHD. We performed a single center retrospective cohort study with patients with TGA and SVP from 2010 to 2017 at Children's Wisconsin. Patient variables were obtained from the medical record. Bayley Scales of Infant Development Third Edition standard scores for cognitive, motor, and language performance were collected from neurodevelopmental visits. Placenta pathology reports were reviewed with tabulation of predetermined anatomical and pathological characteristics. We identified 79 patients in our cohort and 61 (77.2%) had abnormal placentas. There was no significant difference between the two groups in any demographic or clinical variables. For cognitive and motor performance, without adjusting for the covariates, infants with placental abnormalities had significantly lower scores compared to infants without (p = 0.026, p = 0.045 respectively). Conversely, there was no significant difference in language scores between the two groups (p = 0.12). Placenta abnormalities are common in patients with CCHD, and placenta abnormalities are associated with impaired neurodevelopmental outcomes. These results underscore the complex causal pathways of neurodevelopmental impairment in infants with CCHD and offer opportunities for targeted postnatal developmental interventions after discharge.

Is native aortic valvuloplasty at time of Norwood operation in infants with hypoplastic left heart syndrome and aortic stenosis safe?

In hypoplastic left heart syndrome, the size and function of the left ventricle vary and are dependent on the patency of the aortic valve. A patent native aortic valve, permitting left ventricular ejection, can augment cardiac output. We performed a retrospective chart review of patients with hypoplastic left heart syndrome and a stenotic aortic valve who underwent native aortic valvuloplasty at the time of Norwood and found that none of the eight patients identified had clinically significant aortic insufficiency. This case series suggests that surgical aortic valvuloplasty at Norwood is associated with aortic valve patency/augmented systemic cardiac output without the development of clinically significant aortic regurgitation at intermediate follow-up in a limited cohort.

Physiological Approach to Sodium Supplementation in Preterm Infants.

OBJECTIVE: To implement and evaluate a clinical practice algorithm to identify preterm infants with sodium deficiency and guide sodium supplementation based on urine sodium concentrations. STUDY DESIGN: Urine sodium concentration was measured in infants born at 260/7 to 296/7 weeks' gestation at 2-week intervals. Sodium supplementation was based on the urine sodium algorithm. Growth and respiratory outcomes in this cohort were compared with a matched cohort cared for in our neonatal intensive care unit prior to algorithm implementation (2014-2015 cohort). RESULTS: Data were compared for 50 infants in the 2014-2015 cohort and 40 infants in the 2016 cohort. Urine sodium concentration met criteria for supplementation in 75% of the 2016 cohort infants within the first 4 weeks after birth. Average daily sodium intake was greater in the 2016 cohort compared with the 2014-2015 cohort (p < 0.05). Caloric, protein, and total fluid intakes were similar between cohorts. The change in weight Z-score between 2 and 8 weeks of age was significantly greater in the 2016 versus 2014-2015 cohort (0.32 ± 0.05 vs. -0.01 ± 0.08; p < 0.01). No impact on respiratory status at 28 days of age or 36 weeks of postmenstrual age was identified. CONCLUSION: Institution of a clinical practice algorithm to instruct clinicians on sodium supplementation in preterm infants may improve growth outcomes.