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Objectives: Vulnerability to brain injury in infants with congenital heart disease (CHD) may differ by CHD lesion. However, the link between arterial ischemic stroke (AIS) and neurodevelopment, and whether it differs by CHD lesion, is unknown. We sought to determine whether: (1) Stroke topology differs between infants with Transposition of the Great Arteries (TGA) and single ventricle physiology (SVP), and relationship with neurodevelopment;(2) Associations between stroke volume and neurodevelopment are CHD lesion specific. Methods: 64 of 312 CHD infants (TGA n=38, SVP n=26) studied prospectively with pre- and/or post-operative brain MRIs had AIS. AIS were segmented on 3DT1 and/or ADC images. 39 infants completed 18-month neurodevelopmental assessments with Bayley Scales (2 nd or 3 rd Edition); scores were adjusted to account for differences between versions. Adverse neurodevelopment was defined as Results: Most AIS were in MCA territories, with a left-sided predominance (Fig 1A). Stroke volume did not differ between CHD groups (p=0.8). Basal ganglia lesions were most predictive of cognitive (max OR=11) and motor (max OR=6) outcomes (Fig 1B). Stratifying by CHD lesion, AIS volume predicted 18-month cognitive outcomes in infants with TGA (β=-0.6, 95%CI -1.0-(-0.1), p=0.02) but not SVP (β=7.9, 95%CI -72-88). AIS volume did not predict motor outcomes in infants with TGA (β=-0.6, 95%CI -1.6-0.3) or SVP (β=-19.7, 95%CI -91-52). Conclusions: Neonatal AIS topology does not differ between infants with TGA and SVP. AIS location and size are important predictors of neurodevelopment at 18 months though this association differs by CHD lesion.

Introduction: Data suggests there are benefits to both staged (SR) and complete repair (CR) for neonatal Tetralogy of Fallot (nTOF), though data is lacking on whether high-risk groups would benefit from one strategy over another. In a large state with variable practice patterns, we studied the impact of gestational age (GA) and weight by GA (birth weight z-score: BWz) at birth on surgical strategy and outcomes in nTOF, hypothesizing that GA and BWz would influence these outcomes. Methods: Population-based cohort study (2011-2017) of infants with nTOF (defined as catheter or surgical intervention prior to 44 weeks corrected GA) was performed. BWz was categorized as appropriate, small or large for GA. Surgical strategy and timing were compared by GA ( < 36 weeks preterm (n= 96), 37-38 weeks early term (n= 97), > 38 weeks full term (n= 152)) and BWz. Multivariable (MV) models were constructed to evaluate composite outcome of mortality or significant morbidity (BPD, IVH, NEC, PVL etc.) at 1 year and total number of hospital days in first year of life. Results: There were 345 patients included (SR= 194; CR= 151). Surgical strategy did not differ by GA or BWz. Within CR group, full term and early term neonates underwent surgery 20 (95%CI: -27.1, -14.1; p Conclusions: In nTOF, GA and BWz were not associated with surgical strategy though premature neonates were more likely to wait longer for their initial intervention. One-year outcomes appear to be strongly influenced by GA and not surgical strategy; however, there was a trend to suggest that SR may result in better survival probability among premature neonates.

A nearly 4-year-old Ethiopian girl with a 1-year history of weight loss and rapid breathing who had just immigrated to the United States presented to the emergency department with respiratory distress and increasing lethargy. Over the preceding year, she had been found to have increasing tachypnea, anorexia, a 5-kilogram weight loss, fatigue, intermittent emesis, and decreased urine output. There were multiple notable findings on the physical examination. Abnormalities of the vital signs included a respiratory rate of 52 breaths per minute, blood pressure of 70/53, and an oxygen saturation of 85% on room air. In general, she was cachectic and lethargic. There was jugular venous distension with accentuated a waves. There were diffuse fine inspiratory crackles across the lung fields. There was asymmetrical protrusion of the left anterior chest with a visible, dynamic cardiac impulse. On auscultation, there was a regular rhythm with an S3 present. There was a III/VI, holosystolic, midfrequency, blowing murmur in the 5th intercostal space at the midclavicular line with radiation to the apex and the left axilla. A diastolic rumble was not appreciated. The liver edge was 4 centimeters below the right costal margin. Pulses were normal at the brachia and femoral arteries, bilaterally. There was no peripheral edema or clubbing. Blood work revealed a negative troponin and a B natriuretic peptide of 2761 picograms per milliliter. A chest radiograph was performed, which showed cardiomegaly with enlargement of the left superior cardiac border, bilateral pulmonary edema, and superior deviation of the left mainstem bronchus suggestive of left atrial dilatation (Figure 1). A 15-lead electrocardiogram demonstrated sinus rhythm with left atrial enlargement and ST segment elevation in the anterior precordial leads (Figure 2). Figure 1. A chest radiograph taken in an anteroposterior projection demonstrates: pronounced cardiomegaly with a cardiothoracic ratio of 71%; a double contour of …

Objective: Literature suggests that prenatal diagnosis of critical congenital heart disease does not lead to improved mortality. However, it has been associated with better pre-operative conditions in single ventricle lesions and better neurodevelopmental outcome in transposition of the great arteries (TGA). We sought to describe the frequency of pre-operative brain injury in neonates with prenatal and postnatal diagnosis of TGA. We hypothesize that prenatally diagnosed neonates with TGA have a lower prevalence of pre-operative brain injury. Methods: Term newborns with TGA were studied with brain magnetic resonance imaging preoperatively to determine brain injury severity based on the presence of white matter injury and/or stroke. Comparisons were made between prenatal and postnatal diagnosis. Other variables compared included estimated gestational age at birth (EGA), birth weight (BW), lowest oxygen saturation, balloon atrial septostomy (BAS), age at surgery, and hospital mortality. Analysis was performed with Fisher’s exact test or Student t-test. Further analysis will be performed including data from other centers and cardiac diagnoses. Results: The presence of brain injury was significantly higher in postnatally diagnosed neonates than those prenatally diagnosed (Table). There were trends toward lower EGA, lower BW, higher oxygen saturation and younger age at surgery in the prenatal group. BAS did not differ by prenatal diagnosis. Mortality was low and the only two deaths were in the postnatal group. Conclusions: Prenatally diagnosed neonates with TGA have a lower prevalence of preoperative brain injury as compared to those diagnosed postnatally. This finding is significant even when other known risk factors for brain injury did not differ between groups. Our data is consistent with prior literature demonstrating lower EGA and BW in prenatally diagnosed patients. The impact of these findings on neurodevelopmental outcomes will be studied.