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OBJECTIVES Congenital diaphragmatic hernia (CDH) can cause a significant mass effect in the fetal thorax, displacing the heart into the opposite hemithorax. In left-sided CDH (L-CDH), this is associated with smaller left-sided cardiac structures and reduced left-ventricular cardiac output (LVCO). The effect of these physiologic changes on cerebral blood flow is not well understood. We sought to describe the middle cerebral artery (MCA) pulsatility index (PI), a measure of cerebrovascular impedance, in fetuses with L-CDH and those with right-sided CDH (R-CDH) compared with unaffected fetuses, and the relationship between MCA-PI and LVCO. We hypothesized that MCA-PI would be lower in fetuses with L-CDH and similar in those with R-CDH compared to controls, and that MCA-PI would be correlated with LVCO. METHODS We identified all fetuses with CDH evaluated at The University of California San Francisco, San Francisco, CA, USA from 2011 to 2018. Fetal echocardiograms and ultrasound scans were reviewed. Umbilical artery and MCA Doppler examinations were assessed to calculate pulsatility indices. Ventricular outputs were calculated using Doppler-derived stroke volume and fetal heart rate. Lung-to-head ratio (LHR), estimated fetal weight, biparietal diameter (BPD) and head circumference (HC) were obtained from fetal sonograms. Measurements in fetuses with CDH, according to the side of the defect, were compared with those in unaffected, gestational age-matched controls. A subset of CDH survivors had available data on neurodevelopmental outcome, as assessed using the Bayley Scales of Infant Development, 3rd edition. RESULTS A total of 64 fetuses with CDH (L-CDH, n = 53; R-CDH, n = 11) comprised the study groups, with 27 unaffected fetuses serving as controls. Mean gestational age at evaluation was similar between the three groups. Compared to controls, fetuses with L-CDH had significantly lower LVCO expressed as a percentage of combined cardiac output (CCO) (32%; 95% CI, 29-35% vs 38%; 95% CI, 33-42%; P = 0.04) and lower MCA-PI Z-score (-1.3; 95% CI, -1.7 to -1.0 vs 0.08; 95% CI, -0.5 to 0.6; P

Graphical abstract, Highlights • A PAVM was diagnosed in a critically ill cyanotic neonate. • Echocardiography played a pivotal role in establishing the diagnosis. • Urgent transcatheter device occlusion resulted in immediate hemodynamic stabilization.

ObjectiveTo test the hypothesis that delayed brain development in fetuses with d-transposition of the great arteries or hypoplastic left heart syndrome heightens their postnatal susceptibility to acquired white matter injury.MethodsThis is a cohort study across 3 sites. Subjects underwent fetal (third trimester) and neonatal preoperative magnetic resonance imaging of the brain to measure total brain volume as a measure of brain maturity and the presence of acquired white matter injury after birth. White matter injury was categorized as no-mild or moderate-severe based on validated grading criteria. Comparisons were made between the injury groups.ResultsA total of 63 subjects were enrolled (d-transposition of the great arteries: 37; hypoplastic left heart syndrome: 26). White matter injury was present in 32.4% (n=12) of d-transposition of the great arteries and 34.6% (n=8) of those with hypoplastic left heart syndrome. Overall total brain volume (taking into account fetal and neonatal scan) was significantly lower in those with postnatal moderate-severe white matter injury compared with no-mild white matter injury after adjusting for age at scan and site in d-transposition of the great arteries (coefficient: 14.8mL, 95% confidence interval, -28.8 to -0.73, P=.04). The rate of change in total brain volume from fetal to postnatal life did not differ by injury group. In hypoplastic left heart syndrome, no association was noted between overall total brain volume and change in total brain volume with postnatal white matter injury.ConclusionsLower total brain volume beginning in late gestation is associated with increased risk of postnatal moderate-severe white matter injury in d-transposition of the great arteries but not hypoplastic left heart syndrome. Rate of brain growth was not a risk factor for white matter injury. The underlying fetal and perinatal physiology has different implications for postnatal risk of white matter injury.

Background Prenatal diagnosis of congenital heart disease has been associated with early‐term delivery and cesarean delivery (CD). We implemented a multi‐institutional standardized clinical assessment and management plan (SCAMP) through the University of California Fetal‐Maternal Consortium. Our objective was to decrease early‐term (37–39 weeks) delivery and CD in pregnancies complicated by fetal congenital heart disease using a SCAMP methodology to improve practice in a high‐risk and clinically complex setting. Methods and Results University of California Fetal‐Maternal Consortium site‐specific management decisions were queried following SCAMP implementation. This contemporary intervention group was compared with a University of California Fetal‐Maternal Consortium historical cohort. Primary outcomes were early‐term delivery and CD. A total of 496 maternal–fetal dyads with prenatally diagnosed congenital heart disease were identified, 185 and 311 in the historical and intervention cohorts, respectively. Recommendation for later delivery resulted in a later gestational age at delivery (38.9 versus 38.1 weeks, P =0.01). After adjusting for maternal age and site, historical controls were more likely to have a CD (odds ratio [OR],1.8; 95% CI, 2.1–2.8; P =0.004) and more likely (OR, 2.1; 95% CI, 1.4–3.3) to have an early‐term delivery than the intervention group. Vaginal delivery was recommended in 77% of the cohort, resulting in 61% vaginal deliveries versus 50% in the control cohort ( P =0.03). Among pregnancies with major cardiac lesions (n=373), vaginal birth increased from 51% to 64% ( P =0.008) and deliveries ≥39 weeks increased from 33% to 48% ( P =0.004). Conclusions Implementation of a SCAMP decreased the rate of early‐term deliveries and CD for prenatal congenital heart disease. Development of clinical pathways may help standardize care, decrease maternal risk secondary to CD, improve neonatal outcomes, and reduce healthcare costs.

Background Tetralogy of Fallot with absent pulmonary valve is associated with high mortality, but it remains difficult to predict outcomes prenatally. We aimed to identify risk factors for mortality in a large multicenter cohort. Methods and Results Fetal echocardiograms and clinical data from 19 centers over a 10‐year period were collected. Primary outcome measures included fetal demise and overall mortality. Of 100 fetuses, pregnancy termination/postnatal nonintervention was elected in 22. Of 78 with intention to treat, 7 (9%) died in utero and 21 (27%) died postnatally. With median follow‐up of 32.9 months, no deaths occurred after 13 months. Of 80 fetuses with genetic testing, 46% had chromosomal abnormalities, with 22q11.2 deletion in 35%. On last fetal echocardiogram, at a median of 34.6 weeks, left ventricular dysfunction independently predicted fetal demise (odds ratio [OR], 7.4; 95% CI 1.3, 43.0; P =0.026). Right ventricular dysfunction independently predicted overall mortality in multivariate analysis (OR, 7.9; 95% CI 2.1–30.0; P =0.002). Earlier gestational age at delivery, mediastinal shift, left ventricular/right ventricular dilation, left ventricular dysfunction, tricuspid regurgitation, and Doppler abnormalities were associated with fetal and postnatal mortality, although few tended to progress throughout gestation on serial evaluation. Pulmonary artery diameters did not correlate with outcomes. Conclusions Perinatal mortality in tetralogy of Fallot with absent pulmonary valve remains high, with overall survival of 64% in fetuses with intention to treat. Right ventricular dysfunction independently predicts overall mortality. Left ventricular dysfunction predicts fetal mortality and may influence prenatal management and delivery planning. Mediastinal shift may reflect secondary effects of airway obstruction and abnormal lung development and is associated with increased mortality.

Objective To investigate the trends of 1-year mortality and neonatal morbidities in preterm infants with serious congenital heart disease (CHD). Study design This cohort study used a population-based administrative dataset of all liveborn infants of 26-36 weeks gestational age with serious CHD born in California between 2011 and 2017. We assessed 1-year mortality and major neonatal morbidities (ie, retinopathy of prematurity, bronchopulmonary dysplasia, necrotizing enterocolitis, intraventricular hemorrhage grade >2, and periventricular leukomalacia) across the study period and compared these outcomes with those in infants without CHD. Results We identified 1921 preterm infants with serious CHD. The relative risk (RR) of death decreased by 10.6% for each year of the study period (RR, 0.89; 95% CI, 0.84-0.95), and the RR of major neonatal morbidity increased by 8.3% for each year (RR, 1.08; 95% CI, 1.02-1.15). Compared with preterm neonates without any CHD (n = 234 522), the adjusted risk difference (ARD) for mortality was highest at 32 weeks of gestational age (9.7%; 95% CI, 8.3%-11.2%), that for major neonatal morbidity was highest at 28 weeks (21.9%; 95% CI, 17.0%-26.9%), and that for the combined outcome was highest at 30 weeks (26.7%; 95% CI, 23.3%-30.1%). Conclusions Mortality in preterm neonates with serious CHD decreased over the last decade, whereas major neonatal morbidities increased. Preterm infants with a gestational age of 28-32 weeks have the highest mortality or morbidity compared with their peers without CHD. These results support the need for specialized and focused medical neonatal care in preterm neonates with serious CHD.

OBJECTIVE Fetal cerebrovascular resistance is influenced by several factors in the setting of intact autoregulation to allow for normal cerebral blood flow and oxygenation. Maternal hyperoxygenation (MH) allows for acute alterations in fetal physiology and can be a tool to test cerebrovascular reactivity in late-gestation fetuses. In this study, we utilized MH to evaluate cerebrovascular reactivity in fetuses with specific congenital heart disease (CHD). METHODS This was a cross-sectional study of fetuses with complex CHD compared to controls without CHD. CHD cases were grouped according to physiology into: left-sided obstructive lesion (LSOL), right-sided obstructive lesion (RSOL) or dextro-transposition of the great arteries (d-TGA). Subjects underwent MH testing during the third-trimester fetal echocardiogram. The pulsatility index (PI) was calculated for the fetal middle cerebral artery (MCA), umbilical artery (UA) and branch pulmonary artery (PA). The change in PI from baseline to during MH was compared between each CHD group and controls. RESULTS Sixty pregnant women were enrolled (CHD, n = 43; control, n = 17). In the CHD group, there were 27 fetuses with LSOL, seven with RSOL and nine with d-TGA. Mean gestational age was 33.9 (95% CI, 33.6-34.2) weeks. At baseline, MCA-PI Z-score was lowest in the LSOL group (-1.8 (95% CI, -2.4 to -1.2)) compared with the control group (-0.8 (95% CI, -1.3 to -0.3)) (P = 0.01). In response to MH, MCA-PI Z-score increased significantly in the control and d-TGA groups but did not change significantly in the LSOL and RSOL groups. The change in MCA-PI Z-score was significantly higher in the control group than in the LSOL group (0.9 (95% CI, 0.42-1.4) vs 0.12 (95% CI, -0.21 to 0.45); P = 0.03). This difference was more pronounced in the LSOL subgroup with retrograde aortic arch flow. Branch PA-PI decreased significantly in response to MH in all groups, with no difference in the change from baseline to MH between the groups, while UA-PI was unchanged during MH compared with at baseline. CONCLUSIONS The fetal cerebrovascular response to MH varies based on the underlying CHD diagnosis, suggesting that cardiovascular physiology may influence the autoregulatory capacity of the fetal brain. Further studies are needed to determine the clinical implications of these findings on long-term neurodevelopment in these at-risk children. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.

Background: Prenatal detection (PND) has benefits for infants with hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but associations between sociodemographic and geographic factors with PND have not been sufficiently explored. This study evaluated whether socioeconomic quartile (SEQ), public insurance, race and ethnicity, rural residence, and distance of residence (distance and driving time from a cardiac surgical center) are associated with the PND or timing of PND, with a secondary aim to analyze differences between the United States and Canada. Methods: In this retrospective cohort study, fetuses and infants z score using the neighborhood summary score or Canadian Chan index and separated into quartiles. Insurance type and self-reported race and ethnicity were obtained from medical charts. We evaluated associations among SEQ, insurance type, race and ethnicity, rural residence, and distance of residence with PND of HLHS and TGA (aggregate and individually) using bivariate analysis with adjusted associations for confounding variables and cluster analysis for centers. Results: Data on 1862 subjects (HLHS: n=1171, 92% PND; TGA: n=691, 58% PND) were submitted by 21 centers (19 in the United States). In the United States, lower SEQ was associated with lower PND in HLHS and TGA, with the strongest association in the lower SEQ of pregnancies with fetal TGA (quartile 1, 0.78 [95% CI, 0.64–0.85], quartile 2, 0.77 [95% CI, 0.64–0.93], quartile 3, 0.83 [95% CI, 0.69–1.00], quartile 4, reference). Hispanic ethnicity (relative risk, 0.85 [95% CI, 0.72–0.99]) and rural residence (relative risk, 0.78 [95% CI, 0.64–0.95]) were also associated with lower PND in TGA. Lower SEQ was associated with later PND overall; in the United States, rural residence and public insurance were also associated with later PND. Conclusions: We demonstrate that lower SEQ, Hispanic ethnicity, and rural residence are associated with decreased PND for TGA, with lower SEQ also being associated with decreased PND for HLHS. Future work to increase PND should be considered in these specific populations.

Poor and asymmetric fetal growth have been associated with neonatal brain injury (BI) and worse neurodevelopmental outcomes (NDO) in the growth-restricted population due to placental insufficiency. We tested the hypothesis that postnatal markers of fetal growth (birthweight (BW), head circumference (HC), and head to body symmetry) are associated with preoperative white matter injury (WMI) and NDO in infants with single ventricle physiology (SVP) and d-transposition of great arteries (TGA). 173 term newborns (106 TGA; 67 SVP) at two sites had pre-operative brain MRI to assess for WMI and measures of microstructural brain development. NDO was assessed at 30 months with the Bayley Scale of Infant Development-II (n = 69). We tested the association between growth parameters at birth with the primary outcome of WMI on the pre-operative brain MRI. Secondary outcomes included measures of NDO. Newborns with TGA were more likely to have growth asymmetry with smaller heads relative to weight while SVP newborns were symmetrically small. There was no association between BW, HC or asymmetry and WMI on preoperative brain MRI or with measures of microstructural brain development. Similarly, growth parameters at birth were not associated with NDO at 30 months. In a multivariable model only cardiac lesion and site were associated with NDO. Unlike other high-risk infant populations, postnatal markers of fetal growth including head to body asymmetry that is common in TGA is not associated with brain injury or NDO. Lesion type appears to play a more important role in NDO in CHD.

Background Impairments in fetal oxygen delivery have been implicated in brain dysmaturation seen in congenital heart disease (CHD), suggesting a role for in utero transplacental oxygen therapy. We applied a novel imaging tool to quantify fetal cerebral oxygenation by measuring T2* decay. We compared T2* in fetuses with CHD with controls with a focus on cardiovascular physiologies (transposition or left‐sided obstruction) and described the effect of brief administration of maternal hyperoxia on T2* decay. Methods and Results This is a prospective study performed on pregnant mothers with a prenatal diagnosis of CHD compared with controls in the third trimester. Participants underwent a fetal brain magnetic resonance imaging scan including a T2* sequence before and after maternal hyperoxia. Comparisons were made between control and CHD fetuses including subgroup analyses by cardiac physiology. Forty‐four mothers (CHD=24, control=20) participated. Fetuses with CHD had lower total brain volume (238.2 mm 3 , 95% CI, 224.6–251.9) compared with controls (262.4 mm 3 , 95% CI, 245.0–279.8, P =0.04). T2* decay time was faster in CHD compared with controls (beta=−14.4, 95% CI, −23.3 to −5.6, P =0.002). The magnitude of change in T2* with maternal hyperoxia was higher in fetuses with transposition compared with controls (increase of 8.4 ms, 95% CI, 0.5–14.3, P =0.01), though between‐subject variability was noted. Conclusions Cerebral tissue oxygenation is lower in fetuses with complex CHD. There was variability in the response to maternal hyperoxia by CHD subgroup that can be tested in future larger studies. Cardiovascular physiology is critical when designing neuroprotective clinical trials in the fetus with CHD.

Background: Neonates with congenital heart disease are more likely to be small for gestational age. Few studies have investigated the effect of birth weight Z-score on outcomes after congenital heart surgery. Methods: Patients from the Society of Thoracic Surgeons Congenital Heart Surgery Database (2010 to 2016) undergoing cardiac surgery at a corrected gestational age ≤ 44 weeks were included, and classified as severely (birth weight Z-score -4 to -2), moderately (Z-score between -2 and -1) and mildly growth restricted (Z-score -1.0 to -0.5) and compared to a reference (Z-score 0 to 0.5). Multivariable logistic regression clustering on center was used to evaluate the association of birth weight Z-score with operative mortality, postoperative complications and length of stay, adjusting for other patient characteristics. Interaction between Z-score for birth weight and gestational age was assessed. Results: In 25,244 patients, operative mortality was 8.6% and major complications occurred in 19.4%. Compared to the reference group with no growth restriction, the adjusted odds (AOR) of mortality was increased in infants with severe (AOR 2.4, CI 2.0-3.0), moderate (AOR 1.7, CI 1.4-2.0) and mild growth restriction (AOR 1.4, CI 1.2-1.6). The AOR for major postoperative complications was increased for infants with severe (AOR 1.4, CI 1.2-1.7) and moderate growth restriction (AOR 1.2, CI 1.1-1.4), but not in mildly growth restricted infants (AOR 1.0, CI 0.9-1.2). Length of stay was prolonged for all growth restricted cohorts (adjusted Hazard Ratio38 weeks) and then preterm infants ( Conclusions: Even birth weight Z-scores that are slightly below average are independent risk factors for mortality and morbidity in neonates undergoing cardiac surgery. The strongest association between poor fetal growth and operative mortality exists in early-term neonates. These novel findings may account for some of the previously unexplained variation in cardiac surgical outcomes.

BACKGROUND The development of congenital heart disease ( CHD ) is multifactorial with genetic and environmental influences. We sought to determine the relationship between socioeconomic and environmental factors with the incidence of CHD among live‐born infants in California and to determine whether maternal comorbidities are in the causal pathway. METHODS AND RESULTS This was a population‐based cohort study in California (2007–2012). The primary outcome was having significant CHD . Predictors included socioeconomic status and environmental exposure to pollutants determined by U.S. Census data. A social deprivation index and environmental exposure index was assigned based on neighborhood socioeconomic variables, categorized into 4 quartiles. Quartile 1 was the best with the least exposure to pollutants and social deprivation, and quartile 4 was the worst. Multivariate logistic regression and mediation analyses were performed. Among 2 419 651 live‐born infants, the incidence of CHD was 3.2 per 1000 live births. The incidence of CHD was significantly higher among those in quartile 4 compared with quartile 1 (social deprivation index: 0.35% versus 0.29%; odds ratio [OR], 1.31; 95% CI, 1.21–1.41; environmental exposure index: 0.35% versus 0.29%; OR, 1.23; 95% CI, 1.15–1.31) after adjusting for maternal race/ethnicity and age and accounting for the relationship between the 2 primary predictors. Maternal comorbidities explained 13% (95% CI, 10%–20%) of the relationship between social deprivation index and environmental exposure index with the incidence of CHD . CONCLUSIONS Increased social deprivation and exposure to environmental pollutants are associated with the incidence of live‐born CHD in California. Maternal comorbidities explain some, but not all, of this relationship. These findings identify targets for social policy initiatives to minimize health disparities.

BACKGROUND:Brain injury (BI) is reported in 60% of newborns with critical congenital heart disease as white matter injury (WMI) or stroke. Neurodevelopmental (ND) impairments are reported in these patients. The relationship between neonatal BI and ND outcome has not been established. OBJECTIVES:This study sought to determine the association between peri-operative BI and ND outcomes in infants with single ventricle physiology (SVP) and d-transposition of the great arteries (d-TGA). METHODS:Term newborns with d-TGA and SVP had pre-operative and post-operative brain magnetic resonance imaging and ND outcomes assessed at 12 and 30months with the Bayley Scales of Infant Development-II. BI was categorized by the brain injury severity score and WMI was quantified by volumetric analysis. RESULTS:A total of 104 infants had follow-up at 12months and 70 had follow-up at 30months. At 12months, only clinical variables were associated with ND outcome. At 30months, subjects with moderate-to-severe WMI had significantly lower Psychomotor Development Index (PDI) scores (13 points lower) as compared with those with none or minimal WMI for d-TGA and SVP (p=0.03 and p=0.05, respectively) after adjusting for various factors. Quantitative WMI volume was likewise associated. Stroke was not associated with outcome. The Bland-Altman limits of agreement for PDI scores at 12 and 30months were wide (-40.3 to 31.2) across the range of mean PDI values. CONCLUSIONS:Increasing burden of WMI is associated with worse motor outcomes at 30months for infants with criticalcongenital heart disease, whereas no adverse association was seen between small strokes and outcome. Theseresults support the utility of neonatal brain magnetic resonance imaging in this population to aid in predictinglateroutcomes and the importance of ND follow-up beyond 1 year of age.

Objective We hypothesized that infants with fetal growth restrictions have increased mortality and morbidity after congenital heart disease surgery. Methods The study included patients in The Society of Thoracic Surgeons Congenital Heart Surgery Database (2010-2016) who underwent cardiac surgery at a corrected gestational age of ≤44 weeks. Patients were classified as severely (birth weight Z-score −4 to −2), moderately (Z-score −2 to −1), and mildly growth restricted (Z-score −1.0 to −0.5) and compared with a reference population (Z-score 0-0.5). Multivariable logistic regression clustering on center was used to evaluate the association of birth weight Z-score with operative mortality and postoperative complications and its interaction with gestational age was assessed. Results In 25,244 patients, operative mortality was 8.6% and major complications occurred in 19.4%. Compared with the reference group, the adjusted odds ratio (AOR) of mortality was increased in infants with severe (AOR, 2.4; 95% confidence interval [CI], 2.0-3.0), moderate (AOR, 1.7; 95% CI, 1.4-2.0), and mild growth restriction (AOR, 1.4; 95% CI, 1.2-1.6). The AOR for major postoperative complications was increased for severe (AOR, 1.4; 95% CI, 1.2-1.7) and moderate growth restriction (AOR, 1.2; 95% CI, 1.1-1.4). There was significant interaction between birth weight Z-score and gestational age (P = .007). Conclusions Even birth weight Z-scores slightly below average are independent risk factors for mortality and morbidity in infants who undergo cardiac surgery. The strongest association between poor fetal growth and operative mortality exists in early-term infants. These novel findings might account for some of the previously unexplained variation in cardiac surgical outcomes.

Background Infants with critical congenital heart disease ( CCHD ) are more likely to be small for gestational age ( SGA ) or born to mothers with maternal placental syndrome. The objective of this study was to investigate the relationship between maternal placental syndrome, SGA , and gestational age ( GA ) on 1‐year mortality in infants with CCHD . Methods and Results In a population‐based administrative database of all live‐born infants in California (2007–2012) we identified all infants with CCHD without chromosomal anomalies. Our primary predictor was an impaired fetal environment ( IFE ), defined as presence of maternal placental syndrome or SGA . We calculated hazard ratios to quantify the association between different components of IFE and 1‐year mortality and conducted a causal mediation analysis to assess GA at birth as a mediator. We identified 6863 infants with CCHD . IFE was present in 25.1%. Infants with IFE were more likely to die than infants without IFE (16.6% versus 11.1%; hazard ratios 1.55, 95% CI 1.34–1.78). Only SGA (hazard ratios 1.76, 95% CI 1.50–2.05) and placental abruption (hazard ratios 1.70, 95% CI 1.17–2.48) were significantly associated with mortality; preeclampsia and gestational hypertension had no significant association with mortality. The mediation analysis showed that 32.8% (95% CI 24.9–47.0%) of the relationship between IFE and mortality is mediated through GA . Conclusions IFE is a significant contributor to outcomes in the CCHD population. SGA and placental abruption are the main drivers of postnatal mortality while other maternal placental syndrome components had much less of an impact. Only one third of the effect between IFE and mortality is mediated through GA .

Background Tube‐assisted feeding in infancy is common in patients with single‐ventricle physiology ( SVP ). Postnatal brain development is delayed, and injury is common, in patients with SVP . The role of brain findings in feeding outcomes remains unclear. We sought to determine the association between neonatal perioperative brain injury and postnatal brain maturation with feeding‐tube dependency in patients with SVP at neonatal discharge and just before the stage‐2 palliation. Methods and Results We evaluated a cohort of 48 term neonates with SVP who underwent pre‐ and postoperative brain magnetic resonance imaging. Perioperative brain injury and microstructural brain development were measured with diffusion tensor imaging including fractional anisotropy in white matter and apparent diffusion coefficient in gray matter. The primary outcome was defined as being 100% orally fed (binary). Of the patients 79% (38/48) were tube fed at hospital discharge, and 27% (12/45) were tube fed before stage‐2 palliation. Perioperative brain injury did not differ by group. Orally fed patients had a faster rate of decrease in apparent diffusion coefficient (3%, 95% CI 1.7% to 4.6%, P CI 0.6% to 2.2%, P =0.001) at the time of stage‐2 palliation compared with tube‐fed patients, denoting more robust brain development. Conclusions Slower rate of postnatal brain maturation but not perioperative brain injury is associated with feeding modality in infancy. These results support the importance of brain health in optimizing feeding outcomes in patients with SVP .

Background Neonates with congenital heart disease (CHD) are at high risk of punctate white matter injury (WMI) and impaired brain development. We hypothesized that WMI in CHD neonates occurs in a characteristic distribution that shares topology with preterm WMI and that lower birth gestational age (GA) is associated with larger WMI volume. Objective (1) To quantitatively assess the volume and location of WMI in CHD neonates across three centres. (2) To compare the volume and spatial distribution of WMI between term CHD neonates and preterm neonates using lesion mapping. Methods In 216 term born CHD neonates from three prospective cohorts (mean birth GA: 39 weeks), WMI was identified in 86 neonates (UBC: 29; UCSF: 43; UCZ: 14) on pre- and/or post-operative T1 weighted MRI. WMI was manually segmented and volumes were calculated. A standard brain template was generated. Probabilistic WMI maps (total, pre- and post-operative) were developed in this common space. Using these maps, WMI in the term CHD neonates was compared with that in preterm neonates: 58 at early-in-life (mean postmenstrual age at scan 32.2 weeks); 41 at term-equivalent age (mean postmenstrual age at scan 40.1 weeks). Results The total WMI volumes of CHD neonates across centres did not differ (p = 0.068): UBC (median = 84.6 mm3, IQR = 26–174.7 mm3); UCSF (median = 104 mm3, IQR = 44–243 mm3); UCZ (median = 121 mm3, IQR = 68–200.8 mm3). The spatial distribution of WMI in CHD neonates showed strong concordance across centres with predilection for anterior and posterior rather than central lesions. Predominance of anterior lesions was apparent on the post-operative WMI map relative to the pre-operative map. Lower GA at birth predicted an increasing volume of WMI across the full cohort (41.1 mm3 increase of WMI per week decrease in gestational age; 95% CI 11.5–70.8; p = 0.007), when accounting for centre and heart lesion. While WMI in term CHD and preterm neonates occurs most commonly in the intermediate zone/outer subventricular zone there is a paucity of central lesions in the CHD neonates relative to preterms. Conclusions WMI in term neonates with CHD occurs in a characteristic topology. The spatial distribution of WMI in term neonates with CHD reflects the expected maturation of pre-oligodendrocytes such that the central regions are less vulnerable than in the preterm neonates.

Socioeconomic status (SES) is associated with long term outcomes in congenital heart disease (CHD). In other high-risk populations, like prematurity, lower SES has been associated with both early (e.g. neonatal brain injury) and later neurodevelopmental (ND) outcomes. Since this has not been

This review aims to describe how the complex events of cardiovascular transition may affect the brain of infants with congenital heart disease (CHD). In particular, we describe the vulnerabilities of the neonatal brain in the transitional period during and immediately after delivery and propose management strategies that can potentially influence neurodevelopmental outcomes in this patient population. Delayed brain development has been identified in the third trimester fetus with certain forms of CHD. Neonates with critical CHD are at risk for brain injury even before their neonatal operation. The transitional period from fetal to neonatal life is marked with hemodynamic changes that can affect cerebral blood flow and oxygenation and may exacerbate injury and influence outcome. We propose specific perinatal management strategies tailored to maternal-fetal pairs aimed at optimizing hemodynamic stability, especially cerebral blood flow and oxygenation, to maximize the neurodevelopmental potential of infants with CHD.

Background Racial/ethnic and socioeconomic disparities exist in outcomes for children with congenital heart disease. We sought to determine the influence of race/ethnicity and mediating socioeconomic factors on 1‐year outcomes for live‐born infants with hypoplastic left heart syndrome and dextro‐Transposition of the great arteries. Methods and Results The authors performed a population‐based cohort study using the California Office of Statewide Health Planning and Development database. Live‐born infants without chromosomal anomalies were included. The outcome was a composite measure of mortality or unexpected hospital readmissions within the first year of life defined as >3 (hypoplastic left heart syndrome) or >1 readmissions (dextro‐Transposition of the great arteries). Hispanic ethnicity was compared with non‐Hispanic white ethnicity. Mediation analyses determined the percent contribution to outcome for each mediator on the pathway between race/ethnicity and outcome. A total of 1796 patients comprised the cohort (n=964 [hypoplastic left heart syndrome], n=832 [dextro‐Transposition of the great arteries]) and 1315 were included in the analysis (n=477 non‐Hispanic white, n=838 Hispanic). Hispanic ethnicity was associated with a poor outcome (crude odds ratio, 1.72; 95% confidence interval [CI], 1.37–2.17). Higher maternal education (crude odds ratio 0.5; 95% CI , 0.38–0.65) and private insurance (crude odds ratio, 0.65; 95% CI , 0.45–0.71) were protective. In the mediation analysis, maternal education and insurance status explained 33.2% (95% CI , 7–66.4) and 27.6% (95% CI , 6.5–63.1) of the relationship between race/ethnicity and poor outcome, while infant characteristics played a minimal role. Conclusions Socioeconomic factors explain a significant portion of the association between Hispanic ethnicity and poor outcome in neonates with critical congenital heart disease. These findings identify vulnerable populations that would benefit from resources to lessen health disparities.

Background Infants with critical congenital heart disease ( CCHD ) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1‐year mortality separately in preterm (GA CCHD . Methods and Results Live‐born infants with CCHD and GA 22 to 42 weeks born in California 2007–2012 were included in the analysis. The primary predictor was Z score for birth weight and the primary outcome was 1‐year mortality. Multivariable logistic regression was used. Results are presented as adjusted odds ratios and 95% confidence intervals ( CIs ). The authors identified 6903 infants with CCHD . For preterm and full‐term infants, only a Z score for birth weight Z score 0–0.5, adjusted odds ratio, 2.15 [95% CI , 1.1–4.21] and adjusted odds ratio, 3.93 [95% CI , 2.32–6.68], respectively). In contrast, in early‐term infants, the adjusted odds ratios for Z scores CI , 1.93–6.04), 1.78 (95% CI , 1.12–2.83), and 2.03 (95% CI , 1.27–3.23), respectively, versus the reference group. Conclusions GA seems to modify the effect of birth weight Z score on mortality in infants with CCHD . In preterm and full‐term infants, only the most severe small‐for‐GA infants ( Z score Z score

See Article by Vigneswaran et al Fetal echocardiography for disease detection is challenging. Not only are there technical obstacles—maternal body habitus, variable and often unfavorable fetal position, to name a few—but in most obstetric and many cardiology practices, the vast majority of fetuses will be normal. Large numbers are referred for cardiac imaging either as part of routine screening because of risk factors or after an abnormal screen, such as nuchal translucency above 95th percentile; only a small subset will be abnormal. Add to this that nearly all fetal cardiac abnormalities are asymptomatic in fetal life because of the presence of the fetal shunts and the ability of the fetoplacental circulation to redirect flow while maintaining oxygen delivery in a normal range. Hence, a subtle abnormality becomes easy to overlook, a valve or vessel that is just barely subjectively abnormal may incorrectly pass muster in a busy clinic or be passed off as a poor image blamed on the operator or even the fetus. But as imaging platforms and operator expertise have been improving over the decades, so too has our understanding that even apparently minor deviations from normal in early to mid-gestation may have dire consequences for the newborn and the need to accurately and precisely assign a value of normal versus abnormal even for seemingly minor lesions is moving into mainstream.1 Here, though, the fetal echocardiographer encounters another issue, that of the constantly changing ruler by which to judge the fetal cardiac structure dimensions throughout gestation. Luckily, this issue is familiar to pediatric cardiologists: cardiac structures vary with patient size, and patient size varies with patient age in predictable, model-able ways. The most common method for expressing a given patient’s valve and vessel dimensions is to transform a measurement to a Z score or the number of …

OBJECTIVE: We tested the hypothesis that chronic fetal hypoxia, at a severity present in many types of congenital heart disease, would lead to abnormal neurodevelopment. METHODS: Eight mid-gestation fetal sheep were cannulated onto a pumpless extracorporeal oxygenator via the umbilical vessels and supported in a fluid-filled environment for 22 ± 2 days under normoxic or hypoxic conditions. Total parenteral nutrition was provided. Control fetuses (n = 7) were harvested at gestational age 133 ± 4 days. At necropsy, brains were fixed for histopathology. Neurons were quantified in white matter tracts, and the thickness of the external granular layer of the cerebellum was measured to assess neuronal migration. Capillary density and myelination were quantified in white matter. Data were analyzed with unpaired Student t tests or 1-way analysis of variance, as appropriate. RESULTS: Oxygen delivery was reduced in hypoxic fetuses (15.6 ± 1.8 mL/kg/min vs 24.3 ± 2.3 mL/kg/min, P

Copyright © 2014 ISUOG. Published by John Wiley & Sons Ltd. Objectives: Twin-reversed arterial perfusion (TRAP) sequence affects 1% of monochorionic twin pregnancies and is caused by abnormal vascular connections between a pump twin and an acardiac mass. The effects of abnormal vascular connections on cerebral vasculature in the pump twin are unknown. We hypothesize that abnormal cerebral vascular impedance, as assessed by the pulsatility index (PI), is present in pump twins and that fetal intervention alters cerebral impedance. Methods: Fetal echocardiograms performed between 2010 and 2013 in pregnancies diagnosed with TRAP (n = 19), recorded at presentation, and uncomplicated monochorionic twin pregnancies (controls, n = 18; 36 fetuses) were analyzed. In all subjects, the middle cerebral artery (MCA)-PI, combined cardiac output (CCO) and cardiothoracic ratio were calculated, and the values for cases and controls were compared. Results: The mean gestational age at the time of echocardiography was 20 weeks in both groups. MCA-PI was lower in TRAP cases than in controls (1.55 (95%CI, 1.47-1.64) vs 1.74 (95% CI, 1.65-1.82), respectively; P = 0.004). CCO in TRAP cases was mildly elevated for gestational age (199.7 (95%CI, 138.4-261.1) mL/min) compared with that of controls (131.4 (95%CI, 102.2-160.7) mL/min). In six TRAP cases with a second echocardiogram available, the mean MCA-PI increased after intervention, from 1.5 (95%CI, 1.3-1.7) to 1.8 (95% CI, 1.4-2.2). Conclusions: TRAP pump twins have lower cerebral vascular impedance than do controls, suggestive of a brain-sparing effect. MCA-PI appeared to increase in a small group of pump twins after intervention. These findings suggest a fetal cerebral autoregulatory response to a high cardiac output state that begins to change after fetal intervention. The long-term implications for neurodevelopmental outcome warrant further study.

ObjectiveTo assess the trajectory of perioperative brain growth in relationship to cardiac diagnosis and acquired brain injuries.MethodsThis was a cohort study of term neonates with hypoplastic left heart syndrome (HLHS) and d-transposition of the great arteries (d-TGA). Subjects underwent magnetic resonance imaging of the brain pre- and postoperatively to determine the severity of brain injury and total and regional brain volumes by the use of automated morphometry. Comparisons were made by cardiac lesion and injury status.ResultsA total of 79 subjects were included (49, d-TGA; 30, HLHS). Subjects with HLHS had more postoperative brain injury (55.6% vs 30.4%, P=.03) and more severe brain injury (moderate-to-severe white matter [WM] injury, P=.01). Total and regional perioperative brain growth was not different by brain injury status (either pre- or postoperative). However, subjects with moderate-to-severe WM injury had a slower rate of brain growth in WM and gray matter compared with those with no injury. Subjects with HLHS had a slower rate of growth globallyand in WM and deep gray matter as compared with d-TGA (total brain volume: 12cm3/wk vs 7cm3; WM: 2.1cm3/wk vs 0.6cm3; deep gray matter: 1.5cm3/wk vs 0.7cm3; P&nbsp

• Congenital heart disease (CHD) is a common birth defect with improved survival of neonates with complex lesions requiring heart surgery. • Neurodevelopmental (ND) abnormalities are common in school-age children and adolescents after neonatal heart surgery. • Magnetic resonance imaging (MRI) studies have demonstrated evidence of brain injury and delayed brain development, even before having a corrective operation, in patients with hypoplastic left heart syndrome and transposition of the great arteries. • Fetal MRI studies suggest this delayed development begins in the third trimester. • Aberrant fetal physiology resulting in decreased oxygen and substrate supply to the brain may result in these imaging and ND abnormalities in addition to other perioperative risk factors. • Children with complex CHD have a prevalence of pervasive but subtle cognitive problems termed the neurodevelopmental signature of complex congenital heart disease.

PurposeFetal hydrops arises from multiple disease processes and can portend a grim prognosis. We reviewed our experience with hydropic fetuses to understand relevant antenatal anatomic and physiologic predictors of survival.MethodsWe reviewed fetal ultrasounds and echocardiograms of hydropic fetuses evaluated from 1996 to 2013.ResultsOverall neonatal survival in 167 fetuses was 44% (range, 0-75%) and was influenced by the underlying disease process. The anatomic distribution of fluid varied and was not significantly different between survivors and nonsurvivors. Univariate analysis indicated that resolution of hydrops and delivery at a later gestational age were predictive of survival (OR: 5.7 (95% CI: 2.5-13.2) and OR: 1.3 (95% CI: 1.1-1.4), respectively). Fetal intervention also improved survival in some diseases. Echocardiograms were reviewed to group fetuses with similar cardiac physiology and defined categories with high or low/normal cardiothoracic ratio (CTR). Among patients with a high CTR, the cardiovascular profile score was predictive of survival (p=0.009).ConclusionSurvival in hydrops depends on the underlying disease, available fetal therapies to resolve hydrops, and the gestational age of delivery and not on the specific anatomic manifestations of hydrops. In hydropic fetuses with high CTRs, the cardiovascular profile score may be a useful prognostic indicator.

Neurodevelopmental outcomes are impaired in survivors of critical congenital heart disease (CHD) in several developmental domains including motor, cognitive and sensory outcomes. These deficits can extend into the adolescent and early adulthood years. The cause of these neurodevelopmental impairments is multi-factorial and includes patient specific risk factors, cardiac anatomy and physiology as well as brain changes seen on MRI. Advances in imaging techniques have identified delayed brain development in the neonate with critical CHD as well as acquired brain injury. These abnormalities are seen even before corrective neonatal cardiac surgery. This review focuses on describing brain changes seen on MRI in neonates with CHD, risk factors for these changes and the association with neurodevelopmental outcome. There is an emerging focus on the impact of cardiovascular physiology on brain health and the complex heart-brain interplay that influences ultimate neurodevelopmental outcome in these patients.

BACKGROUND AND OBJECTIVES: It is unknown how gestational age (GA) impacts neonatal morbidities in infants with critical congenital heart disease (CCHD). We aim to quantify GA-specific mortality and neonatal morbidity in infants with CCHD. METHODS: Cohort study using a database linking birth certificate, infant hospital discharge, readmission, and death records, including infants 22 to 42 weeks’ GA without chromosomal anomalies (2005–2012, 2 988 925 live births). The International Classification of Diseases, Ninth Revision diagnostic and procedure codes were used to define CCHD and neonatal morbidities (intraventricular hemorrhage, retinopathy, periventricular leukomalacia, chronic lung disease, necrotizing enterocolitis). Adjusted absolute risk differences (ARDs) with 95% confidence intervals (CIs) were calculated. RESULTS: We identified 6903 out of 2 968 566 (0.23%) infants with CCHD. The incidence of CCHD was highest at 29 to 31 weeks’ GA (0.9%) and lowest at 39 to 42 weeks (0.2%). Combined neonatal morbidity or mortality in infants with and without CCHD was 82.8% and 57.9% at CONCLUSIONS: Infants born with CCHD are at high risk of neonatal morbidity. Morbidity remains increased across all GA groups in comparison with infants born at 39 to 42 weeks. This substantial risk of neonatal morbidity is important to consider when caring for this patient population.

Recurrent aortic arch obstruction (RAAO) remains a major cause of morbidity following surgical neonatal repair of coarctation of the aorta (CoA). Elucidating predictors of RAAO can identify high-risk patients and guide postoperative management. The Coarctation index (CoA-I), defined as the ratio of the diameter of the narrowest aortic arch segment to the diameter of the descending aorta, has been used to help diagnose RAAO in neonates following the Norwood Procedure. We sought to assess the predictive value of the CoA-I on RAAO after CoA repair in infants with biventricular circulation. Clinical, surgical, and echocardiographic data of infants with biventricular circulation following neonatal CoA repair between 2010 and 2014 were evaluated. RAAO was defined using a composite quantitative outcome variable: a blood pressure gradient >20, a peak aortic arch velocity >3.5 m/s by echocardiogram, or a catheter-measured peak-to-peak gradient >20 within 2 years of surgery. Univariate and multivariate logistic regression analyses were used. Of the 68 subjects included in the analysis, 15 (22%) met criteria for RAAO. In the multivariate model, only CoA-I (OR 35.89, 95% CI 6.08-211.7, p

Objective Recurrence risk of congenital heart disease (CHD) in families with an affected first-degree relative is increased as compared with the general population. Advances in genetic testing and evidence that preconceptual folic acid supplementation may decrease risk of CHD warrant preventative counseling for at-risk families. Our goal was to document patterns of preconceptual folic acid supplementation and recurrence risk counseling in at-risk families in order to identify opportunities for improved preventative care. Design Mothers referred for a fetal echocardiogram were prospectively enrolled. Cases were defined as mothers deemed to be at higher risk of having an affected fetus with CHD given an affected parent or affected previous pregnancy with CHD. Controls were defined as mothers with no prenatal risk factors. Mothers completed a validated questionnaire assessing use of folic acid supplementation and receipt of recurrence risk counseling. Chi-square analyses were performed to analyze questionnaire responses and demographic data. Results A total of 314 subjects participated (controls = 216, cases = 98). Cases took preconceptual folic acid supplementation more often than controls (P < .001), but only 55% started preconceptually. Maternal advanced education and counseling (P < .001) were associated with preconceptual supplementation, whereas complexity of CHD in the relative was not. While 70% of cases received some recurrence risk counseling, those with advanced education and complex CHD in the affected relative were more likely to receive counseling. Few at-risk cases interacted with genetic services (19%). Conclusions At-risk mothers with lower education are less likely to take preconceptual folic acid supplementation or receive recurrence risk counseling. Health care providers should proactively provide this information to all at-risk patients and develop collaborations with genetic services.

Current recurrence risk counseling for conotruncal cardiac defects (CTD) is based on empiric estimates from multiple studies. We examined the risk of congenital heart disease (CHD) in relatives of probands with CTDs to assist in counseling practices in the current era. One thousand six-twenty probands with CTDs and no reported chromosomal or genetic abnormalities were recruited sequentially. A three-generation pedigree was obtained for each proband by a genetic counselor detailing the presence and type of CHD in each family member. Risks and 95% confidence intervals (CI) were calculated for sub-groups of relatives based on degree of relationship for all probands and by individual lesion of the proband. For pairs of affected relatives, concordance rates were calculated. Severity of CHD in the affected relative was assessed. The risk of CHD was higher in siblings (4.4%, 95% CI 3.4-5.4) than in parents (1.5%, 95% CI 1.1-1.9). Risk varied by the cardiac lesion of the proband with the highest risk in first-degree relatives of probands with tetralogy of Fallot and the lowest in D-transposition of the great arteries. 39% of affected parents and 69% of affected siblings had a concordant lesion (i.e., CTD). Most affected siblings of probands with severe CTDs had complex defects (58%), whereas very few affected parents had complex defects (20%). These data suggest that recurrence risk varies by lesion and relationship, with substantial concordance observed by cardiac lesion and complexity of disease, particularly among siblings. These findings contribute to risk counseling in the current era.

Objectives Fetuses with pulmonary outflow tract obstruction (POTO) have altered blood flow to the pulmonary vasculature. We sought to determine whether pulmonary vascular impedance, as assessed via the pulsatility index (PI), is different in fetuses with POTO compared to normal controls.

Prenatal detection (PND) has benefits for hypoplastic left heart syndrome (HLHS) and transposition of the great arteries (TGA), but socioeconomic and geographic barriers are unknown. We evaluated the association between socioeconomic position, public insurance, race/ethnic group, and distance from

The 22q11.2 deletion syndrome is characterized by multiple congenital anomalies including conotruncal cardiac defects. Identifying the patient with a 22q11.2 deletion (22q11del) can be challenging because many extracardiac features become apparent later in life. We sought to better define the cardiac phenotype associated with a 22q11del to help direct genetic testing. 1,610 patients with conotruncal defects were sequentially tested for a 22q11del. The counts and frequencies of primary lesions and cardiac features were tabulated for those with and those without a 22q11del. Logistic regression models investigated cardiac features that predicted deletion status in tetralogy of Fallot (TOF). Deletion frequency varied by primary anatomic phenotype. Regardless of the cardiac diagnosis, a concurrent aortic arch anomaly (AAA) was strongly associated with deletion status [odds ratio (OR), 5.07; 95 % confidence interval (CI), 3.66-7.04]. In the TOF subset, the strongest predictor of deletion status was an AAA (OR, 3.14; 95 % CI 1.87-5.27; p < 0.001), followed by pulmonary valve atresia (OR, 2.03; 95 % CI 1.02-4.02; p = 0.04). Among those with double-outlet right ventricle and transposition of the great arteries, only those with an AAA had a 22q11del. However, 5 % of the patients with an isolated conoventricular ventricular septal defect and normal aortic arch anatomy had a 22q11del, whereas no one with an interrupted aortic arch type A had a 22q11del. A subset of patients with conotruncal defects are at risk for a 22q11del. A concurrent AAA increases the risk regardless of the intracardiac anatomy. These findings help to direct genetic screening for the 22q11.2 deletion syndrome in the cardiac patient.

Supraventricular tachycardia (SVT), the most common fetal tachycardia, can be difficult to manage in utero. We sought to better understand predictors of the postnatal clinical course in neonates who experienced fetal SVT. We hypothesized that fetuses with hydrops or those with refractory SVT (failure of first-line SVT therapy) are more likely to experience postnatal SVT. This was a retrospective multicenter cohort study of subjects diagnosed with fetal SVT between 2006 and 2014. Fetuses with structural heart disease were excluded. Descriptive comparative statistics and univariate analysis with logistic regression were utilized to determine factors that most strongly predicted postnatal SVT and preterm delivery. The cohort consisted of 103 subjects. Refractory SVT was found in 37% (N = 38) of the cohort with this group more likely to be delivered prematurely (median = 36 vs. 37.5 weeks, p = 0.04). Refractory SVT did not increase the risk of postnatal SVT (p = 0.09). Postnatal SVT was seen in 61% (N = 63). Of those, 68% (N = 43) had postnatal SVT at ≤2 days of age. Postnatal SVT was associated with a later fetal SVT diagnosis (median = 30 vs. 27.5 weeks, p = 0.006). We found a strong correlation between postnatal SVT and later gestational age at fetal SVT diagnosis. Subjects with refractory SVT or hydrops did not have a higher risk of postnatal SVT. We propose strong consideration for term delivery in the absence of significant clinical compromise. Further studies to assess whether outcomes vary for preterm delivery versus expectant management in those with refractory SVT should be performed.

Coarctation of the aorta (CoA) is the most common ductal-dependent lesion missed on neonatal exam screening. Prenatal diagnosis of CoA improves outcomes through early initiation of prostaglandin. Fetal echocardiographic parameters including 2D and Doppler findings have been studied as predictive measures for fetal diagnosis of CoA, but diagnosis rates remain variable. A comprehensive set of predictor variables was applied to fetuses suspected of CoA to analyze which parameters were associated with postnatal CoA. UCSF Fetal Cardiovascular Program databases were queried for fetuses suspected of CoA (2008–2014). Retrospective measurements of aorta/pulmonary artery ratio (AoPA), LV/RV ratio, ascending aorta Z-score (AscAo), isthmus Z-score, isthmus/duct ratio (I/D), posterior “shelf” of descending aorta, and diastolic flow persistence at the isthmus were recorded. ROC analysis identified the parameters most predictive of postnatal CoA. Among 97 fetuses with probable CoA, 62 had complete follow-up. Of these fetuses, 45 (72.5%) had postnatal confirmation of CoA and 17 did not have CoA. The parameters most predictive of postnatal CoA included AscAo, isthmus Z-score, and I/D, with respective AUC of 0.80, 0.89, and 0.90. Diastolic flow persistence was seen more often in fetuses with postnatal CoA, but did not reach statistical significance. Combining 2D and Doppler criteria (AoPA

BackgroundA growing body of evidence indicates that right ventricular dysfunction in patients with palliated hypoplastic left heart syndrome (HLHS) originates in fetal life. In this study, the systolic-to-diastolic time index (SDI) was used to study the presence of ventricular dysfunction in single right ventricles in fetuses with HLHS or evolving HLHS and to assess whether this dysfunction is related to increase preload, myocardial performance, or interventricular interaction.MethodsEchocardiograms from 78 fetuses with HLHS and 10 with evolving HLHS were retrospectively compared with those of 78 normal control fetuses. Fetuses with HLHS were further grouped according to morphology of the left ventricle (LV): not visible (n=35) or visible (n=43). Spectral Doppler signals obtained from right ventricular inflow (blood pool) and tissue Doppler from the tricuspid lateral annulus were analyzed. The SDI was calculated as the ratio of the ejection time plus isovolumic contraction and relaxation times to the diastolic filling time. E/A and E/e' ratios, cardiac output, preload index, and Tei index were also calculated.ResultsFetuses with HLHS demonstrated significantly elevated right ventricular SDI values by both blood pool Doppler and Doppler tissue imaging compared with control subjects (1.89±0.33 vs 1.58±0.29 [P&nbsp

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 …

Infants with critical congenital heart disease (CCHD) are more likely to have fetal growth restriction and subsequently be small for gestational age (SGA) at birth. While SGA infants with CCHD are known to have poor postnatal outcomes, birth weight (BW) has only been evaluated as a dichotomous

Fetal cerebrovascular resistance, as measured clinically by the middle cerebral artery pulsatility index (MCA-PI), has been shown to be associated with neurodevelopmental outcomes in critical congenital heart disease (CCHD). We investigated the relationship between fetal MCA-PI and postnatal, pre

We carried out a prospective randomized trial on 220 couples with nontubal factor infertility to compare pregnancy rates and implantation rates after zygote intrafallopian transfer (ZIFT) and uterine embryo transfer (UET). The zygote was transferred by laparoscopy into the fallopian tube 24 hours after oocyst retrieval. UET was performed 72 hours after retrieval with abdominal sonography guide. Transfer was performed in 102 cycles in the ZIFT and 100 cycles in the U ET group. The pregnancy and implantation rates were significantly higher in the ZIFT group (42.1% and 11.7%) than in the UET group (21.0% and 7.8%) (P < 0.05). ZiFT could be considered for couples who have limited time and adequate financial support.

Supraventricular tachycardia (SVT) is the most common arrhythmia in the fetus and can be difficult to manage in utero. Recognizing that preterm birth before 37 weeks gestation is the most common catastrophic complication of pregnancy, we sought to better understand the pre and postnatal clinical