Critical Critical Congenital Heart Disease Research Articles

Presurgical pulmonary function tests in the first few days of life in neonates with congenital heart disease, a pilot study.

To compare early pulmonary function tests (PFTs) in neonates with critical congenital heart disease (CHD) compared to a historical reference group. Infants ≥ 37 weeks gestation with critical CHD were studied within the first few days of life, prior to cardiac surgery, and compared to data from a published reference group of healthy term neonates without CHD, studied at the same institution. Passive respiratory resistance (Rrs) and compliance (Crs) were measured with the single breath occlusion technique following specific acceptance criteria. The study was powered for a 30% difference in Rrs. PFTs in 24 infants with CHD were compared to 31 historical reference infants. There was no difference in the Rrs between the groups. The infants with CHD had a significantly decreased Crs (1.02 ± 0.26 mL/cmH2O/kg versus 1.32 ± 0.36; (p < 0.05; mean ± SD)). Further prospective studies are required to quantify early PFTs in infants with CHD of different phenotypes.

Utility of Screening Fetal Echocardiograms at aSingle Institution Following Normal Obstetric Ultrasound in Fetuses with Down Syndrome.

Fetal echocardiograms (F-echo) are recommended in all pregnancies when the fetus has Down syndrome (DS) even if there was a prior obstetric scan (OB-scan) that was normal. The utility of a screening F-echo in this high-risk population when an OB-scan is normal is unknown. Goal of this study was to evaluate if any diagnosis of a critical congenital heart disease (CHD) was missed in a fetus with DS who had a normal OB-scan. Secondary goal was to determine if any CHD was missed postnatally when an OB-scan was read as normal. Retrospective chart review of all fetuses that had a F-echo whose indication was DS between 1/1/2010 to 6/30/2022 was performed. Fetuses were included if they had an OB-scan that was read as normal and had a F-echo. Postnatal transthoracic echocardiogram (pTTE) was reviewed when available. Critical CHD was defined as CHD requiring catheterization or surgical intervention < 1month of age. One hundred twenty-two F-echo on fetuses with DS were evaluated, of which 48 met inclusion criteria. OB-scan was performed at 20.4 ± 4.5weeks gestational age and F-echo was performed at 24.0 ± 4.6weeks gestational age. No patient with a normal OB-scan had a diagnosis of a critical CHD by F-echo (n = 48, negative predictive value = 100%). Evaluating those patients that had an OB-scan and a pTTE (n = 38), 14 patients were diagnosed with CHD (muscular ventricular septal defect (VSD) n = 5, perimembraneous VSD n = 3, secundum atrial septal defect (ASD) n = 2, primum ASD n = 1, transitional atrioventricular septal defect (AVSD) n = 2, and aortic valve abnormality n = 1; negative predictive value = 63.2%). F-echo correctly diagnosed 4 of the 14 missed OB-scan CHD (perimembraneous VSD n = 2, muscular VSD n = 1, and transitional AVSD n = 1). Critical CHD was not missed with a normal OB-scan in this high-risk population. F-echo also missed the majority of CHD when an OB-scan was read as normal. The cost/benefit of screening F-echo in fetuses with DS should be evaluated if a normal OB-scan has been performed, considering all these patients would have a pTTE performed per guidelines.

Impact of Prenatal Diagnosis of Critical Congenital Heart Disease on Preoperative and Postoperative Outcomes.

The objective of this study was to assess the relationship of prenatal diagnosis of critical congenital heart disease (CHD) to preoperative and postoperative patient findings. Retrospective analysis of neonates with critical CHD who underwent cardiothoracic surgery at one of four centers in North Carolina between 2008 and 2013. Surgical data collected by sites for submission to the Society of Thoracic Surgeons Congenital Heart Surgery Database (STS-CHSD) and the North Carolina CHD Lifespan Database were queried. There were 715 patients with STS records; 558 linked to the NC-CHD database. Patients with prenatal diagnosis had a lower incidence of preoperative risk factors, including need for mechanical ventilation and presence of shock. However, prenatally diagnosed patients had worse short-term outcomes, including higher operative mortality, higher incidence of select postoperative complications, and longer LOS. There was no difference in one-year mortality. Our findings are consistent with current literature which suggests that prenatal diagnosis of critical CHD is associated with a more optimized preoperative clinical status. However, we found that patients with prenatal diagnoses had less favorable postoperative outcomes. This needs to be investigated further, but may be secondary to patient-specific factors, such as CHD disease severity.

Wayfinding through the "ocean of the great unknown": how lactating parents establish a direct breastfeeding relationship with an infant with critical CHD.

Lactating parents of infants hospitalised for critical congenital heart disease (CHD) face significant barriers to direct breastfeeding. While experiences of directly breastfeeding other hospitalised neonates have been described, studies including infants with critical CHD are scarce. There is no evidence-based standard of direct breastfeeding care for these infants, and substantial practice variation exists. To explain how direct breastfeeding is established with an infant hospitalised for critical CHD, from lactating parents' perspectives. This study is a qualitative grounded dimensional analysis of interviews with 30 lactating parents of infants with critical CHD who directly breastfed within 3 years. Infants received care from 26 United States cardiac centres; 57% had single ventricle physiology. Analysis included open, axial, and selective coding; memoing; member checking; and explanatory matrices. Findings were represented by a conceptual model, "Wayfinding through the 'ocean of the great unknown'." The core process of Wayfinding involved a nonlinear trajectory requiring immense persistence in navigating obstacles, occurring in a context of life-and-death consequences for the infant. Wayfinding was characterised by three subprocesses: navigating the relationship with the healthcare team; protecting the direct breastfeeding relationship; and doing the long, hard work. Primary influencing conditions included relentless concern about weight gain, the infant's clinical course, and the parent's previous direct breastfeeding experience. For parents, engaging in the Wayfinding process to establish direct breastfeeding was feasible and meaningful - though challenging. The conceptual model of Wayfinding explains how direct breastfeeding can be established and provides a framework for research and practice.

Machine Learning to Predict Executive Function in Adolescents with Repaired d-Transposition of the Great Arteries, Tetralogy of Fallot, and Fontan Palliation

To identify predictors of impaired executive function in adolescents after surgical repair of critical congenital heart disease (CHD). We analyzed patient factors, medical and surgical history, and family social class from 3 single-center studies of adolescents with d-transposition of the great arteries (d-TGA), tetralogy of Fallot (TOF), and Fontan repair. Machine learning models were developed using recursive partitioning to predict an executive function composite score based on five subtests (population mean 10, SD 3) of the Delis-Kaplan Executive Function System. The sample included 386 patients (139 d-TGA, 91 TOF, 156 Fontan) of age 15.1±2.1 (mean±SD) years and an executive function composite score of 8.6±2.4. Family social class emerged as the most important predictive factor. The lowest (worst) mean executive function score (5.3) occurred in patients with low to medium social class (Hollingshead index <56) with one or more neurologic events and a diagnosis of TOF. The highest (best) mean score (9.7) occurred in subjects with high social class (Hollingshead index ≥56) and shorter duration of deep hypothermic circulatory arrest. Other factors predicting lower executive function scores included low birth weight and a greater number of catheterizations. In regression tree modeling, family social class was the strongest predictor of executive function in adolescents with critical CHD, even in the presence of medical risk factors. Additional predictors included CHD diagnosis, birth weight, neurologic events, and number of procedures. These data highlight the importance of social class in mitigating risks of executive dysfunction in CHD.

Некротизирующий энтероколит у новорожденных с врожденными пороками сердца: частота и факторы риска

Necrotizing enterocolitis (NEC) is a serious complication in newborns with critical congenital heart disease (CHD), prolonging hospital stay, worsening neurological prognosis, and increasing mortality. The leading role in the pathogenesis of NEC in these infants is the violation of mesenteric perfusion. A decrease in arterial blood oxygenation may be an additional risk factor for intestinal hypoxia. Objective. To assess the incidence and risk factors for NEC in full-term newborns with CHD born in 2019–2021 at the Perinatal Center of Almazov National Research Medical Center. Patients and methods. The article presents an analysis of the frequency and risk factors of NEC in children with CHD born at the Almazov National Medical Research Centre in 2019–2021. In the group of critical and duct-dependent CHD (n = 264), the overall incidence of NEC was 15.9% (42 cases). The frequency of surgical stages of NEC (IIIA) was 1.1% (3 cases). In the preoperative period, there was a trend towards a higher frequency of inotropic therapy in a subgroup of children who subsequently developed NEC. NEC in newborns with duct-dependent CHD developed mainly in the early postoperative period after cardiac surgery. In children with duct-dependent pulmonary blood flow, a higher incidence of NEC after surgery was noted compared to children with duct-dependent systemic blood flow. Conclusion. Risk factors for NEC in the postoperative period were arterial hypotension and hemodynamically significant cardiac arrhythmias. Dysbiosis of the gut microbiota and changes in microbial metabolism may be additional risk factors for NEC in children with CHD. Key words: necrotizing enterocolitis, risk factors, congenital heart disease, cardiopulmonary bypass, intestinal microbiome, metabolomics

In infants with congenital heart disease autonomic dysfunction is associated with pre-operative brain injury.

Background:Brain injury is a serious and common complication of critical congenital heart disease (CHD). Impaired autonomic development (assessed by heart rate variability; HRV) is associated with brain injury in other high-risk neonatal populations.Objective:To determine whether impaired early neonatal HRV is associated with pre-operative brain injury in CHD.Methods:In infants with critical CHD, we evaluated HRV during the first 24 hours of cardiac ICU (CICU) admission using time-domain (RMS1, RMS2, and alpha 1) and frequency-domain metrics (LF, nLF, HF, nHF). Pre-operative brain MRI was scored for injury using an established system. Spearman’s correlation coefficient was used to determine the association between HRV and pre-operative brain injury.Results:We enrolled 34 infants with median birth gestational age (GA) of 38.8 weeks (IQR 38.1-39.1). Median postnatal age (PNA) at pre-operative brain MRI was 2 days (IQR 1-3 days). Thirteen infants had MRI evidence of brain injury. RMS 1 and RMS 2 were inversely correlated with pre-operative brain injury.Conclusions:Time-domain metrics of autonomic function measured within the first 24 hours of admission to the CICU are associated with pre-operative brain injury, and may perform better than frequency domain metrics under non-stationary conditions such as critical illness.

Comparison of motor outcomes between preschool children with univentricular and biventricular critical heart disease not diagnosed with cerebral palsy or acquired brain injury.

This comparison study of two groups within an inception cohort aimed to compare the frequency of motor impairment between preschool children with univentricular and biventricular critical congenital heart disease (CHD) not diagnosed with cerebral palsy/acquired brain injury, describe and compare their motor profiles and explore predictors of motor impairment in each group.Children with an intellectual quotient <70 or cerebral palsy/acquired brain injury were excluded. Motor skills were assessed with the Movement Assessment Battery for Children-2. Total scores <5th percentile indicated motor impairment. Statistical analysis included χ2 test and multiple logistic regression analysis.At a mean age of 55.4 (standard deviation 3.77) months, motor impairment was present in 11.8% of those with biventricular critical CHD, and 32.4% (p < 0.001) of those with univentricular critical CHD. The greatest difference between children with biventricular and univentricular CHD was seen in total test scores 8.73(2.9) versus 6.44(2.8) (p < 0.01) and in balance skills, 8.84 (2.8) versus 6.97 (2.5) (p = 0.001). Manual dexterity mean scores of children with univentricular CHD were significantly below the general population mean (>than one standard deviation). Independent odds ratio for motor impairment in children with biventricular critical CHD was presence of chromosomal abnormality, odds ratio 10.9 (CI 2.13-55.8) (p = 0.004); and in children with univentricular critical CHD odds ratio were: postoperative day 1-5 highest lactate (mmol/L), OR: 1.65 (C1.04-2.62) (p = 0.034), and dialysis requirement any time before the 4.5-year-old assessment, OR: 7.8 (CI 1.08-56.5) (p = 0.042).Early assessment of motor skills, particularly balance and manual dexterity, allows for intervention and supports that can address challenges during the school years.

Germline variants in HEY2 functional domains lead to congenital heart defects and thoracic aortic aneurysms

PurposeIn this study we aimed to establish the genetic cause of a myriad of cardiovascular defects prevalent in individuals from a genetically isolated population, who were found to share a common ancestor in 1728. MethodsTrio genome sequencing was carried out in an index patient with critical congenital heart disease (CHD); family members had either exome or Sanger sequencing. To confirm enrichment, we performed a gene-based association test and meta-analysis in two independent validation cohorts: one with 2685 CHD cases versus 4370 . These controls were also ancestry-matched (same as FTAA controls), and the other with 326 cases with familial thoracic aortic aneurysms (FTAA) and dissections versus 570 ancestry-matched controls. Functional consequences of identified variants were evaluated using expression studies. ResultsWe identified a loss-of-function variant in the Notch target transcription factor-encoding gene HEY2. The homozygous state (n = 3) causes life-threatening congenital heart defects, while 80% of heterozygous carriers (n = 20) had cardiovascular defects, mainly CHD and FTAA of the ascending aorta. We confirm enrichment of rare risk variants in HEY2 functional domains after meta-analysis (MetaSKAT p = 0.018). Furthermore, we show that several identified variants lead to dysregulation of repression by HEY2. ConclusionA homozygous germline loss-of-function variant in HEY2 leads to critical CHD. The majority of heterozygotes show a myriad of cardiovascular defects.

Critical Congenital Heart Disease in Newborn: Early Detection, Diagnosis, and Management

Critical congenital heart disease (CHD) is a type of CHD that requires early intervention in the first year of life to survive. Morbidity and mortality increases significantly if newborns with critical CHD experience delay in the initial diagnosis and management. The infants may develop cyanosis, systemic hypoperfusion, or respiratory distress as the main manifestations of critical CHD. Pulse oximetry screening for early detection of critical CHD must be performed in newborns after 24 hours of age or before discharge from hospital. Generally, infants with critical CHD require patency of the ductus arteriosus with infusion of prostaglandin to maintain pulmonary or systemic blood flow. After initial management, the infants must be immediately referred to tertiary care center for definitive intervention.&#x0D; Keywords: congenital heart disease, duct-dependent circulation, ductus arteriosus, prostaglandin

Low-dose prostaglandin E1 is safe and effective for critical congenital heart disease: is it time to revisit the dosing guidelines?

Prostaglandin E1 is used to maintain ductal patency in critical congenital heart disease (CHD). The standard starting dose of prostaglandin E1 is 0.05 µg/kg/minute. Lower doses are frequently used, but the efficacy and safety of a low-dose regimen of prostaglandin E1 has not been established. We investigated neonates with critical CHD who were started on prostaglandin E1 at 0.01 µg/kg/minute. We reviewed 154 consecutive patients who were separated into three anatomical groups: obstruction to systemic circulation, obstruction to pulmonary circulation, and inadequate mixing (d-transposition of the great arteries). Treatment failure rates and two commonly reported side effects, respiratory depression and seizure, were studied. A total of 26 patients (17%) required a dose increase in prostaglandin E1. Patients with pulmonary obstruction were more likely to require higher doses than patients with systemic obstruction (15/49, 31% versus 9/88, 10%, p = 0.003). Twenty-eight per cent of patients developed respiratory depression and 8% of patients needed mechanical ventilation. Prematurity (<37 week gestation) was the primary risk factor for respiratory depression. No patient required dose escalation or tracheal intubation while on transport. No patient had a seizure attributed to prostaglandin E1. Prostaglandin E1 at an initial and maintenance dose of 0.01 µg/kg/minute was sufficient to maintain ductal patency in 83% of our cohort. The incidence of respiratory depression requiring mechanical ventilation was low and was mostly seen in premature infants. Starting low-dose prostaglandin E1 at 0.01 µg/kg/minute is a safe and effective therapy for critical CHD.

Oxygen saturation among newborns in the first 10 hours of life to detect Critical Congenital Heart Disease - Ductus Dependent

ABSTRACT Delay diagnosis of Critical Congenital Heart Disease (CHD) can be associated with sudden clinical deterioration and dangerous cardiovascular conditions. The oxygen saturation screening among newborns in the first 10 hours of life is essential for early detection of critical CHD. This study aims to prove that measuring oxygen saturation among newborns in the first 10 hours of life can detect critical CHD. This study is a diagnostic experimental with consecutive sampling subjects in the infant care unit of Dr. Soetomo Hospital, including all newborns with birth weight ≥ 1500 grams and oxygen saturation at ≥ 1 hour of age below 90%. The measurement of oxygen saturation uses fingertip pulse oximetry in the right hand and foot at the age of 10 hours. A "positive oxygen saturation" is defined as oxygen saturation ≤ 85% or different oxygen saturation ≥ 3%, while a "negative oxygen saturation" is when the oxygen saturation is 85% to 90% or different oxygen saturation is 3%. Echocardiography is performed for the gold standard. From November 2019 to January 2020, 11 newborns underwent an oxygen saturation examination. Five subjects (45.46%) in the category of positive oxygen saturation, echocardiographic showed all Critical CHD (100%). Six subjects (54.54%) with negative oxygen saturation category, echocardiographic results showed two critical CHD (33.34%) and four non-critical CHD (66.66%). Fisher's exact test p &lt; 0.005 (α). The diagnostic oxygen saturation test among newborns at 10 hours of life shows ≤85%, all subject’s echocardiography (100%) shows detection of critical CHD, while saturation 85% to 90% has of 33.3% for detection of critical CHD. The sensitivity and specificity of oxygen saturation for early diagnosis of critical CHD are 100% and 67%, respectively. Keywords: critical congenital heart disease, oxygen saturation, fingertip pulse oximetry, diagnostic tests*Corresponding Author: wfatchul045@gmail.com

A Comprehensive Clinical Genetics Approach to Critical Congenital Heart Disease in Infancy

To investigate the frequency of genetic diagnoses among infants with critical congenital heart disease (CHD) using a comprehensive cardiovascular genetics approach and to identify genotype-phenotype correlations. A retrospective chart review of patients evaluated by cardiovascular genetics in a pediatric cardiac intensive care unit from 2010 to 2015 was performed. Infants with CHD who were <1month of age were included. CHD was classified using structured phenotype definitions. Cardiac and noncardiac phenotypes were tested for associations with abnormal genetic testing using χ1 and Fisher exact tests. Genetic evaluation was completed in 293 infants with CHD, of whom 213 had isolated congenital heart disease (iCHD) and 80 had multiple congenital anomalies. Overall, the yield of abnormal genetic testing was 26%. The multiple congenital anomalies cohort had a greater yield of genetic testing (39%) than the iCHD cohort (20%) (OR 2.7). Using a non-hierarchical CHD classification and excluding 22q11.2 deletion and common aneuploidies, right ventricular obstructive defects were associated with abnormal genetic testing (P=.0005). Extracardiac features associated with abnormal genetic testing included ear, nose, and throat (P=.003) and brain (P=.0001) abnormalities. A diagnosis of small for gestational age or intrauterine growth retardation also was associated with abnormal genetic testing (P=.0061), as was presence of dysmorphic features (P=.0033, OR 3.5). Infants without dysmorphia with iCHD or multiple congenital anomalies had similar frequencies of abnormal genetic testing. The present study provides evidence to support a comprehensive cardiovascular genetics approach in evaluating infants with critical CHD while also identifying important genotype-phenotype considerations.

Trends in Preterm Delivery among Singleton Gestations with Critical Congenital Heart Disease

To examine state-wide population trends in preterm delivery of children with critical congenital heart disease (CHD) over an 18-year period. We hypothesized that, coincident with early advancements in prenatal diagnosis, preterm delivery initially increased compared with the general population, and more recently has decreased. Data from the Texas Public Use Data File 1999-2016 was used to evaluate annual percent preterm delivery (<37weeks) in critical CHD (diagnoses requiring intervention at <1year of age). We first evaluated for pattern change over time using joinpoint segmented regression. Trends in preterm delivery were then compared with all Texas livebirths. We then compared trends examining sociodemographic covariates including race/ethnicity, sex, and neighborhood poverty levels. Of 7146 births with critical CHD, 1339 (18.7%) were delivered preterm. The rate of preterm birth increased from 1999 to 2004 (a mean increase of 1.69% per year) then decreased between 2005 and 2016 (a mean decrease of -0.41% per year). This represented a faster increase and then a similar decrease to that noted in the general population. Although the greatest proportion of preterm births occurred in newborns of Hispanic ethnicity and non-Hispanic black race, newborns with higher neighborhood poverty level had the most rapidly increasing rate of preterm delivery in the first era, and only a plateau rather than decrease in the latter era. Rates of preterm birth for newborns with critical CHD in Texas first were increasing rapidly, then have been decreasing since2005.

Preoperative Clinical Neurological Assessment in New-borns with Critical Congenital Heart Disease

Objectives: To evaluate preoperative neurological abnormalities in term newborns with critical congenital heart disease (CHD) and to study relationships among the neurological abnormalities, CHD type and clinical characteristics in the group. Methods: Term newborns with critical CHD without genetic disorders were retrospectively studied. Neurological abnormalities were grouped according to Amiel-Tison neurological assessment (ATNA). CHD was categorized into five subgroups according to the physiological presentation. Results: In the period observed 190 newborns with critical CHD were admitted. Of 134 who met the inclusion criteria, ATNA was optimal in 64%; in 28.5% it was mildly, in 6.5% moderately, and in 1% severely abnormal. The difference in the frequency of abnormal ATNA between the five CHD subgroups was not statistically significant (p = 0.098). A weak association was identified between CHD subgroups and an abnormal ATNA (contingency coefficient 0.246). Newborns with abnormal ATNA had more often SpO2 &lt; 92% (p = 0.028) and abnormal results of cerebral ultrasound (p = 0.001). Conclusion: This study establishes the value of Amiel-Tison standardized neurological examination in assessing the risk of neurodevelopmental disorders among newborns with CHD. We found preoperative neurological abnormalities in one-third of newborns with CHD, and the grade of abnormalities was generally mild or moderate. Newborns with an abnormal ATNA more frequently presented an oxygen saturation &lt; 92% and abnormal results on the cranial ultrasound.

Brain Injury in Infants with Critical Congenital Heart Disease: Insights from Two Clinical Cohorts with Different Practice Approaches

To determine prevalence and risk factors for brain injury in infants with critical congenital heart disease (CHD) from 2 sites with different practice approaches who were scanned clinically. Prospective, longitudinal cohort study (2016-2017) performed at Hospital for Sick Children Toronto (HSC) and Wilhelmina Children's Hospital Utrecht (WKZ), including 124 infants with cardiac surgery ≤60days (HSC=77; WKZ=47). Magnetic resonance imaging was performed per clinical protocol, preoperatively (n=100) and postoperatively (n=120). Images were reviewed for multifocal (watershed, white matter injury) and focal ischemic injury (stroke, single white matter lesion). The prevalence of ischemic injury was 69% at HSC and 60% at WKZ (P=.20). Preoperative multifocal injury was associated with low cardiac output syndrome (OR, 4.6), which was equally present at HSC and WKZ (20% vs 28%; P=.38). Compared with WKZ, HSC had a higher prevalence of balloon-atrioseptostomy in transposition of the great arteries (83% vs 53%; P=.01) and more frequent preoperative focal injury (27% vs 6%; P=.06). Postoperatively, 30% of new multifocal injury could be attributed to postoperative low cardiac output syndrome, which was equally present at HSC and WKZ (38% vs 28%; P=.33). Postoperative focal injury was associated with intraoperative selective cerebral perfusion in CHD with arch obstruction at both sites (OR, 2.7). Compared with HSC, WKZ had more arch obstructions (62% vs 35%; P<.01) and a higher prevalence of new focal injury (36% vs 16%; P=.01). Brain injury is common in clinical cohorts of infants with critical CHD and related to practice approaches. This study confirms that the high prevalence of brain injury in critical CHD is a clinical concern and does not simply reflect the inclusion criteria of published research studies.

Brain microstructural development in neonates with critical congenital heart disease: An atlas-based diffusion tensor imaging study.

BackgroundBrain microstructural maturation progresses rapidly in the third trimester of gestation and first weeks of life, but typical microstructural development may be influenced by the presence of critical congenital heart disease (CHD).ObjectiveThe aim of this study was to investigate the pattern of white matter (WM) microstructural development in neonates with different types of critical CHD. The secondary aim was to examine whether there is an association between WM microstructural maturity and neonatal ischemic brain injury.MethodsFor this prospective, longitudinal cohort study, 74 term born neonates underwent diffusion tensor imaging (DTI) before (N = 56) and after (N = 71) cardiac surgery performed <30 days of life for transposition of the great arteries (TGA), single ventricle physiology with aortic arch obstruction (SVP-AO), left- (LVOTO) or right ventricle outflow tract obstruction (RVOTO). Microstructural integrity was investigated by fractional anisotropy (FA) and by mean diffusivity (MD) in 16 white matter (WM) structures in three WM regions with correction for postmenstrual age. Ischemic brain injury was defined as moderate-severe white matter injury or stroke.ResultsBefore cardiac surgery, the posterior parts of the corona radiata and internal capsule showed significantly higher FA and lower MD compared to the anterior parts. Centrally-located WM structures demonstrated higher FA compared to peripherally-located structures. Neonates with TGA had higher FA in projection-, association- and commissural WM before surgery, when compared to other CHD groups. Neonates with LVOTO showed lower preoperative MD in these regions, and neonates with SVP-AO higher MD. Differences in FA/MD between CHD groups were most clear in centrally located WM structures. Between CHD groups, no differences in postoperative FA/MD or in change from pre- to postoperative FA/MD were seen. Neonatal ischemic brain injury was not associated with pre- or postoperative FA/MD.ConclusionsCollectively, these findings revealed brain microstructural WM development to follow the same organized pattern in critical CHD as reported in healthy and preterm neonates, from posterior-to-anterior and central-to-peripheral. Neonates with TGA and LVOTO showed the most mature WM microstructure before surgery and SVP-AO the least mature. Degree of WM microstructural immaturity was not associated with ischemic brain injury.

Visual-spatial processing style is associated with psychopathology in adolescents with critical congenital heart disease

Objective: To determine whether visual-spatial processing style is associated with psychopathology in a large sample of adolescents with critical congenital heart disease (CHD). Local (part-oriented) style was hypothesized to increase risk for internalizing (but not externalizing) forms of psychopathology.Method: Participants included 278 adolescents with critical CHD (dextro-transposition of the great arteries = 134, tetralogy of Fallot = 58, single-ventricle cardiac anatomy requiring the Fontan procedure = 86). Visual-spatial processing style was indexed using Copy Style Ratings from the Rey-Osterrieth Complex Figure-Developmental Scoring System. The Schedule for Affective Disorders and Schizophrenia for School-Aged Children–Present & Lifetime Version was used to determine presence/absence of diagnosable DSM-IV psychiatric disorder(s). Processing style and psychopathology were assessed concurrently.Results: Thirty-three percent of the sample had a part-oriented processing style. In multivariable binary logistic regression models, part-orientation was associated with more than twice the odds of having an anxiety disorder (lifetime: OR = 2.2, p = .02, 95% CI = 1.1–4.1; current: OR = 2.7, p = .03, 95% CI = 1.1–6.5) but was not associated with an increased risk for ADHD, disruptive behavior, or mood disorders (ps > .05).Conclusions: Adolescents with critical CHD who approach complex visual-spatial materials in a local, part-oriented fashion are more likely to meet criteria for an anxiety disorder than those who approach complexity more holistically. Part-orientation may make it more difficult for individuals to judge the relative importance of isolated details and engage in more adaptive perspective-taking.

Amplitude-Integrated Electroencephalography for Early Recognition of Brain Injury in Neonates with Critical Congenital Heart Disease

To study perioperative amplitude-integrated electroencephalography (aEEG) as an early marker for new brain injury in neonates requiring cardiac surgery for critical congenital heart disease (CHD). This retrospective observational cohort study investigated 76 neonates with critical CHD who underwent neonatal surgery. Perioperative aEEG recordings were evaluated for background pattern (BGP), sleep-wake cycling (SWC), and ictal discharges. Spontaneous activity transient (SAT) rate, inter-SAT interval (ISI), and percentage of time with an amplitude <5 µV were calculated. Routinely obtained preoperative and postoperative magnetic resonance imaging of the brain were reviewed for brain injury (moderate-severe white matter injury, stroke, intraparenchymal hemorrhage, or cerebral sinovenous thrombosis). Preoperatively, none of the neonates showed an abnormal BGP (burst suppression or worse) or ictal discharges. Postoperatively, abnormal BGP was seen in 18 neonates (24%; 95% CI, 14%-33%) and ictal discharges was seen in 13 neonates (17%; 95% CI, 8%-26%). Abnormal BGP and ictal discharges were more frequent in neonates with new postoperative brain injury (P = .08 and .01, respectively). Abnormal brain activity (ie, abnormal BGP or ictal discharges) was the single risk factor associated with new postoperative brain injury in multivariable logistic regression analysis (OR, 4.0; 95% CI, 1.3-12.3; P = .02). Postoperative SAT rate, ISI, or time <5 µV were not associated with new brain injury. Abnormal brain activity is an early, bedside marker of new brain injury in neonates undergoing cardiac surgery. Not only ictal discharges, but also abnormal BGP, should be considered a clear sign of underlying brain pathology.

Does prenatal diagnosis of critical congenital heart diseases influence the prereferral mortality in a center without surgical intervention?

Background: Prenatal diagnosis ameliorates some preoperative and postoperative outcomes in critical congenital heart disease (CHD). Despite large variability among anatomical defect types, nearly half of them are diagnosed antenatally. We aimed to investigate the effect of the antenatal diagnosis on prereferral mortality of infants with critical CHD in a center without cardiovascular surgery clinic.Methods: Medical records of the neonates who were diagnosed with critical CHD between the years 2010 and 2016 in Zekai Tahir Burak Women’s health Education and Research Hospital were retrospectively reviewed for the study. Patients were divided in two groups as prenatal and postnatal regarding the time of diagnosis. Groups were compared in terms of demographical, clinical characteristics, and prereferral mortality rates.Results: Seventy seven neonates were included in the study. Of those, 39 (50%) infants had prenatal diagnosis. Most common types of CHD were tetralogy of Fallot (TOF) with pulmonary atresia, hypoplastic left heart syndrome (HLHS), and transposition of the great arteries (TGA), respectively. Demographical and clinical characteristics were similar between the groups. Minor congenital anomalies were more common in the patients with prenatal diagnosis (41 versus 18%, p = .021). Blood gas parameters were similar except pCO2 levels, which were slightly lower in the postnatal diagnosis group (p = .048). There was no difference with regard to prereferral mortality between prenatal and postnatal diagnosed infants.Conclusions: Prenatal diagnosis may not be always associated with improved survival in critical CHD in a center without immediate surgical intervention opportunity.