Outcomes In Congenital Heart Disease Research Articles

Clinical Profile and Outcome of Children with Congenital Heart Disease in First Year of Life

Diagnosis of congenital heart disease (CHD) which is a structural abnormality of the heart or intra thoracic great vessels in the earliest possible time is very important. Identifying the various modes of presentation, early referral and appropriate intervention can save lives and reduce risk of complications. The objectives of this study were to determine the clinical profile, complication and immediate outcome of children with congenital heart disease in first year of life. This cross-sectional observational study was conducted from October 2011 to March 2012 among 50 children from zero to one year of age who was diagnosed as CHD by echocardiography admitted in Department of Paediatrics of ShaheedSuhrawardy Medical College, Dhaka and Department of Paediatric Cardiology of National Institute of Cardiovascular Diseases, Dhaka, Bangladesh. Among 50 infants acyanotic CHD was detected in 70% and cyanotic in 30% infants. Major structural defects were venticularseptal defect 34%, patent ductusarteriosus 30%, tetralogy of fallot’s 14%, transposition of great arteries 12%, atrial septal defect 6%. Presenting features were cough 82%, dyspnea 80%, poor weight gain 70%, feeding difficulty 68% and fever 58%. Frequently observed complications were failure to thrive, pulmonary hypertension and heart failure. Among the outcome of CHD 8% cases closed spontaneously, case fatality rate was 8% and the rest were advised accordingly for surgery, intervention and medical management among which 14% had device closure within the study period. High index of suspicion, early diagnosis, close monitoring and timely intervention can reduce complication of CHD.
 Bangladesh Med J. 2020 Sept; 49(3) : 17-21

Widening our Focus: Characterizing Socioeconomic and Racial Disparities in Congenital Heart Disease

BackgroundSocioeconomic and racial (SER) disparities among patients with congenital heart disease (CHD) may limit access to high-quality care. We characterized the national SER landscape and its relationship to early outcomes and identified interactions among determinants mitigating adverse outcome. MethodsThe Pediatric Health Information System (PHIS) database was queried for patients (age <26 years) with CHD between 2016 and 2018. International Classification of Diseases, 10th Revision, codes were mapped to diagnostic categories for complexity adjustment. Correlational and hierarchical regression analyses identified risk factors and characterized interactions. ResultsWe identified 166,599 unique admissions from 52 hospitals, with 58,395 having interventions. Median age was 0 years (interquartile range [IQR], 4 years). Race/ethnicity was predominantly White (59%), Hispanic (20%), and Black (16%). Median neighborhood household income (NHI) was $41,082 and varied among hospitals. Patient NHI had a parabolic relationship with mortality, with both higher and lower values having increased risk. Black patients had significantly higher death, and this relationship was potentiated by lower NHI and complexity. Hospital length of stay was longer among Black neonates (median, 51 days; IQR, 93 days) compared with neonates of other ethnic groups (median, 32 days; IQR, 71 days; P < .0001. Care pathways, including permanent feeding tubes, were also more prevalent among Black neonates (17.8%) compared with White neonates (15%; P = .02). ConclusionsInteractions among SER disparities modify CHD outcomes. Specific hospitals have more SER fragile patients but may have developed care pathways that prolong length of stay to mitigate risk among Black neonates. Adverse outcomes among SER-disadvantaged patients are magnified in complex CHD, suggesting tangible benefits to targeted resource allocation and population health initiatives.

The association between parent stress, coping and mental health, and neurodevelopmental outcomes of infants with congenital heart disease

Caring for the complex needs of a child with congenital heart disease (CHD) can place significant burden on the family. Parent mental health and coping have important influences on resilience and neurodevelopmental outcomes in children with CHD. Objectives: To describe the uptake of a cardiac neurodevelopmental program (CNP), examine parent mental health and coping specific to parenting a child with CHD, and explore the relationship between parent mental health and child neurodevelopmental outcomes. Method: Implementation and uptake of the CNP was examined, and forty-four parents of children with CHD completed the DASS and RSQ-CHD. Results: The CNP showed significant uptake in follow-up and interventions offered including 100% completed brain MRIs of eligible patients, 35% increase in neonatal neurology consults, and 100% of families counselled on neurodevelopmental outcomes. A significant proportion of parents endorsed moderate/severe levels of anxiety (25%), depression (20%), and CHD-specific stress. Parents predominantly engaged in secondary control engagement coping (F(2,64)=75.04, p<.001, ηp 2=.70). Secondary control engagement coping was associated with lower parent total stress (r=–.48, p=.006) and anxiety (r=–.47, p=.009). Higher parent stress was associated with higher anxiety (r=.45, p=.016), depression (r=.37, p=.05), more severe types of CHD (r=.35, p=.048), older child age (t(30)= –2.33, p=.03), and lower child cognitive scores (r=–.37, p=.045). More severe types of CHD were associated with lower language scores (F(3,35)=3.50, p=.03). Conclusions: This study highlights the relationship between parent mental health and early child cognitive outcomes in CHD and helps inform models of psychological care to reduce family burden and improve child outcomes.

Prenatal Detection of Congenital Heart Disease: the Past, Present, and Future

Prenatal diagnosis of congenital heart disease (CHD) is continuously evolving with each passing decade. Early efforts in fetal cardiology focused on identifying CHD in mid-gestation and understanding of fetal circulation in pathologic conditions. Improving prenatal detection rates for CHD remains an ongoing challenge and increasingly the field of fetal cardiology is moving to not only diagnosing CHD prenatally but also assessing the impact of prenatal diagnosis of CHD outcomes. Future directions include earlier diagnosis of fetal CHD, improved diagnostic rates, widespread sonographer education, and a better understanding of antenatal factors that impact outcomes. Our goal in this review is to describe the past, present, and future of prenatal diagnosis of CHD. There has been a steady improvement in the prenatal diagnosis rate for CHD; however, there remains a significant variation between countries and within the USA. Prenatal diagnosis of CHD allows for counseling and delivery planning in those fetuses with critical CHD, thereby providing parents with resources and important tools while dealing with a challenging situation of carrying a child with heart disease and helping them with important decisions for their family and their future. There are several specific conditions which continue to pose a challenge from a diagnostic standpoint as they may appear mild at the time of initial diagnosis and may be missed but progress through the pregnancy and lead to significant CHD in the neonatal period. In summary, continued efforts aimed at collaborative research and education are needed in order to be able to improve CHD detection rates. We need to cautiously assess lesions that appear minor in mid-gestation but have the potential to progress in late gestation. Earlier detection of CHD by means of a transvaginal or a first-trimester fetal echocardiogram may further help families with delivery planning and decision-making.

Abstract 16431: Outcomes of Left Heart Decompression in Children on Veno-Arterial Extracorporeal Membrane Oxygenation: A Systematic Review and Meta-analysis

Introduction: Left heart decompression (LD) is performed in patients on veno-arterial extra-corporeal membrane oxygenation (ECMO) to decrease myocardial wall stress, enhance recovery and decrease complications. Data on outcomes following LD however, are unclear. We sought to evaluate outcomes of death(D) and hospital parameters with a systematic review and meta-analysis (SR-MA) in children without congenital heart disease (CHD) who underwent LD on ECMO. Methods: We included citations in non-CHD patients &lt;21 years age on ECMO, from a MESH search for ECMO, LD and D in CINAHL, CINAHL PLUS and MEDLINE, in English up to January 31, 2020. Case reports, systematic reviews, database duplicates and CHD outcomes were excluded. Cochrane ROBINS-I risk of bias (ROB) tool for non-randomized studies was used to assess ROB. Results: The search yielded 300 citations, reduced to 36 full text screening of which 7 met inclusion criteria. All were observational retrospective analysis (5 single centers, 2 registries). ROB was critical in 3 studies, and low in 2 (not assessed in 2 abstracts). Of 1,789 included pts, 1,575 were in registries. Median age on ECMO was 6 years and weight 18.7 (range 3-92) kgs. Main diagnoses consisted of myocarditis (n=636,36%) and dilated cardiomyopathy (n=919,52%). Overall, 659 pts died and 75 underwent heart transplantation. Time from ECMO to LD was 15.5 hours (0-113.7 hrs). Dynamic left atrial (LA) LD was performed in 239 pts(13%), static in 135(8%), LA drain in 150(8%) and atrial septal stent in 17(1%). Median LA pressure at the time of decompression was 21 mm Hg. Pts who underwent LD were found to have lower odds ratio for D compared to pts who did not (OR 0.73 [0.5-1.0],I 2 0%). There was inadequate data to assess outcomes of number of days on ECMO (1 study-LD 17+28 vs non-LD 6+4 days), mechanical ventilation (1 study- LD 32+49 vs non-LD 14+19 d), ICU stay (1 study-LD 52+55 vs non-LD 18+10 d) and total length of stay (1 study-LD 60+55 vs non-LD 27+33 d). Conclusions: Our systematic review and meta-analysis found that survival was better in non-CHD pts on ECMO who underwent LD compared to pts who did not. Further outcome analysis was limited by inadequate data. Larger registry level analysis is necessary to assess this further.

Abstract 17205: The Association of Cerebrovascular Autoregulation With Neurodevelopmental Delays Between Infants With and Without Congenital Heart Disease

Background: Children with Congenital Heart Disease ( CHD ) are 5 times more likely to develop neurodevelopmental impairment lasting into adulthood compared to their peers. One theory is that hypoxemia associated with CHD contributes to impaired cerebrovascular autoregulation ( CA ), which in turn may contribute to neurological injuries and impaired neurodevelopmental outcome ( NDO ) in CHD. This study examined the association of CA and NDO between CHD and healthy infants. Hypothesis: Impaired CA will be associated with poorer NDO in CHD infants through 9-month development. Methods: A prospective, longitudinal study was performed at 3, 6, &amp; 9-month time points. CA was indexed by calculating the difference between 2-minute averaged supine and sitting cerebral oxygenation (rcSO 2 ) measured by near-infrared spectroscopy for 3 consecutive tilts (0-90°), resulting in change scores. The Bayley Scales of Infant and Toddler Development III measured NDO. Multiple linear regressions quantified the group-by-CA interaction on NDO at each age. Results: We enrolled 35 CHD and 26 healthy control ( HC ) infants. 3 months: As CA worsened (change score in rcSO 2 increased by 1-unit), cognitive compositive scores decreased by 2.6% in the CHD group compared to the HC during the 3 rd postural change (P=0.003). 6 months: As CA worsened, the language composite scores decreased by 3.7% in the CHD group at compared to the HC for the 1 st postural change (P=0.041). 9 months: As CA worsened, the cognitive composite scores decreased by 2.7% in the CHD group compared to the HC during the 3 rd postural change (P=0.046). See Table 1. Conclusion: We found poorer CA to be significantly associated with worse NDO in CHD infants. These initial results demonstrate the persistence of impaired CA even after surgical repair of CHD, which may be a mechanism of brain injury. Future directions include larger studies and targeted interventions to improve CA to reduce developmental delays in CHD children.

Abstract 16907: Relationship Between 3 rd Trimester Umbilical Arterial Blood Flow and Neurodevelopmental Outcomes at 2 Years in Major Fetal Heart Disease

Introduction: Congenital heart disease (CHD) requiring surgical intervention in infancy is associated with impaired neurodevelopmental (ND) outcomes which may be due in part to prenatal insults. Supporting this, we recently found an inverse relationship between 3 rd trimester umbilical artery Doppler-based (UA) pulsatility index (PI=systolic velocity-diastolic velocity/mean velocity) and growth and ND outcomes at 2 years in hypoplastic left heart syndrome and d-transposition of great arteries. In the present study we explore associations between 2 nd and 3 rd trimester UA-PI in all major fetal CHD and 2-year ND outcomes. Methods: We identified all patients with major structural CHD and fetal echocardiography who underwent cardiac surgery and 2-year ND and biometric assessments (Western Canadian Complex Pediatric Therapies Follow-up Program) from 2007-2017 in our program. Eligible surgeries were: bypass &lt; 6 weeks, shunts at &lt; 6 months or isomerisms with surgery at &lt; 12 months. Patients with genetic syndromes were excluded. 2 nd and 3 rd trimester UA-PI z scores were examined for relationships with 2 year Bayley III ND scales. Data is presented as mean (SD). Results: Data from 147 children was analysed (see Table). 2 nd and 3 rd trimester fetal echo was performed at 22.4(3.7) and 34.7(2.9) weeks, with UA-PI z scores of 0.18 (0.3), and 0.51(1.4) and respectively. The Bayley III cognitive, language and motor scores were 93(13), 89(15) and 89(14) for the entire group. Multivariable regression analysis of the entire group and specific subgroups showed an inverse relationship between 3 rd trimester UA-PI and ND domains (see Table). No association was found for 2 nd trimester UA-PI and ND for the whole group and subgroups. Conclusions: Increased 3 rd trimester UA-PI, which could reflect an altered late gestation placental circulation, inversely relates to abnormal 2 year ND. These relationships are most notably observed in SVs and in CHD associated with AOTO.

Neurocognitive and psychosocial outcomes in adult congenital heart disease: a lifespan approach

### Learning objectives In 1964, Glaser recognised that, ‘Despite benefits derived from these remarkable therapeutic gains, children with congenital heart disease face many difficulties in their efforts toward social and...

Burden of malnutrition and anemia among children with congenital heart disease

Background: Congenital heart disease (CHD) is the most common birth defect and in India, the prevalence is 2.5 to 5/1000 live births. Malnutrition and anemia are common co-morbidities, that determine the outcome in CHD. This study was undertaken to assess the extent of malnutrition and anemia among 1-12-year-old children with CHD.Methods: A total 80 children with CHD, admitted for intervention under the Rashtriya Bal Swasthya Karyakram (RBSK) scheme were enrolled. Nutritional status was assessed using standardized anthropometric measurements. Anemia was estimated using hemoglobin, red cell indices, red cell distribution width and peripheral smear. IEC approval, informed consent and assent from participants were obtained prior to the study. Statistical analysis was performed using SPSS version 19.Results: 2/3rd children were &gt;5-years-old. Acyanotic CHD was more common (81.3%) and 35% had ventricular septal defect. Among cyanotic CHD (18.7%), 13.7% had tetralogy of fallot. In CCHD, there was only one child with normal weight and height. In ACHD, 71% were underweight, 49%, had stunting and 82% had wasting. Anemia was diagnosed in nearly 1/3rd, and iron deficiency was the most common. Even though polycythemia was noted in those with CCHD, increased RDW and reduced red cell indices unmasked iron deficiency.Conclusions: Majority were &gt;5-years-old, due to late referral for intervention. The burden of malnutrition and iron deficiency anemia, that modify the outcome, was very high, Hence, early identification, prompt referral and correction of co-morbidities are of utmost importance, as majority are likely to get surgical/non-surgical interventions under government sponsored schemes like RBSK in India.

Physical Functioning, Mental Health, and Quality of Life in Different Congenital Heart Defects: Comparative Analysis in 3538 Patients From 15 Countries

BackgroundWe compared physical functioning, mental health, and quality of life (QoL) of patients with different subtypes of congenital heart disease (CHD) in a large international sample and investigated the role of functional class in explaining the variance in outcomes across heart defects. MethodsIn the cross-sectional Assessment of Patterns of Patient-Reported Outcome in Adults with Congenital Heart Disease-International Study (APPROACH-IS), we enrolled 4028 adult patients with CHD from 15 countries. Diagnostic groups with at least 50 patients were included in these analyses, yielding a sample of 3538 patients (median age: 32 years; 52% women). Physical functioning, mental health, and QoL were measured with the SF-12 health status survey, Hospital Anxiety and Depression Scale (HADS), linear analog scale (LAS) and Satisfaction with Life Scale, respectively. Functional class was assessed using the patient-reported New York Heart Association (NYHA) class. Multivariable general linear mixed models were applied to assess the relationship between the type of CHD and patient-reported outcomes, adjusted for patient characteristics, and with country as random effect. ResultsPatients with coarctation of the aorta and those with isolated aortic valve disease reported the best physical functioning, mental health, and QoL. Patients with cyanotic heart disease or Eisenmenger syndrome had worst outcomes. The differences were statistically significant, above and beyond other patient characteristics. However, the explained variances were small (0.6% to 4.1%) and decreased further when functional status was added to the models (0.4% to 0.9%). ConclusionsSome types of CHD predict worse patient-reported outcomes. However, it appears that it is the functional status associated with the heart defect rather than the heart defect itself that shapes the outcomes.

CCHD Screening Implementation Efforts in Latin American Countries by the Ibero American Society of Neonatology (SIBEN).

Congenital heart disease (CHD) is among the four most common causes of infant mortality in Latin America. Pulse oximetry screening (POS) is useful for early diagnosis and improved outcomes of critical CHD. Here, we describe POS implementation efforts in Latin American countries guided and/or coordinated by the Ibero American Society of Neonatology (SIBEN), as well as the unique challenges that are faced for universal implementation. SIBEN collaborates to improve the neonatal quality of care and outcomes. A few years ago, a Clinical Consensus on POS was finalized. Since then, we have participated in 12 Latin American countries to educate neonatal nurses and neonatologists on POS and to help with its implementation. The findings reveal that despite wide disparities in care that exist between and within countries, and the difficulties and challenges in implementing POS, significant progress has been made. We conclude that universal POS is not easy to implement in Latin America but, when executed, has not only been of significant value for babies with CHD, but also for many with other hypoxemic conditions. The successful and universal implementation of POS in the future is essential for reducing the mortality associated with CHD and other hypoxemic conditions and will ultimately lead to the survival of many more Latin American babies. POS saves newborns’ lives in Latin America.

The Impact of Neurobehavior on Feeding Outcomes in Neonates with Congenital Heart Disease

To evaluate the association between neonatal neurobehavioral state and oral feeding outcomes following congenital heart disease (CHD) surgery. This single center retrospective cohort study described neonates undergoing cardiac surgery evaluated perioperatively with the Neonatal Intensive Care Unit Network Neurobehavioral Scale (NNNS). We compared NNNS attention scores, which evaluates neonates' ability to orient and fixate on stimuli, with the feeding outcomes percentage of feeds taken orally at discharge and time to reach full oral feeds using regression analyses. Models were constructed for both preoperative and postoperative NNNS evaluations. Between August 2015 and October 2017, 124 neonates underwent 89 preoperative and 97 postoperative NNNS evaluations. In multivariable Cox regression, higher preoperative NNNS attention scores were associated with a shorter time to achieve full oral feeds (hazard ratio 1.4; 95% CI 1.0‒2.0; P=.047). This relationship was not seen for post-operative NNNS attention scores or percentage of oral feeds at discharge. Depending on the model, younger age at surgery, increased ventilator days, increased length of stay, and single or 2-ventricle anatomy with aortic arch obstruction were associated with lower percentage of oral feeds at discharge and/or delay in full oral feeds. Higher neonatal attention before cardiac surgery is associated with improved feeding outcomes. Prospective assessment of neonatal neurobehavioral state may be a novel approach to predict and target interventions to improve feeding outcomes in CHD. Future studies should examine the impact of intrinsic neurodevelopmental delay vs environmental adaptation on the neurobehavioral state of neonates with CHD.

Updates in Congenital Heart Disease: Can Outcomes Improve?

Despite numerous advances in medical and surgical management, congenital heart disease (CHD) remains the number one cause of death in the first year of life from congenital malformations. The current strategies used to approach improving outcomes in CHD are varied. This article will discuss the recent impact of pulse oximetry screening for critical CHD, describe the contributions of advanced cardiac imaging in the neonate with CHD, and highlight the growing importance of quality improvement and safety programs in the cardiac intensive care unit.

Safety and outcomes of catheter ablation for atrial fibrillation in adults with congenital heart disease: A multicenter registry study

An increasing number of adults with congenital heart disease (CHD) are undergoing catheter ablation for atrial fibrillation (AF). Data on ablation strategy and outcomes in CHD are limited. Rhythm control is often believed to be of greater importance among patients with complex CHD. The purpose of this study was to examine the safety and efficacy of AF ablation in adult patients with CHD. A multicenter retrospective analysis was performed of CHD patients undergoing AF ablation. Clinical data were collected, including AF and CHD type, procedural data, and outcomes. Patients were divided into 3 groups (simple, moderate, and severe) based on CHD complexity, as defined by the 2014 PACES/HRS (Pediatric and Congenital Electrophysiology Society/Heart Rhythm Society) consensus statement. One-year procedural success was defined as freedom from recurrent AF, off antiarrhythmic drugs (complete) or off/on previously failed antiarrhythmic drugs (partial). Overall, 84 CHD patients (mean age 51.5 ± 12.1 years; 65.5% male; 45.2% with paroxysmal AF) undergoing AF ablation (51 simple, 22 moderate, 11 severe complexity) were included. Pulmonary vein isolation was performed in 80 (95.2%), of whom 30 (35.7%) underwent pulmonary vein isolation alone. Overall, complete and complete/partial freedom was achieved at 1 year in 53.1% and 71.6%, respectively, with no significant differences between those with simple, moderate, or severe complexity. There were no major complications and 7 minor complications, and 2 patients died during follow-up. There are dramatic differences in the degree of CHD complexity among patients referred for AF ablation. When performed at experienced centers, AF ablation is safe and effective even among patients with the most complex forms of CHD.

Can echocardiographic screening in the early days of life detect critical congenital heart disease among apparently healthy newborns?

BackgroundDelayed diagnosis of critical congenital heart disease (CCHD) carries a serious risk of mortality, morbidity, and handicap. As echocardiography is commonly used to diagnose congenital heart disease (CHD), echocardiographic investigations in newborns may be helpful in detecting CCHD earlier and with higher sensitivity than when using other screening methods. The present study aimed to evaluate the effectiveness of echocardiographic screening for CCHD in a tertiary care center.MethodsA retrospective chart review was conducted among newborns delivered at Hamamatsu University Hospital between June 2009 and May 2016. The study included consecutive newborns who underwent early echocardiographic screening (within the first 5 days of life) performed by pediatric cardiologists, were born at ≥36 weeks of gestation, had a birthweight ≥2300 g, and were cared for in the well-baby nursery. Newborns admitted to the neonatal intensive care unit, as well as those with prenatal diagnosis of CHD and/or clinical symptoms or signs of CHD were excluded. Four CHD outcome categories were defined: critical, serious, clinically significant, and clinically non-significant.ResultsA total of 4082 live newborns were delivered during the study period. Of 3434 newborns who met the inclusion criteria and had complete echocardiography data, 104 (3.0%) were diagnosed as having CHD. Among these, none was initially diagnosed as having critical or serious CHD. Of the 95 newborns who continued follow-up with a cardiologist, 61 (64%) were determined to have non-significant CHDs that resolved within 6 months of life. Review of excluded newborns revealed nine cases of critical or serious CHD; among these newborns, six were diagnosed prenatally and three had some clinical signs of CHD prior to hospital discharge.ConclusionsIn our tertiary care center, echocardiography screening within the first 5 days of life did not help improve CCHD detection rate in newborns without prenatal diagnosis or clinical signs of CHD. Echocardiographic screening may be associated with increased rate of false-positives (defects resulting in clinically non-significant CHDs) in newborns without prenatal diagnosis or suspicion of CHD.

Abstract 16811: Concomitant Fetal Heart and Brain Mri in Congenital Heart Disease Mouse Models With Sub-Nyquist Acquisition

Introduction: Congenital heart disease (CHD) patients suffer higher incidences of neurocognitive impairment. An unanswered clinical question is whether the poor neurodevelopmental outcome in CHD is driven by hemodynamic perturbations arising from the structural heart defects or primarily driven by patient intrinsic/genetic factors. In order to simultaneously evaluate genetic factors, structural heart defects and brain development in CHD mutant mouse models, we have developed a fast MRI methodology for simultaneously imaging fetal mouse hearts and brains. Methods: The specific challenge for fetal mouse MRI is to overcome motion artifacts arising from multiple fetuses and maternal abdominal and respiratory motion. We performed 4D MRI in utero using a hybrid compressed sensing and low-rank imaging method. The two key features of this fetal MRI approach are (1) the ability to express incoherent fetal and maternal motion with reduced degrees of freedom; and (2) a sub-Nyquist sparse sampling scheme to accelerate acquisition and increase temporal resolution. This allows assessments of anatomical structures and the hemodynamic function and its relationship with the developing fetal brain. Results: 4D MRI was performed in utero of a pregnant female mouse on embryonic day E16.5. Outlier respiratory states were automatically identified and removed before image reconstruction. After reconstruction, the image closest to the median image was automatically extracted for analysis. The boundary of each individual yolk sac is indicated by yellow dotted lines, with embryos numbered from E1 to E8. This isotropic 3D data set can be re-oriented according to each embryo’s body axis. FigA shows the sagittal orientation of embryo #2 to highlight brain structures. FigB shows the coronal view of the embryo #7 to highlight stomach, liver, and both kidneys. Placenta can be clearly seen as well (Fig.C). Conclusion: Our fetal MRI can be used to evaluate congenital heart and brain defects.

Estimating Surgical Risk in Younger and Older Children With Congenital Heart Disease

BackgroundAs management of congenital heart disease (CHD) improves, children with CHD increasingly present for noncardiac surgery. Prior studies report conflicting results on the association between CHD and adverse outcomes in noncardiac surgery. Studies reporting no such association predominantly analyze older children and adolescents. We evaluated whether the association between CHD and adverse surgical outcomes was attenuated by increased age. MethodsPatients aged 0-17 y, undergoing elective noncardiac surgery, were identified in the National Surgical Quality Improvement Program-Pediatric registry. CHD status was classified as none, minor, major, or severe. Multivariable logistic regression evaluated the association of CHD status with the occurrence of any postoperative complication for groups defined by quintiles of patient age (<1, 1-3, 4-8, 9-13, and 14-17 y). Specific complications included cardiac arrest, reintubation, infection, renal failure, neurological complication, thromboembolic complication, reoperation, 30-d unplanned revisit, 30-d prolonged hospital stay, and mortality. ResultsThe analysis included 131,164 children, of whom 6420 had minor CHD, 3825 had major CHD, and 963 had severe CHD. The overall rate of complications was 9%. In multivariable analysis, children <1 y old had greater risk of postoperative complications if they had minor (odds ratio [OR] = 1.97; 95% confidence interval [CI]: 1.70, 2.20; P < 0.001), major (OR = 2.58; 95% CI: 2.28, 2.91; P < 0.001), or severe CHD (OR = 4.37; 95% CI: 3.45, 5.54; P < 0.001). In older age groups, however, the presence of CHD was not independently associated with postoperative complications. ConclusionsIn pediatric noncardiac surgery, an independent association of CHD with postoperative complications was only evident among children <1 y old.

Congenital heart disease and cardiac procedural outcomes in patients with trisomy 21 and Turner syndrome.

Congenital heart disease (CHD) is present in approximately 50% of patients with trisomy 21 (T21) and Turner syndrome (TS). According to the American Academy of Pediatrics, every patient with these genetic disorders should have a postnatal echocardiogram. T21 is usually associated with atrioventricular (30%-60%), atrial (16%-21%), or ventricular septal defects (14%-27%). TS is usually associated with left-sided heart disease. However, the spectrum of CHD in these genetic disorders is wider than those mentioned lesions. More cardiac surgical procedures are offered to these patients and that has influenced positively their life expectancy for some CHD conditions. Single ventricular anatomy is associated with high mortality in these genetic disorders (49% in T21 and 83%-91% in TS). The goal of this article is to describe the spectrum of CHD, screening guidelines, and cardiac surgical outcomes in patients with T21 or TS with CHD.

Impact of a Pediatric Cardiology Clinical Program on Congenital Heart Disease Outcomes in Guyana.

Background: Children with congenital heart disease (CHD) in Guyana have not historically been managed with timely intervention, increasing the likelihood of serious, irreversible complications. In 2014, a pediatric cardiology clinical program (Guyana Paediatric Cardiology Steering Committee [GPCSC]) and partnership with International Children’s Heart Foundation (BabyHeart) was developed to improve CHD care. Objectives: To describe the characteristics of CHD in Guyanese children and to determine the impact of GPCSC on CHD outcomes. Methods: Qualitative comparison between CHD patients sent for surgery prior to GPCSC (pre-GPCSC cohort) and those managed through GPCSC (post-GPCSC cohort). Findings: Eighty-eight pre-GPHC patients were identified from 2005 to 2014. A total of 319 CHD patients were referred post-GPCSC. In all, 114 patients required surgical or catheterization procedures, with 74 patients prioritized for interventions within 29 months post-GPCSC. Mean age at surgery was 77 months in both cohorts, with younger children represented in the post-GPCSC cohort. Postoperative follow-up was more frequent post-GPCSC (100% vs 35%). Vital status of 48% of pre-GPCSC patients is unknown, with more pre-GPCSC patients known to be deceased compared with post-GPCSC (9% vs 5%). Pre-GPCSC patients had more incorrect diagnosis and inoperable disease when sent for surgery. Interpretation: Patients undergoing surgery post-GPCSC had more appropriate and timely interventions, better follow-up, and increased survival. The feasibility and positive impact of this collaborative pediatric cardiology clinical program in Guyana is demonstrated, with potential applicability for other low- and middle-income countries. Obstacles to referral of children with CHD in Guyana can begin to be addressed, with the goal of more complete access to timely intervention, and improved outcomes for these children.

FMRI investigation of working memory in adolescents with surgically treated congenital heart disease

ABSTRACTAdolescents and young adults with surgically treated congenital heart disease (CHD) have been shown to exhibit difficulties with executive functions; however, the neural underpinnings of these impairments have not been previously examined with functional magnetic resonance imaging (fMRI). The current study employed fMRI to examine the neural mechanisms during a letter n-back task of working memory compared to vigilance. Seventeen participants with CHD (Mage = 17.76 ± 1.72 years; 88% Caucasian; 30% female; mean IQ = 104.12 ± 15.15) were compared to 17 controls (Mage = 18.40 ± 1.74 years; 70% Caucasian; 30% female; mean IQ = 110.59 ± 5.28) with similar declining performance as the n-back became more challenging. Overall, both groups activated similar frontal-parietal working-memory networks as seen in previous literature; however, some significant differences were detected between the groups. Specifically, the participants with CHD demonstrated differences within the left precuneus and the right inferior frontal gyrus. Secondary analyses indicated that this difference appeared to be due to less task-induced deactivation (TID) in the CHD group during working memory and greater working-memory TID in the control group. In CHD, prefrontal fMRI deactivation on working-memory tasks correlated with improved working-memory performance. Future complementary neuroimaging research with functional connectivity is warranted to further examine the neural underpinnings of disrupted executive function in the long-term outcomes of CHD.