Function In Congenital Heart Disease Research Articles

Association of genetic and sulcal traits with executive function in congenital heart disease.

Persons with congenital heart disease (CHD) are at increased risk of neurodevelopmental disabilities, including impairments to executive function. Sulcal pattern features correlate with executive function in adolescents with single-ventricle heart disease and tetralogy of Fallot. However, the interaction of sulcal pattern features with genetic and participant factors in predicting executive dysfunction is unknown. We studied sulcal pattern features, participant factors, and genetic risk for executive function impairment in a cohort with multiple CHD types using stepwise linear regression and machine learning. Genetic factors, including predicted damaging de novo or rare inherited variants in neurodevelopmental disabilitiesrisk genes, apolipoprotein E genotype, and principal components of sulcal pattern features were associated with executive function measures after adjusting for age at testing, sex, mother's education, and biventricular versus single-ventricle CHD in a linear regression model. Using regression trees and bootstrap validation, younger participant age and larger alterations in sulcal pattern features were consistently identified as important predictors of decreased cognitive flexibility with left hemisphere graph topology often selected as the most important predictor. Inclusion of both sulcal pattern and genetic factors improved model fit compared to either alone. We conclude that sulcal measures remain important predictors of cognitive flexibility, and the model predicting executive outcomes is improved by inclusion of potential genetic sources of neurodevelopmental risk. If confirmed, measures of sulcal patterning may serve as early imaging biomarkers to identify those at heightened risk for future neurodevelopmental disabilities.

Increased Cerebello-Prefrontal Connectivity Predicts Poor Executive Function in Congenital Heart Disease.

Children and adolescents with congenital heart disease (CHD) are at risk for cognitive impairments, such as executive function deficits and motor delays, which can impact their academic and adaptive functioning as well as their quality of life. We investigated whether alterations in connectivity between the prefrontal and cerebellar brain structures exist between CHD and control cohorts and if these alterations could predict cognitive or motor impairment among youths with CHD. 53 participants with CHD and 73 healthy control participants completed multi-modal magnetic resonance imaging (MRI) of the brain, including high-resolution diffusion tensor imaging at 3T. We measured connectivity from masked regions of interest in the cerebellum to the frontal cortex using a probabilistic tractography method. Participants also completed neuropsychological tests of cognitive and motor skills using the NIH Toolbox. In the CHD group, fractional anisotropy (FA) was increased in the cognitive loop connectivity pathways, including from the right cerebellum to the left thalamus (p = 0.0002) and from the left thalamus to the left medial frontal gyrus (MFG) (p = 0.0048) compared with the healthy control group. In contrast, there were no differences between CHD and controls in motor loop connectivity pathways. An increase in FA from the right thalamus to the MFG tract in the cognitive loop (posterior subdivision) predicted (p = 0.03) lower scores on the NIHTB tests, including those of executive functioning. A transient increase in connectivity of the cognitive loop in the adolescent group was observed relative to the child and adult groups. Our results suggest that selective alteration of cerebellum-cerebral connectivity circuitry within the cognitive loops predicts cognitive dysfunction in CHD youth. Our study suggests a critical period of cerebellar circuitry plasticity in the adolescent period in CHD subjects that drives neurocognitive function. Further replication and validation in other pediatric CHD cohorts is warranted for future work.

Extensive identification of genes involved in congenital and structural heart disorders and cardiomyopathy

Clinical presentation of congenital heart disease is heterogeneous, making identification of the disease-causing genes and their genetic pathways and mechanisms of action challenging. By using in vivo electrocardiography, transthoracic echocardiography and microcomputed tomography imaging to screen 3,894 single-gene-null mouse lines for structural and functional cardiac abnormalities, here we identify 705 lines with cardiac arrhythmia, myocardial hypertrophy and/or ventricular dilation. Among these 705 genes, 486 have not been previously associated with cardiac dysfunction in humans, and some of them represent variants of unknown relevance (VUR). Mice with mutations in Casz1, Dnajc18, Pde4dip, Rnf38 or Tmem161b genes show developmental cardiac structural abnormalities, with their human orthologs being categorized as VUR. Using UK Biobank data, we validate the importance of the DNAJC18 gene for cardiac homeostasis by showing that its loss of function is associated with altered left ventricular systolic function. Our results identify hundreds of previously unappreciated genes with potential function in congenital heart disease and suggest causal function of five VUR in congenital heart disease.

Cognitive and Executive Function in Congenital Heart Disease: A Meta-analysis.

Cognitive function and executive function (EF) impairments contribute to the long-term burden of congenital heart disease (CHD). However, the degree and profile of impairments are insufficiently described. To systematically review and meta-analyze the evidence on cognitive function and EF outcomes in school-aged children operated for CHD and identify the risk factors for an unfavorable outcome. Cochrane, Embase, Medline, and PsycINFO. Original peer-reviewed studies reporting cognitive or EF outcome in 5- to 17-year old children with CHD after cardiopulmonary bypass surgery. Results of IQ and EF assessments were extracted, and estimates were transformed to means and SE. Standardized mean differences were calculated for comparison with healthy controls. Among 74 studies (3645 children with CHD) reporting total IQ, the summary estimate was 96.03 (95% confidence interval: 94.91 to 97.14). Hypoplastic left heart syndrome and univentricular CHD cohorts performed significantly worse than atrial and ventricular septum defect cohorts (P = .0003; P = .027). An older age at assessment was associated with lower IQ scores in cohorts with transposition of the great arteries (P = .014). Among 13 studies (774 children with CHD) reporting EF compared with controls, the standardized mean difference was -0.56 (95% confidence interval: -0.65 to -0.46) with no predilection for a specific EF domain or age effect. Heterogeneity between studies was large. Intellectual impairments in CHD are frequent, with severity and trajectory depending on the CHD subtype. EF performance is poorer in children with CHD without a specific EF profile. The heterogeneity in studied populations and applied assessments is large. A uniform testing guideline is urgently needed.

Computational analysis of cardiac structure and function in congenital heart disease: Translating discoveries to clinical strategies

Increased availability and access to medical image data has enabled more quantitative approaches to clinical diagnosis, prognosis, and treatment planning for congenital heart disease. Here we present an overview of long-term clinical management of congenital heart disease and its intersection with novel computational and data science approaches to discovering biomarkers of functional and prognostic importance. Efforts in translational medicine that seek to address the clinical challenges associated with cardiovascular diseases using personalized and precision-based approaches are then discussed. The considerations and challenges of translational cardiovascular medicine are reviewed, and examples of digital platforms with collaborative, cloud-based, and scalable design are provided.

Atrial Function and Its Role in the Non-invasive Evaluation of Diastolic Function in Congenital Heart Disease.

Diastolic dysfunction has correlated with adverse outcomes in various forms of unrepaired and repaired or palliated congenital heart disease (CHD). The non-invasive assessment of diastolic function in pediatric and adult patients with CHD remains challenging. Atrial size has a pivotal role in the evaluation of diastolic function; however, a growing body of evidence supports the additional role of atrial function as a more sensitive parameter of ventricular diastolic dysfunction. While the importance of atrial function is becoming clearer in adult acquired heart disease, it remains ambiguous in those with CHD. In this review we set the stage with the current understanding of diastolic function assessment in CHD, followed by insight into atrial form and function including its non-invasive assessment, and conclude with the current knowledge of atrial function in CHD. A general pattern of decrease in reservoir and conduit function with compensatory increase followed by decompensatory decrease in contractile function seems to be the common pathway of atrial dysfunction in most forms of CHD.

Effects of Exercise Training in Postoperative Patients With Congenital Heart Disease: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

BackgroundThe purpose of this meta‐analysis is to assess the effects of exercise training on quality of life, specific biomarkers, exercise capacity, and vascular function in congenital heart disease (CHD) subjects after surgery.Methods and ResultsWe searched the Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE from the date of the inception of the database through April 2019. Altogether, 1161 records were identified in the literature search. Studies evaluating outcomes before and after exercise training among postoperative patients with congenital heart disease were included. The assessed outcomes were exercise capacity, vascular function, serum NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) levels and quality of life. We analyzed heterogeneity by using the I2 statistic and evaluated the evidence quality according to the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) guidelines. Nine randomized controlled trials were included. The evidence indicated that exercise interventions increased the one of the quality of life questionnaire score (mean difference=3.19 [95% CI, 0.23, 6.16]; P=0.03; I2=39%) from the score before the interventions. However, no alterations in exercise capacity, vascular function, NT‐proBNP or quality of life were observed after exercise training. The results of the subgroup analysis showed that NT‐proBNP levels were lower in the group with exercise training than in the group without exercise training over the same duration of follow‐up. The evidence quality was generally assessed to be low.ConclusionsIn conclusion, there is insufficient evidence to suggest that physical exercise improves long‐term follow‐up outcomes of congenital heart disease, although it has some minor effects on quality of life.

Strain and dyssynchrony in fetuses with congenital heart disease compared to normal controls using speckle tracking echocardiography (STE).

Objective To compare myocardial strain and mechanical dyssynchrony in fetuses with congenital heart disease (CHD) to normal controls using speckle tracking echocardiography (STE). Methods In this comparative cross-sectional study 23 fetuses with CHD and 105 normal controls between 19 and 41 weeks of gestation were assessed with STE. The STE sample box was placed over the myocardium of both ventricles. The parameters of interest included the segmental strain of the left (LV-S) and right lateral ventricle wall (RV-S) and the global ventricular strain of both chambers (2C-S). In order to separately assess the LV, we placed the STE sample box over the myocardium of the LV. We calculated the strain of the LV lateral wall (LW-S), the septum (SEPT-S) and the global ventricular strain of the single LV (1C-S). Furthermore, we analyzed the differences in timing of negative peak myocardial strain between the LV and RV (two-chamber dyssynchrony, 2C-DYS) and also within the LV between the lateral wall and the septum (one-chamber dyssynchrony, 1C-DYS). Results The evaluation of strain and mechanical dyssynchrony was feasible in all cases. Compared to normal controls, fetuses with CHD showed lower segmental and global strain values and the extent of 2C-DYS and 1C-DYS was higher than in the healthy control group. Conclusion The deterioration of myocardial function in CHD can be measured with STE. The assessment of strain and dyssynchrony with STE may be useful for distinguishing fetuses with CHD from healthy fetuses.

Better lung function with increased handgrip strength, as well as maximum oxygen uptake, in congenital heart disease across the lifespan.

The respiratory benefits of muscle strength are well-known in heart-healthy populations, but recommendations and research often focus instead on aerobic fitness (peak oxygen uptake) or total activity. Independent benefits of strength thus may be underestimated, especially in congenital heart disease where perceived dangers of certain types of exercise may outweigh perceived benefits. To assess whether it is plausible that pulmonary benefits of strength in heart-healthy populations also apply in congenital heart disease, we simultaneously correlated these patients' lung function with fitness, strength, and cardiac diagnosis. Lung function (forced expiratory volume in one second percentage predicted (FEV1%pred)) was modeled as function of handgrip strength, congenital heart disease diagnosis, peak oxygen uptake and the interactions of handgrip with sex and diagnosis in 538 Germans (58% male, ages 6-82 years) in linear models corrected for age, sex, height and weight. Congenital heart disease diagnoses were: complex cyanotic; Fallot/Truncus arteriosus communis (common arterial trunk) (TAC); shunts; transposition of the great arteries (TGA); left heart; and other/none. Each kg of handgrip was associated with 0.74% higher FEV1%pred ( p < 0.001) and handgrip explained almost 10% of variance in FEV1%pred. While some groups had higher FEV1%pred than others ( p for global null <0.0001), all experienced similar associations with strength ( p for interaction with handgrip >0.10 for both sex and diagnosis.) Correction for peak oxygen uptake eliminated the association with congenital heart disease, but not handgrip. Strength was associated with better lung function in all ages even after correction for peak oxygen uptake, regardless of sex and congenital heart disease. This suggests that strength may be at least as important for lung function as aerobic fitness. Heart-safe strength training may improve pulmonary function in congenital heart disease.

Atlas-Based Computational Analysis of Heart Shape and Function in Congenital Heart Disease.

Approximately 1% of all babies are born with some form of congenital heart defect. Many serious forms of CHD can now be surgically corrected after birth, which has led to improved survival into adulthood. However, many patients require serial monitoring to evaluate progression of heart failure and determine timing of interventions. Accurate multidimensional quantification of regional heart shape and function is required for characterizing these patients. A computational atlas of single ventricle and biventricular heart shape and function enables quantification of remodeling in terms of z scores in relation to specific reference populations. Progression of disease can then be monitored effectively by longitudinal evaluation of z scores. A biomechanical analysis of cardiac function in relation to population variation enables investigation of the underlying mechanisms for developing pathology. Here, we summarize recent progress in this field, with examples in single ventricle and biventricular congenital pathologies.

Assessment of Diastolic Function in Congenital Heart Disease.

Diastolic function is an important component of left ventricular (LV) function which is often overlooked. It can cause symptoms of heart failure in patients even in the presence of normal systolic function. The parameters used to assess diastolic function often measure flow and are affected by the loading conditions of the heart. The interpretation of diastolic function in the context of congenital heart disease requires some understanding of the effects of the lesions themselves on these parameters. Individual congenital lesions will be discussed in this paper. Recently, load-independent techniques have led to more accurate measurements of ventricular compliance and remodeling in heart disease. The combination of inflow velocities and tissue Doppler measurements can be used to estimate diastolic function and LV filling pressures. This review focuses on diastolic function and assessment in congenital heart disease.

Comparison of Respiratory-Triggered (RT) 3D cine steady-state free precession cardiac mri with standard 2D cine imaging and magnetic resonance angiography in Congenital Heart Disease (CHD)

Background This is a pilot study to evaluate the performance of whole-heart isotropic RT-3D cine SSFP sequence as an efficient single solution for assessment of morphology and function in CHD by comparing it to: 1. Short axis (SA) 2D cine SSFP for LV volumetry and function 2. Multiplanar 2D cine SSFP for intracardiac morphology 3. Cine 2D SSFP and gadolinium (Gd)-enhanced 3D MRA for aortic root size 4. Gd-3D MRA for vessel diameter (VD) 5. Gd-3D MRA, and cine 2D SSFP for morphology of extracardiac vasculature 6. Gd-3D MRA and multiplanar cine 2D SSFP for overall diagnosis.

Platelet and Fibrin Alterations on Thromboelastography in Children Undergoing Congenital Cardiac Surgery with Cardiopulmonary Bypass

Background: Children who undergo congenital heart surgery with cardiopulmonary bypass are at risk for alterations in coagulation due to abnormal flow patterns as well as the stresses of cardiopulmonary bypass circuits, drugs and surgery. They are also at risk for thrombosis including arterial ischemic stroke, shunt obstructions and catheter-related thrombi. Therefore understanding their hemostatic parameters will likely help to manage their disease.Aim: This study determined coagulation and platelet profiles using thromboelastography (TEG) prior to and following surgery in children undergoing congenital heart surgery. Such baseline data are critical to development of clinical trials to prevent thrombosis associated with congenital heart disease.Methods: Children between the ages from birth to 18 years were eligible for this study. With consent and assent (as appropriate) 3 mL of whole blood were drawn prior to surgery and again 12-48 hours after surgery when children were recovering and tolerating oral intake. TEG with platelet mapping was performed using activation with kaolin for whole blood global TEG, an initiator of coagulation for fibrin contribution to TEG, and arachidonic acid (AA) for platelet contribution to TEG. Results were analyzed using means and standard deviations. Statistical differences pre and post operatively were calculated with ANOVA.Results: To date, paired samples have been analyzed on 20 children, mean age 8.2 years, range 3 months to 15.5 years. Whole blood global TEG was normal in all 20 children both pre and post operatively, with the exception of a minimally prolonged reaction time in one child at each time point. No child exhibited clinical bleeding or clotting during the study. Fibrin contribution to TEG increased postoperatively on both maximal amplitude (MA) as well as angle of clot (Ang). In contrast, platelet mapping TEG via AA decreased significantly postoperatively on both MA and ang (figure 1). Overall, TEG platelet mapping showed a mean of 20% inhibition preoperatively (within the reference range) and 81% inhibition postoperatively (below the reference range). Of note, there were 3 children less than 6 months, and all 3 showed increased platelet MA postoperatively.Conclusion: Children with congenital heart defects exhibit normal overall coagulation on TEG in a steady state preoperatively and 12 to 48 hours postoperatively. Postoperatively, platelet function in response to AA is significantly decreased by TEG platelet mapping, which may represent platelet exhaustion, while the fibrin contribution to TEG is significantly increased. Infants less than 6 months of age may show accelerated recovery of platelet function. Studies to better understand coagulation and platelet function in congenital heart disease as well as response to antithrombotic therapy are in process. TEG assays will be compared to more precise assays of coagulation and platelet function. Choices in antithrombotic therapy for clinical care or research trials in children undergoing congenital heart surgery should be data driven. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Speckle Tracking Echocardiography in Pediatric and Congenital Heart Disease

Assessment of myocardial strain using speckle tracking echocardiography is an emerging echocardiographic technique that is increasingly used in the diagnosis and management of acquired heart disease in adults. In pediatric heart disease, this is still mainly considered as a research tool as the application of this technology has been slowed by the lack of vendor-independent technology and of normative data across the different age ranges. We believe that the technology has potential applications for the early detection of myocardial dysfunction, the quantification of ventricular function in congenital heart disease, and the detection of dyssynchrony.

Hypoxia Inducible Factor 1α-Driven Vascular Endothelial Growth Factor and Stromal Derived Factor-1 Signaling Are Maintained in the Hypertrophied Right Ventricle in Congenital Heart Disease: Implications for Pulmonary Hypertension

Purpose Patients with congenital heart disease (CHD) maintain right ventricular hypertrophy (RVH) into adulthood. In contrast, re-acquired RVH, for example in pulmonary hypertension, quickly transitions to failure. Although unclear, myocardial ischemia has been implicated, and augmentation of hypoxia inducible factor 1α (HIF1α)-driven angiogenic signals may prevent this transition. We hypothesized that sustained HIF1α and subsequent angiogenic signaling maintained from physiological neonatal RVH contributes to the sustained RV function in CHD. Methods and Materials Free RVs from neonatal (NRV) and adult (ARV) Sprague Dawleys were analyzed for HIF1α (immunoblot, nuclear HIF1α) and the downstream pro-angiogenic factors vascular endothelial growth factor (VEGF) and stromal derived factor 1 (SDF1) (immunoblot). Secretion (ELISA) and functional CXCR4+ bone marrow stem cell (BMSC) migration (Boyden chamber) and in vitro angiogenesis (matrigel) assays were used. In vivo, vascularity was assessed by CD31 expression (immunoblot) and perfusion (Lectin). Similar endpoints were assessed in human RV samples (CHD-RVH = tetralogy of fallot/hypoplastic left heart; normal RV = pulmonary autograft, left atrial tumor). Results NRVs had elevated HIF1α activation compared to ARVs. This was associated with increased SDF1 and VEGF expression and secretion, as well as CXCR4-cell migration and in vitro angiogenesis. In vivo, NRVs had elevated CD31 expression and perfusion. CHD-RVH samples had higher HIF1α, VEGF, SDF1 and CXCR4 expression compared to normal RVs, which was also associated with increased CD31+cells/field. Conclusions HIF1α signaling is maintained from the neonatal stage in CHD-RVH, and is associated with elevated angiogenic signaling and CD31 expression. Since these RV’s maintain functional compensation, strategies that augment these pathways may offer benefit in patients with acquired RVH in diseases like pulmonary hypertension.

Advances of Tei index in accessment of children's right ventricular in congenital heart disease

The value and detection of right ventricular in circulation have been paid more attention,.Because of the defects in traditional method,Tei index is the method in right ventricular measurement.The Tei index is sensitive in assessing RV function of patients with congenital heart disease.It is not affected by valve regurgitation and RV geometry.The Tei index is accurate and simple in estimating right ventricular function in congenital heart disease and is valuable and invasive index in estimating right ventricular functions. Key words: congenital heart disease; Tei index; Right ventricular function

Assessment of Fetal Global and Regional Ventricular Function in Congenital Heart Disease Using a Novel Feature Tracking Technique

The aim of this study was to assess global and regional longitudinal peak systolic ventricular function in fetuses with congenital heart disease (CHD) and compare measurements derived from feature tracking with reference values of healthy fetuses with a matching gestational age. Global and segmental longitudinal peak systolic strain (LPSS), strain rate and velocity values of the left (LV) ventricular myocardium and right (RV) ventricular myocardium were assessed by a novel feature tracking technique in 17 fetuses with congenital heart disease (10 fetuses with left heart obstruction (LHO), 5 fetuses with tetralogy of Fallot (TOF) and 2 fetuses with double outlet right ventricle (DORV)) and were compared in a second step with 24 matched healthy fetuses (gestational age range 21-36 weeks of gestation). The global LPSS of both ventricles was slightly elevated in fetuses with TOF or DORV compared with controls (RV: p=0.055; LV: p=0.063). The RV strain rate presented a trend toward higher values (p=0.09). Corresponding global LV velocity values of fetuses with TOF or DORV revealed a tendency to decrease compared to healthy controls (p=0.054). In contrast, all measurements of fetuses with LHO did not show any statistical difference regarding deformation parameters or velocity compared to healthy controls. Global longitudinal peak systolic measurements in fetuses with congenital heart disease revealed slightly higher strain (RV/LV) and strain rate (RV) values in the subgroup of fetuses with TOF or DORV compared to healthy controls. Whether the application of this new angle-independent technique is suitable for discriminating between healthy and diseased fetuses has to be verified in further investigations.

Repeatability of cardiac-MRI-measured right ventricular size and function in congenital heart disease

The measurement error for right ventricular (RV) size and function assessed by cardiac MRI (CMRI) in congenital heart disease has not been fully characterized. As CMRI parameters are being increasingly utilized to make clinical decisions, defining error in the clinical setting is critical. This investigation examines the repeatability of CMRI for RV size and function. Forty consecutive people with congenital heart disease involving the RV were retrospectively identified. Contouring of RV volumes was performed by two expert CMRI clinicians. The coefficient of variability and repeatability coefficients were calculated. Repeatability coefficients were multiplied by the mean value for each group studied to define a threshold beyond which measurement error was unlikely to be responsible. The variability for indexed RV end-diastolic volume = 3.2% and 3.3% for intra- and interobserver comparisons, respectively. The repeatability coefficients were 13.2% and 14.9% for intra- and interobserver comparisons, which yielded threshold values of 15.1ml/m^2 and 20.2ml/m^2, respectively. For RV ejection fraction (EF), the repeatability coefficients for intra- and interobserver comparisons were 5.0% and 6.0%, which resulted in threshold values of 2.6 EF% and 3.0 EF%. The threshold values generated can be used during serial assessment of RV size and function.

Cardiovascular magnetic resonance tagging of the right ventricular free wall for the assessment of long axis myocardial function in congenital heart disease.

BackgroundRight ventricular ejection fraction (RV-EF) has traditionally been used to measure and compare RV function serially over time, but may be a relatively insensitive marker of change in RV myocardial contractile function. We developed a cardiovascular magnetic resonance (CMR) tagging-based technique with a view to rapid and reproducible measurement of RV long axis function and applied it in patients with congenital heart disease.MethodsWe studied 84 patients: 56 with repaired Tetralogy of Fallot (rTOF); 28 with atrial septal defect (ASD): 13 with and 15 without pulmonary hypertension (RV pressure > 40 mmHG by echocardiography). For comparison, 20 healthy controls were studied. CMR acquisitions included an anatomically defined four chamber cine followed by a cine gradient echo-planar sequence in the same plane with a labelling pre-pulse giving a tag line across the basal myocardium. RV tag displacement was measured with automated registration and tracking of the tag line together with standard measurement of RV-EF.ResultsMean RV displacement was higher in the control (26 ± 3 mm) than in rTOF (16 ± 4 mm) and ASD with pulmonary hypertension (18 ± 3 mm) groups, but lower than in the ASD group without (30 ± 4 mm), P < 0.001. The technique was reproducible with inter-study bias ± 95% limits of agreement of 0.7 ± 2.7 mm. While RV-EF was lower in rTOF than in controls (49 ± 9% versus 57 ± 6%, P < 0.001), it did not differ between either ASD group and controls.ConclusionsMeasurements of RV long axis displacement by CMR tagging showed more differences between the groups studied than did RV-EF, and was reproducible, quick and easy to apply. Further work is needed to assess its potential use for the detection of longitudinal changes in RV myocardial function.

Cognitive function in congenital heart disease after cardiac surgery with extracorporeal circulation

Congenital heart disease (CHD) may cause deleterious effects on cognitive function in children. This study aimed to evaluate the changes of the cognitive and academic functioning in children aged less than 4 years with serious CHD following cardiac surgery with extracorporeal circulation (ECC). A total of 100 children, aged 0-4 years with cyanotic (35) and acyanotic (65) heart diseases who had undergone cardiac surgery with ECC, were subjected to neuropsychological and behavioral evaluation using the Gesell Developmental Schedule (GDS) before operation and at 1 week, and 1, 3 and 6 months after operation. The GDS scores in the first postoperative week were significantly lower than those before operation, but the scores increased gradually (P<0.01). ECC affected the GDS scores after operation, with the cyanotic children being more significantly affected than the acyanotic children. The GDS scores were negatively correlated with the age at each time point. The GDS scores were significantly lower in the cyanotic children than in the acyanotic children at 1 and 3 months postoperatively (P<0.01), but there was no significant difference at 6 months (P<0.05). After cardiac surgery with ECC, the younger the children who have cardiac surgery, the more significantly the GDS score increases. The ECC mainly affects the cognitive ability and academic performance in cyanotic children in one month postoperatively.