Corrigendum: Comparison of cardiac function between single left ventricle and tricuspid atresia: assessment using echocardiography combined with computational fluid dynamics.

[This corrects the article DOI: 10.3389/fped.2023.1159342.].

Prediabetes is associated with a higher serum neurofilament light chain level in adolescents.

Objective: Serum neurofilament light chain (sNfL) level, which is a biomarker indicative of neuroaxonal damage and cognitive impairment, has been reported in several neurological diseases. There has been a lack of studies on the association between sNfL levels and prediabetes in adolescents. This study investigated whether sNfL levels were higher in adolescents with prediabetes undergoing elective orthopedic surgery. Methods: The sNfL level was measured in 149 adolescents aged from 12 to 18 years who underwent elective orthopedic surgery at the Hunan Children's Hospital (18 with and 131 without prediabetes). We evaluated the association between prediabetes and sNfL level after adjusting for age, sex, and triglycerides using a multivariable linear regression model. Results: The prevalence of prediabetes in adolescents was 12.08%. Univariate logistic regression analysis showed that prediabetes was related to sNfL. In multivariate logistic regression analysis, the association between prediabetes with sNfL levels remained significant after adjustment for age, sex, and triglyceride. The relationship between the two was further visualized by a smoothed curve. Conclusions: Prediabetes is associated with a higher sNfL. Further large-scale and prospective studies are needed to verify the clinical application of sNfL as a monitoring biomarker for adolescent prediabetes in adolescents and to evaluate the performance of sNfL in predicting the incidence of neuropathy and cognitive dysfunction in adolescents with prediabetes.

Comparison of cardiac function between single left ventricle and tricuspid atresia: assessment using echocardiography combined with computational fluid dynamics.

Patients with single left ventricle (SLV) and tricuspid atresia (TA) have impaired systolic and diastolic function. However, there are few comparative studies among patients with SLV, TA and children without heart disease. The current study includes 15 children in each group. The parameters measured by two-dimensional echocardiography, three-dimensional speckle tracking echocardiography (3DSTE), and vortexes calculated by computational fluid dynamics were compared among these three groups. Twist is best correlated with ejection fraction measured by 3DSTE. Twist, torsion, apical rotation, average radial strain, peak velocity of systolic wave in left lateral wall by tissue Doppler imaging (sL), and myocardial performance index are better in the TA group than those in the SLV group. sL by tissue Doppler imaging in the TA group are even higher than those in the Control group. In patients with SLV, blood flow spreads out in a fan-shaped manner and forms two small vortices. In the TA group, the main vortex is similar to the one in a normal LV chamber, but smaller. The vortex rings during diastolic phase are incomplete in the SLV and TA groups. In summary, patients with SLV or TA have impaired systolic and diastolic function. Patients with SLV had poorer cardiac function than those with TA due to less compensation and more disordered streamline. Twist may be good indicator for LV function.

Predictive Value of Glycosylated Hemoglobin for Post-operative Acute Kidney Injury in Non-cardiac Surgery Patients.

Objective: Recent studies have indicated that patients (both with and without diabetes) with elevated hemoglobin A1c (HbA1c) have a higher rate of acute kidney injury (AKI) following cardiac surgery. However, whether HbA1c could help to predict post-operative AKI in patients after non-cardiac surgery is less clear. This study aims to explore the predictive value of pre-operative HbA1c for post-operative AKI in non-cardiac surgery. Methods: We reviewed the medical records of patients (≥ 18 years old) who underwent non-cardiac surgery between 2011 and 2020. Patient-related variables, including demographic and laboratory and procedure-related information, were collected, and univariable and multivariable logistic regression analyses were performed to determine the association of HbA1c with AKI. The area under the receiver operating curve (AUC), net reclassification improvement index (NRI), and integrated discriminant improvement index (IDI) were used to evaluate the predictive ability of the model, and decision curve analysis was used to evaluate the clinical utility of the HbA1c-added predictive model. Results: A total of 3.3% of patients (94 of 2,785) developed AKI within 1 week after surgery. Pre-operative HbA1c was an independent predictor of AKI after adjustment for some clinical variables (OR comparing top to bottom quintiles 5.02, 95% CI, 1.90 to 13.24, P < 0.001 for trend; OR per percentage point increment in HbA1c 1.20, 95% CI, 1.07 to 1.33). Compared to the model with only clinical variables, the incorporation of HbA1c increased the model fit, modestly improved the discrimination (change in area under the curve from 0.7387 to 0.7543) and reclassification (continuous net reclassification improvement 0.2767, 95% CI, 0.0715 to 0.4818, improved integrated discrimination 0.0048, 95% CI, -5e-04 to 0.0101) of AKI and non-AKI cases, NRI for non-AKI improvement 0.3222, 95% CI, 0.2864 to 0.3580 and achieved a higher net benefit in decision curve analysis. Conclusion: Elevated pre-operative HbA1c was independently associated with post-operative AKI risk and provided predictive value in patients after non-cardiac surgery. HbA1c improved the predictive power of a logistic regression model based on traditional clinical risk factors for AKI. Further prospective studies are needed to demonstrate the results and clinical application.

Clinical characteristics and survival of children with hypertrophic cardiomyopathy in China: A multicentre retrospective cohort study.

Background: Few data on paediatric hypertrophic cardiomyopathy (HCM) are available in developing countries. A multicentre, retrospective, cohort study was conducted to profile the clinical characteristics and survival of children with HCM in China. Methods: We collected longitudinal data on children with HCM aged 0-18 years at three participating institutions between January 1, 2010 and December 31, 2019. Patients were identified by searching for the diagnosis using ICD-10 codes from the electronic medical records database. HCM was diagnosed morphologically with echocardiography or cardiovascular magnetic resonance imaging. The exclusion criteria were secondary aetiologies of myocardial hypertrophy. The primary outcomes were all-cause death or heart transplantation. The Kaplan-Meier method was used to estimate the survival rate of different groups. Findings: A total of 564 children were recruited, with a median age at diagnosis of 1.0 year (interquartile range, IQR: 0.4-8.0 years), followed for a median of 2.6 years (1977 patient-years, IQR:0.5, 5.9 years). The underlying aetiology was sarcomeric (382, 67.7%), inborn errors of metabolism (IEMs) (108, 19.2%), and RASopathies (74, 13.1%). A total of 149 patients (26.4%) died and no patients underwent heart transplantation during follow-up. The survival probability was 71.1% (95% confidence interval [CI], 66.3%-75.3%) at 5 years. Patients with IEMs or those diagnosed during infancy had the poorest outcomes, with an estimated 5-year survival rate of 16.9% (95% CI, 7.7%-29.1%) and 56.0% (95% CI, 48.8%-62.5%), respectively. Heart failure was the leading cause of death in the cohort (90/149, 60.4%), while sudden cardiac death was the leading cause in patients with sarcomeric HCM (32/66, 48.5%). Interpretation: There is a high proportion of patients with IEM and a low proportion of patients with neuromuscular disease in children with HCM in China. Overall, mortality remains high in China, especially in patients with IEMs and those diagnosed during infancy. Funding: National Natural Science Fund of China (81770380, 81974029), China Project of Shanghai Municipal Science and Technology Commission (20MC1920400, 21Y31900301).

Automatic Detection of Secundum Atrial Septal Defect in Children Based on Color Doppler Echocardiographic Images Using Convolutional Neural Networks.

Secundum atrial septal defect (ASD) is one of the most common congenital heart diseases (CHDs). This study aims to evaluate the feasibility and accuracy of automatic detection of ASD in children based on color Doppler echocardiographic images using convolutional neural networks. In this study, we propose a fully automatic detection system for ASD, which includes three stages. The first stage is used to identify four target echocardiographic views (that is, the subcostal view focusing on the atrium septum, the apical four-chamber view, the low parasternal four-chamber view, and the parasternal short-axis view). These four echocardiographic views are most useful for the diagnosis of ASD clinically. The second stage aims to segment the target cardiac structure and detect candidates for ASD. The third stage is to infer the final detection by utilizing the segmentation and detection results of the second stage. The proposed ASD detection system was developed and validated using a training set of 4,031 cases containing 370,057 echocardiographic images and an independent test set of 229 cases containing 203,619 images, of which 105 cases with ASD and 124 cases with intact atrial septum. Experimental results showed that the proposed ASD detection system achieved accuracy, recall, precision, specificity, and F1 score of 0.8833, 0.8545, 0.8577, 0.9136, and 0.8546, respectively on the image-level averages of the four most clinically useful echocardiographic views. The proposed system can automatically and accurately identify ASD, laying a good foundation for the subsequent artificial intelligence diagnosis of CHDs.

Perioperative probiotics attenuates postoperative cognitive dysfunction in elderly patients undergoing hip or knee arthroplasty: A randomized, double-blind, and placebo-controlled trial.

Background: Postoperative cognitive dysfunction (POCD) is a common complication in elderly patients following surgery. The preventive and/or treatment strategies for the incidence remain limited. Objective: This study aimed to investigate the preventive effect of perioperative probiotic treatment on POCD in elderly patients undergoing hip or knee arthroplasty. Methods: After obtaining ethical approval and written informed consent, 106 patients (age ≥60 years) were recruited, who scheduled elective hip or knee arthroplasty, from 16 March 2021 to 25 February 2022 for this randomized, double-blind, and placebo-controlled trial. They were randomly assigned with a 1:1 ratio to receive either probiotics or placebo treatment (four capsules, twice/day) from hospital admission until discharge. Cognitive function was assessed with a battery of 11 neuropsychological tests on the admission day and the seventh day after surgery, respectively. Results: A total of 96 of 106 patients completed the study, and their data were finally analyzed. POCD occurred in 12 (26.7%) of 45 patients in the probiotic group and 29 (56.9%) of 51 patients in the placebo group (relative risk [RR], 0.47 [95% confidence interval [CI], 0.27 to 0.81]; P = 0.003). Among them, mild POCD occurred in 11 (24.4%) in the probiotic group and 24 (47.1%) in the placebo group (RR, 0.52 [95% CI, 0.29 to 0.94]; P = 0.022). No significant difference in severe POCD incidence was found between the two groups (P = 0.209). Compared with the placebo group, the verbal memory domain cognitive function was mainly improved in the probiotic group. Conclusion: Probiotics may be used perioperatively to prevent POCD development and improve verbal memory performance in elderly patients receiving hip or knee arthroplasty. Clinical trial registration: www.chictr.org.cn, identifier: ChiCTR2100045620.

Nitrate-functionalized patch confers cardioprotection and improves heart repair after myocardial infarction via local nitric oxide delivery.

Nitric oxide (NO) is a short-lived signaling molecule that plays a pivotal role in cardiovascular system. Organic nitrates represent a class of NO-donating drugs for treating coronary artery diseases, acting through the vasodilation of systemic vasculature that often leads to adverse effects. Herein, we design a nitrate-functionalized patch, wherein the nitrate pharmacological functional groups are covalently bound to biodegradable polymers, thus transforming small-molecule drugs into therapeutic biomaterials. When implanted onto the myocardium, the patch releases NO locally through a stepwise biotransformation, and NO generation is remarkably enhanced in infarcted myocardium because of the ischemic microenvironment, which gives rise to mitochondrial-targeted cardioprotection as well as enhanced cardiac repair. The therapeutic efficacy is further confirmed in a clinically relevant porcine model of myocardial infarction. All these results support the translational potential of this functional patch for treating ischemic heart disease by therapeutic mechanisms different from conventional organic nitrate drugs.

Ventricular function and dyssynchrony in children with a functional single right ventricle using real time three-dimensional echocardiography after fontan operation.

BACKGROUND: This study aimed to evaluate the feasibility and clinical value of real time three-dimensional echocardiography (RT-3DE) for assessing ventricular systolic dysfunction and dyssynchrony in children with an functional single right ventricle (FSRV) having undergone the Fontan procedure. METHODS: Twenty-five children with an FSRV and 25 healthy children were enrolled in our study. RV volume analysis was performed compared with magnetic resonance imaging (MRI) as the reference standard in FSRV patients. The patients were divided into wide and narrow QRS interval groups. Global and regional functions of the RV in three compartments (inflow, body, and outflow) were compared between FSRV and control subjects, including RV systolic dyssynchrony indices of maximal difference of time to minimal volume (Tmsv-Dif), standard deviation of time to minimal volume (Tmsv-SD), maximal difference of time to minimal volume corrected by R-R interval (Tmsv-Dif%), and standard deviation of time to minimal volume corrected by R-R interval (Tmsv-SD%). RESULTS: RT-3DE measurements were significantly lower than MRI measurements for RV-EDV, RV-ESV, RV-SV, and RVEF (p < 0.01).Compared with controls, patients with an FRSV had significantly higher dyssynchrony indices and significantly lower global EF in both narrow QRS interval and wide QRS interval groups. Tmsv-SD% was shown to be most strongly correlated with MRI-RVEF (r = -.570, p = 0.003). CONCLUSIONS: RT-3DE tended to underestimate RV ventricular volume in children with FSRV. Children with an FSRV and either a wide or narrow QRS interval had reduced ventricular function and higher dyssynchrony than normal subjects. Worsening RV dyssynchrony is associated with overall decline in function after the Fontan operation.

Standard Echocardiographic View Recognition in Diagnosis of Congenital Heart Defects in Children Using Deep Learning Based on Knowledge Distillation.

Standard echocardiographic view recognition is a prerequisite for automatic diagnosis of congenital heart defects (CHDs). This study aims to evaluate the feasibility and accuracy of standard echocardiographic view recognition in the diagnosis of CHDs in children using convolutional neural networks (CNNs). A new deep learning-based neural network method was proposed to automatically and efficiently identify commonly used standard echocardiographic views. A total of 367,571 echocardiographic image slices from 3,772 subjects were used to train and validate the proposed echocardiographic view recognition model where 23 standard echocardiographic views commonly used to diagnose CHDs in children were identified. The F1 scores of a majority of views were all ≥0.90, including subcostal sagittal/coronal view of the atrium septum, apical four-chamber view, apical five-chamber view, low parasternal four-chamber view, sax-mid, sax-basal, parasternal long-axis view of the left ventricle (PSLV), suprasternal long-axis view of the entire aortic arch, M-mode echocardiographic recording of the aortic (M-AO) and the left ventricle at the level of the papillary muscle (M-LV), Doppler recording from the mitral valve (DP-MV), the tricuspid valve (DP-TV), the ascending aorta (DP-AAO), the pulmonary valve (DP-PV), and the descending aorta (DP-DAO). This study provides a solid foundation for the subsequent use of artificial intelligence (AI) to identify CHDs in children.

Ionic mechanisms underlying atrial electrical remodeling after a fontan-style operation in a canine model.

Atrial arrhythmia is an important cause of late death in patients after the Fontan-Style operation. However, the detailed electrophysiological characteristics of the post-Fontan atrium and its underlying mechanisms are largely unknown. In this study, we investigated electrophysiological characteristics and the ionic remodeling in the right atrium (RA) of a canine model after the Fontan operation. We performed the operation of RA to pulmonary artery connection to mimic the Fontan operation. We undertook hemodynamic measurements, cardiac electrophysiological studies, and ion current measurements. The expression of ionic channels was analyzed by PCR and western-blotting. Our Fontan model induced RA hypertension, enlarged the size of RA, and increased atrial fibrosis, representing the classic characteristic of Fontan patients. In the Fontan group, the atrial effective refractory period and the active potential duration were reduced, and the atrial tachycardia has been more often to be induced. The electrical conduction mapping showed that the Fontan group reduced the conduction velocity. The Fontan operation significantly down-regulated the expression of KCND3/Kv4.3, CACNA1C/Cav1.2 and SCN5A, but up-regulated the expression of KCNJ2/Kir2.1. Correspondingly, The Fontan operation reduced transient-outward (Ito) and L-type Ca2 (ICa,L) and INa currents, while increasing the inward-rectifier current (IK1). Thus, the net shortening of the action potential in the post-Fontan atrium is associated with the altered expression of ionic channels which disturbed the balance between inward and outward currents. Taken together, the Fontan operation induces the ionic remodeling, and thus altered electrophysiological characteristics of the right atrium, improving our understanding on the pathophysiology of atrial arrhythmias in Fontan patients.

Diagnosis of anomalous origin of the right coronary artery from the pulmonary artery by echocardiography.

PURPOSE: To review the imaging characteristics and evaluate the diagnostic value of echocardiography for diagnosing anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA). METHODS: We retrospectively reviewed the echocardiographic records and compared these images with operative findings in six children with ARCAPA. RESULTS: ARCAPA was characterized by dilation of the left coronary artery, inappropriate origin of the right coronary artery from the pulmonary artery, and collaterals within the interventricular septum. The associated malformations included atrial septal defect in four cases. Four of six cases were diagnosed correctly, while the remaining two cases were misdiagnosed: one of fistula between the pulmonary artery and the left coronary artery and another of ARCAPA diagnosed intraoperatively in one case of atrial septal defect. CONCLUSION: ARCAPA can be diagnosed by echocardiography but is prone to misdiagnosis. This malformation should be distinguished from coronary artery fistula.

Regression equations for calculation of z scores for echocardiographic measurements of left heart structures in healthy Han Chinese children.

OBJECTIVE: Clinical decision making in children with heart disease relies on detailed measurements of cardiac structures using two-dimensional and M-mode echocardiography. However, no echocardiographic reference values are available for the Chinese children. We aimed to establish z-score regression equations for left heart structures in a population-based cohort of healthy Chinese Han children. METHOD: Echocardiography was performed in 545 children with a normal heart. The dimensions of the aortic valve annulus (AVA), aortic sinuses of Valsalva (ASV), sinotubular junction (STJ), ascending aorta (AAO), left atrium (LA), mitral valve annulus (MVA), interventricular septal end-diastolic thickness (IVSd), interventricular septal end-systolic thickness (IVSs), left ventricular end-diastolic diameter (LVIDd), left ventricular end-systolic diameter (LVIDs), left ventricular posterior wall end-diastolic thickness (LVPWd), left ventricular posterior wall end-systolic thickness (LVPWs) were measured. Regression analyses were conducted to relate the measurements of left heart structures to body surface area (BSA). Left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (LVFS) were calculated. Several models were used, and the adjusted R2 values were compared for each model. RESULTS: AVA, ASV, STJ, AAO, LA, MVA, IVSd, IVSs, LVIDd, LVIDs, LVPWd, and LVPWs had a cubic relationship with BSA. LVEF and LVFS fell within a narrow range. CONCLUSIONS: Our results provide reference values for z scores and regression equations for left heart structures in Han Chinese children. These data may help make a quick and accurate judgment of the routine clinical measurement of left heart structures in children with heart disease.

Identification and clinical course of 166 pediatric cardiac tumors.

The aim of this study was to investigate the pathological classifications, clinical features, and natural history of pediatric cardiac tumors to provide a basis for the selection of an appropriate therapeutic method. The medical records of in- or outpatients with cardiac tumors at four hospitals were classified to analyze various types of tumor growth locations, clinical manifestations, surgical indications, and long-term follow-up results. There were 166 patients, including 158 with primary cardiac tumors, six with metastatic cardiac tumors, and two with unclassified cardiac tumors. Among the 158 cases of primary cardiac tumor, 150 were benign and eight were malignant. The rhabdomyoma, fibroma, and myxoma are the most common types of benign cardiac tumors. The major clinical manifestations of cardiac tumors include outflow tract obstruction, arrhythmia, dyspnea, pericardial effusion, heart failure, and seizures. Among the 59 patients who underwent surgery, 49 had primary benign cardiac tumors, eight had primary malignant tumors, and two had malignant metastatic tumors. Post-surgery, nine of the patients had residual tumor tissues that did not significantly affect their hemodynamics. Following surgery, there were two cases of recurrence and nine deaths, including four of benign and five of malignant tumors with mortality rates of 8.2 and 50.0 %, respectively. Of the remaining 107 cases of patients who did not undergo surgery, five (4.7 %) died. CONCLUSION: The primary benign cardiac tumors are the predominant pediatric cardiac tumors, of which rhabdomyoma, fibroma, and myxoma are the most common types. If severe symptoms are nonexistent and the hemodynamics is unaffected, most of the patients can survive in the long term despite the tumors. What is known: • Pediatric cardiac tumors are rare and are predominantly primary and benign. • The symptoms of heart failure, arrhythmia, and outflow obstruction are the most severe complications of cardiac tumors. What is new: • The rhabdomyoma, fibroma, and myxoma are the most common types of primary benign cardiac tumors. • If severe symptoms are not present and the hemodynamics are unaffected, most of the patients can survive in the long term despite the tumors.

Regression equations for calculation of z scores for echocardiographic measurements of right heart structures in healthy Han Chinese children.

OBJECTIVE: Clinical decision making in children with congenital and acquired heart disease relies on measurements of cardiac structures using two-dimensional echocardiography. We aimed to establish z-score regression equations for right heart structures in healthy Chinese Han children. METHODS: Two-dimensional and M-mode echocardiography was performed in 515 patients. We measured the dimensions of the pulmonary valve annulus (PVA), main pulmonary artery (MPA), left pulmonary artery (LPA), right pulmonary artery (RPA), right ventricular outflow tract at end-diastole (RVOTd) and at end-systole (RVOTs), tricuspid valve annulus (TVA), right ventricular inflow tract at end-diastole (RVIDd) and at end-systole (RVIDs), and right atrium (RA). Regression analyses were conducted to relate the measurements of right heart structures to 4body surface area (BSA). Right ventricular outflow-tract fractional shortening (RVOTFS) was also calculated. Several models were used, and the best model was chosen to establish a z-score calculator. RESULTS: PVA, MPA, LPA, RPA, RVOTd, RVOTs, TVA, RVIDd, RVIDs, and RA (R2 = 0.786, 0.705, 0.728, 0.701, 0.706, 0.824, 0.804, 0.663, 0.626, and 0.793, respectively) had a cubic polynomial relationship with BSA; specifically, measurement (M) = ß0 + ß1 × BSA + ß2 × BSA2 + ß3 × BSA.3 RVOTFS (0.28 ± 0.02) fell within a narrow range (0.12-0.51). CONCLUSIONS: Our results provide reference values for z scores and regression equations for right heart structures in Han Chinese children. These data may help interpreting the routine clinical measurement of right heart structures in children with congenital or acquired heart disease. © 2016 Wiley Periodicals, Inc. J Clin Ultrasound 45:293-303, 2017.

Coronary artery indexed diameter and z score regression equations in healthy Chinese Han children.

OBJECTIVE: In children with coronary disease, clinical decision should be based on detailed measurements of the coronary arteries by two-dimensional echocardiography. We aimed to establish coronary artery reference indexed diameter and z scores regression equations in a large cohort of Chinese Han children. METHODS: We measured the diameter of the proximal right (RCA), left main (LMCA), left anterior descending, and left circumflex coronary artery, and of the aortic annulus, and calculated the coronary-aorta index (coronary artery-to-aortic annulus ratio) in 506 Chinese Han children with normal hearts whose ages ranged from 1 day to 18 years. Regression analyses were performed, relating the coronary artery dimensions to body surface area (BSA). Several models were used, and the best model (yielding the maximum adjusted R(2) ) was chosen to establish a z score calculator. RESULTS: Based on cubic regression, (M) = ß0 + ß1 × BSA + ß2 × BSA(2) + ß3 × BSA(3) , the adjusted R(2) values were 0.515, 0.553, 0.505, and 0.518 for the RCA, LMCA, left anterior descending, and left circumflex coronary artery models, respectively. RCA/aortic annulus was 0.14 ± 0.02 (range, 0.07-0.24) and LMCA/AOA was 0.15 ± 0.03 (range, 0.10-0.28). CONCLUSIONS: Our results provide reference values of coronary artery z scores, regression equation, and coronary-aorta index as a quick guide to determine coronary dilation in Chinese Han children.

Familial congenital heart disease: data collection and preliminary analysis.

The aim of this study was to explore genetic mechanisms of congenital heart disease by analysing family data. Families with two or more affected members were studied, and information on family history and risk factors was collected. A total of 25 families with congenital heart disease were identified, and among them the condition was confirmed in 57. The prevalence of congenital heart disease in first-degree relatives was 43.0%, that is 46 out of 107, significantly higher than that in second-degree relatives, that is, 4.4%, 11 out of 252) (χ2 = 83.897, P < 0.01). The prevalence difference between twins (90%) and siblings (62.2%) (χ2 = 4.983, P < 0.05) was also significant among first-degree relatives. Eleven families were found to have the same phenotype (44%), including ventricular septal defect in six families, atrial septal defect in two families, conotruncal defects in two families, and hypoplastic left heart syndrome in one family. Both twins were diagnosed with congenital heart disease in 8 out of 10 twin families - all eight twins were monozygotic. The cardiac phenotype of the twins was consistent in three families (37.5%). The cardiac phenotype of first- and second-degree relatives was not fully consistent with their probands. There was an increased incidence of threatened abortion in early pregnancy in patients with familial congenital heart disease when compared with sporadic congenital heart disease (χ2 = 8.704, P < 0.05). Morbidity in relatives was related to blood relationship, with a closer relationship increasing the risk of congenital heart disease. Genetic factors appear to play an important role in congenital heart disease.

Echocardiographic assessment of juxtaposition of the right atrial appendage in children with congenital heart disease.

The objective of this study was to analyze the echocardiographic characteristics of juxtaposition of the atrial appendages and to determine its prevalence in children with congenital heart disease. From June 1998 to December 2008, 10,880 children underwent selective angiocardiography, magnetic resonance imaging (MRI), and echocardiography for evaluation of congenital heart disease. Juxtaposition of the atrial appendages was diagnosed based on the results of angiocardiography and MRI; the echocardiographic characteristics of this anomaly were analyzed retrospectively. There were 33 patients diagnosed with juxtaposition of the right atrial appendage (JRAA); no patient was diagnosed with juxtaposition of the left atrial appendage. The prevalence of JRAA in children with congenital heart disease was 0.30%. JRAA and abnormal spatial orientation of the atrial septum were visualized by Doppler echocardiography in 28 cases. In the remaining five cases, three cases with suspected JRAA could not be determined by echocardiography and the diagnosis was missed in two cases. The most common associated anomalies were conotruncal malformations (16 cases with double outlet of the right ventricle, 9 cases with pulmonary atresia, 6 cases with transposition of the great arteries, 1 case with tetralogy of Fallot) and tricuspid malformations (6 cases with tricuspid straddling, 3 cases with tricuspid atresia, 2 cases with tricuspid valve stenosis). Based on the characteristic alteration of the plane of the atrial septum and visualization of the malpositioned right atrial appendage, JRAA can be accurately diagnosed by Doppler echocardiography.

Three-dimensional echocardiographic virtual endoscopy for the diagnosis of congenital heart disease in children.

Virtual endoscopy (VE) is a new post-processing method that uses volumetric data sets to simulate the tracks of a "conventional" flexible endoscope. However, almost all studies of this method have involved virtual visualizations of the cardiovascular structures applied to computed tomography (CT) and magnetic resonance (MR) datasets. This paper introduces a novel visualization method called the "three-dimensional echocardiographic intracardiac endoscopic simulation system (3DE IESS)", which uses 3D echocardiographic images in a virtual reality (VR) environment to diagnose congenital heart disease. The aim of this study was to analyze the feasibility of VE in the evaluation of congenital heart disease in children and its accuracy compared with 2DE. Three experienced pediatric cardiologists blinded to the patients' diagnoses separately reviewed 40 two-dimensional echocardiographic (2DE) datasets and 40 corresponding VE datasets and judged whether abnormal intracardiac anatomy was present in terms of a five-point scale (1 = definitely absent; 2 = probably absent; 3 = cannot be determined; 4 = probably present; and 5 = definitely present). Compared with clinical diagnosis, the diagnostic accuracy of VE was 98.7% for ASD, 92.4% for VSD, 92.6% for TOF, and 94% for DORV, respectively. Diagnostic accuracy of VE was significantly higher than that of 2DE for TOF and DORV except for ASD and VSD. The receiver operating characteristic (ROC) curve for VE was closer to the optimal performance point than was the ROC curve for 2DE. The area under the ROC curve was 0.96 for VE and 0.93 for 2DE. Kappa values (range, 0.73-0.79) for VE and 2DE indicated substantial agreement. 3D echocardiographic VE can enhance our understanding of intracardiac structures and facilitate the evaluation of congenital heart disease.

Echocardiographic assessment of conjoined twins with congenital heart disease in Shanghai.

BACKGROUND: The aim of this study is to improve the diagnosis of congenital heart disease in conjoined twins using echocardiography. METHODS: Six sets of conjoined twins at Shanghai Children's Medical Center and Xin-Hua Hospital from 1980 to 2006 were assessed retrospectively. Cardiac anomalies of varying degrees of severity were first evaluated using 2D echocardiography. Tissue Doppler imaging (TDI) was then performed if fused ventricles were suspected. Peak systolic velocities, peak systolic strain rates, and peak systolic strains at the fused myocardium were quantitatively measured using strain rate imaging (SRI). If the M-mode echocardiographic image was poor or difficult to measure, the myocardial performance index (MPI) of the left ventricle was calculated. RESULTS: Intracardiac anomalies were diagnosed correctly using echocardiography and confirmed by surgical findings. Cardiac functional assessment using MPI demonstrated heart failure in three out of the eight individuals. Subtle conjunction of the ventricles, however, were more difficult to visualize and two sets of conjoined twins with fused hearts could not be clearly demonstrated by echocardiography. In one case of suspected ventricular wall conjunction, TDI showed separate ventricular myocardia which was confirmed at surgery. CONCLUSIONS: Intracardiac anomalies and ventricular function can be readily demonstrated by echocardiography, but subtle conjunction of the ventricles may be more difficult to visualize. TDI may be required if there is uncertainty about the degree of cardiac fusion.