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Background Kawasaki disease (KD) is a medium vessel vasculitis that typically occurs in children aged between 6 months and 5 years. It is extraordinarily rare in the neonatal period. KD-related systemic artery aneurysms (SAAs) have never been reported in neonates. Case presentation A male infant was transferred to our institution for persistent high-grade fever lasting 16 days. Symptoms started at day 14 of life, and he was admitted to a children’s hospital on the second day of fever. Physical examination at the time found no signs suggestive of KD. The only laboratory parameters which were of significance were values suggestive of systemic inflammation. However, his fever persisted and inflammatory markers continued to rise despite 2 weeks of antibiotic therapy. KD as a noninfectious cause of fever was considered when he came to our institution, and echocardiographic findings of left and right medium coronary artery aneurysms (CAAs) confirmed our suspicions. Full-body magnetic resonance angiography also revealed bilateral axillary artery aneurysms. Administration of intravenous gamma globulin resulted in rapid improvement. His fever resolved on the next day and CAAs and SAAs regressed to normal at 6 months and 3 months after diagnosis, respectively. Conclusion This unique case of incomplete KD highlights the importance of considering KD in neonates with unexplained prolonged fever and reinforces the need to remain vigilant for SAAs in KD.

BACKGROUND: Coronary artery aneurysms (CAAs) are a well-known complication of Kawasaki disease (KD), but there are no data on incidence or outcomes of systemic artery aneurysms (SAAs) in the current era. METHODS: From April 1, 2016, to March 31, 2019, we screened for SAAs in 162 patients with KD at risk for SAAs with magnetic resonance angiography or peripheral angiography and analyzed incidence and early outcomes of SAAs. RESULTS: Twenty-three patients had SAAs, demonstrating an incidence of 14.2% (23 of 162) in patients who were screened at 1 month after onset. The proportion of patients with SAAs was estimated to be 2% (23 of 1148) of all patients with KD. The median age at onset of KD with SAAs was 5 months. All patients with SAAs had CAAs, with z scores >8. Of patients with giant CAAs, 38.6% (17 of 44) had SAAs. A total of 129 SAAs occurred in 17 different named arteries. The most common sites for SAAs were the axillary (18.6%), common iliac (12.4%), and brachial (11.6%) arteries. During a median follow-up time of 6 months, 92.9% (79 of 85) of SAAs had some degree of regression, with 80% (68 of 85) of SAAs returning to normal. The overall regression rate was higher for medium to large SAAs than for medium to giant CAAs. CONCLUSIONS: Although the incidence of SAAs may not be as dramatically reduced as we expected compared with previous data, SAAs have a high regression rate during short-term follow-up.

OBJECTIVES: Pulse oximetry (POX) has been confirmed as a specific screening modality for critical congenital heart disease (CCHD), with moderate sensitivity. However, POX is not able to detect most serious and critical cardiac lesions (major congenital heart disease [CHD]) without hypoxemia. In this study, we investigated the accuracy and feasibility of the addition of cardiac auscultation to POX as a screening method for asymptomatic major CHD. METHODS: A multicenter prospective observational screening study was conducted at 15 hospitals in Shanghai between July 1, 2012, and December 31, 2014. Newborns with either an abnormal POX or cardiac auscultation were defined as screen positive. All screen-positive newborns underwent further echocardiography. False-negative results were identified by clinical follow-up, parents’ feedback, and telephone review. We assessed the accuracy of POX plus cardiac auscultation for the detection of major CHD. RESULTS: CHD screening was completed in all 15 hospitals, with a screening rate of 94.0% to 99.8%. In total, 167 190 consecutive asymptomatic newborn infants were screened, of which 203 had major CHD (44 critical and 159 serious). The sensitivity of POX plus cardiac auscultation was 95.5% (95% confidence interval 84.9%–98.7%) for CCHD and 92.1% (95% confidence interval 87.7%–95.1%) for major CHD. The false-positive rate was 1.2% for detecting CCHD and 1.1% for detecting major CHD. CONCLUSIONS: In our current study, we show that using POX plus cardiac auscultation significantly improved the detection rate of major CHD in the early neonatal stage, with high sensitivity and a reasonable false-positive rate. It provides strong evidence and a reliable method for neonatal CHD screening.

Summary Background Several pioneering studies have provided evidence for the introduction of universal pulse oximetry screening for critical congenital heart disease. However, whether the benefits of screening reported in studies from high-income countries would translate with similar success to low-income countries is unknown. We assessed the feasibility and reliability of pulse oximetry plus clinical assessment for detection of major congenital heart disease, especially critical congenital heart disease, in China. Methods We did a pilot study at three hospitals in Shanghai to assess the accuracy of pulse oximetry plus clinical assessment for detection of congenital heart disease. We made a data collection plan before recruitment. We then undertook a large, prospective, and multicentre screening study in which we screened all consecutive newborn babies (aged 6–72 h) born at 18 hospitals in China between Aug 1, 2011, and Nov 30, 2012. Newborn babies with positive screen results (either an abnormal pulse oximetry or abnormal clinical assessment) were referred for echocardiography within 24 h of screening. We identified false-negative results by clinical follow-up and parents' feedback. We calculated sensitivity, specificity, positive and negative predictive values, and positive and negative likelihood ratios for pulse oximetry alone, and in combination with clinical assessment, for detection of major and critical congenital heart disease. Findings In the pilot study, 6785 consecutive newborn babies were screened; 46 of 49 (94%) cases of asymptomatic major congenital heart disease and eight of eight (100%) cases of asymptomatic critical disease were detected by pulse oximetry and clinical assessment. In the prospective multicentre study, we screened 122 738 consecutive newborn babies (120 707 asymptomatic and 2031 symptomatic), and detected congenital heart disease in 1071 (157 critical and 330 major). In asymptomatic newborn babies, the sensitivity of pulse oximetry plus clinical assessment was 93·2% (95% CI 87·9–96·2) for critical congenital heart disease and 90·2% (86·4–93·0) for major disease. The addition of pulse oximetry to clinical assessment improved sensitivity for detection of critical congenital heart disease from 77·4% (95% CI 70·0–83·4) to 93·2% (87·9–96·2). The false-positive rate for detection of critical disease was 2·7% (3298 of 120 392) for clinical assessment alone and 0·3% (394 of 120 561) for pulse oximetry alone. Interpretation Pulse oximetry plus clinical assessment is feasible and reliable for the detection of major congenital heart disease in newborn babies in China. This simple and accurate combined method should be used in maternity hospitals to screen for congenital heart disease. Funding Key Clinical Research Project sponsored by Ministry of Health, Shanghai Public Health Three-Year Action Plan sponsored by Shanghai Municipal Government, and National Basic Research Project of China.

Shone's complex is a rare and severe type of congenital left-sided obstructive lesion of multiple cardiovascular levels. This report aims to present the authors' experience diagnosing atypical Shone's complex using echocardiography. Atypical Shone's complex was diagnosed for 38 consecutive patients (18 boys and 20 girls, ages 1 day to 15 years) using echocardiography. All the diagnoses were confirmed by cardioangiography, surgery, or both. Among the congenital left-sided obstructive lesions, four levels of obstruction coexisted in 3 cases (7.9%), three levels in 10 cases (26.3%), and two levels in 25 cases (65.8%). Supra-annular mitral stenosis caused by a supravalvular mitral ring was diagnosed in 1 case. Mitral valvular stenosis was diagnosed in 24 cases including a parachute mitral valve (4 cases), a double-orifice mitral valve (1 case), and partly fused thickened mitral valvular leaflets (19 cases). Subaortic stenosis was diagnosed in 10 cases including circumferential membranous subaortic stenosis (1 case), tunnel-type subaortic narrowing (2 cases), and asymmetric obstructive fibrous ridge below the aortic valve (7 cases). Aortic stenosis was diagnosed in 25 cases including the bicuspid aortic valve (12 cases), the hypoplasia aortic annulus (3 cases), and partly fused or thickened tri-leaflet aortic valves (10 cases). Supra-aortic stenosis was diagnosed in 6 cases including the localized type (4 cases) and the diffused type (2 cases). Coarctation of the aorta was diagnosed in 26 cases. Of these, focal coarctation was described in 15 cases, long segment coarctation in 7 cases, and dysplastic isthmus and/or descending aorta in 4 cases. Secondary changes in Shone's complex manifested as left atrial dilation (26 cases), left ventricular hypertrophy (26 cases), and pulmonary hypertension (19 cases). Echocardiography plays an important role in the diagnosis of atypical Shone's complex, in comprehensive evaluation of the number, position, morphology, and severity of left-sided obstructions.

Background: This study aimed to test the hypothesis that alteration of left ventricular (LV) torsional mechanics occurs in patients after repair of tetralogy of Fallot (TOF) and is associated with right ventricular (RV) volume overload and changes in LV configuration. Methods and Results: Fifty-five TOF patients aged 19.0±8.1 years and 27 age-matched healthy controls were studied. The LV and RV volumes were measured using 3-dimensional echocardiography while LV geometry was quantified by the diastolic eccentricity index (EI). The LV peak systolic torsion and systolic twisting and diastolic untwisting velocities were determined by speckle tracking. Compared with controls, patients had significantly greater RV end-systolic (P

Left ventricular (LV) dysfunction is 1 of the major determinants of late adverse clinical outcomes in patients after surgical repair of tetralogy of Fallot (TOF). The aim of this study was to test the hypothesis that LV myocardial deformation is impaired in patients after TOF repair and related to right ventricular (RV) dilation and exercise capacity. Longitudinal, radial, and circumferential LV myocardial deformation was determined using speckle-tracking echocardiography in 23 postoperative patients with TOF and compared to that of 23 age-matched controls. Relations between LV strain and strain rate (SR) and RV volumes and exercise parameters were determined in patients. Compared to controls, patients had reduced global LV longitudinal, radial, and circumferential strain (all p values0.05). Patients with significantly increased RV end-systolic volume (2 SDs higher than normal; n = 17) had reduced global LV circumferential strain (p = 0.048) and SR (p = 0.038), but similar longitudinal and radial speckle-tracking echocardiographic parameters, compared to those without (n = 6). RV end-systolic volume was correlated inversely with global LV circumferential strain and SR (r = -0.58, p = 0.004, and r = -0.58, p = 0.005, respectively), while RV end-diastolic volume was correlated only with global LV circumferential strain (r = -0.43, p = 0.047). In patients, the LV ejection fraction was correlated with global LV circumferential strain (r = 0.54, p = 0.01) and SR (r = 0.66, p = 0.001) but not with longitudinal or radial speckle-tracking echocardiographic parameters. Using multivariate analysis, global LV circumferential SR (beta = 0.66, p = 0.001) and male gender (beta = 0.46, p = 0.012) were identified as independent predictors of peak oxygen consumption. In conclusion, the negative impact of RV dilation on LV function relates to its influence on LV circumferential strain and SR in patients after TOF repair.

AIM The relationship was investigated between endothelial progenitor cells (EPCs) level and arterial functions in the convalescence of Kawasaki disease (KD). METHODS Sixty-three children were divided into coronary artery lesion (CAL) group (group 1, n = 21), non-CAL group (group 2, n = 20) and control group (group 3, n = 22). EPCs were examined by flow cytometry and arterial functions (flow-mediated dilation [FMD], carotid artery stiffness index [SI]) were measured by ultrasound. RESULTS From group 1 to group 3, FMD was 4.5%+/- 1.5%, 9.5%+/- 2.8% and 12.1%+/- 2.3% (p < 0.01 between any two groups); carotid artery SI was 4.10 +/- 0.44, 3.81 +/- 0.50 and 3.59 +/- 0.46 (group 1 vs. group 2, p < 0.05; group 1 vs. group 3, p < 0.01; group 2 vs. group 3, p = 0.142) and the number of EPCs was 2.0 +/- 0.6/microL, 4.2 +/- 0.8/microL, 4.5 +/- 0.7/microL (p < 0.01 for group 1 vs. group 2 and group 1 vs. group 3; group 2 vs. group 3, p = 0.292). Multiple linear regressions analysis and correlation analysis identified that FMD and carotid artery SI were significant determinants of EPCs level and were all independently correlated with EPCs level. CONCLUSIONS Our results indicate decreased EPCs are associated with arterial dysfunction in patients with CAL in the convalescence of KD. Our findings suggest EPCs may have a role in alteration of arterial functions.

OBJECTIVE. We tested the hypothesis that single-nucleotide polymorphisms of inflammatory genes C-reactive protein (CRP) and tumor necrosis factor α (TNF-α) may exert influence on susceptibility to Kawasaki disease and its arterial sequelae. METHODS. We analyzed the CRP +1444 C→T and TNF-α −308 G→A polymorphisms in 167 patients aged 8.9 ± 4.1 years with a history of Kawasaki disease (73 with and 94 without coronary aneurysms) and 124 healthy control subjects. For patients with Kawasaki disease, we further determined whether these single-nucleotide polymorphisms were associated with coronary aneurysms, carotid arterial stiffening, and intima-media thickness. RESULTS. Genotypic and allelic frequencies of CRP +1444 for T carrier and TNF-α −308 for A carrier were significantly higher in patients than in control subjects. The genotypic and allelic distributions did not differ between patients with and those without coronary aneurysms; however, patients with CRP +1444 CT/TT genotype compared with those with a CC genotype and patients with TNF-α −308 GA/AA genotype compared with those with a GG genotype had significantly greater carotid arterial stiffness and intima-media thickness. Carriers of both CRP +1444 T allele and TNF-α −308 A allele had the highest susceptibility to Kawasaki disease and a significant trend of increased arterial stiffness and intima-media thickness compared with those who carried either 1 or none of the rare alleles. Multiple linear regression analysis identified CRP +1444 allele carrier as a significant determinant of both carotid stiffness and carotid intima-media thickness and TNF-α −308 A allele carrier as a determinant of only intima-media thickness. CONCLUSIONS. Our findings suggest that CRP +1444 C→T and TNF-α −308 G→A polymorphisms are associated with predisposition to Kawasaki disease and, in patients with Kawasaki disease, increased carotid arterial stiffness and intima-media thickness in the long-term.

Recent data suggest potential benefits of cardiac resynchronization therapy in the management of right ventricular (RV) dysfunction in congenital heart disease. The aim of this study was to determine the nature, prevalence, and functional implications of mechanical RV dyssynchrony in patients after Senning or Mustard procedures for transposition of the great arteries. Twenty-eight patients (mean age 21.1 +/- 3.5 years) at 19.9 +/- 3.2 years after atrial switch operations and 29 healthy controls were studied. The times from the onset of QRS to peak systolic strain (T epsilon) at the base of and the mid RV free wall, the ventricular septum (VS), and the left ventricular (LV) free wall were determined using tissue Doppler echocardiography. Intraventricular mechanical delay was defined as Delta T epsilon(RV-VS) and interventricular mechanical delay as Delta T epsilon(RV-LV). In patients, the magnitude of RV intra- and interventricular mechanical delay was correlated with cardiac magnetic resonance-derived RV volumes and ejection fractions (n = 26) and treadmill exercise testing parameters (n = 20). Compared with controls, patients had significantly longer Delta T epsilon(RV-VS) (48.1 +/- 50.9 vs 17.0 +/- 16.1 ms, p0.001) and Delta T epsilon(RV-LV) (63.1 +/- 49.5 vs 19.0 +/- 12.9, p0.001). Nine patients (32%) exhibited RV dyssynchrony (Delta T epsilon(RV-VS)49 ms, control mean +/- 2SD), and 16 patients (57%) showed interventricular dyssynchrony (Delta T epsilon(RV-LV)45 ms). In patients, RV intra- and interventricular mechanical delay was correlated negatively with the RV ejection fraction (both r = -0.42, p = 0.03) and percentage predicted maximum oxygen consumption (r = -0.50, p = 0.03, and r = -0.52, p = 0.02, respectively) and positively with minute ventilation/carbon dioxide production slope (r = 0.49, p = 0.03, and r = 0.56, p = 0.01, respectively). In conclusion, RV dyssynchrony is common in young adults after atrial switch operations and is associated with RV systolic dysfunction and impaired exercise performance.

Objective: To evaluate the role of transoesophageal echocardiography and problems related to safety during the surgical repair of congenital cardiac malformations in children. Methods: We examined the transoesophageal recordings made in 350 children with congenital cardiac diseases, aged from 2 months to 17 years and 9 months, with a median age of 2.7 years, tracings having been taken both before and after cardiopulmonary bypass. All patients had been scanned by transthoracic echocardiography before the operative procedures. Results: Preoperative transoesophageal echocardiography added additional findings, or changed the diagnoses made using transthoracic echocardiography, in 33 cases (9.4%), among which the findings had therapeutic significance in 23 cases (6.6%) that altered the planned surgical procedures. Residual problems or sequels were detected by postoperative transoesophageal echocardiography in 57 cases (16.3%), with 13 patients (3.7%) requiring instant intervention or return to bypass for modifications of the surgical procedures. We encountered no severe complications due to the performance of transoesophageal echocardiography. Mild complications occurred in only 2 patients (0.6%). Conclusions: Transoesophageal echocardiography is a useful tool with which to determine the strategies for treatment in the perioperative period, and to improve the quality of surgical procedures in children with congenital cardiac diseases. Complications were few, but still deserved careful attention to detail.

To evaluate the value of echocardiography in the diagnosis of noncompaction of the ventricular myocardium (NCVM) and to elucidate the echocardiographic characteristics of NCVM.This study included 53 patients (28 boys and 25 girls), with an age for initial diagnosis of 15 days to 18 years, who were diagnosed with NCVM by echocardiography between May 2006 and May 2015. Transthoracic two-dimensional echocardiography and color Doppler were performed for qualitative diagnosis, and the end-diastolic non-compacted layer/compacted layer (N/C) ratio measured in the parasternal ventricular short-axis sectional view was selected as the criterion for quantitative diagnosis.The excessively prominent ventricular trabeculae and deep inter-trabecular recesses were all seen in 53 cases, and the blood flow in the cardiac chambers was connected to the inter-trabecular recesses. The areas involved in NCVM were mainly the apex (100%) and the middle segment of the left ventricular lateral wall (98%), followed by the middle segment of the left ventricular posterior wall (49%) and the middle segment of the left ventricular inferior wall (42%). In 53 children with NCVM, the N/C ratio was 4.3±1.9 (2.1-10.0). Cardiac insufficiency was found in 83% (44/53) of the children with NCVM, and the left ventricular ejection fraction for these children was (43±9)%.Echocardiography can be used in the qualitative and quantitative diagnosis of NCVM and in the evaluation of cardiac function. The apex and the middle segment of the left ventricular lateral wall are often involved in NCVM, accompanied by decrease in the left ventricular ejection fraction.

Background Real-time three-dimensional echocardiography (RT-3DE) has made revolutionized improvements of cardiac imaging during the past few years. However, there is no standard examination method for RT-3DE so far. This study aimed to establish the diagnostic method of RT-3DE and evaluate its application in the diagnosis of complex congenital heart diseases (CHD). Methods Fifty patients with complex CHD were examined by RT-3DE with modes of Live 3DE and Full Volume. A series of novel volumetric views combined with Van Praagh sequential segmental approach were introduced to reveal the pathological morphology of the hearts, which were compared with the findings of two-dimensional echocardiography (2DE), angiography and cardiac surgery. Results In 50 patients, 190 image acquisitions of Full Volume were performed at several acoustic windows including subcostal, apical and parastemal regions. Among them, 94.2% (179/190) of image acquisitions were successful. Most sectional volumetric views could be clearly displayed in 92.6% of the successful image acquisitions. However, sectional volumetric views could not be clearly displayed in 7.4%, which was mainly due to poor perspective conditions of examination location, improper instrument multi-parameter setting and insufficient information of whole heart captured in Full Volume acquisitions. As compared with surgical findings and angiography, RT-3DE made correction to the diagnoses in 2 cases including 1 with corrected transposition of the great arteries and the other with single atrium and mitral cleft. The diagnoses initially made by 2DE for these 2 patients were double outlet right ventricle with transposition of the great arteries and complete atrio-ventricular septal defect. Conclusions RT-3DE can clearly display the pathological morphology of complex CHD by a series of novel volumetric views combined with sequential segmental approach through providing more spatial informative cardiovascular structures, which provides a practical method for RT-3DE diagnosis.

To assess the accuracy of cardiac output (CO) measured by transpulmonary thermodilution technique (TPTD)and explore the validity of intrathoracic blood volume index (ITBVI) for assessment of circulatory volume status.Ten immature pigs with a mean weight of (20.6±1.9)kg were studied during the conditions including normovolemia, hypervolemia, and hypovolemia. Simultaneous CO was measured in each condition using pulmonary artery thermodilution (PATD) method and TPTD. More specifically, CO (COPA) was determined with PATD, while CO (COTP) and ITBVI were determined with TPTD. All measurements were repeated 3 times. Central venous pressure (CVP) and heart rate were measured at the same time. The potential correlations of CVP and ITBVI with cardiac index (CI) and stroke volume index (SVI) in each blood volume status were analyzed.A total of 90 simultaneous measurements of COPA and COTP in 3 different blood volume conditions were made. The correlation coefficient between the two measurements was 0.977 (P0.001) and the mean difference was (0.25±0.26)L/min (95%CI:0.20-0.30 L/min, P0.001). The coefficient of variation of COTP was 3.7%, while COPA was 5.4%. Compared with those in normovolemia, CVP and ITBVI in hypervolemia significantly increased (P=0.002, 0.019), ITBVI in hypovolemia decreased significantly (P0.001), and CVP in hypovolemia decreased insignificantly (P=0.05). Correlation analysis revealed a significant correlation between ITBVI with CI and SVI in normovolemia (r=0.741, P=0.014; r=0.885, P=0.001). In contrast, correlations between CVP with CI and SVI were poor.TPTD can accurately and precisely measure CO in different blood volume conditions. ITBVI measured by TPTD has better validity for the assessment of circulatory volume status than CVP.

To assess the effects and potential role of percutaneous balloon valvuloplasty as an alternative therapy of surgery in young infants with severe and critical pulmonary valve stenosis.Eighteen patients aged 8 days to 6 months with severe and critical pulmonary valve stenosis admitted to our hospital from June 2006 to August 2008 underwent balloon valvuloplasty. Among them, 11 infants including 2 neonates had critical pulmonary stenosis. Severe tricuspid regurgitation was seen in 5 and moderate in 3. Right ventricular systolic pressure in all patients was greater than systemic pressure with right-to-left or bi-directional shunt at atrial level. Dilatation was performed under general anesthesia with intubation in 12 patients and caudal block combined with sedation in 6 patients. Dilatation with 2 balloons sequentially in one procedure was performed in 6 patients and dilatation with 1 balloon in other 10 patients.Of the 18 patients, there was failure to cross the pulmonary valve with balloon catheter in one and cardiac tamponade in one. The dilatation success rate was 88.9%. Immediately after dilatation, the systemic pressure gradient from right ventricle to pulmonary artery decreased from (87 +/- 24) mm Hg to (30 +/- 19) mm Hg (P0.01). No complication was found in all patients during or post dilation. During a follow-up of 6 to 32 months, pressure gradient crossing pulmonary valve measured by echocardiography further decreased or remained stable in 16 cases, except one neonate and one infant whose pressure gradient gradually increased and required a second dilatation. Re-dilatation rate was 12.5%. Tricuspid regurgitation was reduced in all patients. Mild pulmonary regurgitation was seen in most of patients post-dilatation, except moderate in one. All patients fared well and stayed asymptomatic.Percutaneous balloon valvuloplasty for severe and critical pulmonary stenosis in infants is relatively safe and effective and should be considered a valid alternative to surgical operation. It should be the first choice for such patients based on its excellent outcome, less trauma and fewer complications.

Background The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle. Hypothesis Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS. Methods A total of 27 patients, age 16.5 ± 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity. Results Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%–94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05). Conclusion Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS. Copyright © 2010 Wiley Periodicals, Inc.

We tested the hypothesis that diastolic ventricular interaction occurs after atrial switch operation for transposition of the great arteries (TGA) and that subpulmonary LV diastolic function is influenced by septal geometry.Twenty-nine patients (male 19) after atrial switch operation for TGA aged 20.8 ± 4.1 years and 27 healthy controls were studied. Two-dimensional longitudinal systolic strain, systolic (SRs), early diastolic (SRe), and late diastolic (SRa) strain rates of both ventricles were determined using speckle tracking echocardiography. Early diastolic trans-atrioventricular velocity (E) and myocardial early diastolic myocardial velocity (e) at the ventricular free wall-annular junction were measured. Geometry of the morphologic left ventricle was quantified by the diastolic eccentricity index (EI).In both systemic and subpulmonary ventricles, SRe and SRa were significantly lower and trans-atrioventricular E/e ratios higher in patients than controls (all p0.001). In patients, RV SRe correlated with left ventricular (LV) SRe (r=0.49, p=0.008), and RV SRa correlated with LV SRa (r=0.46, p=0.01). Significant leftward shifting of the septum in patients was reflected by the greater LV EI (p0.001). In patients, LV EI correlated with age- and sex-adjusted z score of LV end-diastolic volume. As a group, LV EI correlated negatively with LV SRe (r=-0.62, p0.001) and LV SRa (r=-0.51, p0.001), and positively with mitral E/e ratio (r=0.33, p=0.02).Systemic RV diastolic dysfunction occurs after atrial switch operation and correlates with subpulmonary LV diastolic dysfunction. The observed diastolic ventricular interaction may potentially be mediated through alteration of septal geometry.

Background: Increasing data suggest that parameters of myocardial deformation are strong indices of ventricular systolic and diastolic function. We sought to determine myocardial deformation of the left ventricle and assess relationship of deformation rates with myocardial iron load in patients with beta-thalassemia major. Methods: The left ventricular longitudinal, circumferential, and radial myocardial deformation was determined using speckle tracking echocardiography in 42 thalassemia patients aged 24.4 ± 6.4 years. The results were compared with those of 38 age-matched controls. The rates of longitudinal and circumferential deformation were correlated with cardiac T2* magnetic resonance findings. Results: Compared with controls, patients had significantly greater global systolic radial strain (P = 0.001), but similar global systolic longitudinal (P = 0.12) and circumferential strain (P = 0.84). On the other hand, patients had significantly lower longitudinal systolic strain rate (SR) (P = 0.019), longitudinal early diastolic SR (P = 0.036), and circumferential early diastolic SR (P = 0.04) than controls. The cardiac T2* findings correlated positively with longitudinal (r = 0.44, P = 0.004) and circumferential early diastolic SR (r = 0.37, P = 0.019), but not with the respective systolic SRs and left ventricular ejection fraction (all P > 0.05). Patients with iron overload (T2*< 20 msec), compared to those without, had significantly lower longitudinal (1.45 ± 0.33/sec vs. 1.76 ± 0.27/sec, P = 0.002) and circumferential (1.01 ± 0.31/sec vs. 1.22 ± 0.31/sec, P = 0.03) early diastolic SR. Conclusions: Patients with beta-thalassemia major have reduced longitudinal systolic SR, longitudinal early diastolic SR, and circumferential early diastolic SR. The rates of diastolic deformation in the longitudinal and circumferential dimensions are inversely related to myocardial iron overload. (Echocardiography 2010;27:253-259)

This study aimed to test the hypothesis that right ventricular (RV) volume overload may result in left ventricular (LV) systolic dysfunction through induction of LV systolic dyssynchrony after tetralogy of Fallot (TOF) repair. Sixty patients with TOF repair were studied at 14.3 +/- 7.2 years after TOF repair. Real-time 3-dimensional echocardiographic data sets were acquired for measurement of LV and RV volumes and derivation of ejection fractions and pulmonary regurgitant volume. The LV systolic dyssynchrony index (SDI) was derived from the dispersion of time to minimum regional volume using a 16-segment model. The results were compared with those of 29 healthy controls. LV SDI was significantly higher in patients than controls (7.4% +/- 2.0% vs 3.0% +/- 0.9%, p0.001). The prevalence of LV mechanical dyssynchrony (SDI4.7%) in patients was 93% (95% confidence interval 87% to 100%). The time to minimum regional volume was significantly longer in all of the 6 basal segments and the midposterior segment (all p0.05) in patients than controls. Multivariate analysis identified RV end-diastolic volume (beta = 0.58, p0.001), LV ejection fraction (beta = -0.38, p0.001), and LV end-diastolic volume (beta = 0.26, p = 0.002) as significant correlates of LV SDI. In conclusion, in patients after TOF repair, RV volume overload has a negative effect on LV systolic function through induction of global LV mechanical dyssynchrony.

To determine the usefulness of new two-dimensional strain indices, based on speckle tracking imaging, for assessment of systemic right ventricular (RV) function after an atrial switch operation for transposition of the great arteries.Cross-sectional study.Tertiary paediatric cardiac centre.26 patients, mean (SD) age 21.0 (3.6) years at 19.9 (3.2) years after an atrial switch operation, and 27 age-matched controls were studied. Two-dimensional imaging at the four-chamber view was obtained with tracing of the entire RV endocardial border. The RV global longitudinal strain (GLS) and GLS rate were derived using automated software (EchoPAC, GE Medical) and correlated with tissue Doppler-derived RV isovolumic acceleration (IVA), and, in the patient cohort, with cardiac magnetic resonance-derived RV ejection fraction.Intra- and interobserver variability for measurement of GLS, as determined from the mean (SD) of differences in two consecutive results from 20 studies, were 0.06 (1.39)% and 0.24 (1.77)%, respectively. Compared with controls, patients had lower RV GLS (17.1 (1.9)% vs 26.3 (2.9)%, p0.001), a reduced GLS rate (0.78 (0.11)/s vs 1.33 (0.23)/s, p0.001), lower RV IVA (1.10 (0.36) m/s(2) vs 1.56 (0.53) m/s(2), p0.001) and increased RV myocardial performance index (0.52 (0.09) vs 0.38 (0.09), p0.001). Both RV GLS and GLS rate correlated positively with RV IVA (r = 0.43, p = 0.001 and r = 0.46, p0.001, respectively), and negatively with RV myocardial performance index (r = -0.65, p0.001 and r = -0.57, p0.001, respectively). In patients, the GLS rate correlated positively with RV ejection fraction (r = 0.62, p = 0.001).Two-dimensional RV GLS and GLS rate are new, potentially useful indices for assessment of systemic RV function.

To explore the endothelial function, carotid artery stiffness index and carotid intima-media thickness in children with a history of Kawasaki disease (KD).A cohort of 204 children was studied, which comprised 51 patients with Kawasaki disease with coronary artery lesion (CAL group), 50 patients with Kawasaki disease with normal coronary arteries (normal coronary arteries group), and 103 healthy age matched children (control group). Their systemic blood pressure, fasting cholesterol concentrations, flow-mediated dilation (FMD) of the brachial artery, carotid artery stiffness index and carotid intima-media thickness (IMT) were compared.FMD of the brachial artery in CAL group (5.2% +/- 1.9%) and normal coronary arteries group (6.8% +/- 2.0%) were significantly lower than that of control group (13.2% +/- 4.1%, both P0. 01); carotid artery stiffness index in CAL group (4.0 +/- 0.6) was significantly greater than that of normal coronary arteries group (3.6 +/- 0.6) and control group (3.4 +/- 0.5, P = 0.05, P0.01); likewise, IMT in CAL group (0.447 mm +/- 0.024 mm) was significantly higher than that of normal coronary arteries group (0.426 mm +/- 0.016 mm) and control group (0.424 mm +/- 0.016 mm, both P0.01). In multiple linear regression analysis, age and patient grouping were significant determinants of carotid artery stiffness index, FMD and IMT. There were significant correlations between carotid artery stiffness index, FMD and IMT in both healthy group and KD group after adjustment of age.There were endothelial dysfunction and increased systemic arterial stiffness and IMT late after the acute phase of Kawasaki disease. flow-mediated dilation of the brachial artery, carotid artery stiffness index combined carotid intima-media thickness can evaluated completely the prognosis of vascular lesion in convalescence after KD.

Background - We determined the usefulness of novel 2-dimensional strain indices, based on speckle tracking imaging, for assessment of systemic right ventricular (RV) function after atrial switch operation for transposition of the great arteries. Methods - Twenty-six patients, aged 21.0±3.6 years at 19.9±3.2 years after atrial switch operation, and 27 age-matched controls were studied. Two-dimensional imaging at the 4 chamber view was obtained with tracing of the entire RV endocardial border. The RV global longitudinal strain (GLS) and GLS rate were derived using an automated software (EchoPAC, GE Medical) (fig ), and correlated with tissue Doppler-derived RV isovolumic acceleration (IVA), and, in patient cohort, with cardiac magnetic resonance-derived RV ejection fraction. Results - Intraobserver and interobserver variability for measurement of GLS from 20 studies were 0.06±1.93% and 0.24±1.77% respectively. Compared with controls, patients had lower RV GLS (17.1±1.9% vs 26.3 ±2.9%, pvs 1.33 ±0.23/s, p 2 vs 1.56 ±0.53 m/s 2 , p=0.001), and increased RV myocardial performance index (0.52±0.09 vs 0.38 ±0.09, p=0.001). Both RV GLS and GLS rate correlated positively with RV IVA (r=0.43, p Conclusion - Two-dimensional RV GLS and GLS rate are novel indices potentially useful for assessment of systemic RV function.

To investigate the clinical features of coarctation of aorta (CoA).The clinical data of 96 pediatric patients with CoA, 60 male and 36 female, aged 3.7 months (7 - 12 years), were analyzed.The male to female ratio was 1.7:1. Infants aged less than 6 months accounted for 60% (57/96). The proportion of CoA in all hospitalized patients with congenital heart diseases admitted in this hospital was 0.5% (5/924) in 1996, increased every year, and reached 4.3% (15/330) in 2005. The coarctation was situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus in 93 cases, between the left common carotid artery and left subclavian artery in 2 cases, and at the opening of the left subclavian artery in 1 case. Thirteen patients (4%) suffered only from CoA, 47 patients were complicated with patent ductus arteriosus (PDA, 47/96, 49%) and/or ventricular septum defect (VSD, 47/96, 49%), 22 of the 96 patients (23%) complicated with both PDA and VSD. Eighty-nine cases were diagnosed by echocardiography, however, echocardiography failed to diagnose CoA in 7 cases (7.3%). Fifty-five patients underwent surgical repair and 2 of them died with a mortality of 3.8%. Seven patients with large VSD and severe pulmonary hypertension underwent two-stage repair. Immediate post-operative echocardiography showed satisfactory outcome.The morbidity of CoA among Chinese is similar to that among the Western population. Most of the coarctation is situated at the distal end of the left subclavian artery opposite to or near the ductus arteriosus, and most of the cases are complicated by PDA and/or VSD. Echocardiography is the first choice in the diagnosis of CoA; however, angiography is still necessary in some cases. Primary radical operation is indicated for infants with CoA, but older patients, especially those complicated with VSD and severe pulmonary hypertension, should undergo two-stage procedure.