Search

Right ventricular (RV) function is a prognostic marker of cardiac disease in children. Speckle tracking has been developed to assess RV longitudinal shortening, the dominant deformation during systole; little is known about its feasibility in children with congenital heart disease (CHD).To evaluate the feasibility and reproducibility of RV two-dimensional (2D) strain assessed by speckle tracking in infants undergoing CHD surgery compared with conventional markers.In this prospective single-centre study, RV peak systolic strain (RV-PSS) was measured using 2D speckle tracking in 37 consecutive children undergoing CHD surgery. Examinations were performed the day before surgery, a few hours after surgery and before discharge. Relationships with the z score of tricuspid annular plane systolic excursion (TAPSE) and tricuspid annular systolic velocity (TA Sa) were assessed.Median (interquartile range) age was 19 months (5-63); median weight was 9.2 kg (5.3-18.0). RV-PSS analysis was feasible in 92.9% (95% confidence interval [CI]: 86.0-97.1) of examinations. The coefficient of variation was 9.7% (95% CI: 7.4-11.9) for intraobserver variability and 15.1% (95% CI: 12.7-17.6) for interobserver variability. Correlations between RV-PSS and z score of TAPSE and TA Sa were strong (r=0.71, P0.0001 and r=0.70, P0.0001, respectively). RV-PSS was significantly reduced after surgery compared with baseline (-10.5±2.9% vs. -19.5±4.8%; P0.0001) and at discharge (-13.5±4.0% vs. -19.5±4.8%; P0.0001). Similar evolutions were observed with TAPSE and TA Sa (both P0.0001).RV longitudinal strain by speckle tracking is a feasible and reproducible method of assessing perioperative evolution of RV function in children with CHD.

Morphologic description of ventricular septal defect (VSD) is mandatory before performing the newly developed transcatheter closure procedure. Inaccurate estimation of defect size has been reported using conventional two-dimensional (2D) transthoracic echocardiography (TTE). The aim of this study was to assess VSD morphology and size using three-dimensional (3D) TTE compared with 2D TTE and surgery.Forty-eight children aged 21.4 ± 29.3 months with isolated muscular (n = 11 [22.9%]) and membranous (n = 37 [77.1%]) VSDs were prospectively included. Three-dimensional images were acquired using full-volume single-beat mode. Minimal diameter, maximal diameter, and systolic and diastolic VSD areas were measured from 3D data sets using multiplanar reconstruction mode (QLAB 9). Maximal-to-minimal VSD diameter ratio was used to assess VSD geometry. Linear regression analysis and the Bland-Altman method were used to compare 3D measurements with 2D and surgical measurements in a subgroup of 15 patients who underwent surgical VSD closure.VSD 3D diameters and areas were measured in all patients (100%; 95% CI, 92.6%-100%). Maximal diameter was lower on 2D TTE compared with 3D TTE (7.3 vs 11.3 mm, P .0001). Mean bias was 4 mm, with 95% of values ranging from -1.76 to 9.75 mm. Correlation between 3D maximal diameter and surgical diameter was strong (r(2) = 0.97, P .0001), while correlation between maximal 2D diameter and surgical diameter was moderate (r(2) = 0.63, P .0001). VSDs had an oval shape when assessed by 3D TTE. Maximal-to-minimal diameter ratio assessed by 3D TTE was significantly higher in muscular VSDs compared with membranous VSDs (3.20 ± 1.51 vs 2.13 ± 1.28, respectively, P = .01). VSD area variation throughout the cardiac cycle was 32% and was higher in muscular compared with membranous VSDs (49% vs 26%, P = .0001).Three-dimensional TTE allows better VSD morphologic and maximal diameter assessment compared with 2D TTE. VSD shape and its changes during the cardiac cycle can be visually and quantitatively displayed. Three-dimensional echocardiography may thus be particularly useful before and during percutaneous VSD closure.

Background A new three-dimensional echocardiographic (3DE) automated software (HeartModel) is now available to quantify left heart chamber. Our aims were to assess the feasibility of this software in children and its correlation with manual 3DE and cardiac magnetic resonance (CMR). Methods Ninety-two children were prospectively included in 2 separate protocols. In protocol 1, 73 healthy children (8.8 ± 3.0 years) underwent 2D and 3D transthoracic echocardiography (EPIQ 7 C). LV end-diastolic volume (LVEDV), LV end-systolic volume (LVESV), LVEF and LA volume at ventricular end-systole (LAV) obtained with automated 3DE were compared with the manual 3DE measurements. In protocol 2, automated 3DE measurements from 19 children with cardiopathy (12.8 ± 2.9 years) were compared with CMR values. Intra and inter-observer variability were assessed. Results Automated 3DE was feasible in 77% of datasets and reduced significantly time required for indices analysis compared with manual 3DE (20 vs. 125 seconds). There was excellent correlation for LVEDV, LVESV and LAV between automated and manual 3DE (R = 0.95) but less for LVEF (R = 0.57). Compared with manual 3DE, automated 3DE slightly overestimated LVEDV, LVEF and LAV and slightly underestimated LVESV. There were excellent correlation for LV volumes between automated 3DE and CMR (R = 0.94) but the correlation for LVEF was moderate (R = 0.46). Compared with CMR, automated 3DE slightly underestimated LVEDV and LVESV (biase-7.5%), underestimated LAV (− 31.6%), and had a negligible bias for LVEF (1.0%). Intraobserver and interobserver variability for automated 3DE measurements were low ( Fig. 1 )). Conclusions HeartModel is promising software for fast assessment of left heart chamber volume and function. Its feasibility in children aged more than 5 years is good, with high reproducibility. The automated 3DE measurements were comparable to manual 3DE. Compared with CMR, LVEDV and LVEF measured by automated 3DE seem interesting in clinical practice.

Background The SAPIEN 3 valve (S3) is the latest iteration of the SAPIEN valve family. It is deployed using a low profile flexible Commander delivery system. Hemodynamic outcomes and device durability are promising with SAPIEN valves, but percutaneous pulmonary valve implantation (PPVI) remains difficult. Moreover, the lack of valve covering increases the risk of tricuspid valve injury. Objectives To described the early results of a modified procedure for PPVI of the SAPIEN S3 valve using a delivery sheath. Methods We report the first consecutive cases of a modified procedure for PPVI of the SAPIEN S3 valve using a DryGore Seal 26French 65 cm delivery sheath. The sheath was used to stent the right ventricular outflow tract and then for the valve implantation. Results PPVI was performed using the modified technique in 15 patients (mean age 31 yo; mean weight 72.5 kg). The most common diagnoses were conotruncal defect (53.3%) and congenital aortic valve disease with Ross surgery (13.3%). A right-ventricle-to-pulmonary-artery tube had been inserted in 20.0% and a homograft or bioprosthesis implanted in 13.3% and 17.7% of patients, respectively. PPVI was performed on the native or patched RVOT in 73.3% of patients. Prestenting was performed in 12 cases with Andrastent XXL in 11 cases and eV3 LD max in 1 case. Prestenting was performed during the same procedure in all cases. While advancing the sheath through the stent, stent embolization occurred in one case. The stent was impacted and moved back with post dilatation and implantation of a second stent. SAPIEN 3 No. 23, No. 26 and No. 29 were implanted in 2, 3 and 10 cases. Valves were successfully implanted in all cases using the delivery sheath. Mean post valve gradient was 3 mmHg. Minor paravalvular leak was reported in 2 cases. Mean fluoroscopy time was 26.8 min. Groin hematoma were reported in 4 cases (26.7%). Conclusion A delivery sheath allows a fast and safe PPVI of SAPIEN S3 valve.

Introduction 3D printed models enhance understanding of complex anatomy in congenital heart disease. However few studies assessed the accuracy of measurements in printing model. Our aim was to assess the feasibility and accuracy of 3D printing in children with conotruncal ventricular septal defect (VSD). Methods This prospective study included patients with conotruncal VSD who underwent cardiac CT scan. VSD and aortic annulus diameters in two orthogonal axes were measured using Multiplanar Reformating (MPR) mode on CT images. CT DICOM files were imported to dedicated software (Mimics medical 19, Materialise, Belgium) for segmentation and to generate the 3D object. A hollow model was created using 3-Matic software (Materialise, Belgium) and cropped to better visualize the relationship between VSD and great arteries. VSD and aortic annulus diameters were measured on 3D models in two orthogonal axes and compared to measurements obtained from CT images ( Fig. 1 ). Results Fourteen patients were included (1 TA, 1 PA-VSD, 1 DOLV, 2 TOF, 9 DORV). Mean age 5.5 months (range 3 weeks–48 months), mean weight 6.7 kg (range 3.5–16 kg). 3D models were successfully obtained in all patients. Correlation between measurements obtained from 3D models with those obtained from source CT images was high (R = 0.977). Mean measurements of VSD diameters from CT images and 3D models were 8.5 mm and 8.1 respectively (P = NS). Mean measurements of aortic annulus diameters from CT images and 3D models were 15.8 mm and 14.9 mm respectively (P = NS), indicating the absence of any significant difference. Conclusion 3D printing is feasible in pediatric population with complex congenital heart disease and demonstrates correctly the spatial relationship between VSD and great arteries. Structure measurements are not significantly different between 3D model and source CT images.

SummaryCardiac catheterization has contributed to the progress made in the management of patients with congenital heart disease (CHD). First, it allowed clarification of the diagnostic assessment of CHD, by offering a better understanding of normal cardiac physiology and the pathophysiology and anatomy of complex malformations. Then, it became an alternative to surgery and a major component of the therapeutic approach for some CHD lesions. Nowadays, techniques have evolved and cardiac catheterization is widely used to percutaneously close intracardiac shunts, to relieve obstructive valvar or vessel lesions, and for transcatheter valve replacement. Accurate imaging is mandatory to guide these procedures. Cardiac imaging during catheterization of CHD must provide accurate images of lesions, surrounding cardiac structures, medical devices and tools used to deliver them. Cardiac imaging has to be ‘real-time’ with an excellent temporal resolution to ensure ‘eyes–hands’ synchronization and ‘device–target area’ accurate positioning. In this comprehensive review, we provide an overview of conventional cardiac imaging tools used in the catheterization laboratory in daily practice, as well as the effect of recent evolution and future imaging modalities.

Introduction The Echonavigator® software allows real-time imaging fusion between 2D or 3D echocardiography and fluoroscopy in the catheterization laboratory. We aim to test the accuracy of the imaging fusion in a pediatric population. Methods After percutaneous closure of atrial septal defect, occluder devices, visualized on echocardiography and fluoroscopy, were used as a reference tool. First, distance between the thread pitch on the 3D echo view and on fluoroscopy was measured on 4 orthogonal views (right anterior oblique, left anterior oblique, right posterior oblique, left posterior oblique), in systole and diastole (distance 1). Second, distance between a marker positionned on echo and the thread pitch on the fluoroscopy view was measured on the 4 orthogonal views in systole and diastole (distance 2). Results Thirty-one patients (7.9 years old [6.0–9.8]; 26 Kg [21–37]) were included. Fusion and marker positioning were feasible in all cases. No complication related to the echocardiography was reported. In 5 cases (16.1%), fusion was instable during the procedure with transient loss of the automatic tracking of the probe. Quality of the fusion imaging was rated good in 21 cases (67.7%) and excellent in 10 cases (32.3%). Fusion was available in real time in all patients. However there was a slight temporal delay between the fluoroscopic and the echo images. Minimal and maximal distances 1 were 0.5 [0–0.5] and 2 mm [1.5–2.5]. The marker positionned on echo was fixed and did not follow the movement of the thread pitch during the cardiac cycle. On the fusion view, minimal and maximal distances 2 during the cardiac cycle were 0.5 [0.3–1] and 2 mm [1.5–2.5]. Bias was not significantly different among the 4 axis ( Fig. 1 ). Conclusion Feasibility and accuracy of the echonavigator software is high in a pediatric population weighting more than 20 Kg. It could be improved by the development of a pediatric 3D trans-esophageal probe and by applying 3D speckle tracking technology.

The pathophysiology of Ventricular Septal Defect (VSD) is determined by the size of the defect and the state of the pulmonary vascular resistance. We assessed the morphology of VSD using 3D transthoracic echocardiography (3D-TTE) and the ability to estimate the pathophysiology.Methods48 children with isolated unique muscular (77%) and membranous VSD (23%) were included. Severity of the VSD was rated according to their pathophysiology. (Type 1: minor left-to-right (L-R) shunt; 2a: significant L-R shunt (left ventricular end diastolic diameter (LVEDD) Z-score >2); 2b: VSD associated with pulmonary hypertension). 3D VSD measurements were obtained after a multi-planar reconstruction of a TTE 3D full volume (X5-1 or X7-2 matrix probes, ie33, Philips). Diagnosis properties of the ratio of 3D VSD area to aortic annulus area (3DA/AAA), 3D VSD area to body surface area (3DA/BSA) and 2Dmax diameter to BSA (2DD/BSA) were compared.ResultsMedian age was 8.5 month-old (min 1 max 123). LVEDD Zscore was>2 in 19 children (40%), (12 (25%) had pulmonary hypertension). 3DVSD systolic area, 3D and 2D max. diameters were correlated with LVEDD. (r=0.71, r=0.52, r=0.55, p0.39 has a sensitivity of 92% and a specificity of 97% to diagnose a 2b-VSD.Conclusion3D-TTE allows a morphological and a quantitative assessment of muscular and membranous VSD. The 3DA/AAA ratio is an accurate diagnostic tool to assess the pathophysiology of the VSD.

Malignant ventricular arrhythmias (VA) may happen remote from tetralogy of Fallot (TOF) repair. Monomorphic ventricular tachycardia (VT) using surgical scar-related reentry circuits is the most common form of VA, but ventricular fibrillation (VF) unrelated to VT has been described. Right ventricular fibrosis may be part of the substrate for VF and also VT. We aimed to assess right ventricular fibrosis in young children with TOF. Methods We prospectively included 9 children requiring surgery for the management of TOF. Right ventricle tissue of the outflow tract resected during surgery was analyzed under conventional microscopy. Results Surgical indications were complete repair of TOF with pulmonary stenosis ( n = 7), complete repair with right-ventricle-to pulmonary-artery-conduit of TOF with pulmonary atresia ( n = 1) and redo surgery for outflow tract and pulmonary valve stenosis six years after the complete repair ( n = 1). Median age at complete repair was 5.5 months old (range: 3 to 75, 3 children coming from developing countries having been operated after 2 year-old). Median Sp0 2 was 85% (range: 65 to100%). Pulmonary annulus section was required in 62.5%. Morphological analysis revealed a marked sub-endocardial fibrosis, fibrosis of the interstitium and of the support vessels. Morphology of cardiomyocytes suggests that they remain immature in most cases. However, an early maturation was observed in some myocardial sectors in a 27-month-old child with cardiomyocytes exhibiting acquisition of a mature brick-wall micropattern. Cardiomyocytes are often multinucleated with some being trinucleated. MIB-1 revealed some cardiomyocytes to be in mitosis cycle. Conclusion Early fibrosis and cardiomyocytes alterations of the right ventricle outflow tract before any surgery in TOF are already present in very young children. If these early abnormalities are evolutive and if they represent a facilitating substrate for late VA remains to be demonstrated.

Introduction Shortly after birth, under changes in loading conditions of the heart, a shift from predominantly hyperplasic to hypertrophic cardiac growth occurs. In rodents, at birth, neonatal proliferative cardiomyocytes (CMs) exhibit a fusiform shape and differentiated into non-proliferative rhod-shape CM around P20 defined as the mature state. In human, CM differentiation supposedly ended around 6 years old but the exact timing still remains unknown. Moreover, numerous stimuli most probably contribute to the CM maturation, including pressure. In a human model of pressure overload named tetralogy of Fallot (ToF), we thus hypothesized the occurence of an earlier maturation of the cardiac tissue. Methods we prospectively included 15 children around 6 month-old (min. 3.5 max. 27), who required surgery for the management of ToF. We assessed criteria of maturation from right ventricle tissue of infundibulum that was resected during the surgery. Results As previously described, heart sections analysis revealed a marked sub-endocardial fibrosis (473±444µm) and a significant fibrosis of the interstitium (13.0±6.3%). This criteria was correlated to the severity of the disease represented by the degree of desaturation (r=0,623; p=0,017). In all children analysed, CMs were hypertrophied but unlike healthy myocardium, CM size was heterogeneous (CV=40,4%), with alternating immature and mature area. In mature area, as expected, CMs proliferation stopped as indicated by the loss of Ki67 staining and exhibit a mature rod-shape. Ultrastructurally, CMs had structured intercalated disk and elongated contractile apparatus with an alignment of Z-strikes and apparent I-band. The lateral membrane between two CMs was compacted with periodic crests and holes. Conclusion Our data highly suggest that the increase of pressure during childhood may act as a maturation factor. Myocardium in ToF is characterized by a shortening of the hyperplasia stage and a subsequent early hypertrophy stage.

BackgroundRV systolic strain evolution during peri-operative management of congenital heart diseases (CHD) is unknown.MethodsIn this prospective study, RV peak systolic strain (PSS) was measured using 2D speckle tracking echocardiography (Qlab10.0 software, Philips) in 39 children undergoing surgery of a CHD (Median age: 17 months, min 6 day-old, max 14.3 year-old). Three measures were performed the day before surgery, few hours after the surgery and before discharge and compared to conventional echocardiographic parameters of RV and left ventricular (LV) function. The relationships between the evolution of RV-PSS, peri-operative parameters and the type of CHD were assessed.ResultsMean RV-PSS at baseline was –19.5±4.8. RV-PSS was moderately correlated with the heart rate (r=0.49), the LV Tmad (r=–0.48), the TAPSE (r=–0.54) and the tricuspid S’ wave (r= –0.44)(all p