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BACKGROUND: Repair of complete atrioventricular canal (CAVC) is often complicated by atrioventricular valve regurgitation, particularly of the left-sided valve. Understanding the three-dimensional (3D) structure of the atrioventricular canal annulus prior to repair may help to inform optimized repair. However, the 3D shape and movement of the CAVC annulus has yet to be quantified nor has it been rigorously compared to a normal mitral valve annulus. METHODS: The complete annuli of 43 patients with CAVC were modeled in 4 cardiac phases using transthoracic 3D echocardiograms and custom code. The annular structure was compared to the annuli of 20 normal pediatric mitral valves using 3D metrics and statistical shape analysis (Procrustes analysis). RESULTS: The unrepaired CAVC annulus varied in shape significantly throughout the cardiac cycle. Procrustes analysis visually demonstrated that the average normalized CAVC annular shape is more planar than the normal mitral annulus. Quantitatively, the annular height to valve width ratio of the native left CAVC atrioventricular valve was significantly lower than that of a normal mitral valve in all systolic phases(p

Patients with borderline hypoplastic right or left ventricle and VSD may be candidates for either single ventricle palliation or staged ventricular recruitment (SVR) followed by eventual biventricular conversion. Components of SVR include restriction of atrial septal defects (ASD) without ventricular septal defects (VSD) closure and addition of accessory pulmonary blood flow. This study evaluated the impact of ASD restriction on ventricular growth and function. We retrospectively reviewed patients with borderline ventricular hypoplasia and VSD who underwent a staged ventricular recruitment (SVR) procedure from 2012 to June 2019. Pre- and post-recruitment MRI and echocardiogram data were compared and analyzed. We excluded cases in which we intentionally restricted VSD with simultaneous ASD restriction. Forty-six patients (41 with right-dominant ventricle, 25 with risk factors for Fontan procedure) underwent SVR at a median age of 15.1 months' (interquartile range (IQR), 7.2-37.2 months'). The median indexed ventricular end-diastolic volume, end-systolic volume, and stroke volume according to cardiac MRI significantly increased at median 11.0 months' (IQR:7.8~14.1 months') after recruitment. Among them, except 2 operative mortalities after SVR, 26 patients underwent bi-ventricular repair (56.5% including one and a half ventricle repair) at a median of 8.0 months' (IQR: 6.2-12.2 months') after recruitment. Fifteen patients await biventricular completion, and 3 patients underwent single ventricle palliation. Pulmonary blood flow (Qp) tended to increase after recruitment regardless of type of pulmonary blood flow modification without statistical significance. Six patients died at a median duration of 6.5 months' (IQR: 2.9-11.7) after SVR; 3 patients died after biventricular completion, 2 after recruitment, and 1 after returning to single ventricle palliation. All of them were considered poor Fontan candidates due to severe atrioventricular valve regurgitation, pulmonary hypertension, pulmonary vein stenosis, or airway stenosis. Restriction of the atrial septum leads to the growth of hypoplastic ventricle in patients with ventricular septal defects who undergo SVR regardless of the preoperative characteristics, and eventual biventricular repair can be performed in a subgroup of these patients. Future work is necessary to optimize timing of SVR and method of accessory pulmonary blood flow.

Objectives Aortic valve reconstruction (AVRec) with neocuspidization or the Ozaki procedure with complete cusp replacement for aortic valve disease has excellent mid-term results in adults. Limited results of AVRec in pediatric patients have been reported. We report our early outcomes of the Ozaki procedure for congenital aortic and truncal valve disease. Methods A retrospective analysis was performed on all 57 patients with congenital aortic and truncal valve disease who had a 3-leaflet Ozaki procedure at a single institution from August 2015 to February 2019. Outcome measures included mortality, surgical or catheter-based reinterventions, and echocardiographic measurements. Results Twenty-four patients had aortic regurgitation (AR), 6 had aortic stenosis (AS), and 27 patients had AS/AR. Two patients had quadricuspid valves, 26 had tricuspid, 20 had bicuspid, and 9 had unicusp aortic valves. Four patients had truncus arteriosus. Thirty-four patients had previous aortic valve repairs and 5 had replacements. Preoperative echocardiography mean annular diameter was 20.90 ± 4.98 cm and peak gradient for patients with AS/AR was 53.62 ± 22.20 mm Hg. Autologous, Photofix, and CardioCel bovine pericardia were used in 20, 35, and 2 patients. Eight patients required aortic root enlargement and 20 had sinus enlargement. Fifty-one patients had concomitant procedures. Median intensive care unit and hospital length of stay were 1.87 and 6.38 days. There were no hospital mortalities or early conversions to valve replacement. At discharge, 98% of patients had mild or less regurgitation and peak aortic gradient was 16.9 ± 9.5 mm Hg. Two patients underwent aortic valve replacement. At median follow-up of 8.1 months, 96% and 91% of patients had less than moderate regurgitation and stenosis, respectively. Conclusions The AVRec procedure has acceptable short-term results and should be considered for valve reconstruction in pediatric patients with congenital aortic and truncal valve disease. Longer-term follow-up is necessary to determine the optimal patch material and late valve function and continued annular growth.

This study describes a single center experience with the use of cardiac resynchronization therapy (CRT) in a difficult patient population, including single systemic right ventricles (RVs), subpulmonary RVs, and left ventricles (LVs) with right bundle branch block (RBBB).CRT remains challenging in the congenital heart disease population.Consecutive patients undergoing resynchronization of single RVs, subpulmonary RVs, or LVs in the setting of RBBB were identified between 2016 and 2019. Patients who had CRT performed for complete heart block or had3 months of follow-up were excluded. Patients underwent pre-procedural advanced imaging by echocardiogram, computed tomography, or cardiac magnetic resonance to assess ventricular function and synchrony; intraoperative mapping was performed to identify optimal lead placement.All patients undergoing resynchronization presented with at least moderate systolic ventricular dysfunction in the setting of intrinsic atrioventricular nodal conduction and RBBB. Seven patients were identified. Two patients underwent CRT of a single RV, 3 with subpulmonary RVs and 2 with systemic LVs. The median age at CRT was 5 years (range 0.6 to 48 years). The median follow-up was 9 months (range 3 to 18 months). The median baseline QRS duration was 180 ms (range 115 to 260ms). Post-CRT, the QRS duration decreased by a median of 34% (range 19% to 38%). All patients had improvement in their systolic ventricular function.Targeted resynchronization in systemic and subpulmonary RVs can be used to improve ventricular function and heart failure in the congenital heart disease population. Similar techniques can be applied to successfully treat patients with LV dysfunction and RBBB and improve their long-term outcomes.

Evaluate outcomes of single leaflet aortic valve reconstruction using Ozaki sizer and template. Single institute retrospective analysis between August 2015 and August 2019. Thirty-three patients, median age 9.3 years and weight 29.2 kg underwent single leaflet Ozaki repair. Preoperative indications were: AR (n = 17), AS (n = 3) or AS/AR (n = 13). Baseline anatomy was unicuspid (n = 15), bicuspid (n = 9) or tricuspid (n = 9). Two patients had endocarditis. Prior interventions included balloon valvuloplasty (n = 22) and aortic valve repair (n = 9). Pre-op average native annulus diameter was 19.6 mm and peak echo gradient was 36 mm Hg. Autologous pericardium, Photofix and CardioCel bovine pericardium were used in 26, 5, and 2 patients. Non-coronary sinus enlargement was required in 3 and aortic root reduction in 9 patients. Single leaflet reconstruction was done for the right coronary cusp (n = 25), non-coronary cusp in (n = 6) and left coronary cusp (n = 2). Additional procedures were done in 30 patients. Median ICU and hospital LOS were 2.1 and 6.3 days. There were no early re-interventions or conversions to valve replacement and one unrelated mortality.en At discharge, all patients hadmoderate AR and/or AS with average peak gradients of 15 mm Hg. The median follow-up was 1.1 year, (IQR 0.7-1.8 years). Freedom from ≥ moderate AR and AS at 2 years was 76% and 86%. One patient required surgical re-intervention for severe AR 1.5 years after surgery for inflammatory infiltrate with calcification and fibrosis. Single-leaflet aortic valve leaflet reconstruction utilizing the Ozaki technique has promising early results and can be considered in patients when there are acceptable native leaflets.

Background: Management of the congenitally corrected transposition of the great arteries (ccTGA) with intact ventricular septum (IVS) is controversial and includes either anatomic repair with systemic left ventricle (morphological left ventricle) or maintenance of a systemic right ventricle (morphological right ventricle). The purpose of this study was to describe outcomes of ccTGA-IVS with either strategy. Methods: A single-institution, retrospective review of all patients with a diagnosis of ccTGA-IVS who either underwent double switch operation (DSO) or managed with systemic morphological right ventricle (no-DSO). The composite end points included death, heart transplantation, or development of significant systemic ventricular dysfunction or atrioventricular valve regurgitation. Results: Between January 1980 and October 2019, 128 patients with ccTGA-IVS underwent primary treatment at a single institution (91 no-DSO and 37 DSO). Mean age at initial presentation was younger in the DSO compared with the no-DSO cohort (2.1 versus 18.2 years; P P =0.039). Among patients with initial age at presentation ≤5 years, DSO was associated with 0.16× the hazard compared with no-DSO (hazard ratio, 0.16 [95% CI, 0.04–0.62]; P =0.008). Pacemaker implantation was not associated with increased hazard of the composite outcome. Conclusions: Systemic ventricular dysfunction is a late complication of ccTGA-IVS regardless of the strategy pursued. Compared with those undergoing anatomic repair, patients with systemic morphological right ventricle may have lower long-term freedom from cardiovascular complications.

Cone repair (CR) uses native tissue for tricuspid valve (TV) repair and provides potential for growth. Results after CR were investigated in different age groups including several surgical modifications. Single institution retrospective analysis of all CR excluding neonatal procedures. Endpoints included TV reoperation, late tricuspid regurgitation (TR) and death. Between April 2006 and August 2019, 157 patients underwent CR at a median age of 11.7 years (range, 0.3-57.2). 20% (n=32) of patients had previous surgery. Repair modifications included atrial reduction (n=111,71%), right ventricular plication (n=85,55%), leaflet augmentation (n=36,23%), papillary muscle repositioning (n=50,32%), ring annuloplasty (n=70,45%). Early re-operation for recurrent TR occurred in 11 patients. Median follow-up time was 4.3 years (range, 9d-12.3y). There was no significant association between age at repair and time to TV reoperation (p=0.25). However, age4 years at CR was identified as the most discriminating binary age threshold for the patients with TV reoperation (25.0% in4y group vs 9.3% in the ≥4y group). Placement of an annuloplasty ring was protective against ≥moderate TR (OR=0.39, 95% CI 0.16-0.95, p=0.039). Freedom from late TV re-operation was 94.1% at 7 years. Survival was 97.9% at 6 years. Repair after age 18 years was associated with mortality in early follow-up (p=0.037). Mid-term results for CR are favorable in children and adults. Time to TV reoperation may be shorter when CR is performed before age four years, but this result requires confirmation in a larger sample. An annuloplasty ring should be considered when appropriate.

Background: Three-dimensional echocardiography (3DE) evaluation of left ventricular (LV) volume and function in pediatrics compares favorably with cardiac magnetic resonance imaging. The aim of this study was to establish from a multicenter, normal pediatric z-score values of 3DE left ventricular volumes and function. Methods: Six hundred and ninety-eight healthy children (ages 0 to 18 years) were recruited from five centers. LV 3DE was acquired from the 4-chamber view. A vendor independent software analyzed end-diastolic volume (EDV), end-systolic volume (ESV), stroke volume (SV), and ejection fraction (EF) using semi-automated quantification. Body surface area (BSA) based z-scores were generated. Intraobserver and interobserver variability were calculated using intraclass correlation (ICC) and repeatability coefficient (RC). Results: Z-scores were generated for ESV, EDV, and SV. The ICC for intraobserver variability for EDV, ESV, and SV were 0.99, 0.99, and 0.99 respectively. The ICC for interobserver variability for EDV, ESV, and SV were 0.98, 0.94, and 0.98 respectively. The RC for intraobserver and interobserver variability for LV EF was 4.39% (95% CI: 3.01, 5.59) and interobserver was 7.08% (95%CI: 5.51, 8.42). Conclusions: We report pediatric Z-scores for normal LV volumes using the semi-automated method from five centers, enhancing its generalizability. 3DE evaluation of LV volumes and EF in pediatric patients is highly reproducible.

Background. The aim of this study was to evaluate early and mid-term outcomes (mortality and prosthetic valve reintervention) after mitral valve replacement with 15- to 17-mm mechanical prostheses.Methods. A multicenter, retrospective cohort study was performed among patients who underwent mitral valve replacement with a 15- to 17-mm mechanical prosthesis at 6 congenital cardiac centers: 5 in The Netherlands and 1 in the United States. Baseline, operative, and follow-up data were evaluated.Results. Mitral valve replacement was performed in 61 infants (15 mm, n = 17 [28%]; 16 mm, n = 18 [29%]; 17 mm, n = 26 [43%]), of whom 27 (47%) were admitted to the intensive care unit before surgery and 22 (39%) required ventilator support. Median age at surgery was 5.9 months (interquartile range [IQR] 3.2-17.4), and median weight was 5.7 kg (IQR, 4.5-8.8). There were 13 in-hospital deaths (21%) and 8 late deaths (17%, among 48 hospital survivors). Major adverse events occurred in 34 (56%). Median follow-up was 4.0 years (IQR, 0.4-12.5) First prosthetic valve replacement (n = 27 [44%]) occurred at a median of 3.7 years (IQR, 1.9-6.8). Prosthetic valve endocarditis was not reported, and there was no mortality related to prosthesis replacement. Other reinterventions included permanent pacemaker implantation (n = 9 [15%]), subaortic stenosis resection (n = 4 [7%]), aortic valve repair (n = 3 [5%], and aortic valve replacement (n = 6 [10%]).Conclusions. Mitral valve replacement with 15- to 17-mm mechanical prostheses is an important alternative to save critically ill neonates and infants in whom the mitral valve cannot be repaired. Prosthesis replacement for outgrowth can be carried out with low risk. (C) 2020 by The Society of Thoracic Surgeons. Published by Elsevier Inc.

To assess the feasibility and outcomes of biventricular conversion following takedown of Fontan circulation.Retrospective analysis of patients who had takedown of Fontan circulation and conversion to biventricular circulation at a single center from September 2007 to April 2020. Failing Fontan physiology was defined as Fontan circulation pressure15 mm Hg and/or the presence of associated complications.Biventricular conversion was performed in 23 patients at a median age of 10.0 (7.5-13.0) years. Indications included failing Fontan physiology in 15 (65%) and elective takedown in 8 (35%) patients. A subset of patients (n = 6) underwent procedures for staged recruitment of the nondominant ventricle before conversion. Median z score of end-diastolic volume of borderline ventricle before takedown was -2.3 (-3.3, -1.3). Hypoplastic left heart syndrome (P .01) and sub-/aortic stenosis (P .01) were more common in these patients. Biventricular conversion with or without staged ventricular recruitment led to a significant increase in indexed end-diastolic volume (P .01), indexed end-systolic volume (P .01), and ventricular mass (P .01) of the nondominant ventricle (14 right, 9 left ventricle). There were 5 (22%) deaths (1 [4%] early death). All who underwent elective biventricular conversion survived, whereas 2-year survival rate for patients with a failing Fontan circulation was 72.7% (95% confidence interval, 37%-90%). The overall, 3-year reoperation-free survival was 86.7% (95% confidence interval, 56%-96%). Left dominant atrioventricular canal defect (P .01) and early era of biventricular conversion (P = .02) were significant predictors for mortality.A primary as well as a staged biventricular conversion is feasible in patients who have had previous Fontan procedure. Although this provides an alternative to transplantation in patients with failing Fontan, outcomes are worse in those with failing Fontan compared with elective takedown of Fontan circulation. Optimal timing needs further evaluation.

To determine the contemporary outcomes of the double switch operation (DSO) (ie, Mustard or Senning + arterial switch).A single-institution, retrospective review of all patients with congenitally corrected transposition of the great arteries undergoing a DSO.Between 1999 and 2019, 103 patients underwent DSO with a Mustard (n = 93) or Senning (n = 10) procedure. Segmental anatomy was (S, L, L) in 93 patients and (I, D, D) in 6 patients. Eight patients had heterotaxy and 71 patients had a ventricular septal defect. Median age was 2.1 years (range, 1.8 months-40 years), including 34 patients younger than age 1 year (33%). Median weight was 10.9 kg (range, 3.4-64 kg). Sixty-one patients had prior pulmonary artery bands for a median of 1.1 years (range, 14 days-12.9 years; interquartile range, 0.7-3.1 years). Median intensive care unit and hospital lengths of stay were 5 and 10 days, respectively. Median follow-up was 3.4 years (interquartile range, 1-9.8 years) and 5.2 years (interquartile range, 2.3-10.7 years) in 79 patients withgt;1 year follow-up. At latest follow-up, aortic, mitral, tricuspid valve regurgitation, and left ventricle dysfunction was less than moderate in 96%, 98%, 96%, and 93%, respectively. Seventeen patients underwent reoperation: neoaortic valve intervention (n = 10), baffle revision (n = 5), and ventricular septal defect closure (n = 4). At latest follow-up, 17 patients (17%) had a pacemaker and 27 (26%) had cardiac resynchronization therapy devices. There were 2 deaths and 2 transplants. Transplant-free survival was 94.6% at 5 years. Risk factors for death or transplant included longer cardiopulmonary bypass time and older age at DSO.The outcomes of the DSO are promising. Earlier age at operation might favor better outcomes. Progressive neoaortic regurgitation and reinterventions on the neo-aortic valve are anticipated problems.

Background Strain and synchrony can be calculated from a variety of software packages, but there is a paucity of data with inter-vendor comparisons in children. To test the hypothesis that different packages may affect results, independent of acquisition, we compared values obtained using two commercially available analysis tool (QLAB and TomTec), with several different settings. Methods The study population included 108 children; patients were divided into three groups: (1) normal cardiac structure and conduction; (2) ventricular paced rhythm; and (3) flattened ventricular septum (reflecting right ventricular pressure or volume load lesions). We analyzed the same image acquired from the apical 4-chamber (AP4) and short-axis at the mid-papillary level (SAXM) views in both QLAB (versions 10.5 and 10.8) and TomTec (version 1.2). In QLAB version 10.8, low, medium, and high quantification smoothness settings were employed. In TomTec, images were analyzed with both low and high frame rates. Tracking quality for each package was graded. AP4 and SAXM strain and synchrony values were recorded. A mixed-effects linear regression model was used, with main effect considered significant if the p-value was Results Tracking scores were high for all packages except QLAB 10.5 in the SAXM view. AP4 and SAXM strain values varied significantly between QLAB 10.5 and the other packages. Synchrony values varied widely for all strain values (p Conclusion Synchrony values varied substantially among all packages in children. Strain values varied widely between QLAB 10.5 and all other software packages, recommending avoidance of QLAB 10.5 for future studies. Quantification smoothness settings in QLAB 10.8 resulted in minimal strain differences. In TomTec, low and high frame rate strain values differed only in a subset of patients (flattened septum). These data suggest that reliable comparisons between strain values derived from QLAB and TomTec is possible in certain cases, but that caution should be used especially in different hemodynamics conditions.

Tracheobronchomalacia and airway obstruction from severely dilated pulmonary arteries in tetralogy of Fallot with absent pulmonary valve (TOF-APV) has been associated with high rates of respiratory failure and mortality (15% to 25%). It is not known whether aggressive pulmonary artery (PA) or direct airway intervention during early definitive cardiac repair improves outcomes.A retrospective observational study was made of all patients undergoing surgical repair for TOF-APV at our center between 2006 and 2018.Twenty patients underwent repair at a median age of 51 days and PA Z-scores of 8.1. Twelve patients had a valve implanted, 6 of whom required reoperation for valve replacement at a median of 9 months (range, 3 to 28) compared with 8 who had initial transannular patch, and only 1 patient required subsequent valve replacement (P.05). Seven patients had central PAs replaced with thin-walled Gore-Tex (WL Gore, Flagstaff, AZ) grafts; none of these required PA reoperation during a median follow-up of 26.5 months, whereas 3 of 13 patients who did not have PA replacement with Gore-Tex required subsequent PA reoperation (P.05). Concomitant airway interventions (eg, tracheobronchopexy/plasty) were performed in 4 patients and none required subsequent airway interventions, whereas 2 patients not having initial airway intervention required subsequent tracheopexy (P.05). Three patients in the cohort eventually required tracheostomy (15%), and 2 patients died (10%; on postoperative days 30 and 326); none had received initial airway intervention.Pulmonary artery replacement and aggressive direct airway management at initial definitive repair of cardiac TOF-APV can be performed safely with acceptable survival outcomes and low rates of airway and PA reintervention.

Background Aortic valve neocuspidization (AVNeo) (Ozaki procedure) has excellent midterm results in adults. Outcomes in patients with a small native aortic annulus are unknown. We report early outcomes in young patients with small native aortic valve annuli. Methods Retrospective data of patients undergoing AVNeo between 2015 and 2019 were reviewed. Patients with native aortic annulus less than 21 mm undergoing 3-leaflet AVNeo were included. Results A total of 51 patients were identified (median age 7.9 years; median weight 21 kg), and 80% patients were less than or equal to 12 years age. Preoperative indication was aortic regurgitation (AR) (n = 23), aortic stenosis (AS) (n = 22), or mixed AS and AR (n = 6). Baseline anatomy was quadricuspid (n = 1), tricuspid (n = 23), bicuspid (n = 15), or unicuspid (n = 12) valve. Preoperative peak gradient for AS and mixed AS and AR patients was 55.36 mm Hg. Median native annulus diameter was 17 mm; sinus and annular enlargements were required in 22 patients and 9 patients, respectively. Median intensive care unit and hospital length of stay were 2.0 days and 7.2 days, respectively. There were no reinterventions, and there was 1 hospital mortality unrelated to aortic valve. At discharge, 94% of patients had less than or equal to mild AR, and the median peak gradient was 18 mm Hg. At mean follow-up of 11.9 months, 80% and 82% of patients had less than moderate AR and AS, respectively. Three patients required surgical reintervention. In annular enlargement patients, mean annulus Z score remained greater than 0 at follow-up. Conclusions The Ozaki procedure has acceptable short-term results in young patients with small aortic annuli. A larger aortic annulus can be achieved with surgical annular enlargement. Long-term follow-up is necessary to determine late valve function and potential continued annular growth.

Normal pediatric values of three-dimensional (3D) left ventricular (LV) volumes and strain are not well established; moreover, no reports exist of the stress-strain relation and the heart rate-corrected velocity of circumferential fiber shortening (VCFc) based upon 3D imaging in children. Three-dimensional LV datasets were obtained in pediatric patients (≤18 years of age) with structurally normal hearts. Ventricular volumes and strain components (longitudinal, GLS; circumferential, GCS; and 3D strain, 3DS) were analyzed using a commercial 3D speckle-tracking analysis package. LV mid-wall global average end-systolic fiber stress was calculated from 3D LV volumes. A total of 238 patients were included in the analysis with a median age of 13.1 years (range 0.4 to 17.9 years). Regression equations were derived for 3D volume parameters, permitting body surface area-based Z score calculation. Overall, 3DS values were more negative than GLS and GCS (mean ± SD = -33.8 ± 2.8; -27.8 ± 2.9; and -21.7 ± 3.1, respectively); only GLS varied significantly with age (r = 0.22; p 0.001). Both global average end-systolic fiber stress and 3D VCFc increased significantly with age (p 0.001 for both). Stress-adjusted 3DS and VCFc both varied with age (p 0.001 for both), consistent with increased contractility. In conclusion, 3D echocardiography may be used to calculate LV stress, strain, and volumes in pediatric patients with strong reproducibility. Among strain parameters, significant age-related changes were seen only in GLS. Both indexes of contractility investigated (3DS and VCFc indexed to wall stress) improved with age. Future studies of the 3D echocardiography stress-strain relation may yield new insights into maturational changes in myocardial contractility.

Since the first successful palliation was performed by Norwood et al. in 1983, there have been substantial changes in diagnosis, management, and outcomes of hypoplastic left heart syndrome, Survival for stage 1 palliation has increased to 90% in many centres, with patients potentially surviving into adulthood. However, the associated morbidity and mortality remain substantial. Although the principles of staged surgical palliation of hypoplastic left heart syndrome are well established, there is significant variability in surgical procedure and management between centres, and several controversial aspects remain unresolved. In this review, we summarize the current surgical and management options for newborns with hypoplastic left heart syndrome and their outcomes.

Objectives Endothelial-to-mesenchymal transition (EndMT) has been identified as the underlying mechanism of endocardial fibroelastosis (EFE) formation. The purpose of this study was to determine whether hemodynamic alterations due to valvar defects promote EndMT and whether age-specific structural changes affect ventricular diastolic compliance despite extensive surgical resection of EFE tissue. Material and Methods We analyzed EFE tissue from 24 patients with hypoplastic left heart syndrome (HLHS) who underwent left ventricular (LV) rehabilitation surgery at Boston Children's Hospital between December 2011 and March 2018. Six patients with flow disturbances across the aortic valve and/or mitral valve but no HLHS diagnosis and macroscopic appearance of “EFE-like tissue” in the LV were included for comparison. All samples were examined for amount of collagen/elastin production and degradation, and presence of active EndMT by histologic analysis. Results EFE tissue from patients with and without HLHS consisted predominantly of elastin and collagen fibers. There was no alteration in degradation activity for collagen or elastin as shown by in situ zymography. Active EndMT was found in all patients with and without HLHS with flow disturbances (“EFE-like”). In patients with HLHS, EFE infiltrated into the underlying myocardium with increasing age. Conclusions Patients with and without HLHS with flow disturbances due to stenotic or incompetent valves develop EndMT-derived fibrotic tissue covering the LV. When EFE recurs, it is directly associated with flow disturbances and switches to an infiltrative growth pattern with increasing age, leading to increased diastolic stiffness of the LV.

Review echocardiography and outcomes before and after Ross procedures, including patients undergoing biventricular conversion with Damus-Kaye-Stansel (DKS) takedown. A retrospective review was performed on 62 patients undergoing simple (control group) and complex Ross procedures, including 12 patients who underwent biventricular conversion with Ross operation and DKS takedown (complex). Echocardiography was reviewed preoperatively and at discharge and late follow-up. Kaplan-Meier estimates of patient survival and freedom from reintervention were obtained. In all, 62 patients had a median age of 4.5 years (interquartile range [IQR]: 1-12.5), weight of 16.4kg (IQR: 8-41), and follow-up of 3.8 years (IQR: 1.3-6). The complex DKS takedown group had 2 deaths, no neoaortic valve or root reinterventions, and 3 right ventricular outflow tract (VOT) reinterventions. There were no differences from the control group in left VOT or right VOT reinterventions. Neither group showed differences between pre- and late follow-up aortic root and ascending aorta dimensions, and no correlations were found among preoperative pulmonary valve (PV) size, late aortic regurgitation (AR), aortic root, or ascending aortic Z-scores. Aortic valve size increased from discharge to late follow-up for both groups (P ≤ .05); 90% of patients at late follow-up had mild or less AR with similar distributions in severity between complex and control groups. Severity of late AR showed no correlation with preoperative PV size and is independent of it. The Ross procedure has good short-term results in simple and complex patients and should be considered in those undergoing Ross operation with biventricular conversion and DKS takedown. Moreover, native PV size should not be a contraindication for Ross procedure.

Purpose of review With a combination of fetal catheter interventions and postnatal catheter and surgical interventions, the left ventricle in patients with hypoplastic left heart can undergo significant increase in size and remodeling to support cardiac output. However, some will have persistent high left atrial pressures resulting in pulmonary edema and high pulmonary artery pressures, that is, diastolic dysfunction. Recent findings The manuscript details the medical and surgical strategies to diagnose and treat diastolic dysfunction in this patient population to promote biventricular circulations. Summary Often times, this diastolic dysfunction is related to the presence of endocardial fibroelastosis, which affects left ventricular filling and mitral valve function. Ongoing research is investigating what causes and promotes the development of endocardial fibroelastosis, and means to improve the medical and surgical treatment of diastolic dysfunction.

The spectrum of congenital mitral valve stenosis (MS) consists of a complex of defects that result in obstruction to left ventricular inflow. This spectrum includes patients with underdeveloped left heart structures (Fig. 1) to those with isolated congenital MS. The specific mitral valve defects can further be divided into categories based on the relationship to the mitral valve annulus including valvar, supravalvar and subvalvar components. Clinically, these patients present based on the degree of obstruction, associated mitral regurgitation, secondary pulmonary hypertension, associated lung disease and/or associated cardiac lesions. There are a number of factors that contribute to the successful outcomes in these patients including pre-operative imaging, aggressive surgical techniques and peri-operative management.

Background Assessment of left atrial (LA) size and function is important in a number of pediatric cardiac conditions including those affecting the diastolic performance of the left ventricle. Measurements of LA volume and strain by two-dimensional echocardiography rely upon inaccurate geometric assumptions and are hampered by out-of-plane motion. The objective of this study was to characterize LA volumes and strain by three-dimensional echocardiography in healthy children. Methods LA volumes and strain were retrospectively measured by three-dimensional echocardiography in healthy children with no known structural or functional heart disease using a commercial speckle-tracking package applied to the LA to compute maximum volume ( V max ), minimum volume ( V min ), ejection volume (LAEV), ejection fraction (LAEF), and the following components of global strain: 3D principal (3DS), longitudinal (GLS), and circumferential (GCS). Results The study population included 196 normal subjects (median age, 12 years; range, 4 days to 20.9 years). V max , V min , and LAEV increased with age and body surface area. Significant age-related declines were present in all measured functional indices including LAEF, 3DS, GLS, and GCS. Analysis of a subset of 50 subjects showed excellent agreement between V max derived by three-dimensional and two-dimensional biplane area-length method. Regression equations with standard deviations were generated to enable calculation of Z scores. Conclusions LA volume and functional indices can be reliably calculated using a commercial three-dimensional analysis software. All components of LA strain decline modestly with age. Normative data generated in this study have the potential to greatly enhance the utility of three-dimensional echocardiography-derived measurements in a wide range of cardiac pathologies.

> What’s done is done. > > —Lady Macbeth to Macbeth (Act 3, Scene 2) The theory of fetal programming, known alternatively as the developmental origins of health and disease hypothesis, the fetal origins of adult disease concept, or simply the thrifty phenotype, posits that the maternal uterine environment to which a fetus is exposed can have substantial and long-lasting effects on the health of the individual, throughout childhood and beyond. Indeed, alterations in the maternal milieu comprising the early fetal environment have been implicated in the development of a wide variety of postnatal disturbances, ranging from neuropsychiatric and neurodevelopmental disorders1,2 to the metabolic syndrome and diabetes mellitus3,4, to endocrinologic dysfunction5,6, and to lung cancer.7 See Article by Sarvari et al First reported by Barker et al,8,9 this thrifty phenotype states that a hostile maternal environment engenders a process of fetal change that is adaptive during the initial time of fetal stress but leads to a permanent programming that later proves maladaptive over the long term. Barker’s original assertion,8–10 based on the observations of low birth weight babies in England and Wales in the 1920s and 1930s, focused on the adverse cardiovascular outcomes experienced by these small fetuses, including long-term increased risk of coronary heart disease, type 2 diabetes mellitus, stroke, and hypertension. To this day, however, specific mechanisms linking fetal growth restriction (FGR) with these poor long-term cardiovascular outcomes remain speculative, with a variety of …

Quantitative assessment of right ventricular (RV) size and function is important in congenital heart disease. Although three-dimensional (3D) echocardiography overcomes geometric challenges that limit two-dimensional echocardiography, its feasibility and reproducibility in children have not been systematically evaluated. The goal of this study was to determine the feasibility and reproducibility of 3D echocardiographic RV measurements in children.Patients aged 0 to 20 years were prospectively enrolled. Full-volume 3D echocardiographic RV data sets were acquired in each from the subxiphoid and apical four-chamber views by a trained sonographer. Another sonographer then acquired a second image data set from the same patient. RV volumes and ejection fraction were then measured offline. Outcomes included successful acquisition, measurable data set, and observer and interstudy variability.Three-dimensional echocardiographic RV data sets were obtainable in 67 of 70 patients (96%) and were measurable from at least one view in 39 (58%). Factors associated with nonmeasurable data sets by univariate analysis included older age, larger body surface area and body mass index, no sedation, and female gender. Multivariate analysis identified older age (odds ratio, 1.34; 95% confidence interval, 1.17-1.55, P.0001) and female gender (odds ratio, 6.06; 95% confidence interval, 1.45-25.3, P = .0135) as independently associated with nonmeasurable RV data sets. Intraobserver, interobserver, and interstudy agreement was excellent for analyzable RV volumes and modest for RV ejection fraction.Transthoracic 3D echocardiographic RV volumes were measurable in 58% of children with and without congenital heart disease. Older age and female gender were independently associated with nonanalyzable data sets. When feasible, measurements of RV volumes are highly reproducible.

Patients with heterotaxy syndrome (HS) have a range of anomalies and outcomes. There are limited data on perinatal outcomes after prenatal diagnosis. To determine the factors influencing perinatal and infant outcomes, we analyzed prenatal and postnatal variables in fetuses with HS from 1995 to 2011. Of 154 fetuses with HS, 61 (40%) had asplenia syndrome (ASP) and 93 (60%) had polysplenia syndrome (PSP). In the ASP group, 22 (36%) patients were elected for termination of pregnancy, 4 (10%) had fetal death, and 35 of 39 (90%) continued pregnancies were live born. In the PSP group, 12 (13%) patients were elected for termination of pregnancy, 5 (6%) had fetal death (4 with bradyarrhythmia), and 76 of 81 (94%) continued pregnancies were live born. Bradyarrhythmia was the only predictor of fetal death. In the live-born ASP group, 43% (15 of 35) died, 7 because of pulmonary vein stenosis, 4 postoperatively, and 4 because of noncardiac causes. In the live-born PSP group, 13% (10 of 76) died, 5 postoperatively, 2 from bradyarrhythmia, 1 from a cardiac event, and 2 from noncardiac causes. Pulmonary vein stenosis and noncardiac anomalies were independent risk factors for postnatal death. Only 8% of ASP patients achieved biventricular circulation, compared with 65% of PSP patients. In the live-born cohort, the 5-year survival rate was 53% for ASP and 86% for PSP. In conclusion, most PSP patients are currently alive with biventricular circulation in contrast with few ASP patients. Bradyarrhythmia was the only predictor of fetal death. Pulmonary vein stenosis and noncardiac anomalies were predictors of postnatal death.

Objective Early results for anatomic repair of congenitally corrected transposition of the great arteries (ccTGA) are excellent. However, the development of left ventricular dysfunction late after repair remains a concern. In this study we sought to determine factors leading to late left ventricular dysfunction and the impact of cardiac resynchronization as a primary and secondary (upgrade) mode of pacing. Methods From 1992 to 2012, 106 patients (median age at surgery, 1.2 years; range, 2 months to 43 years) with ccTGA had anatomic repair. A retrospective review of preoperative variables, surgical procedures, and postoperative outcomes was performed. Results In-hospital deaths occurred in 5.7% (n = 6), and there were 3 postdischarge deaths during a mean follow-up period of 5.2 years (range, 7 days to 18.2 years). Twelve patients (12%) developed moderate or severe left ventricular dysfunction. Thirty-eight patients (38%) were being paced at latest follow-up evaluation. Seventeen patients had resynchronization therapy, 9 as an upgrade from a prior dual-chamber system (8.5%) and 8 as a primary pacemaker (7.5%). Factors associated with left ventricular dysfunction were age at repair older than 10 years, weight greater than 20 kg, pacemaker implantation, and severe neo-aortic regurgitation. Eight of 9 patients undergoing secondary cardiac resynchronization therapy (upgrade) improved left ventricular function. None of the 8 patients undergoing primary resynchronization developed left ventricular dysfunction. Conclusions Late left ventricular dysfunction after anatomic repair of ccTGA is not uncommon, occurring most often in older patients and in those requiring pacing. Early anatomic repair and cardiac resynchronization therapy in patients requiring a pacemaker could preclude the development of left ventricular dysfunction.

Objectives The durability of valve-sparing aortic root procedures with aortic regurgitation due to leaflet disease is questioned. Here, we review our experience in combined aortic root and valve reconstruction in children and young adults. Methods All valve-sparing aortic root procedures from 2000 to 2012 were reviewed, and patients with aortic valve repair beyond resuspension were included. Root procedures were classified as replacement with reimplantation, root remodeling, or aortic annular and sinotubular junction stabilization. The primary end point was structural valve deterioration, a composite of aortic valve reoperation and/or moderate or greater regurgitation at follow-up. Results Thirty-four patients were included during the study period. The surgery consisted of reimplantation in 13 patients, remodeling in 16 patients, and annular and sinotubular junction stabilization in 5 patients. Valve repair consisted of leaflet procedures in 26 patients and subannular reduction in 15 patients. During a median follow-up of 4.2 months (range, 2 weeks-8 years), there were 5 reoperations for aortic valve replacement due to aortic regurgitation, and 2 patients presented with moderate or greater regurgitation. Freedom from structural valve deterioration was 70.1% ± 10.3% at 1 year and remained stable thereafter, although it was significantly worse in the reimplantation group ( P = .039). A more severe degree of preoperative aortic regurgitation ( P = .001) and smaller graft to aortic annulus ratio ( P = .003) were predictors of structural valve deterioration. Conclusions Valve-sparing root and valve reconstruction can be done with low operative risk and allows valve preservation in most patients. These data should question the assumption that reimplantation is superior when associated with complex valve reconstruction.

Introduction High-titre factor VIII (FVIII) inhibitors complicate peri-operative haemostasis. Recombinant porcine FVIII (r-pFVIII) may provide an alternative haemostatic agent for high-risk procedures and allow FVIII activity monitoring. Aim Devise an effective haemostatic plan for repair of a progressively symptomatic aortic coarctation in a 5-year-old male with immune tolerance induction (ITI) refractory high-titre FVIII inhibitors. Methods Preprocedure human FVIII inhibitor titre was 58 Bethesda Units mL−1 (BU) and cross-reacted to neutralize porcine FVIII at 30 BU. Daily ITI with plasma-derived FVIII concentrate was supplemented with anti-B-cell and anti-plasma cell immunotherapy to reduce FVIII inhibitor titres. Potential haemostatic agents were evaluated in comparative ex vivo thrombin generation assays (TGA). Results Four weeks after immunosuppression, human and porcine inhibitor titres declined to 16 and 2 BU respectively. TGA with r-pFVIII was less robust than with activated prothrombin complex concentrate (aPCC); however, r-pFVIII was selected for cardiac surgery to secure the ability to assay FVIII levels throughout this high-bleeding risk procedure. Haemostasis with r-pFVIII was excellent; initial trough FVIII activity levels ranged from 0.81–1.17 IU mL−1. On postoperative day 3, peak and trough levels markedly declined suggesting a rising porcine inhibitor titre. Postprocedure prophylaxis was transitioned to aPCC, informed by TGA. Conclusions R-pFVIII provided effective peri-procedural haemostasis with no adverse events. Rapid neutralization of r-pFVIII after the first 60 hours, despite intensive immune suppression, accentuates the importance of careful monitoring. Use of TGA can support bypassing agent selection for convalescence. The comparative cost of r-pFVIII may limit its use to high morbidity clinical scenarios.

Personalized medicine requires physicians to employ complex and expensive diagnostic analyses. However, without evidence-based standards, there is over-utilization of testing that complicates patient care, diminishes quality, and increases costs. To address this, we implemented the diagnostic management team (DMT), a multi-disciplinary system for development and deployment of diagnostic testing guidelines for hematologic malignancies. The team created evidence-based standard ordering protocols (SOPs) for cytogenetic and molecular testing that were applied by pathologists to bone marrow biopsies on adult patients. Testing on 780 biopsies performed during the 6 months before SOP implementation was compared with 1,806 biopsies performed during the subsequent 12 months. After implementation, there were significant decreases in tests discordant with SOPs, omitted tests, and the estimated cost of testing to payers. The fraction of positive tests increased. Clinicians reported acceptance of the new procedures and perceived time savings. This process is a model for optimizing complex and personalized diagnostic testing.

BACKGROUND-: Mitral valve prolapse (MVP) is common in children with Marfan syndrome (MFS) and is associated with varying degrees of mitral regurgitation. However, the 3-dimensional (3D) morphology of the mitral valve (MV) in children with MFS and its relation to degree of mitral regurgitation (MR) are not known. The goals of this study were to describe the 3D morphology of the MV in children with MFS and to compare it to that of normal children. METHODS-: 3D transthoracic echocardiography was performed in 27 patients (age 3–21 years) meeting revised Ghent criteria for MFS as well as 27 normal children matched by age (±1 year). The 3D geometry of the MV apparatus in mid-systole was measured, and its association with clinical and 2D echocardiographic parameters was examined. RESULTS-: Compared to age-matched controls, children with MFS had larger 3D annular area (p

Background Management of unbalanced common atrioventricular canal (UCAVC) defect by a single-ventricle (SV) approach frequently results in poor outcomes, especially in trisomy 21 patients. In this report we describe our results with conversion to biventricular circulation in UCAVC patients with SV palliation. Methods Retrospective review of patients with UCAVC undergoing biventricular conversion from prior SV palliation between 2003 and 2011 was conducted. Mortality and freedom from reinterventions were analyzed using nonparametric methods. Results Sixteen children with UCAVC (8 patients [50%] were left dominant) and prior SV palliation underwent conversion to biventricular circulation between 2003 and 2011. Median follow-up was 18 months (range, 3 to 94 months). Surgical indications included worsening cyanosis, severe atrioventricular valve regurgitation, or failing bidirectional Glenn or Fontan physiology. All patients had either unequal distribution of the common atrioventricular valve of greater than 60% or one hypoplastic ventricle. By magnetic resonance imaging or computed tomography, 8 patients with right dominant atrioventricular canal had a median left ventricular end-diastolic volume of 32 mL/m 2 (range, 22 to 35 mL/m 2 ). Eight patients with a left dominant atrioventricular canal had a median right ventricular end-diastolic volume of 42 mL/m 2 (range, 26 to 64 mL/m 2 ). Eleven patients (69%) had trisomy 21, and 3 patients (19%) had heterotaxy. Stages of palliation included stage I in 2 patients, bidirectional Glenn in 10 patients, hemi-Fontan in 2 patients, and Fontan in 2 patients. There was 1 (6%) operative (right ventricle dominant) and 1 (6%) late death (left ventricle dominant). Eight patients required reinterventions, 3 (19%) surgical and 6 (38%) catheter-based. On follow-up, all had improvement in cyanosis and symptoms. Conclusions Biventricular conversion from failing SV palliation in UCAVC can be accomplished with an acceptable early and late morbidity and mortality, although need for reintervention was not uncommon.

ObjectivesThe goal of this study was to review results of a novel management strategy intended to rehabilitate the left heart (LH) in patients with LH hypoplasia who have undergone single-ventricle palliation (SVP).BackgroundManagement of patients with hypoplastic LH syndrome and borderline left ventricle (LV) involves 2 options: SVP or biventricular repair. We hypothesized that staged LV recruitment and biventricular conversion may be achieved after SVP by using a strategy consisting of relief of inflow and outflow tract obstructions, resection of endocardial fibroelastosis, and promotion of flow through the LV.MethodsPatients with hypoplastic LH and borderline LV who underwent traditional SVP (n = 34) or staged LV recruitment (n = 34) between 1995 and 2010 were retrospectively analyzed and compared with a control SVP group.ResultsMean initial z-scores for LH structures before stage 1 SVP were not significantly different between groups. Mortality occurred in 4 of 34 patients after LV recruitment and in 7 of 34 after traditional SVP. LH dimension z-scores increased significantly over time after LV recruitment, whereas they declined after traditional SVP, with significant interaction between stage of palliation and treatment group. Restriction of the atrial septum (conducted in 19 of 34 patients) was the only predictor of increase in left ventricular end-diastolic volume (p < 0.001). Native biventricular circulation was achieved in 12 patients after staged LV recruitment; all of these patients had restriction at the atrial septum.ConclusionsIn these patients with borderline LH disease who underwent SVP, it is possible to increase LH dimensions by using an LV recruitment strategy. In a subset of patients, this strategy allowed establishment of biventricular circulation.

Mitral valve replacement (MVR) in young children is limited by the lack of small prostheses. Our institution began performing MVR with modified, surgically placed, stented jugular vein grafts (Melody valve) in 2010. We sought to describe key echocardiographic features for pre- and postoperative assessment of this novel form of MVR.The pre- and postoperative echocardiograms of 24 patients who underwent Melody MVR were reviewed. In addition to standard measurements, preoperative potential measurements of the mitral annulus were performed whereby dimensions were estimated for Melody sizing. A ratio of the narrowest subaortic region in systole to the actual mitral valve dimension (SubA:MV) was assessed for risk of postoperative left ventricular outflow tract obstruction (LVOTO).Melody MVR was performed at a median of 8.5 months (5.6 kg) for stenosis (5), regurgitation (3), and mixed disease (16). Preoperatively, actual mitral z scores measured hypoplastic (median -3.1 for the lateral [lat] dimension; -2.1 for the anteroposterior [AP] dimension). The potential measurements often had normal z scores with fair correlation with intraoperative Melody dilation (ρ = 0.51 and 0.50 for lat and AP dimensions, respectively, both P = .01). A preoperative SubA:MV0.5 was associated with postoperative LVOTO, which occurred in 4 patients. Postoperatively, mitral gradients substantially improved, with low values relative to the effective orifice area of the Melody valve. No patients had significant regurgitation or perivalvar leak.Preoperative echocardiographic measurements may help guide intraoperative sizing for Melody MVR and identify patients at risk for postoperative LVOTO. Acute postoperative hemodynamic results were favorable; however, ongoing assessment is warranted.

The increasing use of ventricular assist devices (VADs) in terminal heart failure patients provides new challenges to cardiac rehabilitation physicians. Structured cardiac rehabilitation strategies are still poorly implemented for this special patient group. Clear guidance and more evidence for optimal modalities are needed. Thereby, attention has to be paid to specific aspects, such as psychological and social support and education (e.g., device management, INR self-management, drive-line care, and medication).In Germany, the post-implant treatment and rehabilitation of VAD Patients working group was founded in 2012. This working group has developed clear recommendations for the rehabilitation of VAD patients according to the available literature. All facets of VAD patients' rehabilitation are covered. The present paper is unique in Europe and represents a milestone to overcome the heterogeneity of VAD patient rehabilitation.

There are hardly any publications about the outcome of cardiac rehabilitation considering patients with an increased need for medical, nursing and therapeutic care. The aim of this study, which consecutively included n=387 statutory health insurance inpatients over a period of 2 years, was to find out differences in outcome in self-care patients (Barthel index70) as compared to patients with a need for complex care (Barthel index≤70).Rehabilitation outcomes concerning physical capacity, emotional status and activities of daily living as measured by Barthel index, FIM index, HADS, clinical complications, exercise test, duration of rehabilitation and form of dismission were analyzed and compared between both groups.The inpatients with a Barthel index ≤70 at admission were older, had a longer stay in hospital and in rehabilitation, developed more complications and more often suffered from concomitant diseases. They were readmitted to hospital more often. They showed a comparatively higher increase in indices of self-care and a significant increase in physical performance tests.Higher medical care expenses of multimorbid cardiac inpatients are no contraindication against rehabilitation, because even in this group the specific rehabilitation aims of the healthcare payers can be reached.

Three-dimensional echocardiography (3DE) has become important in the management of patients with congenital heart disease (CHD), particularly with pre-surgical planning, guidance of catheter intervention, and functional assessment of the heart. 3DE is increasingly used in children because of good acoustic windows and the non-invasive nature of the technique. The aim of this paper is to provide a review of the optimal application of 3DE in CHD including technical considerations, image orientation, application to different lesions, procedural guidance, and functional assessment.

Parachute left atrioventricular (AV) valve can complicate repair of common atrioventricular canal (CAVC), and single-ventricle palliation is sometimes preferred. The goal of this study is to review our single institutional experience in biventricular repair in this patient group.The demographic, procedural and outcome data were obtained for all children who underwent biventricular repair for complete CAVC with parachute [single left ventricular (LV) papillary muscle] or forme fruste parachute left AV valve (closely spaced LV papillary muscles) from 2001 to 2012. Primary outcomes were survival, freedom from left AV valve stenosis (defined as an inflow gradient ≥7 mmHg and post-capillary pulmonary hypertension) and freedom from left AV valve replacement.A total of 24 patients were included (21 parachutes, 3 forme frustes). There was 1 early death (4.2%). At discharge, no patient had more-than-mild regurgitation and 1 had stenosis. During a median follow-up of 3.7 years (IQR 4 months to 5 years), there were 2 late deaths (8.3%), 6 patients (25%) presented significant left AV valve stenosis and 2 patients (8.3%) required valve replacement. Freedom from stenosis was 95 ± 4.9% at 1 year, 83.1 ± 8.9% at 3 years, 64.7 ± 13.5% at 5 years and 51.7 ± 15.8% at 10 years. Complete cleft closure was not associated with a significantly different freedom from left AV valve reoperation (log-rank test, P = 0.89) or significant stenosis (P = 0.47).Biventricular repair in parachute left AV valve and CAVC is feasible with acceptable mortality and freedom from stenosis. The burden of reoperation remains significant in this patient group.

Background Left ventricular noncompaction (LVNC) cardiomyopathy is variably defined by numerous trabeculations, deep intertrabecular recesses, and noncompacted-to-compacted (NC/C) ratio >2. Limited studies exist on the reproducibility of diagnosing LVNC. Methods Clinical records of patients diagnosed with LVNC by echocardiography were reviewed. Blinded review of the index echocardiogram for all patients and a 1:1 match without LVNC was performed independently by two observers, measuring the number of trabeculations and the NC/C ratio. Results A total of 104 patients with LVNC were included in the study, 52 with no congenital heart disease (NCongHD) and 52 with congenital heart disease (CongHD). The duration of follow-up was 7.2 years (range, 0.5–23.1 years) for NCongHD and 8.2 years (range, 0–33.3 years) for CongHD. Agreement between observers in determining zero to three versus more than three trabeculations was 59% (NCongHD) and 73% (CongHD). Agreement in measuring an NC/C ratio ≤ 2 versus > 2 was 79% (NCongHD) and 74% (CongHD). Agreement with the original reader in diagnosing LVNC was 67%. There was no association between the number of trabeculations or the NC/C ratio and the likelihood of a major event. Patients with moderate or severe left ventricular dysfunction at the time of diagnosis were more likely to undergo cardiac transplantation or die compared with those with normal or mild dysfunction (NCongHD, 22% vs 0%, P = .01; CongHD, 39% vs 3%, P = .001). Conclusions The reproducibility of making measurements to diagnose LVNC by accepted criteria is poor. Heart transplantation and death are associated with significant ventricular dysfunction and not with increased trabeculations or NC/C ratios.

Summary Background Development of FVIII inhibitors represents a major challenge in patients with mild haemophilia A (HA), because they tend to occur at an older age and classical immune tolerance induction appears to be less effective. Case report A man (age: 60 years) with mild HA due to the missense mutation, Leu1929Arg, received a single dose of rFVIII at 35 IU/kg prior to routine colonoscopy, totalling 25 lifetime exposure days. Two months later, rFVIII was infused for a traumatic hip haematoma. However, FVIII recovery was inappropriate, and a FVIII inhibitor of 19 BU with type-2 kinetics was detected, resulting in FVIII:C of < 1%. Two weeks later, the patient experienced spontaneous iliopsoas bleeding. Parallel to bypassing therapy, we started single-agent immunosuppression with prednisolone at 1.5 mg/kg. FVIII : C “normalized” at 10.2% after four weeks. After five months, the inhibitor titre fell to < 0.4 BU with sustained remission after one year of follow-up. Conclusion In mild HA, FVIII inhibitors may share characteristic features with FVIII autoantibodies commonly observed in acquired HA. Therefore, immunosuppressive therapy alone could be successful at least in a subset of patients.

Echocardiography is critical in the assessment of patients with hypoplastic left heart syndrome. Fundamental techniques and standardised approaches are useful when evaluating patients with hypoplastic left heart syndrome prenatally, after birth, and before the Norwood operation (Stage 1); after the Norwood operation, before and after the superior cavopulmonary anastomosis (Stage 2); before and after the Fontan operation (Stage 3); and for chronic surveillance after the Fontan operation. From foetal assessment to ongoing surveillance after the Fontan procedure, echocardiography remains the primary technique for cardiac monitoring in this growing population of children and adults.

The shape of the mitral valve annulus is used in diagnostic and modeling applications, yet methods to accurately and reproducibly delineate the annulus are limited. This paper presents a mitral annulus segmentation algorithm designed for closed mitral valves which locates the annulus in three-dimensional ultrasound using only a single user-specified point near the center of the valve. The algorithm first constructs a surface at the location of the thin leaflets, and then locates the annulus by finding where the thin leaflet tissue meets the thicker heart wall. The algorithm iterates until convergence metrics are satisfied, resulting in an operator-independent mitral annulus segmentation. The accuracy of the algorithm was assessed from both a diagnostic and surgical standpoint by comparing the algorithm's results to delineations made by a group of experts on clinical ultrasound images of the mitral valve, and to delineations made by an expert with a surgical view of the mitral annulus on excised porcine hearts using an electromagnetically tracked pointer. In the former study, the algorithm was statistically indistinguishable from the best performing expert (p=0.85) and had an average RMS difference of 1.81+/-0.78 mm to the expert average. In the latter, the average RMS difference between the algorithm's annulus and the electromagnetically tracked points across six hearts was 1.19+/-0.17 mm .

Objectives The significant morbidity of long-term pulmonary regurgitation (PR) has driven the development of pulmonary valve (PV) sparing repair strategies in patients with tetralogy of Fallot (ToF). We assessed mid-term PV function in patients who underwent primary ToF repair with valve-sparing intraoperative balloon dilation (IBD) technique. Methods We evaluated 162 consecutive patients with ToF and pulmonary stenosis (ToF-PS) who underwent valve-sparing repair with IBD under 1 year of age. Results Median age at surgery was 98 days (interquartile range [IQR], 72-126) and median follow-up was 2.5 years (IQR, 0.6-4.9). Median preoperative PV annulus z score was −2.2 (IQR, −2.5 to −1.8). Twenty-five patients (15.4%) required reintervention for residual valvular stenosis. Multivariable analysis demonstrated preoperative annulus z score less than −2.45 ( P = .036) and younger age at surgery ( P = .001) were independent risk factors for early reintervention for stenosis. Freedom from at least moderate PR was 77%, 61%, and 43% at 1, 3, and 5 years postrepair. Right ventricular dimensions were not significantly different compared with a matched cohort of patients undergoing transannular patch repair at midterm follow-up. Conclusions Patients with ToF-PS who undergo valve-sparing repair with IBD develop progressive PR. Compared with transannular patch repair, the extent of RV dilation at midterm follow-up is not significantly different. Patients younger than 3 months of age and those with an annulus z score less than −2.45 experience higher rates of early reintervention for PV stenosis. In these patient subgroups, alternative strategies should be considered. This study suggests valve-sparing repair with IBD does not preserve long-term PV function in patients with ToF-PS.

Background— Aortic stenosis in the midgestation fetus with a normal-sized or dilated left ventricle predictably progresses to hypoplastic left heart syndrome when associated with certain physiological findings. Prenatal balloon aortic valvuloplasty may improve left heart growth and function, possibly preventing evolution to hypoplastic left heart syndrome. Methods and Results— Between March 2000 and October 2008, 70 fetuses underwent attempted aortic valvuloplasty for critical aortic stenosis with evolving hypoplastic left heart syndrome. We analyzed this experience to determine factors associated with procedural and postnatal outcome. The median gestational age at intervention was 23 weeks. The procedure was technically successful in 52 fetuses (74%). Relative to 21 untreated comparison fetuses, subsequent prenatal growth of the aortic and mitral valves, but not the left ventricle, was improved after intervention. Nine pregnancies (13%) did not reach a viable term or preterm birth. Seventeen patients had a biventricular circulation postnatally, 15 from birth. Larger left heart structures and higher left ventricular pressure at the time of intervention were associated with biventricular outcome. A multivariable threshold scoring system was able to discriminate fetuses with a biventricular outcome with 100% sensitivity and modest positive predictive value. Conclusions— Technically successful aortic valvuloplasty alters left heart valvar growth in fetuses with aortic stenosis and evolving hypoplastic left heart syndrome and, in a subset of cases, appeared to contribute to a biventricular outcome after birth. Fetal aortic valvuloplasty carries a risk of fetal demise. Fetuses undergoing in utero aortic valvuloplasty with an unfavorable multivariable threshold score at the time of intervention are very unlikely to achieve a biventricular circulation postnatally.

In patients with ST-segment elevation myocardial infarction (STEMI), myocardial reperfusion is associated with an inflammatory response leading to adverse effects on further myocardial damage. Therefore, we investigated the effects of the thrombolytic regimen with half-dose reteplase (r-PA) combined with abciximab on different cytokines involved in the local and systemic inflammatory scenario in STEMI patients. Thirty-eight STEMI patients were enrolled in this study. We investigated the effects of the regimen with half-dose r-PA plus abciximab versus full-dose r-PA on interleukin-6 (IL-6), interleukin-8 (IL-8), interleukin-10 (IL-10), MCP-1 and tumor necrosis factor-alpha (TNF-alpha) up to 48 h after the start of therapy. The full-dose r-PA group had a significant IL-6 increase after 48 h compared with the combination group. Furthermore, the full-dose r-PA group showed a marked increase of IL-10 (up to 3 h after) compared with a 41% IL-10 decrease in the combination group. MCP-1 decreased significantly after 3 h in the combination group compared with patients on r-PA therapy only. The combination group showed a nonsignificant increase in IL-8 within the first 6 h. There were no differences in TNF-alpha levels between the two infarct groups. In vivo, the investigated combined thrombolytic regimen consisting of half-dose r-PA plus abciximab causes less of an IL-6 increase and marked decreases in IL-10 and MCP-1 in STEMI patients compared with the pure r-PA thrombolytic regimen. This observation suggests that besides the known beneficial effects on systemic coagulation and leukocyte-platelet aggregates, this regimen may exert a favorable influence on myocardial tissue damage and thus on cardiac repair following myocardial infarction.

We are working to develop beating-heart atrial septal defect (ASD) closure techniques using real-time 3D ultrasound guidance. The major image processing challenges are the low image quality and the processing of information at high frame rate. This paper presents comparative results for ASD tracking in time sequences of 3D volumes of cardiac ultrasound. We introduce a block flow technique, which combines the velocity computation from optical flow for an entire block with template matching. Enforcing adapted similarity constraints to both the previous and first frames ensures optimal and unique solutions. We compare the performance of the proposed algorithm with that of block matching and region-based optical flow on eight in-vivo 4D datasets acquired from porcine beating-heart procedures. Results show that our technique is more stable and has higher sensitivity than both optical flow and block matching in tracking ASDs. Computing velocity at the block level, our technique tracks ASD motion at 2 frames/s, much faster than optical flow and comparable in computation cost to block matching, and shows promise for real-time (30 frames/s). We report consistent results on clinical intra-operative images and retrieve the cardiac cycle (in ungated images) from error analysis. Quantitative results are evaluated on synthetic data with maximum tracking errors of 1 voxel.

Background— Quantitative assessment of ventricular volumes and mass in pediatric patients with single-ventricle physiology would aid clinical management, but it is difficult to obtain with 2-dimensional echocardiography. The purpose of the present study was to compare matrix-array 3-dimensional echocardiography (3DE) measurements of single-ventricle volumes, mass, and ejection fraction with those measured by cardiac magnetic resonance (CMR) in young patients. Methods and Results— Twenty-nine patients (median age, 7 months) with a functional single ventricle undergoing CMR under general anesthesia were prospectively enrolled. The 3DE images were acquired at the conclusion of the CMR. Twenty-seven of 29 3DE data sets (93%) were optimal for 3DE assessment. Two blinded and independent observers performed 3DE measurements of volume, mass, and ejection fraction. The 3DE end-diastolic volume correlated well ( r =0.96) but was smaller than CMR by 9% ( P P P Conclusions— In young pediatric patients with a functional single ventricle, matrix-array 3DE measurements of mass and volumes compare well with those obtained by CMR. 3DE will provide an important modality for the serial analysis of ventricular size and performance in young patients with functional single ventricles.

ObjectiveWe sought to identify and characterize a subgroup of patients with hypoplastic left heart syndrome who might be at higher risk for stage I failure.MethodsFrom January 2001 through December 2006, all patients with hypoplastic left heart syndrome who underwent stage I palliation at Children’s Hospital Boston were retrospectively reviewed. The subgroup with the mitral stenosis–aortic atresia variant was studied separately. We evaluated preoperative echocardiographic data, operative characteristics, and postoperative factors associated with death or the need for transplantation. The Kaplan–Meier method was used to assess survival.ResultsThirty-eight (23%) of 165 patients had mitral stenosis–aortic atresia. Hospital mortality or need for transplantation for patients with mitral stenosis–aortic atresia was significantly higher than for other anatomic subgroups (29% vs 7.9%, P = .002). Left ventricle–subepicardial coronary artery communications were present in 20 (53%) patients with mitral stenosis–aortic atresia and were associated with a significantly higher hospital mortality (50% vs 6%, P = .004). No difference in outcome was demonstrated between different sources of pulmonary blood flow. A longer cardiopulmonary bypass time (P = .02) and the need for postoperative extracorporeal membrane oxygenation support (P < .001) were associated with a higher mortality rate.ConclusionsWith improved outcomes in the management of neonates with hypoplastic left heart syndrome, those with the mitral stenosis–aortic atresia variant and left ventricle–subepicardial coronary artery fistulae have emerged as a higher-risk subgroup for failure of stage I palliation. Further investigation is required, and a change in clinical management strategy for this particular subgroup might be warranted.

Background The slope of the minute ventilation versus CO 2 production relationship (VE/VCO 2 slope) is an index of gas exchange efficiency during exercise. In patients with repaired tetralogy of Fallot (rTOF), it correlates negatively with exercise capacity and is one of the best predictors of peak oxygen consumption (VO 2 ). In these patients, the magnitude of the VE/VCO 2 slope is related to the severity of pulmonary blood flow maldistribution (PBFM). The purpose of this study was to determine whether, in patients with rTOF, improvements in PBFM after a successful balloon angioplasty procedure (BAP) result in improvements in peak VO 2 and gas exchange during exercise. Methods Seventeen patients with rTOF and residual pulmonary artery stenoses referred for BAP were recruited. Exercise tests were performed and PBFM determined before and after BAP. Results Nine patients (group 1) had a successful BAP (ie, improvement of >5 percentage points in PBFM); 8 did not (group 2). Patients in group 1 had significantly greater improvements in VE/VCO 2 slope, peak VO 2 , and peak oxygen pulse (an index of forward stroke volume at peak exercise) than did patients in group 2. A significant correlation existed between the improvement in PBFM and the decline in the VE/VCO 2 slope ( r = −0.70, P = .002). Changes in peak oxygen pulse accounted for 89% of the improvement in peak VO 2 . Conclusions In these patients, a successful BAP resulted in improved peak VO 2 and more efficient gas exchange during exercise. The improvement in peak VO 2 appeared to be mediated by an increase in forward stroke volume.

Objective Severe aortic stenosis in the mid-gestation fetus can progress to hypoplastic left heart syndrome (HLHS). @ In-utero aortic valvuloplasty is an innovative therapy to promote left ventricular growth and function and potentially to prevent HLHS. This study evaluated the effects of mid-gestation fetal balloon aortic valvuloplasty on subsequent fetal left ventricular function and left heart Doppler characteristics. Methods We reviewed fetuses with aortic stenosis that underwent attempted in-utero aortic valvuloplasty between 2000 and 2006. Pre-intervention and the latest post-intervention fetal echocardiograms were analyzed to characterize changes in left heart function and Doppler characteristics in utero. Results Forty-two fetuses underwent attempted aortic valvuloplasty during the study period, 12 of which were excluded from analysis secondary to inadequate follow-up data, termination or fetal demise. Study fetuses (n = 30) underwent pre-intervention echocardiography at a median gestational age of 23 weeks, and were followed for a median of 66 ± 23 days post-intervention. In 26 fetuses, aortic valvuloplasty was technically successful. Among these 26, left heart physiology was abnormal pre-intervention and improved or normalized after intervention in most cases: biphasic mitral inflow was present in 5/25 (20%) cases pre-intervention and in 21/23 (91%) post-intervention (P < 0.001); moderate or severe mitral regurgitation was present in 14/26 (54%) cases pre-intervention and in 5/23 (22%) post-intervention (P = 0.02); bidirectional flow across the patent foramen ovale was present in 0/26 cases pre-intervention and in 6/25 (24%) post-intervention (P = 0.01); antegrade flow in the transverse arch was present in 0/25 cases pre-intervention and in 17/26 (65%) post-intervention (P < 0.001). The left ventricular ejection fraction increased from 19 ± 10% pre-intervention to 39 ± 14% post-intervention (P < 0.001). These changes were not observed in control fetuses (n = 18). Conclusion Fetal aortic valvuloplasty, when technically successful, improves left ventricular systolic function and left heart Doppler characteristics. Copyright © 2007 ISUOG. Published by John Wiley & Sons, Ltd.

The optimal reperfusion strategy in elderly patients with ST-segment elevation myocardial infarction (STEMI) remains a topic of debate. Therefore, we investigated in the MITRA PLUS registry clinical outcome variables in 5455 patients aged70 years and STEMI on admission at hospitals without the facilities of coronary catheterization and PCI. Outcome was compared after thrombolysis, transfer to PCI and after no reperfusion therapy. Data of this registry in STEMI patients older than 70 years, who were transferred to another hospital for PCI, showed a strong trend for lower in-hospital mortality rates compared with a strategy with sole fibrinolysis and significantly lower in hospital death rates compared with a conservative treatment without (medical or mechanical) reperfusion. Additionally, the PCI group also had a reduced incidence of the combined events: death, myocardial reinfarction, stroke in comparison with both other infarct groups. Data of the presented MITRA PLUS registry in STEMI patients older than 70 years support data of several other studies, that patients with STEMI benefit from a transfer to primary PCI even after a time delay of symptom onset to hospital admission of more than 2 h compared with a strategy using sole fibrinolytic therapy.