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Single-stage ventricular septation for double-inlet left or right ventricle (DILV or DIRV) has historically been associated with poor outcomes. We hypothesize that staged ventricular septation may demonstrate favorable clinical outcomes to be an alternative to Fontan palliation. This single-center retrospective study reviewed patients with DILV or DIRV who underwent staged ventricular septation between 2015-2021. The strategy involves pulmonary artery banding or Norwood procedure during infancy (stage 1), followed by partial ventricular septation to anchor the septum, while maintaining systemic RV pressure to avoid septal shift (stage 2). Residual septal defects are closed with pulmonary artery band removal at stage 3. Results are reported as median (interquartile range). Twelve patients underwent partial ventricular septation. At a median follow-up time of 17 months (8-30) after stage 2, there were no interstage deaths or cardiac transplants; LV dysfunction was observed in one patient. Hemodynamic evaluation after stage 2 demonstrated median left atrial pressure of 9.5 mm Hg (8.9-11.5), cardiac index of 3.4 L/min/m 2 (3.2-3.6), and RV and LV indexed end-diastolic volumes of 52 ml/m 2 (41-67) and 105 ml/m 2 (81-115), respectively. Five patients have progressed to stage 3; one required pacemaker for complete heart block. Unplanned reintervention was required in 4 patients after stage 1, 2 patients after stage 2, and 3 patients after stage 3. Staged ventricular septation is an alternative to single-ventricle palliation in a subset of double-inlet ventricle patients and is associated with acceptable early outcomes. Further studies are necessary to determine long-term outcomes., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Objective: In this study, we sought to identify independent risk factors for mortality and reintervention after early surgical correction of truncus arteriosus using a novel statistical method., Methods: Patients undergoing neonatal/infant truncus arteriosus repair between January 1984 and December 2018 were reviewed retrospectively. An innovative statistical strategy was applied integrating competing risks analysis with modulated renewal for time-to-event modeling., Results: A total of 204 patients were included in the study. Mortality occurred in 32 patients (15%). Smaller right ventricle to pulmonary artery conduit size and truncal valve insufficiency at birth were significantly associated with overall mortality (right ventricle to pulmonary artery conduit size: hazard ratio, 1.34; 95% confidence interval, 1.08-1.66, P = .008; truncal valve insufficiency: hazard ratio, 2.5; 95% confidence interval, 1.13-5.53, P = .024). truncal valve insufficiency at birth, truncal valve intervention at index repair, and number of cusps (4 vs 3) were associated with truncal valve reoperations (truncal valve insufficiency: hazard ratio, 2.38; 95%, confidence interval, 1.13-5.01, P = .02; cusp number: hazard ratio, 6.62; 95% confidence interval, 2.54-17.3, P < .001). Right ventricle to pulmonary artery conduit size 11 mm or less was associated with a higher risk of early catheter-based reintervention (hazard ratio, 1.54; 95% confidence interval, 1.04-2.28, P = .03) and reoperation (hazard ratio, 1.96; 95% confidence interval, 1.33-2.89, P = .001) on the right ventricle to pulmonary artery conduit., Conclusions: Smaller right ventricle to pulmonary artery conduit size and truncal valve insufficiency at birth were associated with overall mortality after truncus arteriosus repair. Quadricuspid truncal valve, the presence of truncal valve insufficiency at the time of diagnosis, and truncal valve intervention at index repair were associated with an increased risk of reoperation. The size of the right ventricle to pulmonary artery conduit at index surgery is the single most important factor for early reoperation and catheter-based reintervention on the conduit., (Copyright © 2021 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objectives: The Super Glenn procedure involves targeted increased in blood flow to left sided heart structures with fenestrated atrial septation. The objective of this study was to examine the outcomes of patients who had this procedure as a part of biventricular staging and specifically evaluate the effect on dimensions of left heart structures., Methods: Data for patients who had this procedure between 2005 and 2019 were retrospectively identified., Results: Thirty-seven patients were identified. Most common diagnosis was hypoplastic left heart syndrome in 40% (n = 15). On echocardiography, the median mitral valve z score was -2.26. On cardiac magnetic resonance imaging, median indexed left ventricular end-diastolic volume was 31.5 ml/m2 and mitral/tricuspid inflow ratio was 0.35. The median age at Super Glenn was 2.3 years (interquartile range 1.5-3.6) while median weight was 12 kg (interquartile range 9.8-14). There were no early/hospital deaths. The median intensive care unit length of stay was 4 days, and median hospital length of stay was 10 days. Median follow-up for the entire cohort was 3 years (range 15 days to 13.2 years). There was a statistically significant increase in indexed left ventricular dimensions. There were 5 deaths (14%). Three patients (8%) underwent heart transplant. Freedom from death/transplant was 79% at 5 years. Seven patients (19%) needed a reoperation. Twenty-three patients (62%) underwent biventricular conversion after a median of 11.3 months after Super Glenn., Conclusions: The Super Glenn procedure achieves consistent increase in left ventricular dimensions. This may be a useful strategy to help achieve a successful biventricular circulation in patients with borderline left ventricle. The superiority/non-inferiority of this approach over the conventional Fontan pathway is unclear., (© The Author(s) 2021. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

The use of the left ventricle as the subpulmonary ventricle to achieve a 1.5 or biventricular circulation is feasible in heterotaxy patients with complex intracardiac anatomy and acceptable right ventricular function. It is an alternative in patients who are not ideal candidates for single-ventricle palliation. We highlight 2 cases in which patients were rescued from a failed Fontan palliation and demonstrated improved functional status with normal saturations., (Copyright © 2020 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Patient-specific in vivo ventricle mechanical wall stress and strain conditions are important for cardiovascular investigations and should be calculated from correct zero-load ventricle morphologies. Cardiac magnetic resonance (CMR) data were obtained from 6 healthy volunteers and 12 Tetralogy of Fallot (TOF) patients with consent obtained. 3D patient-specific CMR-based ventricle models with different zero-load diastole and systole geometries due to myocardium contraction and relaxation were constructed to qualify right ventricle (RV) diastole and systole stress and strain values at begin-filling, end-filling, begin-ejection, and end-ejection, respectively. Our new models (called 2G models) can provide end-diastole and end-systole stress/strain values which models with one zero-load geometries (called 1G models) could not provide. 2G mean end-ejection stress value from the 18 participants was 321.4% higher than that from 1G models (p = 0.0002). 2G mean strain values was 230% higher than that of 1G models (p = 0.0002). TOF group (TG) end-ejection mean stress value was 105.4% higher than that of healthy group (HG) (17.54±7.42kPa vs. 8.54±0.92kPa, p = 0.0245). Worse outcome group (WG, n = 6) post pulmonary valve replacement (PVR) begin-ejection mean stress was 57.4% higher than that of better outcome group (BG, 86.94±26.29 vs. 52.93±22.86 kPa; p = 0.041). Among 7 selected parameters, End-filling stress was the best predictor to differentiate BG patients from WG patients with prediction accuracy = 0.8208 and area under receiver operating characteristic curve (AUC) value at 0.8135 (EE stress). Large scale studies are needed to further validate our findings., Competing Interests: The authors have declared that no competing interests exist.

Purpose: The goal of this study was to develop an algorithm that enhances the temporal resolution of two-dimensional color Doppler echocardiography (2D CDE) by reordering all the acquired frames and filtering out the frames corrupted by out-of-plane motion and arrhythmia., Methods: The algorithm splits original frame sequence into the fragments based on the correlation with a reference frame. Then, the fragments are aligned temporally and merged into a resulting sequence that has higher temporal resolution. We evaluated the algorithm with 10 animal epicardial 2D CDE datasets of the right ventricle and compared it with the existing approaches in terms of resulting frame rate, image stability and execution time., Results: We identified the optimal combination of alternatives for each step, which resulted in an increase in frame rate from 14 ± 0.87 to 238 ± 93 Hz. The average execution time was 7.23 ± 0.48 s in comparison with 0.009 ± 0.001 s for ECG gating and 1167.37 ± 587.85 s for flow reordering. Our approach demonstrated a significant (p < 0.01) increase in image stability compared with ECG gating and flow reordering., Conclusion: This work presents an offline algorithm for temporal enhancement of 2D CDE. Unlike previous frame reordering approaches, it can filter out-of-plane or corrupted frames, increasing the quality of the results, which substantially increases diagnostic value of 2D CDE. It can be used for high-frame-rate intraoperative imaging of intraventricular and valve regurgitant flows and is potentially modifiable for real-time use on ultrasound machines.

Objectives: Patients with borderline ventricles and ventricular septal defects (VSDs) who have previously undergone single ventricle palliation might be candidates for staged ventricular recruitment with the ultimate goal of biventricular conversion. This study aimed to determine the effect of atrial septal defect (ASD) restriction without VSD closure on ventricular growth in patients with borderline right or left ventricles and VSDs., Methods: Patients with borderline ventricles and VSD who underwent a staged ventricular recruitment procedure with strategies to increase blood flow through hypoplastic ventricle via ASD restriction without VSD closure after single ventricle palliation were retrospectively reviewed. Pre- and postrecruitment ventricular volumes were compared using Wilcoxon signed rank test., Results: A total of 21 patients underwent staged ventricular recruitment via ASD restriction without VSD closure at median age of 20.0 months (interquartile range [IQR], 8.0-52.5 months). At a median of 9.0 months (IQR, 8.0-11.8 months) after the recruitment procedure, there were increases in the median indexed ventricular diastolic volume (31.7 mL/m 2 [IQR, 24.5-37.1] to 48.5 mL/m 2 [IQR, 38.4-58.0; P < .01]), median indexed systolic volume (13.3 mL/m 2 [IQR, 9.7-18.7] to 19.5 mL/m 2 [IQR, 16.8-29.7]; P < .01), and the median indexed stroke volume (18.4 mL/m 2 [IQR, 14.8-21.1] to 28.1 mL/m 2 [IQR, 21.3-31.8]; P < .01). Biventricular conversion was ultimately performed in 14 (67%). Two patients died after biventricular conversion., Conclusions: Staged ventricular recruitment via ASD restriction without VSD closure is associated with growth of the hypoplastic ventricle. In patients who are deemed high-risk for single ventricle, this approach might facilitate eventual biventricular conversion. Further studies are needed to identify optimal candidates for this approach., (Copyright © 2018 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: Patients with a borderline left ventricular hypoplasia in the hypoplastic left heart syndrome variant or an unbalanced atrioventricular canal who undergo initial single-ventricle palliation may be candidates for biventricular (BiV) conversion following left ventricle (LV) recruitment procedures. We investigated associations among preoperative parameters and postoperative outcomes in patients undergoing BiV conversion., Methods: We performed a retrospective review of patients who underwent BiV conversion to determine variables associated with clinical outcomes. Predictor variables included cardiac diagnosis, age and weight, LV dimension, LV end diastolic volume, LV mass, preoperative LV end diastolic pressure (LVEDP), and preoperative left atrial pressure. Primary outcome was a composite of death, heart transplant, or BiV takedown., Results: Of 51 patients, 11 experienced primary outcome (22%). Patients with hypoplastic left heart syndrome variant were more likely to experience primary outcome than those with an unbalanced atrioventricular canal (30% vs 6%; P = .03). Receiver operating characteristic analysis demonstrated that preoperative LVEDP had good predictive accuracy in classifying patients with and without the primary outcome (area under the curve, 0.757; 95% confidence interval, 0.594-0.919; P = .012). The Youden J-index indicated a cutoff of LVEDP ≥ 13 mm Hg as optimal for predicting the primary outcome. Multivariable Cox regression demonstrated that LVEDP ≥ 13 mm Hg (adjusted hazard ratio, 4.00; P = .037) and postoperative right ventricle pressure > 3/4 (adjusted hazard ratio, 21.75; P < .001) were significantly associated with primary outcome, independent of age, weight, and diagnosis., Conclusions: Elevated preoperative LVEDP is a risk factor for suboptimal postoperative hemodynamic parameters and adverse outcome following BiV conversion from single-ventricle palliation., (Copyright © 2017 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

In recent decades, significant progress has been made in the diagnosis and management of congenitally corrected transposition of the great arteries (ccTGA). Nevertheless, gradual dysfunction and failure of the right ventricle (RV) in the systemic circulation remain the main contributors to mortality and disability for patients with ccTGA, especially after adolescence. Anatomic repair of ccTGA effectively resolves the problem of failure of the systemic RV and has good early and midterm results. However, this strategy is applicable primarily in infants and children up to their teens and has associated risks and limitations, and new challenges can arise in the late postoperative period. Patients with ccTGA manifesting progressive systemic RV dysfunction beyond adolescence represent the major challenge. Several palliative options such as cardiac resynchronization therapy, tricuspid valve repair or replacement, pulmonary artery banding, and implantation of an assist device into the systemic RV can be used to improve functional status and to delay the progression of ventricular dysfunction in patients who are not suitable for anatomic correction of ccTGA. For adult patients with severe systemic RV failure, heart transplantation currently remains the only long-term lifesaving procedure, although donor organ availability remains one of the most limiting factors in this type of therapy. This review focuses on current surgical and medical strategies and interventional options for the prevention and management of systemic RV failure in adults and children with ccTGA., (© 2016 American Heart Association, Inc.)

Background: Accurate calculation of ventricular stress and strain is critical for cardiovascular investigations. Sarcomere shortening in active contraction leads to change of ventricular zero-stress configurations during the cardiac cycle. A new model using different zero-load diastole and systole geometries was introduced to provide more accurate cardiac stress/strain calculations with potential to predict post pulmonary valve replacement (PVR) surgical outcome., Methods: Cardiac magnetic resonance (CMR) data were obtained from 16 patients with repaired tetralogy of Fallot prior to and 6 months after pulmonary valve replacement (8 male, 8 female, mean age 34.5 years). Patients were divided into Group 1 (n = 8) with better post PVR outcome and Group 2 (n = 8) with worse post PVR outcome based on their change in RV ejection fraction (EF). CMR-based patient-specific computational RV/LV models using one zero-load geometry (1G model) and two zero-load geometries (diastole and systole, 2G model) were constructed and RV wall thickness, volume, circumferential and longitudinal curvatures, mechanical stress and strain were obtained for analysis. Pairwise T-test and Linear Mixed Effect (LME) model were used to determine if the differences from the 1G and 2G models were statistically significant, with the dependence of the pair-wise observations and the patient-slice clustering effects being taken into consideration. For group comparisons, continuous variables (RV volumes, WT, C- and L- curvatures, and stress and strain values) were summarized as mean ± SD and compared between the outcome groups by using an unpaired Student t-test. Logistic regression analysis was used to identify potential morphological and mechanical predictors for post PVR surgical outcome., Results: Based on results from the 16 patients, mean begin-ejection stress and strain from the 2G model were 28% and 40% higher than that from the 1G model, respectively. Using the 2G model results, RV EF changes correlated negatively with stress (r = -0.609, P = 0.012) and with pre-PVR RV end-diastole volume (r = -0.60, P = 0.015), but did not correlate with WT, C-curvature, L-curvature, or strain. At begin-ejection, mean RV stress of Group 2 was 57.4% higher than that of Group 1 (130.1±60.7 vs. 82.7±38.8 kPa, P = 0.0042). Stress was the only parameter that showed significant differences between the two groups. The combination of circumferential curvature, RV volume and the difference between begin-ejection stress and end-ejection stress was the best predictor for post PVR outcome with an area under the ROC curve of 0.855. The begin-ejection stress was the best single predictor among the 8 individual parameters with an area under the ROC curve of 0.782., Conclusion: The new 2G model may be able to provide more accurate ventricular stress and strain calculations for potential clinical applications. Combining morphological and mechanical parameters may provide better predictions for post PVR outcome., Competing Interests: Dr. Geva is a consultant to Medtronic.

Objectives: Publicly available data from the Pediatric Heart Network's Single Ventricle Reconstruction Trial was analyzed to determine the prevalence, timing, risk factors for, and impact of second- and third-degree heart block (HB) on outcomes in patients who underwent stage 1 palliation (S1P) for hypoplastic left heart syndrome (HLHS)., Methods: The presence and date of onset of post-S1P HB occurring within the first year of life, potential risk factors for HB, and factors known to predict poor outcomes after S1P were extracted. Multivariable logistic and Cox regression analyses were performed to identify risk factors for HB and to determine the effect of HB on 3-year transplantation-free survival., Results: Among the 549 patients in the cohort, 33 (6%) developed HB after S1P. The median interval between S1P and HB was 8 days (interquartile range, 0-133 days). Regression analysis showed that tricuspid valve repair during S1P and obstruction of pulmonary venous drainage requiring pre-S1P intervention were independently associated with HB (adjusted odds ratio [aOR], 11.6, 95% confidence interval [CI] 3.3-40; P < .001 and aOR, 5.1; 95% CI, 1.3-20.6; P = .02, respectively). Transplantation-free survival at 3 years was lower for those with HB (39% vs 65%; P = .004). HB remained associated with transplantation-free survival after controlling for known risk factors (adjusted hazard ratio, 3.1; 95% CI, 1.9-5.0; P < .001). Nine children (27%) had a pacemaker implanted, and 7 of these children (78%) died or underwent heart transplantation., Conclusions: HB after S1P is rare but heralds a poor outcome. Careful monitoring of these patients is recommended given their significantly increased risks of death and heart transplantation., (Copyright © 2016 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objectives: A uniform management strategy has not been established for the treatment of large ventricular fibromas identified in children. The goal of the present study was to review our experience with surgical resection of this uncommon tumor., Methods: We performed a retrospective review of all patients with a diagnosis of ventricular fibromas who had undergone surgical intervention at our institution from July 1990 to June 2013. The clinical records and pathology reports were reviewed and available follow-up data obtained., Results: A total of 20 patients had a ventricular fibroma. The median age at presentation was 4 years (range, 5 months to 12 years). Of these, 18 (89%) presented with ventricular tachycardia (n = 15) or frequent ectopy (n = 3), and 1 (6%) with outflow tract obstruction; 1 (6%) was asymptomatic and diagnosed during routine evaluation for a murmur. One patient had undergone previous ablation, and one had had an automated internal cardiac defibrillator implanted for ventricular tachycardia control. Before tumor resection at our institution, 1 patient had been listed for transplantation at an outside institution and 1 had undergone previous extracardiac Fontan without tumor resection. All patients underwent successful tumor excision with no recurrence of ventricular arrhythmia during a median follow-up period of 3.3 years (range, 1 month to 14.7 years). One patient underwent planned staged tumor resection. No patient died., Conclusions: Primary ventricular fibromas, even when very large, can be managed by surgical resection, with excellent short- and intermediate-term ventricular function and relief of arrhythmic events., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

Objective: With advances in valve repair and ventricular recruitment strategies, patients initially palliated with single ventricle physiology have been increasingly brought to biventricular circulation. Few data are available on the technical aspects and outcomes after takedown of the superior cavopulmonary anastomosis (bidirectional Glenn [BDG]). We reviewed a single-institutional experience in BDG takedown., Methods: The demographic, procedural, and outcome data were obtained for all children who had undergone BDG takedown at our institution from 2000 to 2012. The primary outcome measures were achievement of biventricular circulation, reoperation, and mortality. The secondary outcome measures were postoperative arrhythmias, superior vena cava (SVC)-right atrium (RA) or pulmonary artery stenosis at the BDG takedown site., Results: A total of 40 patients were included during the study period, with a mean age of 4.4 years (range, 7 months to 22 years). Primary SVC-RA anastomosis was performed in 7 patients (18%), and an anterior patch was used in 33 patients (82%). Of the 40 patients, 38 were discharged with biventricular physiology (98%) and mild or less ventricular dysfunction. During a mean follow-up period of 3.4±2.9 years, 3 patients died and 1 required heart transplantation; 2 patients developed more than mild SVC stenosis requiring reintervention and 1 developed pulmonary artery stenosis. Finally, 34 patients were in normal sinus rhythm and 4 had heart block (1 pacemaker placement)., Conclusions: BDG takedown can be undertaken with a low operative risk and a low incidence of SVC or pulmonary artery stenosis or sinus node dysfunction. Additional follow-up is required to see how the reconstructed SVC grows., (Copyright © 2014 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.)

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., (Copyright © 2013 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Although hypoplasia of left heart structures presents with a spectrum of severity, management decisions are typically dichotomous: single-ventricle palliation or biventricular repair. Since the long-term outcomes of single-ventricle palliation are sub-optimal, strategies to aggressively pursue biventricular circulation in patients with borderline left heart structures have been developed. Recent strategies and surgical techniques to rehabilitate the left heart in patients with borderline left heart are described., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Objectives: The 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)., Background: Management 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., Methods: Patients 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., Results: Mean 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., Conclusions: In 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., (Copyright © 2012 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Objectives: Annuloplasty for functional tricuspid regurgitation may sometimes be ineffective because of chamber dilation and valve tethering. This study compared a novel technique, right ventricle (RV)-papillary muscle approximation, with annuloplasty in experimentally-produced tricuspid regurgitation., Methods: RVs of isolated porcine hearts (n = 10) were statically pressurized, which led to RV dilation and central tricuspid regurgitation. Regurgitant flow was measured with a saline solution-filled column. The head of the anterior papillary muscle was approximated to 4 points on the ventricular septum. Next, a prosthetic ring was implanted, and then RV-papillary muscle approximation was combined. Tricuspid annular dimension, RV geometry, and tricuspid valve tethering were analyzed with 3-dimensional echocardiography., Results: Tricuspid regurgitation (2270 ± 186 mL/min) was reduced by RV-papillary muscle approximation alone (214 ± 45 mL/min; P < .05) more than by annuloplasty alone (724 ± 166 mL/min; P < .05). Combined RV-papillary muscle approximation and annuloplasty resulted in the least regurgitation (80 ± 39 mL/min). RV-papillary muscle approximation reduced tricuspid septolateral diameter (25%; P < .05), and annular area (23%; P < .05), as did annuloplasty. RV-papillary muscle approximation also reduced RV sphericity index (33%; P < .05) and tricuspid tethering height (54%; P < .05), whereas annuloplasty did not. Direction of RV-papillary muscle approximation did not independently affect outcomes., Conclusions: This ex vivo study suggests that RV-papillary muscle approximation potentially repairs tricuspid regurgitation better than annuloplasty by improving ventricular sphericity and valve tethering as well as annular dimension., (Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.)

Primary cardiac tumours in children are rare. Despite the majority of these tumours being benign, they may cause blood flow obstruction, ventricular dysfunction, and life-threatening arrhythmias. We present a case of a 4-year-old boy with repeated presyncope episodes in whom we found a large left ventricular fibroma. Our case emphasises that surgical resection is safely feasible even with large fibromas, avoiding cardiac transplantation in childhood.

This study aimed to evaluate clinical outcomes including hemodynamics, right ventricle (RV) function, and tricuspid valve (TV) function in patients with hypoplastic left heart syndrome (HLHS) at midterm after completion of staged palliation based on the source of pulmonary blood flow provided at stage 1. The records of all patients with HLHS who completed Fontan palliation between 2001 and 2007 were retrospectively reviewed. The outcome variables were RV dysfunction, TV, and neo-atrioventricular (neo-AV) regurgitation (from latest echocardiogram), cardiac index (CI), pulmonary vascular resistance (PVR), pulmonary artery pressure (PAp), and right ventricular end-diastolic pressure (RVEDp) (from latest catheterization). Clinical status was obtained from medical records and by contact with the referring cardiologist if necessary. Of 118 patients undergoing a Fontan for HLHS, 116 had a fenestrated lateral tunnel and 2 had an extracardiac conduit. At the time of stage 1 palliation, 36 patients had a right ventricle-to-pulmonary artery (RV-PA) conduit, and 82 patients had a modified Blalock-Taussig shunt (mBTS). All the patients except one who died of sepsis on extracorporeal membrane oxygenation (ECMO) survived the Fontan operation and were discharged home. At a mean follow-up post-Fontan period of 28.4 months (range, 0.16-95.3 months), three patients had died (2 on the transplantation list and 1 from pulmonary vein stenosis), and one patient had the Fontan circulation taken down. No patient had a heart transplantation. A follow-up echocardiogram was performed for 115 patients (after a mean of 15.6 months for RV-PA and 32.1 months for BTS), and 66 patients underwent a post-Fontan catheterization (after a mean of 15.8 months for RV-PA and 29.3 months for BTS). The hemodynamic results for RV-PA conduit versus BTS were a CI of 3.4 ± 0.8 versus 3.4 ± 1.2, a PVR of 1.8 ± 0.7 versus 1.7 ± 0.8, a PAp of 14.3 ± 3.1 versus 14.2 ± 4.5, and an RVEDp of 7.1 ± 3.3 versus 8.9 ± 5.3. No statistically significant differences were found between shunt types regarding survival or degree of RV dysfunction or in terms of neo-AV regurgitation, CI, PVR, PAp, RVEDp, or rhythm problems. Patients in the BTS group required more tricuspid valvuloplasties and had more tricuspid regurgitation at follow-up evaluation. The patients in the RV-PA group had more PA interventions. In conclusion, the contemporary results after Fontan palliation for HLHS were excellent. At the midterm follow-up evaluation, outcomes and hemodynamic data were similar between shunt types. However, the patients in the BTS group exhibited more tricuspid regurgitation, and the patients in the RV-PA group had increased pulmonary artery interventions.

Background: Progressive left ventricular (LV) dysfunction can be a major late complication in patients with chronic right ventricular pressure overload (eg, tetralogy of Fallot). Therefore, we examined LV function (serial echocardiography and ex vivo Langendorff) and histology in a model of infant pressure-load right ventricular hypertrophy (RVH)., Methods and Results: Ten-day-old rabbits (n=6 per time point, total n=48) that underwent pulmonary artery banding were euthanized at 2 to 8 weeks after pulmonary artery banding, and comparisons were made with age-matched sham controls. LV performance (myocardial performance index) decreased during the progression of RVH, although the LV ejection fraction was maintained. In addition, RVH caused significant septal displacement, reduced septal contractility, and decreased LV end-systolic and end-diastolic dimensions, resulting in LV diastolic dysfunction with the appearance of preserved ejection fraction. Significant septal and LV free-wall apoptosis (myocyte-specific TUNEL and activated caspase-3), fibrosis (Masson trichrome stain), and reduced capillary density (CD31 immunostaining) occurred in the pulmonary artery banding group after 6 to 8 weeks (all P<0.05)., Conclusions: This is the first study showing that pressure overload of the right ventricular resulting in RVH causes LV diastolic dysfunction while preserving ejection fraction through mechanical and molecular effects on the septum and LV myocardium. In particular, the development of RVH is associated with septal and LV apoptosis and reduced LV capillary density.

Objective: The need for conduit replacement in the growing child remains a major problem after right ventricular outflow tract reconstruction. We compared two diverse surgical centers with considerable practice variation in Europe and the United States to identify modifiable risk factors that can increase conduit longevity., Design: Retrospective analysis of 194 patients (56 Europe, 138 United States) who underwent primary right ventricular to pulmonary artery conduit placement between January 1987 and March 2003., Patients: Diagnoses included tetralogy of Fallot with pulmonary atresia, truncus arteriosus, transposition of the great arteries with ventricular septal defect and pulmonary stenosis, and double-outlet right ventricle., Results: Median age was 7.3 months (range 2 days-29.9 years). Types of conduits included aortic homografts (n = 111), pulmonary homografts (n = 48), Contegra conduits (Medtronic, Inc, Minneapolis, MN) (n = 23), and synthetic conduits (n = 12). Freedom from conduit failure at 5 years was 50% (58% Europe, 48% United States, P = NS). On multivariate analysis, smaller conduit diameter (hazard ratio [HR] 1.15, P < .001) and conduits other than pulmonary homografts (synthetic conduits [HR 3.17, P = .01], Contegra conduits [HR 2.80, P = .02], aortic homografts [HR 1.56, P = .05]) predicted shorter time to conduit failure. In addition, time to failure was longer for patients undergoing transcatheter intervention. Different surgical techniques in conduit preparation and insertion did not influence conduit longevity., Conclusions: Analysis of the two diverse surgical centers showed that to increase conduit longevity, one should choose the largest possible conduit, use a pulmonary homograft, and consider children whose conduits develop obstruction as candidates for transcatheter intervention.

Objectives: The use of extracorporeal membrane oxygenation to support patients with shunted single-ventricle physiology has been controversial. Variable survivals are reported in a number of small case series. We sought to evaluate outcomes and identify predictors of survival for patients with shunted single-ventricle physiology who require extracorporeal membrane oxygenation support., Methods: We retrospectively reviewed the medical records of all patients aged less than 1 year with shunted single-ventricle physiology who were supported with extracorporeal membrane oxygenation at Children's Hospital Boston between 1996 and 2005. Survivors and nonsurvivors were compared with respect to demographics, diagnosis, operative variables, indication for extracorporeal membrane oxygenation, and extracorporeal membrane oxygenation variables., Results: Forty-four infants with shunted single-ventricle physiology were supported with extracorporeal membrane oxygenation. Diagnoses included hypoplastic left heart syndrome (24), other single-ventricle lesions (12), and pulmonary atresia/intact ventricular septum or a variant (8). Overall survival to discharge was 48%. Indication for extracorporeal membrane oxygenation was the strongest predictor of survival to discharge, with 81% of patients cannulated for hypoxemia but only 29% of those cannulated for hypotension surviving to discharge. Specifically, patients cannulated for shunt obstruction had the highest survival (83%)., Conclusions: Overall survival to discharge for patients with shunted single-ventricle physiology is similar to survival reported in the Extracorporeal Life Support Organization registry for all infants supported with cardiac extracorporeal membrane oxygenation. Thus, shunted single-ventricle physiology should not be considered a contraindication to extracorporeal membrane oxygenation. Patients cannulated for hypoxemia, particularly shunt thrombosis, had markedly improved survival compared with those supported primarily for hypotension/cardiovascular collapse. Survival did not differ depending on anatomic diagnosis.

This report describes the use of myocardial delayed-enhancement magnetic resonance imaging to delineate the extent of endocardial fibroelastosis (EFE) in 4 infants after fetal balloon aortic valvuloplasty. The preoperative mapping of EFE followed by extensive resection led to biventricular physiology in 2 patients.

Background and Aim of the Study: Lateral tunnel Fontan operation patients with atrioventricular valve (AVV) regurgitation have an increased incidence of Fontan failure (death, take-down or transplant). The outcomes of patients undergoing AVV repair during Fontan palliation were reviewed to determine the optimal technique and timing of repair., Methods: Hospital records for all patients with AVV regurgitation at the time of their Fontan procedure were reviewed retrospectively. Patients with staged single-ventricle palliation culminating in a lateral tunnel Fontan operation who had their first AVV repair at the Children's Hospital, Boston, were included. AVV regurgitation was graded by semi-quantitative color Doppler echocardiography on a scale of 1 to 4, as was ventricular dysfunction., Results: Among 859 lateral tunnel Fontan patients, 27 (3%) had a total of 30 AVV repairs (18 tricuspid, six mitral, six common AVV). Of the 27 first-time AVV repairs (16 TV, six MV, five CAVV), six were performed pre-Fontan, and 21 at or after Fontan. The median age at the first AVV repair was 3 years (range: 0.6-9.4 years). Preoperatively, the median echocardiographic severity of AVV regurgitation was grade 3 (range: 2-4). At median follow up of 1.2 years (range: 0-9.5 years) the severity of AVV regurgitation was reduced significantly to median grade 2 (range: 0-4; p <0.001). No patient with initial AVV repair at the time of Fontan underwent reoperation for AVV regurgitation. In all cases, ventricular function was maintained or improved, with preoperative median systemic ventricular function grade 1.5 (range: 1 to 4) versus postoperative grade 1 (range: 1-4; p = NS). There were no Fontan failures in the intermediate term., Conclusion: AVV regurgitation and ventricular systolic function can be maintained or improved in the intermediate term following AVV repair in single-ventricle patients.

Objective: Thromboembolic events in patients who have undergone the Fontan operation have been reported to be as high as 20% to 33%. A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair., Methods: After informed consent had been obtained, coagulation factors were assayed in 36 infants (mean age, 7.7 +/- 3.6 months) with single-ventricle cardiac defects immediately before undergoing the bidirectional Glenn procedure; 34 infants (mean age, 8.4 +/- 2.6 months) without cardiac disease were assayed as control subjects. Concentration of factors II, V, VII, VIII, IX, and X; antithrombin III; plasminogen; proteins C and S; fibrinogen; serum albumin; and liver enzymes were measured. Normal reference intervals on the basis of the control subjects were determined by using 95% confidence limits. Patient demographic and hemodynamic variables were evaluated as possible predictors of coagulation abnormalities., Results: Concentrations of protein C; factors II, V, VII, IX, and X; plasminogen; fibrinogen; and antithrombin III were significantly lower in the pre-Glenn infants compared with the age-matched control subjects (all P <.001, Student t test). On the basis of multiple logistic regression, no specific hemodynamic variables were predictive of a procoagulant or anticoagulant deficiency. Ventricular dysfunction did predict the presence of multiple coagulation abnormalities (P <.001)., Conclusion: Procoagulant and anticoagulant factor abnormalities occur early in the course of single-ventricle repair and precede the cavopulmonary connection.

Right ventricular (RV) and left ventricular (LV) myocardium differ in their pathophysiological response to pressure-overload hypertrophy. In this report we use microarray and proteomic analyses to identify pathways modulated by LV-aortic banding (AOB) and RV-pulmonary artery banding (PAB) in the immature heart. Newborn New Zealand White rabbits underwent banding of the descending thoracic aorta [LV-AOB; n = 6]. RV-PAB was achieved by banding the pulmonary artery (n = 6). Controls (n = 6 each) were sham-manipulated. After 4 (LV-AOB) and 6 (RV-PAB) wk recovery, the hearts were removed and matched RNA and proteins samples were isolated for microarray and proteomic analysis. Microarray and proteomic data demonstrate that in LV-AOB there is increased transcript expression levels for oxidative phosphorylation, mitochondria energy pathways, actin, ILK, hypoxia, calcium, and protein kinase-A signaling and increased protein expression levels of proteins for cellular macromolecular complex assembly and oxidative phosphorylation. In RV-PAB there is also an increased transcript expression levels for cardiac oxidative phosphorylation but increased protein expression levels for structural constituents of muscle, cardiac muscle tissue development, and calcium handling. These results identify divergent transcript and protein expression profiles in LV-AOB and RV-PAB and provide new insight into the biological basis of ventricular specific hypertrophy. The identification of these pathways should allow for the development of specific therapeutic interventions for targeted treatment and amelioration of LV-AOB and RV-PAB to ameliorate morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objective: Borderline left heart disease is characterized by left heart obstructive lesions (coarctation, aortic and mitral stenoses, left ventricular hypoplasia) and endocardial fibroelastosis. The multilevel obstruction and impaired left ventricular systolic and diastolic function contribute to failure of biventricular circulation. We studied the effects of left ventricular rehabilitation—endocardial fibroelastosis resection with mitral or aortic valvuloplasty—on left ventricular function and clinical outcomes. Methods: All patients with borderline left heart structures and endocardial fibroelastosis who underwent a primary left ventricular rehabilitation procedure were retrospectively analyzed to determine operative mortality, reintervention rates, and hemodynamic status. Left heart dimensions and hemodynamics were recorded from preoperative and postoperative echocardiogram and cardiac catheterization. Postoperative left atrial pressure was obtained from the intracardiac line early after left ventricular rehabilitation. Preoperative and postoperative values were compared by paired t test. Results: Between 1999 and 2008, 9 patients with endocardial fibroelastosis and borderline left heart disease underwent left ventricular rehabilitation at a median age of 5.6 months (range, 1–38 months). There was no operative mortality, and at a median follow-up of 25 months (6 months to 10 years) there was 1 death from noncardiac causes and 2 patients required reoperations. Significant increases in ejection fraction and left ventricular end-diastolic volume were observed, whereas left atrial pressure and right ventricular/left ventricular pressure ratios decreased postoperatively. Conclusion: In patients with borderline left hearts, primary left ventricular rehabilitation with endocardial fibroelastosis resection and mitral and aortic valvuloplasty results in improved left ventricular systolic and diastolic performance and decreased right ventricular pressures. This approach may provide an alternative to single-ventricle management in this difficult patient group. [Copyright &y& Elsevier]

Background: There are distinct physiologic differences between patients with single-ventricle lesions who have undergone the Norwood procedure with a right ventricle–to–pulmonary artery conduit (NW-RVPA) compared with those patients who have undergone the Norwood operation with a Blalock-Taussig shunt (NW-BTS). We evaluated bidirectional Glenn operation outcomes and compared the two groups to assess whether the type of Norwood operation influenced outcomes. Methods: A retrospective chart review compared bidirectional Glenn operation outcomes for children undergoing the Norwood operation with NW-RVPA or NW-BTS at Children’s Hospital Boston from January 1, 2002, to December 31, 2003. Results: Of 80 patients undergoing the Norwood operation, 56 (NW-BTS, 27 versus NW-RVPA, 29) returned for the bidirectional Glenn operation at our institution. The NW-RVPA group had a lower median age at presentation for bidirectional Glenn (4.5 months versus 5.8 months; p = 0.01), but had better weight gain (20.6 g/day versus 16.5 g/day; p = 0.03) than the NW-BTS group. No interstage deaths occurred in the NW-RVPA group. There were no differences in morbidity or mortality after the BDG between the two groups. Conclusions: There were no differences in morbidity and mortality outcomes after the bidirectional Glenn operation between the NW-RVPA and NW-BTS groups. Despite younger age at presentation, the NW-RVPA patients had better growth rate, which may have contributed to the similar postoperative outcomes. [Copyright &y& Elsevier]

Objective: Patients with repaired tetralogy of Fallot account for most cases of late-onset right ventricle failure. The current surgical approach, which includes pulmonary valve replacement/insertion, has yielded mixed results. A new surgical option of placing an elastic band in the right ventricle is proposed to improve right ventricular cardiac function as measured by the ejection fraction. Methods: A total of 20 computational right ventricular/left ventricular/patch/band combination models using cardiac magnetic resonance imaging from a patient with tetralogy of Fallot were constructed to investigate the effect of band material stiffness variations, band length, and active contraction. These models included 4 different band material properties, 3 band length, 3 active contracting band materials, and models with patch and scar replaced by contracting tissue. Results: Our results indicated that the band insertion, combined with active band contraction and tissue regeneration techniques that restore right ventricular myocardium, has the potential to improve right ventricular ejection fraction by 7.5% (41.63% ejection fraction from the best active band model to more than 34.10% ejection fraction from baseline passive band model) and 4.2% (41.63% from the best active band model compared with cardiac magnetic resonance imaging-measured ejection fraction of 37.45%). Conclusions: The cardiac magnetic resonance imaging-based right ventricular/left ventricular/patch/band model provides a proof of concept for using elastic bands to improve right ventricular cardiac function. Band insertion, combined with myocardium regeneration techniques and right ventricular remodeling surgical procedures, has the potential to improve ventricular function in patients with repaired tetralogy of Fallot and other similar forms of right ventricular dysfunction after surgery. Additional investigations using in vitro experiments, animal models, and, finally, patient studies are warranted. [ABSTRACT FROM AUTHOR]

Abstract: Multi-physics right and left ventricle (RV/LV) fluid–structure interaction (FSI) models were introduced to perform mechanical stress analysis and evaluate the effect of patch materials on RV function. The FSI models included three different patch materials (Dacron scaffold, treated pericardium, and contracting myocardium), two-layer construction, fiber orientation, and active anisotropic material properties. The models were constructed based on cardiac magnetic resonance (CMR) images acquired from a patient with severe RV dilatation and solved by ADINA. Our results indicate that the patch model with contracting myocardium leads to decreased stress level in the patch area, improved RV function and patch area contractility. [Copyright &y& Elsevier]