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In this manuscript, we describe the design and rationale of a randomized controlled trial in pediatric Fontan patients to test the hypothesis that a live-video-supervised exercise (aerobic+resistance) intervention will improve cardiac and physical capacity; muscle mass, strength, and function; and endothelial function. Survival of children with single ventricles beyond the neonatal period has increased dramatically with the staged Fontan palliation. Yet, long-term morbidity remains high. By age 40, 50% of Fontan patients will have died or undergone heart transplantation. Factors that contribute to onset and progression of heart failure in Fontan patients remain incompletely understood. However, it is established that Fontan patients have poor exercise capacity which is associated with a greater risk of morbidity and mortality. Furthermore, decreased muscle mass, abnormal muscle function, and endothelial dysfunction in this patient population is known to contribute to disease progression. In adult patients with 2 ventricles and heart failure, reduced exercise capacity, muscle mass, and muscle strength are powerful predictors of poor outcomes, and exercise interventions can not only improve exercise capacity and muscle mass, but also reverse endothelial dysfunction. Despite these known benefits of exercise, pediatric Fontan patients do not exercise routinely due to their chronic condition, perceived restrictions to exercise, and parental overprotection. Limited exercise interventions in children with congenital heart disease have demonstrated that exercise is safe and effective; however, these studies have been conducted in small, heterogeneous groups, and most had few Fontan patients. Critically, adherence is a major limitation in pediatric exercise interventions delivered on-site, with adherence rates as low as 10%, due to distance from site, transportation difficulties, and missed school or workdays. To overcome these challenges, we utilize live-video conferencing to deliver the supervised exercise sessions. Our multidisciplinary team of experts will assess the effectiveness of a live-video-supervised exercise intervention, rigorously designed to maximize adherence, and improve key and novel measures of health in pediatric Fontan patients associated with poor long-term outcomes. Our ultimate goal is the translation of this model to clinical application as an "exercise prescription" to intervene early in pediatric Fontan patients and decrease long-term morbidity and mortality. [ABSTRACT FROM AUTHOR]

Objective: To test feasibility of tele-clinic visits using parentally acquired vital signs and focused echocardiographic images in patients with Marfan syndrome.Study Design: We included patients with Marfan syndrome aged 5-19 years followed in our clinic. We excluded patients with Marfan syndrome and history of previous aortic root (AoR) surgery, cardiomyopathy, arrhythmia, or AoR ≥4.5 cm. We trained parents in-person to acquire focused echocardiographic images on their children using a hand-held device as well as how to use a stadiometer, scale, blood pressure (BP) machine, and a digital stethoscope. Before tele-clinic visits, parents obtained the echocardiographic images and vital signs. We compared tele-clinic and on-site clinic visit data. Parental and clinic echocardiograms were independently analyzed.Results: Fifteen patient/parent pairs completed tele-clinic visits, conducted at a median of 7.0 (IQR 3.0-9.9) months from the in-person training session. Parents took a median of 70 (IQR 60-150) minutes to obtain the height, weight, heart rate, BP, cardiac sounds, and echocardiographic images before tele-clinic visits. Systolic BP was greater on-site than at home (median +13 mm Hg, P = .014). Height, weight, diastolic BP, heart rate, and AoR measurements were similar.Conclusions: This study provides information for implementing tele-clinic visits using parentally acquired vital signs and echocardiographic images in patients with Marfan syndrome. The results show that tele-clinic visits are feasible and that parents were able to obtain focused echocardiographic images on their children.Trial Registration: ClinicalTrials.gov: NCT03581682. [ABSTRACT FROM AUTHOR]

Background Major aortopulmonary collateral arteries (MAPCAs) are frequently found in association with pulmonary atresia with ventricular septal defect (PA/VSD). However, some patients with MAPCAs do not have PA/VSD but have a variety of other “atypical” anatomic diagnoses. Methods This was a retrospective review of patients with MAPCAs and atypical anatomy. The 50 patients with MAPCAs could be divided into two subgroups: (1) single ventricle anatomy (n = 33) and (2) two ventricle anatomy (n = 17). Results The 33 patients with MAPCAs and single ventricle included 15 with unbalanced complete atrioventricular canal (CAVC), 6 with pulmonary atresia-intact ventricular septum, and 12 with other forms of single ventricle. The initial cardiac operation included unifocalization/shunt in 24 patients and creation of aortopulmonary window or central shunt in 9 patients. There were seven operative and eight late deaths. Sixteen patients have had a bidirectional Glenn procedure and 6 had a Fontan procedure. The 17 patients with MAPCAs and two ventricles included 5 with CAVC, 4 with corrected transposition, 3 with double outlet right ventricle, 3 with scimitar syndrome, and 2 with complex D-transposition. The initial cardiac operation included single-stage complete repair in 5 patients, unifocalization/shunt in 10 patients, and aortopulmonary window in 2 patients. There were two operative and two late deaths. Thirteen patients have achieved complete repair status. Conclusions The data demonstrate the wide diversity of anatomy seen in patients with MAPCAs when evaluating diagnoses other than PA/VSD. Two-thirds of the patients had single ventricle and was associated with a relatively high mortality. [ABSTRACT FROM AUTHOR]

Hematopoietic cell transplantation (HCT) is curative for many pediatric malignant and nonmalignant disorders but is associated with significant morbidity and mortality, including the development of pericardial effusion (PEF). We report the results of a retrospective chart review performed to assess the incidence, risk factors, and prognostic significance of PEF in pediatric HCT recipient at Lucile Packard Children's Hospital of Stanford University. A total of 119 patients undergoing HCT between January 2010 and December 2013 were selected through the hospital's Pediatric Stem Cell Transplant Program database. A retrospective chart review, including review of documentation, correspondence, imaging reports, laboratory values, and death records, was performed to collect data. The overall incidence of PEF in our population was 21%. Risk factors for the development of PEF included unrelated donor transplants and cord blood as the stem cell source ( P  = .005), whereas HLA mismatch approached significance ( P  = .05). The risk for development of PEF was found to not be significantly associated with acute or chronic graft-versus-host disease (GVHD), age at transplantation, sex, conditioning regimen, or viral reactivation status. Of interest, 6 of the 119 patients were found to have transplant-associated thrombotic microangiopathy (TA-TMA). Four of those 6 patients developed PEF, suggesting TA-TMA as a risk factor for PEF. Eight of the 25 patients who developed PEF (32%) required pericardiocentesis. Five out of the 8 patients requiring pericardiocentesis died owing to causes unrelated to the procedure or to PEF itself. Pericardial fluid testing in 4 of these patients (50%) was positive for human herpesvirus 6, Epstein-Barr virus, cytomegalovirus, and/or adenovirus. Finally, of significant interest, patients with PEF had a statistically significant higher likelihood of mortality compared with those without PEF (44% versus 17%; P  = .007). [ABSTRACT FROM AUTHOR]

Background In neonates, it is challenging to diagnose aortic coarctation in the setting of a patent ductus arteriosus (PDA). Frequently, serial echocardiograms are performed, and diagnosis is delayed until the PDA closes. The purpose of this study was to identify echocardiographic predictors of neonatal coarctation in the presence of a PDA in cases in which diagnosis is uncertain. Methods We retrospectively identified neonates diagnosed with possible but not definitive coarctation in the presence of a PDA by echocardiography (January 2004 through August 2013). The carotid-subclavian artery index (CSAi) was defined as the distal transverse arch diameter divided by the distance between the left common carotid and left subclavian arteries. Medical records were reviewed to identify patients who underwent coarctation repair within 1 year. A separate validation group was identified with the same methodology (September 2013 through April 2015). Results Thirty-three patients were identified (median age 1, range 0-8 days). Twelve patients (36%) underwent coarctation repair. The coarctation group had smaller aortic and mitral valves, distal transverse arch, and isthmus z scores, larger right innominate artery, and longer transverse arch compared with the remaining group ( p < 0.05). The CSAi was lower in the coarctation group ( p = 0.014), and a cutoff of less than 0.85 yielded a sensitivity of 0.83 and specificity of 0.86 for coarctation (area under the receiver operating characteristic curve, 0.91). In the validation group (n = 12; median follow-up, 316 days), none of the 8 patients with a CSAi of greater than 0.85 have required surgery. The intraclass correlation coefficient for CSAi was 0.79 (95% confidence interval, 0.18 to 0.95). Conclusions The CSAi, a simple and reproducible measure, can identify neonates at risk for aortic coarctation even in the presence of a PDA, prevent multiple echocardiographic evaluations, and hence guide appropriate resource utilization. [ABSTRACT FROM AUTHOR]

Background Surgical repair of pulmonary atresia with ventricular septal defect and major aortopulmonary collaterals (PA/VSD/MAPCAs) requires insertion of a conduit from the right ventricle to the reconstructed pulmonary arteries. Although there is extensive literature on conduit longevity for many forms of congenital heart defects, there is currently limited information for PA/VSD/MAPCAs. It is likely that conduits will have a different longevity in PA/VSD/MAPCAs because pulmonary artery pressures are higher after unifocalization than in other congenital heart defects. The purpose of this study was to evaluate the fate of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs. Methods This was a retrospective review of 103 infants who underwent complete repair of PA/VSD/MAPCAs with insertion of an aortic homograft conduit. The average age at complete repair was 5.1 ± 2.8 months, average conduit size was 13 ± 2 mm, and average conduit z score was a positive 1.6 ± 0.9. Results Conduit z scores were linearly correlated with increasing homograft conduit size. The average longevity of small homografts (8 to 11 mm) was 15 ± 4 months, medium homografts (12 to 14 mm) was 39 ± 7 months, and large homografts (15 to 17 mm) was 50 ± 14 months. There was a negative correlation between increasing pulmonary artery pressure and conduit longevity. Conclusions Conduit z scores and conduit size worked synergistically to predict the longevity of right ventricle to pulmonary artery conduits after complete repair of PA/VSD/MAPCAs. Pulmonary artery pressure was an additional modifier of longevity. These data provide a framework for expectations of aortic homograft conduits used in the repair of PA/VSD/MAPCAs. [ABSTRACT FROM AUTHOR]

Background Chronic right ventricular (RV) pacing has been associated with decreased exercise capacity and left ventricular (LV) function in adults with congenital complete atrioventricular block (CCAVB), but not in children. Objective The purpose of this study was to evaluate the exercise capacity and LV function in pediatric patients with CCAVB receiving chronic RV pacing. Methods We prospectively evaluated pediatric patients with isolated CCAVB receiving atrial synchronous RV pacing for at least 5 years. Supine bicycle ergometry was performed, and LV ejection fraction (EF) was evaluated by echocardiography. Results Ten CCAVB subjects and 31 controls were matched for age, gender, and body surface area. CCAVB subjects had normal resting EF (63.1% ± 4.0%) and had been paced for 7.9 ± 1.4 years. Exercise testing demonstrated reduced functional capacity in CCAVB patients compared to controls with a lower VO 2peak (26.0 ± 6.6 mL/kg/min vs 39.9 ± 7.0 mL/kg/min, P <.001), anaerobic threshold (15.6 ± 3.9 mL/kg/min vs 18.8 ± 2.7 mL/kg/min, P = .007), and oxygen uptake efficiency slope (1210 ± 406 vs 1841 ± 452, P <.001). Maximum heart rate (165 ± 8 bpm vs 185 ± 9 bpm, P <.001) and systolic blood pressure (159 ± 17 mm Hg vs 185 ± 12 mm Hg, P <.019) also were reduced in CCAVB patients despite maximal effort (respiratory exchange ratio 1.2 ± 0.1). EF was augmented with exercise in controls but not in CCAVB patients (13.2% ± 9.3% vs 0.2% ± 4.8% increase, P <.001). Conclusion Clinically asymptomatic children with chronic RV pacing due to CCAVB have significant reductions in functional capacity accompanied by chronotropic incompetence and inability to augment EF with exercise. [ABSTRACT FROM AUTHOR]

Background Children requiring a permanent epicardial pacemaker (PM) traditionally have a single lead placed on the right ventricle. Lead failure in pacemaker-dependent (PMD) children, however, can result in cardiovascular events (CVEs) and death. Objective The purpose of this study was to determine if redundant ventricular lead systems (RVLS) can safeguard against CVE and death in PMD children. Methods This was a single-center study of PMD patients undergoing placement of RVLS from 2002–2013. Patients ≤21 years of age who were PMD were included. Patients with a biventricular (BiV) system placed for standard resynchronization indications were excluded. RVLS patients were compared to PMD patients with only a single pacing lead on the ventricle (SiV). Results Seven hundred sixty-nine patients underwent PM/implantable cardioverter-defibrillator placement with 76 BiV implants; 49 patients (6%) were PMD. Thirteen patients underwent implantation of an RVLS. There was no difference between the RVLS group (n = 13) and SiV PMD control group (n = 24) with regard to age (RVLS 9.5 ± 5.8 years vs SiV 9.4 ± 6.7 years, P = .52), weight (RVLS 38.2 ± 32.6 kg vs SiV 35.2 ± 29.3 kg, P = .62), indication for pacing, procedural complications, or time to follow-up. There were 2 lead fractures (17%) in the RVLS group (mean follow-up 3.8 ± 2.9 years), with no deaths or presentations with CVE. The SiV control group had 3 lead fractures (13%) (mean follow-up 2.8 ± 2.9 years), with no deaths, but all 3 patients presented with CVE and required emergent PM placement. Conclusion RVLS systems should be considered in children who are PMD and require permanent epicardial pacing. BiV pacing and RVLS may decrease the risk of CVE in the event of lead failure in PMD patients. [ABSTRACT FROM AUTHOR]

Background. Aortic valve disease in association with pulmonary atresia, ventricular septal defect, and major aortopulmonary collaterals (PA/VSD/MAPCAs) is an extremely rare combination of congenital heart defects. The presence of aortic stenosis or insufficiency or both imposes an additional physiologic burden that complicates the management of PA/VSD/MAPCAs. This report summarizes our experience with 7 patients who underwent surgical repair of this rare combination of defects. Methods. This was a retrospective review of patients who had both aortic valve disease and PA/VSD/MAPCAs. Four patients had predominantly aortic stenosis, 2 patients had aortic insufficiency, and 1 had mixed stenosis and insufficiency. Anatomically, this corresponded to a dysplastic trileaflet valve in 4, bicuspid valve in 2, and quadricuspid valve in 1. Results. Four of the 7 patients underwent aortic valve surgery at the time of their first operation for PA/VSD/MAPCAs. The remaining 3 patients had aortic valve surgery at subsequent operations. All 7 patients are alive, with a median duration of follow-up of 3 years. One patient has required aortic valve replacement 2 years after repair of an insufficient bicuspid valve. Conclusions. This manuscript summarizes our experience with 7 patients who had the rare combination of aortic valve disease and PA/VSD/MAPCAs. Although aortic valve disease has been reported for other conotruncal defects, we believe this is the first report of its presence in association with PA/VSD/MAPCAs. [ABSTRACT FROM AUTHOR]

Background: Pulmonary stenosis with ventricular septal defect and major aortopulmonary collaterals (PS/VSD/MAPCAs) is an extremely rare form of congenital heart defect. Although it has been assumed that PS/VSD/MAPCAs would be similar to pulmonary atresia (PA) with VSD/MAPCA, there are currently no data to support this conjecture. This study reviewed our surgical experience with reconstruction of PS/VSD/MAPCA. Methods: This was a retrospective review of 25 patients (14 boys, 11 girls) who were born with PS/VSD/MAPCA and underwent surgical reconstruction. Preoperative pulmonary angiography was used to define the central branch pulmonary arteries and MAPCA. Patients were a median age of 4 months at the first operation. Results: There was one operative death (4%) in this cohort of 25 patients, and complete repair was achieved in the 24 survivors (96%). There were two distinct subgroups of patients: 11 demonstrated cyanosis in the neonatal timeframe and underwent an initial procedure to augment pulmonary blood flow (+PBF). The remaining 14 patients formed the second group (–PBF). The median age at the first operation was 0.8 months in the +PBF group and 5.2 months in the –PBF group (p < 0.005). Complete repair was achieved in 91% of patients in the +PBF group and in 100% in the –PBF group; however, the average number of procedures to achieve complete repair was 2.8 in the +PBF group vs 1.0 in the –PBF group (p < 0.005). Conclusions: Outcomes for PS/VSD/MAPCAs as a whole were excellent, with a low surgical mortality and high rate of complete repair. There were two identifiable subgroups with distinctive differences required in their surgical management. These results provide a prognostic outlook for patients with PS/VSD/MAPCAs that can be compared and contrasted with PA/VSD/MAPCAs. [Copyright &y& Elsevier]

Objectives: Peripheral pulmonary artery stenosis is a rare congenital heart defect frequently found in association with Williams and Alagille syndromes. Controversy exists regarding the optimal treatment of peripheral pulmonary artery stenosis, with most centers favoring catheter-based interventions. In contrast, we have preferentially used surgical reconstruction of peripheral pulmonary artery stenosis. The purpose of the present study was to review our experience with surgical reconstruction of peripheral pulmonary artery stenosis. Methods: We performed a retrospective review of patients who underwent surgical reconstruction of peripheral pulmonary artery stenosis. A total of 16 patients were identified: 7 had Williams syndrome, 6 had Alagille syndrome, and 3 had no identifiable syndrome. Detailed pulmonary angiography was performed in all patients to define stenoses at the main, branch, lobar, and segmental arterial levels. The mean preoperative right ventricular/left ventricular pressure ratio was 0.88 ± 0.07. The surgical approach was a median sternotomy with cardiopulmonary bypass. All peripheral stenoses were augmented with pulmonary artery homograft tissue. The median age at surgery was 14 months, and concomitant procedures were performed in 9 of the 16 patients. Results: There was 1 operative mortality (6%). The mean right ventricular/left ventricular pressure ratio decreased to 0.40 ± 0.04 postoperatively (P < .005), representing a 55% reduction compared with the preoperative values. The patients were followed up for a median of 5 years. No late mortality occurred and reoperation was not required. Conclusions: The data have demonstrate that this comprehensive surgical approach to the treatment of peripheral pulmonary artery stenosis was associated with low early and no late mortality. Surgical reconstruction of the peripheral pulmonary artery stenosis resulted in a significant decrease in right ventricular pressure. We hypothesize that this reduction in right ventricular pressures will confer a long-term survival advantage for this cohort of patients. [Copyright &y& Elsevier]

BACKGROUND: Cardiac resynchronization therapy has been proposed for treatment of hypoplastic left heart syndrome (HLHS) patients with right ventricular (RV) failure. The role of dyssynchrony, however, is poorly understood in this population. OBJECTIVE: The purpose of this study was to better understand the relationship between electrical and mechanical dyssynchrony in HLHS using 3-dimensional electrical mapping, tissue Doppler indices of wall motion, and vector velocity imaging. METHODS: Eleven HLHS subjects with normal RV function and ten normal subjects (age 3–18 years) were studied. Electrical and mechanical activation times and dyssynchrony indices (electrical dyssynchrony index, mechanical dyssynchrony index) were calculated using 3-dimensional electrical mapping, tissue Doppler indices, and vector velocity imaging. RESULTS: No differences in measures of electrical dyssynchrony were seen when comparing HLHS patients and normal patients (electrical activation time 63.3±22.8 ms vs 56.2±11.2 ms, P = .38; electrical dyssynchrony index 13.7±6.3 ms vs 11.6±3.0 ms, P = .34). However, measures of mechanical dyssynchrony were markedly abnormal in HLHS patients despite normal RV function (mechanical activation time 16±11.3 ms vs 0.9±1.9 ms, P = .01; mechanical dyssynchrony index 7.5±5.5 vs 0.4±0.8, P<.01). CONCLUSION: Patients with HLHS and preserved RV systolic function have normal electrical activation when compared to patients with normal right and left ventricles. In contrast, these patients demonstrate mechanical dyssynchrony compared to patients with normal right and left ventricles. This finding raises important questions about the indications for cardiac resynchronization therapy in this patient population. [Copyright &y& Elsevier]

This review focuses on the embryology and anatomy of the aorta with some imaging examples. Dividing the aorta by segments of unique function and embryogenesis facilitates organizing the group of potential anomalies encountered. A basic understanding of the embryologic development of the aorta and its major branches helps in understanding and recognizing typical and atypical anatomic findings. Diagnostic imaging of the aorta and its major branches can be accomplished by invasive and noninvasive methods, based on the clinical scenario and the age of the patient. In this review, computed tomography and magnetic resonance imaging examples are emphasized. [Copyright &y& Elsevier]

Background: Pulmonary atresia and major aortopulmonary collaterals (PA/MAPCAs) is a complex form of congenital heart disease. One to two percent of patients with PA/MAPCAs will also have total anomalous pulmonary venous connection (TAPVC). This study summarizes our surgical experience with this rare combination of life-threatening congenital heart defects. Methods: A retrospective review was performed to identify patients who had surgery for PA/MAPCAs in association with TAPVC. From November 2001 to March 2011, 9 patients presented with this combination of defects. Eight of the 9 patients had heterotaxy with an unbalanced atrioventricular canal and functional single ventricle. The ninth patient had double outlet right ventricle (two ventricles). Timing of surgical intervention was typically predicated on the degree of pulmonary venous obstruction. The median age at surgery was 14 days. All nine patients had surgical correction of TAPVC, unifocalization of MAPCA''s into a central confluence, and placement of a shunt. Results: There was one early mortality (< 30 days) and two late mortalities. For the 6 survivors, 5 have subsequently undergone a bidirectional Glenn procedure, and 3 had completion of their Fontan. Two patients are currently at the bidirectional Glenn stage; one is a good candidate for Fontan completion while the other is not suitable. The sixth patient is awaiting further assessment. Conclusions: The PA/MAPCAs, in association with TAPVC, is a challenging combination of defects. The data suggest that the combination of PA/MAPCAs and TAPVC can be undertaken with a reasonable midterm prognosis. [ABSTRACT FROM AUTHOR]

Corrected transposition is a relatively uncommon congenital heart defect characterized by discordance at both the atrioventricular and ventriculoarterial levels. A rare subset of patients with corrected transposition also have criss-cross atrial morphology. We report the surgical treatment of 2 patients with corrected transposition in association with criss-cross atrial morphology, emphasizing the novel atrial baffle required for this operation. [ABSTRACT FROM AUTHOR]

Objective: To test the hypothesis that a fetal stratification pathway will effectively discriminate between infants at different levels of risk for surgical coarctation and reduce unnecessary medicalization.Study Design: We performed a pre-post nonrandomized study in which we prospectively assigned fetuses with prenatal concern for coarctation to 1 of 3 risk categories and implemented a clinical pathway for postnatal management. Postnatal clinical outcomes were compared with those in a historical control group that were not triaged based on the pathway.Results: The study cohort comprised 109 fetuses, including 57 treated along the fetal coarctation pathway and 52 historical controls. Among mild-risk fetuses, 3% underwent surgical coarctation repair (0% of those without additional heart defects), compared with 27% of moderate-risk and 63% of high-risk fetuses. The combined fetal aortic, mitral, and isthmus z-score best discriminated which infants underwent surgery (area under the curve = 0.78; 95% CI, 0.66-0.91). Compared with historical controls, infants triaged according to the fetal coarctation pathway had fewer delivery location changes (76% vs 55%; P = .025) and less umbilical venous catheter placement (74% vs 51%; P = .046). Trends toward shorter intensive care unit stay, hospital stay, and time to enteral feeding did not reach statistical significance.Conclusions: A stratified risk-assignment pathway effectively identifies a group of fetuses with a low rate of surgical coarctation and reduces unnecessary medicalization in infants who do not undergo aortic surgery. Incorporation of novel measurements or imaging techniques may improve the specificity of high-risk criteria. [ABSTRACT FROM AUTHOR]

Pulmonary regurgitation developing late after tetralogy of Fallot repair is now recognized as a serious threat to the long-term welfare of these patients. This article summarizes our experience with 5 patients who underwent reoperations for treatment of severe pulmonary regurgitation after transannular patch repair of tetralogy of Fallot. In each case, the intraoperative findings revealed anatomy favorable for valve repair and enabled preservation of the native pulmonary valves. [Copyright &y& Elsevier]

Objective Palliation of hypoplastic left heart syndrome with a standard nonvalved right ventricle to pulmonary artery conduit results in an inefficient circulation in part due to diastolic regurgitation. A composite right ventricle pulmonary artery conduit with a homograft valve has a hypothetical advantage of reducing regurgitation, but may differ in the propensity for stenosis because of valve remodeling. Methods This retrospective cohort study included 130 patients with hypoplastic left heart syndrome who underwent a modified stage 1 procedure with a right ventricle to pulmonary artery conduit from 2002 to 2015. A composite valved conduit (cryopreserved homograft valve anastomosed to a polytetrafluoroethylene tube) was placed in 100 patients (47 aortic, 32 pulmonary, 13 femoral/saphenous vein, 8 unknown), and a nonvalved conduit was used in 30 patients. Echocardiographic functional parameters were evaluated before and after stage 1 palliation and before the bidirectional Glenn procedure, and interstage interventions were assessed. Results On competing risk analysis, survival over time was better in the valved conduit group ( P = .040), but this difference was no longer significant after adjustment for surgical era. There was no significant difference between groups in the cumulative incidence of bidirectional Glenn completion ( P = .15). Patients with a valved conduit underwent more interventions for conduit obstruction in the interstage period, but this difference did not reach significance ( P = .16). There were no differences between groups in echocardiographic parameters of right ventricle function at baseline or pre-Glenn. Conclusions In this cohort of patients with hypoplastic left heart syndrome, inclusion of a valved right ventricle to pulmonary artery conduit was not associated with any difference in survival on adjusted analysis and did not confer an identifiable benefit on right ventricle function. [ABSTRACT FROM AUTHOR]