Tricuspid valve repair concomitant with the Norwood operation among babies with hypoplastic left heart syndrome.

OBJECTIVES: Among patients with hypoplastic left heart syndrome (HLHS), tricuspid valve regurgitation (TR) portends a poor prognosis. Our goal was to describe the outcomes of tricuspid valve reconstruction (TVR) concomitant with the Norwood operation and using two-dimensional echocardiography and evaluate the structural factors associated with successful functional correction. METHODS: We performed a retrospective, single-centre study of patients with HLHS undergoing TVR at the time of the Norwood operation. Structural echocardiographic parameters were compared between patients with successful correction (≤ mild TR) and those with ≥ moderate regurgitation at discharge. Preoperative dimensions of matched HLHS controls with ≤ trivial TR were used as a reference. RESULTS: Of 205 patients with HLHS undergoing the Norwood operation, 18 patients had a concomitant TVR. Ten (56%) patients had an improved TR grade postoperatively, 8 (44%) of whom had ≤ mild TR at discharge. Improvement in TR grade (P = 0.001) and having ≤ mild TR at discharge (P = 0.011) were associated with an improved reintervention and TR-free survival. Patients with successful functional correction had smaller preoperative tricuspid annulus lateral dimensions (P = 0.023), tricuspid valve area (P = 0.007) and right ventricle mid-width (P = 0.064). Preoperatively, the successful TVR cases tended to have had higher anterior leaflet excursion (80 ± 20 vs 55 ± 11, P = 0.010), and a higher proportion of anterior leaflet prolapse (63% vs 10%, P = 0.043) compared to cases where TVR was not successful. CONCLUSIONS: Patients with HLHS with significant tricuspid regurgitation undergoing the stage 1 operation were more likely to have successful concomitant tricuspid valve repair if they had less tricuspid annular dilation, less-severe RV enlargement and predominantly anterior leaflet prolapse. Successful tricuspid valve repair was associated with improved mid- and long-term outcomes.

Mortality and Reoperation Risk After Bioprosthetic Aortic Valve Replacement in Young Adults With Congenital Heart Disease.

Bioprosthetic aortic valve replacement (bAVR) in patients with congenital heart disease is challenging due to age, size and complexity. Our objective was to assess survival and identify predictors of re-operation. Data were retrospectively collected for 314 patients undergoing bAVR at 8 centers from 2000-2014. Kaplan-Meier estimation of time to re-operation and Cox regression were utilized. Average age was 45.2 years (IQR 17.8-71.1) and 30% were <21. Indications were stenosis (48%), regurgitation (28%) and mixed (18%). Twenty-eight (9%) underwent prior AVR. Median valve size was 23mm (IQR 21, 25). Implanted valves included CE (Carpentier-Edwards) Perimount (47%), CE Magna/Magna Ease (29%), Sorin Mitroflow (9%), St Jude (2%) and other (13%). Median follow-up was 2.9 (IQR 1.2, 5.7) years. Overall, 11% required re-operation, 35% of whom had a Mitroflow and 65% were <21 years old. Time to re-operation varied among valve type (p=0.020). Crude 3-year rate was 20% in patients ≤21. Smaller valve size indexed to BSA was associated with re-operation (21.7 vs. 23.5 mm/m2). Predictors of reintervention by multivariable analysis were younger age (29% increase in hazard per 5-year decrease, p<0.001), Mitroflow (HR=4 to 8 versus other valves), and smaller valve size (20% increase in hazard per 1 mm decrease, p=0.002). The overall 1, 3 and 5-year survival rates were 94%, 90% and 85% without differences by valve (p=0.19). A concerning reduction in 5-year survival after bAVR is shown. Re-operation is common and varies by age and valve type. Further research is needed to guide valve choice and improve survival.

Reintervention rates after bioprosthetic pulmonary valve replacement in patients younger than 30 years of age: A multicenter analysis.

OBJECTIVES: To assess the difference in time to and predictors of reintervention according to valve type in surgical bioprosthetic pulmonary valve replacement (PVR) in patients younger than 30 years of age from multiple centers. METHODS: Data were retrospectively collected for 1278 patients <30 years of age undergoing PVR at 8 centers between 1996 and 2015. RESULTS: Mean age at PVR was 19.3 ± 12.8 years, with 719 (56.3%) patients ≤18 years of age. Diagnosis was tetralogy of Fallot in 626 patients (50.5%) and 165 (12.9%) had previous PVR. Median follow-up was 3.9 years (interquartile range, 1.2, 6.4). Multiple valve types were used, most commonly CE PERIMOUNT, 488 (38.2%), CE Magna/Magna Ease, 361 (28.2%), and Sorin Mitroflow 322 (25.2). Reintervention occurred in 12.7% and was most commonly due to pulmonary stenosis (68.8%), with most reinterventions occurring in children (85.2%) and with smaller valve sizes (P < .001) Among adults aged 18 to 30 years, younger age was not a significant risk factor for reintervention. Surgical indication of isolated pulmonary regurgitation was associated with a lower risk of reintervention (P < .001). Overall, 1-, 3-, 5-, and 10-year freedom from reintervention rates were 99%, 97%, 92%, and 65%. The only independent risk factors for reintervention after controlling for age and valve size were lack of a concomitant tricuspid valve procedure (P = .02) and valve type (P < .001); Sorin and St Jude valves were associated with similar time to reintervention, and deteriorated more rapidly than other valve types. CONCLUSIONS: In this large multicenter study, 8% of patients have undergone reintervention by 5 years. Importantly, independent of age and valve size, reintervention rates vary by valve type.

Three-Patch Aortic Root Reconstruction With Extended Left Main Coronary Artery Patch Augmentation in Neonates and Infants.

Left main coronary artery (LMCA) stenosis is present in approximately 5% of patients with congenital supravalvular aortic stenosis (SVAS) (Fig. 1)1 and is associated with an increased risk of sudden cardiac death.2 However, patients undergoing coronary artery intervention at the time of SVAS repair are at the highest risk of experiencing major adverse cardiac events.3 Literature reports of surgical techniques and outcomes of concomitant coronary artery repair in these high-risk patients are diverse and inconsistently described. We have recently adopted a standardized surgical technique for management of this complex pathology by combining extended LMCA patch augmentation with a 3-patch aortic root reconstruction (Brom's technique). In this report, we describe our contemporary surgical technique of 3-patch aortic root reconstruction with extended LMCA patch augmentation for patients with congenital SVAS with ostial LMCA stenosis and bilateral outflow tract obstruction. Institutional review board approval was obtained for retrospective review of patient charts.

Pathology of valved venous homografts used as right ventricle-to-pulmonary artery conduits in congenital heart disease surgery.

OBJECTIVES: Although valved venous homografts (VVHs) are used for establishing right ventricle-to-pulmonary artery continuity in some complex heart defects, the tissue changes that occur in situ have not been described. We review the gross and microscopic changes observed in explanted VVH conduits and their effects on functionality. METHODS: In total, 20 explanted VVH conduits were evaluated for valve integrity, presence of thrombus, and stenosis. Hematoxylin and eosin- and trichrome-stained sections were reviewed for neointima formation, wall remodeling, inflammation, and calcification. Regurgitation and narrowing were assessed on pre-explant echocardiogram, and angiographic video clips were correlated with tissue findings. The source of the proliferating cells within the conduits was investigated by fluorescent in situ hybridization. RESULTS: Thirteen male and 7 female infants underwent VVH implantation either as part of a composite Sano shunt (65%) or to establish right ventricle-to-pulmonary artery continuity in biventricular hearts (35%). The median duration of conduits in situ was 140 days (range: 98-340 days). Conduits were predominantly explanted for staged conversion to bidirectional Glenn (60%) and conduit upsizing (20%). The valves remained intact and functional in 75% of cases. Occlusive thrombosis was absent in all. Wall thickening due to neointima formation and wall remodeling was uniformly present and appeared to be driven by smooth muscle actin-expressing cells, which by fluorescent in situ hybridization are predominantly of recipient origin. Minimal calcification and mild adventitial chronic inflammation were present. CONCLUSIONS: Vein wall thickening is a uniform finding and can cause stenosis. The valves remain functional in most, and vein walls undergo remodeling with only minimal inflammation and calcification.

Outcomes after mechanical aortic valve replacement in children and young adults with congenital heart disease.

OBJECTIVES: There are little recent data on the outcomes of mechanical aortic valve replacement (AVR) in children and young adults with congenital aortic valve disease. We sought to review the survival and associated thromboembolic or bleeding complications after mechanical AVR at a single center. METHODS: Data were retrospectively collected for 121 patients undergoing prosthetic AVR from 2000 to 2014. Kaplan-Meier estimates and Cox proportional hazards were employed. RESULTS: Median age at AVR was 16 years (interquartile range, 12-22.8 years). The valves implanted were the St Jude valve (St Jude Medical Inc, St Paul, Minn) in 79 patients (62%), the On-X valve (On-X Life Technologies Inc, Austin, Tex) in 45 patients (35%), and CarboMedics (Sorin SpA, Milan, Italy) in 3 patients (2.4%). Median valve size was 23 mm (range, 21-25 mm). There were 5 early deaths (3.9%). Median follow-up was 5 years (range, 1.6-9.2 years; 600 patient-years). There were 14 deaths during follow-up. Survival was 90.6% ± 2.8% at 1 year, 85.4% ± 3.7% at 5 years, and 81.5% ± 4.5% at 10 years. Freedom from aortic valve reoperation was 98% ± 1.4% at 1 and 5 years, 91.5% ± 3.9% at 7 years, and 78.4% ± 6.9% at 10 years and at latest follow-up. Univariable analysis identified younger age, lower weight, and use of a 16-mm CarboMedics valve as predictors of reoperation. Valve sizes of 16 or 17 mm have a significantly higher risk of reoperation compared with larger valves (log-rank test, P < .001). At multivariable analysis, only younger age was a significant independent predictor of reoperation (hazard ratio, 0.84; 95% confidence interval, 0.71-0.99; P = .038). All patients were treated with warfarin to a goal international normalized ratio of 2.0 to 3.0. Four patients (3.1%; 0.66% per patient-year) had thromboembolic complications, and 5 patients (3.9%; 0.83% per patient-year) had bleeding events during follow-up. CONCLUSIONS: Mechanical AVR in patients with congenital heart disease has excellent short- and midterm outcomes. Younger age was an independent predictor of reoperation.

Right ventricular outflow tract reintervention after primary tetralogy of Fallot repair in neonates and young infants.

OBJECTIVE: To assess the outcomes following primary tetralogy of Fallot (TOF) repair in neonates and young infants with pulmonary stenosis (PS) and pulmonary atresia and compare differences in reintervention on the right ventricular outflow tract (RVOT) among those undergoing valve sparing repair (VSR), transannular RVOT patch (TAP), and right ventricle-to-pulmonary artery (RV-PA) conduit surgeries. METHODS: Data were collected retrospectively in 101 patients who underwent TOF repair over a 10-year period between January 2005 and September 2015. The primary endpoint was reintervention on the RVOT, defined as a surgical procedure or cardiac catheterization-based RVOT reintervention. RESULTS: Forty-three patients had TOF/PS, of whom 24 (56%) underwent TAP and 19 (44%) underwent VSR. Fifty-eight patients had TOF/PA, 14 (24%) underwent TAP and 44 (76%) underwent RV-PA conduit repair. Overall patient mortality was 2.9% (3 of 101). Thirty-three patients underwent surgical reintervention, and 52 underwent catheterization-based reintervention. Patients with TOF/PA who underwent RV-PA conduit repair had a higher surgical reintervention rate than those who underwent TAP (45% vs 21%). Patients with TOF/PSs undergoing VSR with a lower median birth weight (2.5 kg vs 3.7 kg) required more surgical reintervention. CONCLUSIONS: Neonatal TOF repair can be performed with low mortality but frequent RVOT reinterventions. Surgical reintervention is earlier and the rate is higher among patients with TOF/PA undergoing RV-PA conduit repair compared with those undergoing TAP. Although there were no overall differences in RVOT reintervention rate between patients with TOF/PS undergoing VSR and those undergoing TAP, a lower birth weight in the patients undergoing VSR is associated with a higher surgical reintervention rate.

Morphologic and histologic findings in bioprosthetic valves explanted from the mitral position in children younger than 5 years of age.

BACKGROUND: Mitral valve replacement (MVR) in very young children is challenging. This study investigates the mechanisms for early bioprosthetic valve failure in very young patients through review of the macroscopic and microscopic findings in explanted bioprosthetic valves. METHODS: Patients who underwent MVR with a bioprosthetic valve at Boston Children's Hospital between January 2010 to April 2016 at <5 years of age were the subjects of this study. Valve failure was defined as prosthetic mitral valve explantation with mitral valve re-replacement. RESULTS: Bioprosthetic valves were used in 31 of 77 MVRs during the study period. Valve failure occurred in 10 patients (32%). Freedom from valve failure was 80% at 1 year and was associated with older age at implantation. On gross and microscopic evaluation, valve failure (predominantly stenosis) was found to be due to pannus deposition and intrinsic leaflet calcification. CONCLUSIONS: Successful long-term use of bioprosthetic valves in the mitral position in very young children continues to be a challenge. In addition to intrinsic calcification, excessive pannus deposition can lead to early bioprosthetic valve failure in this population. Early exuberant pannus growth appears due to thrombus deposition on the valves themselves and to the host's reaction to foreign material.

Augmentation of Bridging Leaflets in Repair of Atrioventricular Canal Defects.

Left atrioventricular (AV) valve regurgitation is the most common complication after a atrioventricular canal defect (AVCD) repair. Despite what appears to be a less complex repair, patients with partial and transitional AV canal have higher reoperation rates for left AV valve regurgitation and left ventricular outflow tract (LVOT) obstruction. Retraction of bridging leaflets with secondary attachments to the septal crest commonly produces increased tension and flattening of the medial left AV valve leaflet and LVOT obstruction after cleft closure. We describe a novel technique of detachment and patch augmentation of bridging leaflets to avoid these complications.

Photo-oxidized bovine pericardium in congenital cardiac surgery: single-centre experience.

Objectives: Dye-mediated photo-oxidation of pericardium is an alternative method to chemical treatment with glutaraldehyde for cross-linking collagen, providing biostability of the patch material while avoiding late calcification and cytotoxicity. There are few data available, on using photo-oxidation-treated pericardium, in congenital cardiac surgery. This study reports the outcomes using Photofix™ bovine pericardium in neonates, infants, children and young adults undergoing paediatric cardiac surgery. Methods: A total of 490 patches in 383 consecutive operations (364 patients) were used in the surgical repair of congenital heart defects at our institution from October 2008 to October 2011. Recorded variables included demographic data, age at operation, primary cardiac diagnosis, associated complications and number, type and location of patches placed and patch-related reintervention. Results: Median age at operation was 5.3 years, ranging from <1 month to 56 years. The overall survival rate at late follow-up was 92%, and no deaths were related to failure of the tissue substitute. Two patients (0.5%) underwent reintervention late due to patch material failure: one for residual shunt after Rastelli repair and one for aneurysmal dilatation of a right ventricular outflow tract patch. The patch material was explanted in 8 patients at a mean of 20 months (range, 1-72 months) following implantation. Histological examination revealed mild to moderate inflammation with variable calcification. Conclusions: Photo-oxidized bovine pericardium demonstrated excellent performance when used as a patch material in cardiovascular repair in children. Its handling characteristics and biocompatibility are consistent with a wide range of applications.

Outcome and performance of bioprosthetic pulmonary valve replacement in patients with congenital heart disease.

OBJECTIVES: The goal of this single-center series was to assess differences in reintervention by the type of valve used for surgical bioprosthetic pulmonary valve replacement and to identify independent predictors of reintervention. METHODS: Data were retrospectively collected for 611 patients undergoing pulmonary valve replacement from 1996 to 2014. Kaplan-Meier estimation and Cox proportional hazards regression methodologies were used. RESULTS: The median age of patients was 17.8 years (interquartile range, 11.9-27.3). The diagnosis was tetralogy of Fallot in 69% of patients. The median follow-up was 3.0 years (interquartile range, 1.1-5.3). Valve types included Sorin Mitroflow (Milan, Italy), 316 (50%; median age 16.5 years); Carpentier-Edwards (Irvine, Calif) Magna/MagnaEase, 223 (35%; median age, 19.3 years); and Carpentier-Edwards Perimount, 72 (11%; median age, 21.9 years). Reintervention occurred in 6.7% of patients (41/633) and was higher in children than adults (hazard ratio, 4.8). Age-adjusted 5-year reintervention rates were Sorin Mitroflow, 13.4%; Carpentier-Edwards Magna/MagnaEase, 2.1%; and Carpentier-Edwards Perimount, 0%. Reintervention was not associated with gender, valve insertion method, or concurrent procedures. The only independent risk factor for reintervention after controlling for age was valve type (P < .001). The Sorin Mitroflow valve had a shorter time to reintervention than the other 2 valve types (hazard ratios both >7, each P < .001). Differences by valve type did not depend on age (interaction P = .61). CONCLUSIONS: Bioprosthetic pulmonary valve replacement in patients with congenital heart disease has excellent short-term outcomes, but children have an approximately 5-fold greater risk of reintervention than adults. Independently of age, reintervention rates vary by valve type. These differences may be important in valve selection and follow-up.

Outcomes and Short-Term Follow-Up in Complex Ross Operations in Pediatric Patients Undergoing Damus-Kaye-Stansel Takedown.

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.

Histology of Pericardial Tissue Substitutes Used in Congenital Heart Surgery.

Pericardium is used as a tissue substitute during pediatric heart surgery. However, little is known about the histological characteristics of pericardial tissue substitutes. We searched our clinical and Pathology databases to identify cases in which glutaraldehyde-preserved autologous pericardium, PhotoFix bovine pericardium, or Peri-Guard Repair Patch glutaraldehyde-preserved bovine pericardium was used as patch material during cardiac surgery and in which explanted tissue substitute was available for histologic examination. Tissue sections were stained with hematoxylin and eosin, Masson trichrome, and Movat pentachrome, and were graded for inflammation, tissue substitute degeneration, neointima formation, and calcification. Nonparametric statistical methods were used to test differences between groups because of small sample size. The eight patients who received PhotoFix pericardium were older (median 10 months vs 10 days, P < 0.05) and the material was in situ longer (median 14 vs 2.5 months, P < 0.05) compared to the eight who received autologous pericardium. Only three patients received glutaraldehyde-preserved bovine pericardium precluding statistical comparison. Inflammation and tissue degeneration were greater in PhotoFix pericardium compared to autologous pericardium but were no more than moderate. Neointima formation and calcification did not differ significantly between the two groups. PhotoFix bovine pericardium is associated with more inflammation and material degeneration but calcification, and neointima formation are similar to autologous pericardium. Although the short-term outcomes are acceptable, calcification and degeneration seen in some cases suggest that long-term outcomes and performance at certain anatomic locations need further study.

Biventricular repair for common atrioventricular canal defect with parachute left atrioventricular valve.

OBJECTIVES: 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. METHODS: 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. RESULTS: 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). CONCLUSION: 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.

Review of Congenital Mitral Valve Stenosis: Analysis, Repair Techniques and Outcomes.

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.

Outcomes of surgery for young children with multivessel pulmonary vein stenosis.

OBJECTIVE: We pursued a multimodality approach to the treatment of patients with pulmonary vein stenosis, incorporating sutureless surgical repair, catheter interventions, and adjunctive chemotherapy. We report our outcomes after surgery. METHODS: Between January 2007 and August 2013, 49 patients with multivessel pulmonary vein stenosis underwent operations at our institution. We retrospectively reviewed data from a pulmonary vein stenosis registry and the medical records. RESULTS: At the time of the index operation, the median patient age was 6 months (range, 32 days-48 months) and weight was 4.9 kg (range, 2.1-13.4 kg). Fourteen patients (28%) died during the follow-up period (median follow-up was 0.5 years [range, 0.04-4.9 years]). There were 2 deaths (4%) within 30 days. Age at repair <6 months, weight at repair <3 kg, and a preoperative right ventricular systolic pressure < ¾ systemic were found to be associated with mortality. One patient required repeat operation for recurrent stenosis. Thirty-nine patients (80%) underwent postoperative catheterizations. The median number of catheterizations per patient was 2 (range, 0-14). Twenty-nine patients (59%) underwent catheterizations with pulmonary vein intervention. The median number of catheterizations with intervention per patient was 1 (range, 0-14). There were no identifiable associations with need for or number of catheterizations with intervention. Ten patients were listed for lung transplantation: 4 patients were de-listed, 3 patients died waiting, and 3 patients underwent transplant. CONCLUSIONS: Using a multimodality approach, we observed acceptable early survival after operation in patients with pulmonary vein stenosis, despite the need for catheter reinterventions. Lung transplantation remains a viable option.

Ring-reinforced Sano conduit at Norwood stage I reduces proximal conduit obstruction.

BACKGROUND: Reinterventions for proximal conduit obstruction or on the pulmonary arteries are frequent after the Sano-modified stage I Norwood palliation of hypoplastic left heart syndrome. We report our initial experience with a modified Sano technique using a ring-reinforced graft inserted transmurally through the right ventricle with a limited ventriculotomy. METHODS: All patients who underwent the Sano-modified stage I Norwood procedure using a modified "dunked" technique from September 2010 to September 2012 at our institution were reviewed. An historical control group consisted of patients undergoing the traditional Sano right ventricle-to-pulmonary artery conduit anastomosed to the epicardium. The primary outcome measures included death, reintervention on the Sano and pulmonary arteries, and ventricular function. RESULTS: The study included 29 patients. No patients required intervention on the Sano conduit, pulmonary arteries, or aortic arch before discharge after the stage I procedure. During a median follow-up of 20 months (range, 26 days to 3.3 years), survival was estimated at 96.6% ± 3.4% at 1 month and 86.2% ± 6.4% at the latest follow-up. One patient underwent heart transplantation. No interstage intervention was required on the proximal or distal Sano conduit. Intervention was required on the midportion of the conduit in 1 patient and on the pulmonary arteries in 3 patients. At the time of the bidirectional Glenn anastomosis, freedom from conduit and pulmonary artery intervention was estimated at 92.3% ± 7.4% and 90.1% ± 8.7%, respectively, and global right ventricular dysfunction was mild or less in 84% (16 of 19) of patients. CONCLUSIONS: The ring-reinforced right ventricle-to-pulmonary artery Sano conduit transmurally inserted into the right ventricle provides acceptable results, with a low incidence of interstage reinterventions in patients undergoing stage I palliation.

Mechanisms of tricuspid regurgitation in patients with hypoplastic left heart syndrome undergoing tricuspid valvuloplasty.

OBJECTIVES: Tricuspid regurgitation (TR) remains a risk factor for morbidity and mortality through staged palliation in patients with hypoplastic left heart syndrome (HLHS). Reports on the mechanisms associated with TR in patients with HLHS are limited. Thus, we sought to describe our experience with tricuspid valve (TV) repair in these patients, focusing on the mechanisms of TR and corresponding surgical techniques. METHODS: We performed a retrospective single-center review (January 2000 to December 2012) of patients with HLHS undergoing TV repair and completing Fontan circulation. We evaluated the pre- and postoperative echocardiograms, intraoperative findings, and surgical techniques used. RESULTS: A total of 53 TV repairs were performed in 35 patients with HLHS completing staged palliation. TV repairs were performed at stage II in 15, between stage II and III in 4, at stage III in 27, and after stage III in 7. The surgical techniques for valvuloplasty included annuloplasty (38%), anteroseptal (AS) commissuroplasty (66%), anterior papillary muscle repositioning (11%), multiple commissuroplasties (9%), septal-posterior commissuroplasty (9%), and fenestration closure (4%). The predominant jet of TR emanated along the AS commissure in 68% of the cases. All patients survived the procedure and were discharged. Preoperative echocardiography showed a dilated TV annulus on the lateral dimension, anteroposterior dimension, and area that was significantly reduced after TV repair (P < .0001). The preoperative mean TR, as assessed by lateral (P = .002) and anteroposterior (P = .005) vena contracta, was also significantly reduced after TV repair. TV repair did not significantly affect right ventricular systolic function immediately after surgery (P = .17) or at the most recent follow-up visit (P = .52). Patients with anterior leaflet prolapse were at increased risk of worse outcomes, including moderate or greater right ventricular dysfunction (P = .02). Patients requiring reoperation for TV repair were younger (6.3 vs 28.1 months, P < .0001) at the initial operation. One patient died of heart failure. Freedom from TV replacement and transplant-free survival were both 97% at the most recent follow-up point. CONCLUSIONS: TR in patients with HLHS commonly emanates from the AS commissure. The associated mechanisms are often annular dilatation and anterior leaflet prolapse. Preoperative anterior leaflet prolapse was associated with worse outcomes. Annuloplasty, closure of the AS commissure, and repositioning of the anterior papillary muscle are effective in addressing TR in the short- and mid-term in this challenging population.

Takedown of cavopulmonary shunt at biventricular repair.

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.

Accelerated degeneration of a bovine pericardial bioprosthetic aortic valve in children and young adults.

BACKGROUND: Experience with aortic valve replacement (AVR) with current-generation pericardial bioprostheses in young patients is limited. The death of a child with accelerated bioprosthetic aortic stenosis prompted enhanced surveillance of all such patients at our institution. METHODS AND RESULTS: We reviewed records of 27 patients who had undergone AVR (median follow-up, 13.7 months) with a bovine pericardial bioprosthesis at ≤30 years of age. In the Mitroflow LXA valve group (n=15), freedom from valve failure was 100% at 1 year, 53% (95% confidence interval, 12-82) at 2 years, and 18% (95% confidence interval, 1-53) at 3 years. No Magna/Magna Ease valves (n=12) failed by 3 years. Among valve failure patients, median age at AVR was 12 years (range, 10-21 years). Life-threatening prosthetic aortic stenosis was detected at a median of 6 months after prior echocardiograms showing mild or less gradients. Patients with Mitroflow LXA compared with Magna/Magna Ease valves were smaller (median body surface area, 1.42 versus 1.93 m(2); P=0.002) and younger (median age, 13.0 versus 20.9 years; P=0.02) at AVR. Pathology demonstrated diffuse intrinsic leaflet calcification, not associated with inflammation or infection, and virtually immobile leaflets in closed position. CONCLUSIONS: Young patients undergoing AVR with Mitroflow LXA pericardial valves are at high risk for rapid progression from mild or less to severe aortic stenosis over months, highlighting their need for heightened echocardiographic surveillance and suggesting that this aortic bioprosthesis should not be implanted in the young. Current data are insufficient to assess the safety of AVR with other pericardial bioprostheses in children and the youngest adults.