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Background: Despite pharmaceutical industry promise and enthusiasm, abuse-deterrent formulation (ADF) opioid use is relatively low. While some barriers to use have been addressed through state laws and policy, pharmacists' experiences with and opinions of ADF opioids are unclear.Objectives: The objective of this study was to evaluate pharmacists' perceptions of dispensing ADFs.Methods: This was a cross-sectional survey of community pharmacists licensed and practicing in Kentucky conducted in late 2019. The survey asked about perception, experience dispensing, and insurance coverage of 5 ADF opioids available at the time.Results: Most respondents (421/629, 67.9%) were familiar or very familiar with ADFs, and 63.1% agreed that all opioids should meet U.S. Food and Drug Administration standards for abuse deterrence. Aside from OxyContin, most ADF opioid formations were not stocked (range: 46.7%-73.6%). Third-party payer claims were occasionally or almost always rejected for most ADFs (range: 56.3%-75.4%). Contrary to intended mechanism of deterrence, ADFs were rated as the least effective strategy to reduce opioid misuse/abuse, with over half (51.2%) of respondents believing ADFs were not effective or somewhat effective. ADFs were rated as effective or very effective at reducing opioid abuse by swallowing intact by 37.4% of respondents.Conclusion: Pharmacists are familiar with ADFs but do not dispense them frequently. Pharmacists appear skeptical about the effectiveness of ADFs but support policies that could increase ADF uptake. [ABSTRACT FROM AUTHOR]

To examine the association of race/ethnicity and geography with service-connected disability (SCD) for traumatic brain injury (TBI) and how such determinants affect monthly total SCD compensation by TBI SCD status. Retrospective observational cohort study. Veterans Health Administration (VHA) /Veterans Benefits Administration (VBA). Veterans from LIMBIC-CENC Phenotype study with ≥1 SCD rating from the VBA. N/A. SCD designation for TBI based on VA Schedule for Rating Disabilities (VASRD) diagnostic codes (8045 [residuals of TBI] and 9304 [neurocognitive disorder due to TBI]) and monthly VBA compensation per veteran during 2022. Results 1,225,679 Veterans without and 93,911 with TBI SCD were included. Veterans living in highly rural areas (OR 1.44, 95% CI 0.06,0.05) and on Tribal Lands (OR 1.28, 95% CI 0.25,0.31) had the highest odds of receiving a TBI SCD award in unadjusted models. In adjusted models for all factors except TBI severity, Native Americans (AOR 1.12, 95% CI 1.06,1.18) and Asian American Pacific Islanders (AOR 1.19, 95% CI 1.16, 1.22), had the highest odds of a TBI SCD award relative to non-Hispanic Whites. In the unadjusted model examining monthly overall SCD compensation, a TBI award was associated with an additional $362 (95% CI 353,370) in compensation. Veterans on Tribal Lands (OR $-80 95% CI -157,-4) had significantly lower compensation relative to urban areas. Native Americans (OR $169 95% CI 153, 185) had the lowest additional SCD compensation relative to Non-Hispanic Whites, and other Non-Hispanic White race/ethnicities. Native American Veterans and those living on Tribal Lands were disproportionately affected by observed disparities in TBI SCD award status and monthly compensation. We wish to acknowledge the inspiration and mentoring of Ralph G. DePalma, MD, FACS, In Memoria. Work supported by U.S. Army Medical Research Acquisition Activity LIMBIC Award/W81XWH-18-PH/TBIRP-LIMBIC. Dr. Pugh supported by VA HSR&D Research Career Scientist Award (IK6HX003762). The views expressed do not represent the official policy or position of the Department of Defense, or Department of Veterans Affairs. [ABSTRACT FROM AUTHOR]

Patients receiving a right ventricle to pulmonary artery conduit (PC) in infancy will require successive procedures or replacements, each with variable longevity. We sought to identify factors associated with time-related risk of a subsequent surgical replacement (PC3) or transcatheter pulmonary valve insertion (TPVI) after a second surgically placed PC (PC2). From 2002 to 2016, 630 patients from 29 Congenital Heart Surgeons' Society member institutions survived to discharge after initial valved PC insertion (PC1) at age ≤ 2 years. Of those, 355 underwent surgical replacement (PC2) of that initial conduit. Competing risk methodology and multiphase parametric hazard analyses were used to identify factors associated with time-related risk of PC3 or TPVI. Of 355 PC2 patients (median follow-up, 5.3 years), 65 underwent PC3 and 41 TPVI. Factors at PC2 associated with increased time-related risk of PC3 were smaller PC2 Z score (hazard ratio [HR] 1.6, P <.001), concomitant aortic valve intervention (HR 7.6, P =.009), aortic allograft (HR 2.2, P =.008), younger age (HR 1.4, P <.001), and larger Z score of PC1 (HR 1.2, P =.04). Factors at PC2 associated with increased time-related risk of TPVI were aortic allograft (HR: 3.3, P =.006), porcine unstented conduit (HR 4.7, P <.001), and older age (HR 2.3, P =.01). Aortic allograft as PC2 was associated with increased time-related risk of both PC3 and TPVI. Surgeons may reduce risk of these subsequent procedures by not selecting an aortic homograft at PC2, and by oversizing the conduit when anatomically feasible. [ABSTRACT FROM AUTHOR]

The stentless porcine aortic root prosthesis (SPAR) has been described as a suitable valve for right ventricular outflow tract reconstruction (RVOTR). Indiana University Methodist Hospital in Indianapolis, Indiana began using this valve for RVOTR in 1998. This study reports medium-term to late- term outcomes of the valve in the pulmonary position. A retrospective chart review was conducted of patients older than 18 years of age who underwent RVOTR with a SPAR between April 2000 and October 2019. Primary outcomes included survival and freedom from any valvular reintervention. Secondary outcomes included endocarditis and conduit dysfunction detected by routine echocardiography or cardiac magnetic resonance imaging. A total of 135 patients underwent RVOTR with a SPAR at a median age of 32.4 years (range, 18 to 71 years). Of these patients, 129 had previous surgery. Indications included pulmonary insufficiency (90.4%), stenosis (34.8%), endocarditis (7.4%), and carcinoid (4.4%). Median follow-up was 2.97 years (interquartile range, 0.6 to 8.0 years). Overall survival was 93.3%, with 3 perioperative death and 6 late deaths. Endocarditis developed in 4 patients (2.9%), 2 of whom required reoperation. Progressive conduit degradation was evident at 10 years, with 22.2% and 7.7% having moderate stenosis and insufficiency, respectively. Eight (5.9%) reinterventions included 2 surgical replacements, 3 percutaneous replacements, and 3 balloon valvuloplasties at means of 8.5, 7.4, and 2.2 years, respectively. Overall freedom from reintervention at 1, 5, and 10 years was 99.1%, 94.7% and 90.7%, respectively. In this large, single-institution experience with a long follow-up period, use of the SPAR demonstrated excellent midterm to long-term durability, low rates of endocarditis, and high freedom from reintervention. [ABSTRACT FROM AUTHOR]

Total shoulder arthroplasty is an accepted treatment for glenohumeral osteoarthritis. The Arthrex Eclipse shoulder prosthesis is a stemless, canal-sparing humeral prosthesis with bone ingrowth capacity on the trunnion, as well as through the fenestrated hollow screw, that provides both diaphyseal and metaphyseal load sharing and fixation. Between 2013 and 2018, 16 sites in the United States enrolled 327 patients (Eclipse in 237 and Arthrex Univers II in 90). All patients had glenohumeral arthritis refractory to nonsurgical care. Strict exclusion criteria were applied to avoid confounding factors such as severe patient comorbidities, arthritis not consistent with osteoarthritis, and medical or prior surgical treatments that may have affected outcomes. Patients were randomized to the Eclipse or Univers II group via block randomization. In total, 149 Eclipse and 76 Univers II patients reached 2-year follow-up (139 Eclipse patients [93.3%] and 68 Univers II patients [89.5%] had complete data). The success rate using the Composite Clinical Success score was 95% in the Eclipse group vs. 89.7% in the Univers II group. No patient exhibited radiographic evidence of substantial humeral radiolucency, humeral migration, or subsidence at any point. Reoperations were performed in 7 patients (3.2%) in the Eclipse group and 3 (3.8%) in the Univers II group. The Arthrex Eclipse shoulder prosthesis is a safe and effective humeral implant for patients with glenohumeral arthritis at 2-year follow-up, with no differences in outcomes compared with the Univers II shoulder prosthesis. [ABSTRACT FROM AUTHOR]

Multiple conduits for right ventricular outflow tract reconstruction exist, although the ideal conduit that maximizes outcomes remains controversial. We evaluated long-term outcomes and compared conduits for right ventricular outflow tract reconstruction in children with truncus arteriosus. Records of patients who underwent truncus arteriosus repair at our institution between 1981 and 2018 were retrospectively reviewed. Primary outcomes included survival and freedom from catheter reintervention or reoperation. Secondary analyses evaluated the effect of comorbidity, operation era, conduit type, and conduit size. One hundred patients met inclusion criteria. Median follow-up time was 15.6 years (interquartile range, 5.3-22.2). Actuarial survival at 30 days, 5 years, 10 years, and 15 years was 85%, 72%, 72%, and 68%, respectively. Early mortality was associated with concomitant interrupted aortic arch (hazard ratio, 5.4; 95% confidence interval, 1.7-17.4; P =.005). Median time to surgical reoperation was 4.6 years (interquartile range, 2.9-6.8; n = 58). Right ventricle to pulmonary artery continuity was established with an aortic homograft (n = 14), pulmonary homograft (n = 41), or bovine jugular vein conduit (n = 36) in most cases. Multivariate analysis revealed longer freedom from reoperation with the bovine jugular vein conduit compared with the aortic homograft (hazard ratio, 3.1; 95% confidence interval, 1.3-7.7; P =.02) with no difference compared with the pulmonary homograft. Larger conduit size was associated with longer freedom from reoperation (hazard ratio, 0.7; 95% confidence interval, 0.6-0.9; P <.001). The bovine jugular vein conduit is a favorable conduit for right ventricular outflow tract reconstruction in patients with truncus arteriosus. Concomitant interrupted aortic arch is a risk factor for early mortality. [ABSTRACT FROM AUTHOR]

The increased bioeconomy targets for the biomass share of renewable energy production across Europe should be met using land unsuitable for food production. Miscanthus breeding programs targeted the production of plants with a diverse range of traits allowing a wider utilization of land resources for biofuel production without competing with arable crops. These traits include increasing tolerances to drought, chilling, and to metal(loid)s excess. Two novel Miscanthus hybrids, GNT41 and GNT34, were compared against Miscanthus x giganteus (M x g) on metal-contaminated arable land in Poland. This study aimed at evaluating their yield, biomass quality and quantifying seasonal differences in photosynthetic and transpiration parameters. A secondary objective was to identify key physiological mechanisms underlying differences in metal accumulation between the investigated plants. The new hybrids produced a similar yield to M x g (13–15 t ha−1 yr−1), had shorter shoots, higher Leaf Area Index and stem number. Based on gas exchange measurements, GNT34 exhibited isohydric (water-conserving) behavior. The stomatal response to light of the new hybrids was at least twice as fast as that of M x g , a trait that is often associated with increased seasonal water use efficiency. This contributed to the almost 40% reduction in shoot Pb and Cd concentrations for the new hybrids as compared to M x g. This suggested that promoting stomatal regulation in conjunction with improved water conservation may be a target for improving plants for wider use on metals contaminated land. High yielding Miscanthus hybrids with combined drought resilience and fast stomatal response produced less contaminated biomass. Image 1 • Miscanthus spp. plantation was established on metal-contaminated arable land. • All investigated plants produce similar yield, ca. 13–15 t ha−1 yr−1. • New, seed-based hybrids, showed more water-conserving behavior than M. x giganteus. • Plants exhibiting more isohydric behavior had lower shoot Pb and Cd concentrations. [ABSTRACT FROM AUTHOR]

Deferment cutting is a two-aged regeneration method in which the majority of the stand is harvested and a dispersed component of overstory trees—approximately 15–20% of the basal area – is retained for at least one-half rotation and up one full rotation for reasons other than regeneration. Careful consideration of residual trees, in both characteristics and harvesting, is necessary to improve the chances that individual trees will survive until the next planned harvest. A long-term experimental deferment cutting study was established on the Fernow Experimental Forest and Monongahela National Forest in West Virginia, USA in the early 1980s. Repeated tree measurements spanning the approximately 35-year study period permitted a survival analysis of the overstory cohort. The effects on survival were tested for three endogenous factors (dbh, species, crown class) and one exogenous factor (logging damage) using a Cox Proportional Hazards model, with stand effects (multiple trees measured within a stand) accounted for by using as frailty model. Survival rates were high, with 92% of trees surviving (910 of 985 trees). The mortality rate was low at six percent, (60 of 985 trees), and the number of trees cut or destroyed during logging was two percent (15 of 985 trees). Trees injured in the deferment harvest did not show increased risk of mortality, p = 0.91. Crown class also did not contribute to increased mortality, p = 0.35, as the majority of overstory trees retained were in the dominant and codominant classes. Six species, Liriodendron tulipifera L., Prunus serotina Ehrh., Quercus alba L., Quercus montana Willd., Quercus rubra L., Quercus velutina Lam., had sufficient sample sizes to test for species differences. However, species was not significant for survival, p = 0.10. The only significant factor in survival was dbh, p < 0.01, with larger trees having increased probability of survival. These results demonstrate that overstory trees in a deferment cutting can be acceptably maintained midway through the next rotation and likely until the next regeneration harvest. [ABSTRACT FROM AUTHOR]

A long-term forest management case study on the Fernow Experimental Forest in West Virginia referred to as the Cutting Practice Level study is evaluated after 60 years. Treatments include a commercial clearcut (one time application), a 39 cm diameter-limit (applied 4 times), uneven-aged management using two variations of single-tree selection (applied 7 and 8 times, respectively), and an unmanaged reference area. We examine productivity, species composition and diversity, structure, tree quality, and revenues generated related to each treatment since establishment. The diameter-limit treatment resulted in greatest average periodic annual increment (PAI) of sawtimber volume of 3.1 m 3 ha −1 yr −1 while the unmanaged reference area resulted in the least of 2.2 m 3 ha −1 yr −1 (based on the difference in standing volume from 1956 to 2008). All types of partial harvesting resulted in greater sawtimber productivity than either the commercial clearcut or the reference area. Post-harvest tree quality, as measured by proportion of grade 1 butt logs, has improved from 1988 to 2008 for all but the diameter-limit treatment, which is similar to conditions in 1968. In 2008, the proportion of grade 1 trees in the residual stand ranged from a high of 0.22 for single-tree selection to 0.15 for diameter-limit harvesting. Species composition is becoming less diverse and more dominated by shade-tolerant species in all treatment groups but the change has been the greatest in the two single-tree selection treatments. Initially, size-class distributions were somewhat unimodal and reflective of even-aged stands with shade tolerant species persisting in the understory. In 2008, the single-tree selection treatments were both characterized by a reverse-J size class distribution and it appears this structure can be maintained due to recruitment of shade-tolerant species in the smaller size classes with concomitant reductions in species diversity. The net present value for each treatment in 2008, the time of the last management intervention, ranged from $20,000 ha −1 for reference area to almost $34,000 ha −1 for the single-tree selection treatment that included management of pole-sized trees based on all revenue and the value of standing timber using an internal rate of return of 4%. [ABSTRACT FROM AUTHOR]

Background Structural deterioration of allografts over time is believed to be at least partly related to an immune response mounted against human leukocyte antigen specific to the transplanted tissue. SynerGraft (SG) processing (CryoLife, Kennesaw, GA) is a technology that decellularizes an allograft leaving only connective tissue, therefore, reducing immunogenicity and potentially increasing durability of the implant. Methods We performed a retrospective review of 163 SG patients and 124 standard allograft controls from 3 medical centers. Patient demographics were tabulated, and conduit stenosis and insufficiency were measured by echocardiography. Results There were 28 deaths (15 of 163 [9%] SG patients vs 13 of 124 [11%] standard patients; p = 0.72), but no deaths were attributed to structural failure of the conduit. The actuarial survival for SG vs standard cohorts was not different at 5 and 10 years. Among the 274 hospital survivors, 17% SG vs 42% standard had evidence for significant conduit dysfunction at the most recent follow-up or before conduit replacement. Freedom from conduit dysfunction was significantly worse at 10 years in the standard group (58%) than in the SG group (83%, p < 0.001). Conclusions This study represents a multiinstitutional retrospective comparison of SG and standard cryopreserved allografts used in right ventricular outflow tract reconstruction in a broad range of patient ages. Our results demonstrate that at an intermediate-term to long-term follow-up, conduit dysfunction and pulmonary insufficiency and stenosis are higher among patients receiving standard allografts. We postulate that the improved durability of SG is related to decreased immunogenicity of the SG technology. [ABSTRACT FROM AUTHOR]

Objectives This study sought to evaluate this transcatheter aortic valve (TAV) bioprosthesis in patients who are poorly suitable for surgical aortic valve (AV) replacement. Background A novel self-expandable TAV bioprosthesis was designed to provide a low-profile delivery system, conformable annular sealing, and the ability to resheath and reposition during deployment. Methods The Evolut R U.S. study included 241 patients with severe aortic stenosis who were deemed to be at least high risk for surgery treated at 23 clinical sites in the United States. Clinical outcomes at 30 days were evaluated using Valve Academic Research Consortium-2 criteria. An independent echocardiography laboratory was used to evaluate hemodynamic outcomes. Results Patients were elderly (83.3 ± 7.2 years of age) and had high surgical risk (Society of Thoracic Surgeons predicted risk of mortality of 7.4 ± 3.4%). The majority of patients (89.5%) were treated by iliofemoral access. Resheathing or recapturing was performed in 22.6% of patients; more than 1 valve was required in 3 patients (1.3%). The 30-day outcomes included all-cause mortality (2.5%), disabling stroke (3.3%), major vascular complications (7.5%), life-threatening or disabling bleeding (7.1%), and new permanent pacemaker (16.4%). AV hemodynamics were markedly improved at 30 days: the mean AV gradient was reduced from 48.2 ± 13.0 mm Hg to 7.8 ± 3.1 mm Hg (p < 0.001) and AV area increased from 0.6 ± 0.2 cm 2 to 1.9 ± 0.5 cm 2 (p < 0.001). Moderate residual paravalvular leak was identified in 5.3% of patients. Conclusions We conclude that this novel self-expanding TAV bioprosthesis is safe and effective for the treatment of patients with severe aortic stenosis who are suboptimal for surgery. (Medtronic CoreValve Evolut R U.S. Clinical Study; NCT02207569 ). [ABSTRACT FROM AUTHOR]

Partial anomalous pulmonary venous return is a rare congenital heart defect characterized by 1 or more but not all of the pulmonary veins draining somewhere other than the left atrium, thereby creating a left-to-right shunt. Over time, right-sided volume overload may develop with its subsequent complications. We present a case of isolated partial anomalous pulmonary venous return in an older patient who underwent a Warden procedure at age 77 years, with rapid improvement in right ventricular size and function. [ABSTRACT FROM AUTHOR]

Patients with tricuspid atresia and ventricular septal defect have in the past occasionally undergone a Fontan with "Björk" modification to create a connection between the right atrium and the right ventricular outflow tract. Although rarely performed now, patients with this physiology often face severe complications requiring reintervention. We hypothesize that surgical conversion to a 2-ventricle or 1.5-ventricle circulation can improve hemodynamics, clinical status, and thus increase time to transplant. We present 2 successful cases to illustrate this idea. [ABSTRACT FROM AUTHOR]

In patients with tetralogy of Fallot (TOF), pulmonary atresia (PA), and other congenital right ventricular outflow tract (RVOT) malformations, polytetrafluoroethylene (PTFE) monocusp outflow tract patches (MOTP) relieve obstruction and provide pulmonary valve competence. The purpose of this study was to determine whether our PTFE-MOTP was an acceptable short- and mid-term remedy for patients with TOF or PA as assessed by freedom from severe pulmonary regurgitation and freedom from reoperation. From 1994-2014, 171 patients (mean age 1.5 ± 1.5 years; median 1.1 years) with TOF or PA underwent initial right ventricular outflow tract (RVOT) reconstruction with a PTFE-MOTP. Patients were studied intraoperatively and serially postoperatively using echocardiography and cardiac magnetic resonance imaging (CMR) to determine pulmonary valve dysfunction defined as a peak gradient >40mmHg or valve regurgitation>moderate. The mean follow-up duration was 10.9 ± 5.8 years (range: 1 month-20 years). There were 5 late deaths and 1 early death. There was a significant difference between the preoperative and postoperative peak RVOT gradients (74.0 vs 25.2mmHg). Of the 171 patients, 25 were lost to follow-up, and 42 have required replacement of their monocusp valves 10.1 ± 5.0 years (range: 5 months-19 years) after original monocusp insertion. At 10-year follow-up, severe pulmonary regurgitation was seen in less than 25% of patients, and severe pulmonary stenosis was seen in less than 10% of patients. Since 2007, CMR was used in 44 patients to characterize cardiac function in patients under consideration for PTFE-MOTP replacement. The average right ventricular-to-left ventricular (RV/LV) ratio on CMR was 1.7 ± 0.5 in these patients. CMR also showed that RV ejection fraction (52 ± 9%) and left ventricular ejection fraction (58 ± 7%) were both preserved in most patients. The PTFE-MOTP is an excellent short-term and mid-term option for initial RVOT reconstruction, particularly in children with TOF with nonsalvageable pulmonary valve or PA-ventricular septal defect. [ABSTRACT FROM AUTHOR]

Background The Ross aortic valve replacement (AVR) has been the AVR of choice for children at our center since 1993. Absence or inadequate quality of the pulmonary valve or, less commonly, family or surgeon preference caused us to select an alternative AVR prosthesis for some children. This review compares the outcomes of 42 children who received a non-Ross AVR with 115 children undergoing Ross root replacement at our institution during the most recent 22 years. Methods A retrospective chart review of the 42 pediatric non-Ross AVRs was compared with 115 Ross AVRs. The mean age at AVR was 11.0 ± 6.5 years (range, 1 month to 18 years) for the non-Ross and 11.4 ± 5.5 years (range, 6 weeks to 18 years) for the Ross groups. Follow-up was 7.8 ± 6.2 years for the Ross group and 8.7 ± 6.5 years for the non-Ross group. The Ross AVR technique was modified in 2000; these modifications were applied to 72 patients (63%) of the total Ross AVR group. Results All-cause early and late mortality of the non-Ross AVR group was 17% compared with 4% for the Ross cohort ( p = 0.017). The actuarial survival rate at 20 years for the non-Ross AVR group was 81% compared with 94% for the Ross group ( p = 0.018). Reintervention rate was 29% (12/38) in the non-Ross group and 28% (32/115) in the entire Ross group (60% [26/43] before 2000, and 8% [6/72] after 2000). Thromboemboli, bleeding, and endocarditis occurred in 2.4%, 1.4%, and 10% in the non-Ross group compared with 0.9%, 2.6%, and 1.7% in the Ross AVR group ( p = 0.46, 0.01, and 0.02), respectively. Patients in the non-Ross group had a significantly higher recurrent or persistent aortic valve gradient (>20 mm Hg) than did patients in the Ross group at most recent follow-up ( p < 0.05). Conclusions Lower mortality, valve-related complications, and better hemodynamics were seen after the Ross AVR than with other types of AVR prostheses. The Ross AVR remains the treatment of choice for children who have an adequate pulmonary valve. Reoperation for Ross root dilatation, regurgitation, or both (only 6% since our modifications in 2000) has markedly reduced the main drawbacks of this AVR technique. The Ross AVR affords the growing child the advantages of a growing, autologous, viable valve substitute and eliminates the lifelong disadvantages of mechanical and xenograft valves. [ABSTRACT FROM AUTHOR]

Background Transcatheter aortic valve replacement (TAVR) is currently offered to patients who are high-risk candidates for conventional surgical aortic valve replacement. For the past 37 years, off-pump aortic valve bypass (AVB) has been used in elderly patients at our center for this similarly high-risk group. Although TAVR and AVB were offered to similar patients at our center, comparisons of clinical outcomes and hospital economics for each strategy were not reported. Methods We reviewed the clinical and financial records of 53 consecutive AVB procedures performed since 2008 with the records of 51 consecutive TAVR procedures performed since 2012. Data included demographics, hemodynamics, The Society of Thoracic Surgeons (STS) risk score, extent of coronary disease, and ventricular function. Follow-up was 100% in both groups. Hospital financial information for both cohorts was obtained. Mean risk score for the TAVR group was 10.1% versus 17.6% for AVB group ( p < 0.001). Results Kaplan-Meier hospital rates of 3- and 6-month survival and of 1-year survival were 88%, 86%, 81%, and 61% and 89%, 83%, 83%, and 70% for the TAVR and AVB groups, respectively ( p = 0.781). Two patients who had undergone TAVR had a procedure-related stroke. The one stroke in an AVB recipient was late and not procedure related. At discharge, mild and moderate perivalvular and central aortic insufficiency were present in 31% and 16% of TAVR recipients, respectively; no AVB valve leaked. Transvalvular gradients were reduced to less than 10 mm Hg in both groups. The average hospital length of stay for the AVB-treated patients was 13 days, and it was 9 days for the TAVR-treated patients. Median hospital charges were $253,000 for TAVR and $158,000 for AVB. Mean payment to the hospital was $65,000 (TAVR) versus $64,000 (AVB), and the mean positive contribution margin (profit) to the hospital was $14,000 for TAVR versus $29,000 for AVB. Conclusions TAVR and AVB relieve aortic stenosis and have similar and acceptable procedural mortality rates. AVB-treated patients had 1.75 times the STS risk score when compared with the TAVR cohort. Hospital charges for TAVR were nearly twofold those of AVB. Hospital reimbursement was similar, but AVB had two to four times the profit margin of TAVR. Longer follow-up for the TAVR cohort will determine whether survival is comparable to that after AVB at 3 and 5 years. [ABSTRACT FROM AUTHOR]

Objectives This study sought to compare the health status outcomes for patients treated with either self-expanding transcatheter aortic valve replacement (TAVR) or surgical aortic valve replacement (AVR). Background In patients at increased surgical risk, TAVR with a self-expanding bioprosthesis is associated with improved 1-year survival compared with AVR. However, elderly patients may be just as concerned with quality-of-life improvement as with prolonged survival as a goal of treatment. Methods Between 2011 and 2012, 795 patients with severe aortic stenosis at increased surgical risk were randomized to TAVR or AVR in the CoreValve US Pivotal Trial. Health status was assessed at baseline, 1 month, 6 months, and 1 year using the Kansas City Cardiomyopathy Questionnaire, Medical Outcomes Study Short-Form 12 Questionnaire, and EuroQOL 5-dimension questionnaire; growth curve models were used to examine changes over time. Results Over the 1-year follow-up period, disease-specific and generic health status improved substantially for both treatment groups. At 1 month, there was a significant interaction between the benefit of TAVR over AVR and access site. Among surviving patients eligible for iliofemoral (IF) access, there was a clinically relevant early benefit with TAVR across all disease-specific and generic health status measures. Among the non-IF cohort, however, most health status measures were similar for TAVR and AVR, although there was a trend toward early benefit with TAVR on the Short-Form 12 Questionnaire’s physical health scale. There were no consistent differences in health status between TAVR and AVR at the later time points. Conclusions Health status improved substantially in surviving patients with increased surgical risk who were treated with either self-expanding TAVR or AVR. TAVR via the IF route was associated with better early health status compared with AVR, but there was no early health status benefit with non-IF TAVR compared with AVR. (Safety and Efficacy Study of the Medtronic CoreValve® System in the Treatment of Symptomatic Severe Aortic Stenosis in High Risk and Very High Risk Subjects Who Need Aortic Valve Replacement; NCT01240902 ) [ABSTRACT FROM AUTHOR]

Background: The purpose of this study is to compare morbidity and mortality between fenestrated (F, 61 patients) and nonfenestrated (NF, 54 patients) extracardiac Fontan patients during two eras from July 1995 to December 2010: era 1(1995 to 2004) and era 2 (2005 to 2010). Methods: Variables evaluated included morphology, hemodynamics, chest tube volume and duration, intensive care and hospital stay, oxygen saturation, neurologic events, rhythm, and readmissions for chylous effusions. Follow-up in 114 hospital survivors was longer in the nonfenestrated cohort (F, 5.0 ± 3.3 years; NF, 7.1 ± 4.6 years; p < 0.005). Results: Cohorts were similar in body size, morphology, and hemodynamics. Fenestration in hypoplastic left heart syndrome was appreciatively higher in era 2. Bypass time (F, 69 ± 27 minutes; NF, 57 ± 21 minutes) and conduit size (F, 18.8 mm; NF, 19.1 mm) were similar. There was 1 early nonfenestrated Fontan death (1 of 54; 2%) and 4 late deaths (F, 2 of 61, 5%; NF, 2 of 53, 4%; p = 0.86). Room air saturation was higher in NF patients (F, 89%; NF, 94%; p < 0.05). Total chest tube volume was similar, but fenestration was associated with greater chest tube drainage among hypoplastic left heart patients (HLHS, 5,582 ± 3,286 mL; non-HLHS, 3,405 ± 2,533 mL; p = 0.06; odds ratio; 2.0). Readmission to treat chylous effusions, loss of sinus rhythm, actuarial freedom from death, all neurologic events, pacemaker insertion, and Fontan takedown were similar in both cohorts. Conclusions: Fenestration was associated with lower discharge oxygen saturations, but late outcomes in fenestrated and nonfenestrated patients are equivalent. [Copyright &y& Elsevier]

Background: Treatment of congenital heart disease may include placement of a right ventricle to pulmonary artery conduit that requires future surgical replacement. We sought to identify surgeon-modifiable factors associated with durability (defined as freedom from surgical replacement or explantation) of the initial conduit in children less than 2 years of age at initial insertion. Methods: Since 2002, 429 infants were discharged from 24 Congenital Heart Surgeons' Society member institutions after initial conduit insertion. Parametric hazard analysis identified factors associated with conduit durability while adjusting for patient characteristics, the institution where the conduit was inserted, and time-dependent interval procedures performed after conduit insertion but before replacement/explantation. Results: In all, 138 conduit replacements (32%) and 3 explantations (1%) were performed. Conduit durability at a median follow-up of 6.0 years (range, 0.1 to 11.7) was 63%. After adjusting for interval procedures and institution, placement of a conduit with smaller z-score was associated with earlier replacement/explantation (p = 0.002). Moreover, conduit durability was substantially reduced with aortic allografts (p = 0.002) and pulmonary allografts (p = 0.03) compared with bovine jugular venous valved conduits (JVVC). The JVVC were 12 mm to 22 mm in diameter at insertion (compared with 6 mm to 20 mm for allografts); therefore, a parametric propensity-adjusted analysis of patients with aortic or pulmonary allografts versus JVVC with diameter of 12 mm or greater was performed, which verified the superior durability of JVVC. Conclusions: Pulmonary conduit type and z-score are associated with late conduit durability independent of the effects of institution and subsequent interval procedures. Surgeons can improve long-term conduit durability by judiciously oversizing, and by selecting a JVVC. [Copyright &y& Elsevier]

Abstract: Since activities of daily living do not exist in isolation this prospective study examined biomechanical function during level walking and sit-to-stand activities over two months of brace use by subjects with varying grades of knee osteoarthritis. Kinematic and kinetic data were collected using infrared cameras and force platforms; clinical scores compared perceived and measured functional effects. There was no significant change in pain for any grades of OA but activity levels were significantly higher at one month in the moderate OA grades and at two months for the low OA grades. Mechanical constraints and changes in movement strategies were consistent across tasks and OA grade. This study provides valuable data for further studies aimed at refining the use and optimization of valgus bracing efficacy. [Copyright &y& Elsevier]

Objective: Interrupted aortic arch is a rare congenital anomaly affecting 1.5% of infants with congenital heart disease. Multiple surgical modalities exist to address this defect. We evaluate the long-term outcome of interrupted aortic arch with the left carotid artery turndown technique from a single institution. Methods: Patients with interrupted aortic arch who underwent the carotid turndown procedure were identified between September 1982 and March 2010. Medical and surgical records were reviewed. Mortality data were obtained from state death records. Results: Forty-seven patients met inclusion criteria. Median follow-up was 6.2 years (5 days to 23.2 years). Twenty-one patients (45%) had genetic syndromes. There were 4 operative deaths (8.5%) and 9 late deaths (19%). One-year and 5-year survivals were 80.2% and 72.6%, respectively. Seventeen patients (36.2%) required reoperation or other interventions on the aortic arch. Conclusions: Left carotid artery turndown offers a favorable surgical outcome. It compares with end-to-end repair, while providing a tension-free anastomosis and avoiding neonatal circulatory arrest and cardiopulmonary bypass. Disadvantages include a 2-stage repair and a significant reintervention rate, particularly when compared with the aortic arch advancement technique. Nevertheless, the reduced exposure to circulatory arrest and bypass and avoidance of left bronchial obstruction are important considerations that may offset these limitations. [ABSTRACT FROM AUTHOR]

Background: For children with congenital aortic stenosis (AS) who are selected for biventricular repair, valvuloplasty can be achieved by surgical aortic valvuloplasty (SAV) or by transcatheter balloon aortic dilation (BAD). A retrospective study was undertaken to compare the effectiveness of BAD versus SAV, evaluating the long-term survival, incidence of aortic valve restenosis or aortic insufficiency (AI) or both, and freedom from reoperation for repeated valve repair or replacement. Neonates less than 2 months of age were excluded from this comparison. Methods: We reviewed the outcomes of children undergoing repair by SAV (n = 89) and BAD (n = 69) at our institution during a recent 20-year period. Clinical and echocardiographic follow-up were analyzed. The patient groups were compared with regard to the persistence or recurrence of postoperative aortic gradients and valve insufficiency and valve-related reintervention, including aortic valve replacement (AVR). Results: There was no significant difference between the groups with respect to mean age, body surface area, valve anatomy, sex, and preoperative gradients. Our data demonstrate that gradient reduction, AI, and the need for reintervention were worse for BAD. Aortic gradients at last follow-up were similar in both cohorts, but return of a significant gradient occurred sooner for patients who had BAD. Aortic gradient at discharge was significantly better for the patients who underwent SAV. Kaplan-Meier analysis showed that at 10 years, comparison of SAV and BAD was as follows: freedom from reintervention, 72% versus 53% (p = 0.02) and freedom from AVR, 80% versus 75% (p = 0.32). Conclusions: BAD yields less gradient reduction, more postprocedural AI, and a shorter interval between initial and subsequent reintervention than does SAV. Our results demonstrate that SAV is safe and effective and that residual gradients and degree of AI are low. After SAV, the need for AVR can usually be delayed until the child is significantly older. The long-term functional stability after SAV is excellent. BAD in comparison is associated with an increased frequency and severity of AI and the need for earlier reintervention and valve replacement. SAV should be offered to all patients beyond the newborn period because it gives superior and longer lasting palliation. [Copyright &y& Elsevier]

Objective: The study objective was to determine the effects of implementing a blood conservation algorithm on blood product use and outcomes in a community cardiac surgery program. Methods: A blood management strategy including lower hemoglobin transfusion threshold and algorithm-driven decisions was adopted. Intraoperatively, point-of-care testing was used to avoid inappropriate component transfusion. A low prime perfusion circuit was adopted. Blood was withdrawn from patients before initiating bypass when possible. Patients undergoing coronary and valve procedures were included. Outlier patients receiving more than 10 units packed red blood cells were excluded. Data were collected for 6 months as a baseline group (group I). A 3-month period of program implementation was allotted. Data were subsequently collected for 6 months and comprised the study patients (group II). Prospective data were collected on demographics, blood use, and outcomes. Results: Group I comprised 481 patients, and group II comprised 551 patients. Group II received fewer units of packed red blood cells, fresh-frozen plasma, and cryoprecipitate than group I. There was no difference in platelets transfused. Total blood product use was reduced by 40% in group II (P < .001). The overall 30-day mortality was 1.3%. There were no differences in mortality, reoperation for bleeding, or other postoperative outcomes between the groups. Conclusions: Implementation of a comprehensive blood conservation algorithm can be rapidly introduced, leading to reductions in blood and component use with no detrimental effect on early outcomes. Point-of-care testing can direct component transfusion in coagulopathic cases, with most coagulopathic patients requiring platelets. Further research will determine the effects of reduced transfusions on long-term outcomes. [ABSTRACT FROM AUTHOR]

Abstract: The term ductal carcinoma in situ (DCIS) of the breast encapsulates a biologically, morphologically, clinically and genetically heterogeneous group of lesions. These have a wide spectrum of histological features but are characterized by a non-invasive proliferation of malignant epithelial cells confined to the parenchymal structures of the breast and thus contained within basement membrane-bound structures. Analysis at the molecular and genetic level has improved our understanding of these entities as non-obligate precursors of invasive breast cancer. It is clear that the linear progression model from normal epithelium through hyperplasia to atypical hyperplasia to DCIS to invasive breast cancer is inaccurate. Here we examine current methods for classifying DCIS and some recent molecular advances, including the impact of genetic profiling and immunohistochemistry, upon our understanding of current pathological definitions of DCIS. [Copyright &y& Elsevier]

Background: This report reviews our 40-year experience with pediatric mitral valve replacement (MVR) with respect to mortality, valve-related morbidity, and reoperation risk factors. Methods: From 1970 to 2010, 97 patients have undergone a total of 136 MVRs. Median age was 8 years (2 weeks to 18 years), 41 patients (42%) were less than 5 years, and 16 were infants (17%). Etiology was congenital in 65 patients (67%), rheumatic in 27 (28%), and endocarditis in 5 (5%). Regurgitation was the predominant lesion in 67 patients (69%), stenosis in 23 (24%), and mixed in 7 (7%) patients. Mechanical valves (ball, n = 11; or bileaflet disc, n = 66) and xenografts (porcine, n = 14; bovine, n = 2) were used in 93 initial MVR patients. Since 2002, 5 children have undergone Ross MVR with a pulmonary autograft in 3 and an aortic homograft in 2. Results: Hospital mortality was 6% (6 of 97). There were 23 late deaths and 5 patients have required cardiac transplantation. Thirty-five year actuarial survival was 71%. Age less than 2 years, MVR prior to 1980, atrioventricular septal defect, univentricular heart, and additional left side obstructions were significant predictors of death. Mean follow-up was 12.8 ± 10.1 years (range, 2 months to 38 years). Seventeen patients with mechanical valves experienced systemic emboli in 9 (10%), valve thrombosis in 5 (6%), and bleeding requiring transfusion in 3 (3%) patients. Thirty-two patients required reoperations (35%) from 3 months to 14 years (mean, 6.5 ± 4.4 years) after initial MVR. Actuarial freedom from reoperation at 35 years was 63%. Variables associated with mitral re-replacement were younger age, small weight, valve diameter less than 23 mm, MVR prior to 1980, and type of implanted valves (xenograft, single-leaflet disk, ball-caged, or human valves). Conclusions: Pediatric MVR can be performed with low initial mortality but should be reserved for medical and reconstruction failure because reoperation, valve-related complications, and late mortality are high. Bileaflet prostheses larger than 23 mm have the lowest reoperation risk. Ross MVR may offer select patients a durable tissue valve without lifelong anticoagulation and its associated complications. [Copyright &y& Elsevier]

Purpose: The technical difficulty of performing the left ventricular apical anastomosis has limited the adoption of aortic valve bypass surgery for the treatment of aortic stenosis. We report the successful use of an automated coring and apical connector device to perform aortic valve bypass surgery. Description: A 74-year-old man, with a history of prior coronary bypass surgery with patent grafts and a porcelain ascending aorta, presented with symptomatic critical aortic stenosis. Through a left anterolateral thoracotomy, a valved conduit was anastomosed to the descending thoracic aorta. The automated coring and apical connector insertion device was used to core a plug of apical myocardium and simultaneously insert an 18-mm apical connector into the left ventricular apex. Evaluation: There were no procedural complications, cardiopulmonary bypass was not used, and estimated blood loss was minimal. The patient was discharged on postoperative day 5, and at 3-month follow-up demonstrated significant clinical and hemodynamic improvement. Conclusions: The automated coring and apical connector insertion device facilitated the safe and effective performance of aortic valve bypass surgery. [ABSTRACT FROM AUTHOR]

Background: The Ross aortic valve replacement (AVR) offers excellent hemodynamic and clinical outcomes in most patients undergoing aortic root replacement. Because of ongoing debate regarding the durability of this procedure, long-term follow-up in a large adult Ross experience might be of interest. Methods: Between 1994 and 2010, 230 adult patients underwent modified Ross root procedures. Mean age was 42.4 ± 12.1 years (range, 20 to 68 years), 62% were male. Fifty-six patients (24%) had undergone one or more prior aortic valve interventions. Concomitant procedures were performed in 126 patients (55%), including 90 ascending aortic procedures. Presenting pathology was aortic insufficiency in 114 (50%) and aortic stenosis in 116 (50%). Results: Hospital mortality or within 30 days was 2 of 230 patients (0.9%). During follow-up (mean, 7.8 years), 12 more patients died. At 15 years, survival was 94%, and freedom from autograft and allograft reoperation was 91% and 98%, respectively. Reoperation was required for 23 patients. Eighteen patients required reoperation on the autograft root; 11 of 18 (61%) had preoperative aortic insufficiency as their predominate aortic valve lesion. Freedom from autograft reoperation was not significantly different for patients with preoperative aortic insufficiency (87%) compared with patients who had aortic stenosis (94%; p = 0.15). On multivariable analysis, no risk factors were significant for reoperation except for surgery before 2000 (p < 0.0001) and previous AVR (p = 0.05). Conclusions: Preoperative aortic regurgitation was not a significant risk factor for late autograft reoperation in adults. The Ross AVR provides excellent hemodynamics and survival for adults willing to accept a small risk of reoperation as opposed to a life-long risk of thromboemboli and anticoagulation therapy as exist with mechanical aortic prostheses. Reoperation risk for Ross AVR has decreased with Ross annulus sinotubular junction reinforcement replacement of a dilated ascending aorta and postoperative hypertension management. [Copyright &y& Elsevier]

Background: We retrospectively reviewed the 12-year (1999 to 2010) clinical and echocardiographic performance of 232 bovine jugular vein conduits for extracardiac right ventricular outflow tract reconstruction in non-Ross patients. Methods: The bovine jugular vein conduit cohorts, group 1 (12 to 14 mm), group 2 (16 to 18 mm), and group 3 (20 to 22 mm), had mean follow-up of 48 ± 30 months. Graft dysfunction is defined as right ventricular outflow tract obstruction with peak echo Doppler gradient greater than 40 mm Hg or grade 3/4 valve regurgitation. Graft failure is the need for conduit replacement or transcatheter or surgical reintervention. Results: Early mortality (4 of 232; 2%) and late mortality (8 of 228; 3.5%) were not conduit related. Twenty-four conduits (10%) were explanted. Mean implant Z score was significantly lower for group 1 (1.7 ± 0.08 versus group 2, 2.7 ± 0.6, or group 3, 2.5 ± 1.5; p = 0.001). Ten-year actuarial survival (group 1, 84% versus 2, 100%, and 3, 99%; p = 0.001) and freedom from conduit dysfunction (group 1, 64%; group 2, 92%; and group 3, 90%) and failure (group 1, 75%; 2, 82%; and 3, 91%; p = 0.002) were significantly better for groups 2 and 3. Conclusions: Bovine jugular vein is an excellent immediate substitute for right ventricular outflow tract reconstruction, with early durability superior to that of pulmonary homografts reported at similar follow-up. Conduits larger than 14 mm have improved performance. Longer follow-up will define the structural integrity and efficacy of this prosthesis. [ABSTRACT FROM AUTHOR]

Background: Since the advent of cisplatin-based chemotherapy, nonseminomatous germ cell tumors (NSGCT) have been considered one of the most curable solid neoplasms and a model for multimodality cancer therapy. We undertook an institutional review of testicular NSGCT patients who underwent operations to remove lung or mediastinal metastases after chemotherapy in the cisplatin era to determine outcomes. Methods: From 1980 to 2006, 431 patients underwent 640 postchemotherapy surgical procedures to remove lung (n = 159, 36.8%), mediastinal (n = 136, 31.6%), or both lung and mediastinal (n = 136, 31.6%) metastases within 2 years of chemotherapy. Multiple variables potentially predictive of survival were analyzed. Results: The overall median survival was 23.4 years, with 295 (68%) patients alive and well after an average follow-up of 5.6 years. There was no survival difference in patients who underwent removal of lung or mediastinal metastases. Pathologic categories of resected residual disease were necrosis (21.5%), teratoma (52.7%), persistent NSGCT (15.0%), and degenerative non-germ cell cancer (10.1%). Multivariable analysis identified older age at time of diagnosis (p = 0.001), non-germ cell cancer in testes specimen (p = 0.004), and pathology of residual disease (p < 0.001) as significantly predictive of survival. Conclusions: Patients who undergo resection of residual lung or mediastinal disease for metastatic testicular NSGCT as a planned approach after cisplatin-based chemotherapy have overall excellent long-term survival. Survival is equivalent comparing hematogenous and lymphatic routes of metastases but depends on the pathology of the resected disease. These results justify an aggressive surgical approach, particularly to remove residual teratoma in the lung or mediastinum after chemotherapy, including multiple surgical procedures if necessary. [ABSTRACT FROM AUTHOR]

Background: In the Ross aortic valve replacement (AVR), a pulmonary allograft normally replaces the autotransplanted pulmonary valve. Despite the Ross advantages vs other AVR procedures, there has been a small but unpredictable risk of early structural allograft valve deterioration, usually manifested by shrinkage and right ventricular outflow tract obstruction. This study analyzed our results of the Ross AVR using a new CryoValve SynerGraft (CryoLife Inc, Kennesaw, GA) decellularized pulmonary allograft (SGDPA) and compared it with the standard cryopreserved allograft (SCA) used during the same period. Methods: Between 2000 and 2009, 29 patients received a SGDPA and 34 received the SCA during Ross AVR. Patients were a mean age at implant of 28.6 ± 16.0 years (range, 4 months to 58 years). Retrospective data included reported adverse events, and the most recent hemodynamic data were collected. Results: No early or late deaths or significant morbid events occurred during the mean follow-up of 4.9 ± 2.7 years (range, 2 months to 9 years). No patient required conduit reoperation. The median peak gradient at discharge was 12 mm Hg and was not significant at last follow-up. No deterioration in conduit valve function occurred in the SGDPA group. Mild conduit regurgitation developed in several SCA patients, and one patient had moderate regurgitation. No patient reached our definition of conduit dysfunction (peak gradient: 40 mm Hg or >2+ regurgitation). Conclusions: The SGDPA conduit is an alternative to the SCA for the Ross AVR. The early clinical and hemodynamic results are encouraging but were not significantly different from the SCA. SynerGraft technology may provide a more durable option for patients who need right ventricular outflow tract reconstruction. Further long-term follow-up is needed to see if this decellularization process improves long-term allograft durability. [ABSTRACT FROM AUTHOR]

Background: The optimal surgical treatment of patients with transposition of the great arteries, ventricular septal defect, and pulmonary stenosis is controversial. Although the Rastelli operation has been standard surgical management of this lesion, aortic root translocation with right ventricular outflow tract (RVOT) reconstruction (Nikaidoh) and the pulmonary artery translocation (Lecompte) or REV (réparation a l''étage ventriculaire) are surgical alternatives more recently introduced to treat this complex lesion. This report reviews our 20-year experience with the Rastelli procedure and attempts to compare our outcomes with those recently published using the Nikaidoh and REV procedures. Methods: Between 1988 and 2008, 40 patients (median age, 4 years; range, 9 months to 17 years) underwent Rastelli operation at our institutions. The RVOT was obstructed in 32 and atretic in 8. Follow-up was available for all but one patient (mean follow-up, 8.6 ± 5.6 years). The RVOT was reconstructed with homograft (n = 25), bovine jugular vein (n = 8), nonvalved Dacron tube (n = 5), or a porcine valved conduit (n = 2). Two patients required a pacemaker. Results: There were no early, but three late deaths and one heart transplantation 12 years postoperative the Rastelli operation. Kaplan-Meier survival was 93% at 5, 10, and 20 years. Univariate risk factors for death or transplantation included surgery before 1998 (p = 0.03) and concomitant noncardiac anomalies (p = 0.001). Sixteen patients (40%) had reoperation for right ventricular-pulmonary artery conduit stenosis (mean, 7.8 ± 3.8 years) without mortality. Freedom from conduit replacement was 86%, 74%, 63%, and 59% at 5, 10, 15, and 20 years, respectively. Multivariate analysis revealed that the risk factors of conduit replacement were younger age at operation (p = 0.001) and surgery before 1998 (p < 0.001). Two patients (5%) required reoperation for left ventricular outflow tract obstruction. At follow-up, there were no sudden unexplained deaths, and New York Heart Association functional class is I or II. Conclusions: The Rastelli procedure is a low-risk operation with regard to early and late mortality and reoperation for left ventricular outflow tract obstruction. Conduit change operations will be required in most patients regardless of the technique of repair, but currently can be performed with low morbidity and mortality. These midterm outcomes after the Rastelli operation should serve as a basis for comparison with surgical alternatives more recently introduced for transposition of the great arteries and ventricular septal defect with RVOT obstruction. [Copyright &y& Elsevier]

Objective: In a univentricular Fontan circulation, modest augmentation of existing cavopulmonary pressure head (2–5 mm Hg) would reduce systemic venous pressure, increase ventricular filling, and thus substantially improve circulatory status. An ideal means of providing mechanical cavopulmonary support does not exist. We hypothesized that a viscous impeller pump, based on the von Kármán viscous pump principle, is optimal for this role. Methods: A 3-dimensional computational model of the total cavopulmonary connection was created. The impeller was represented as a smooth 2-sided conical actuator disk with rotation in the vena caval axis. Flow was modeled under 3 conditions: (1) passive flow with no disc; (2) passive flow with a nonrotating disk, and (3) induced flow with disc rotation (0–5K rpm). Flow patterns and hydraulic performance were examined for each case. Hydraulic performance for a vaned impeller was assessed by measuring pressure increase and induced flow over 0 to 7K rpm in a laboratory mock loop. Results: A nonrotating actuator disc stabilized cavopulmonary flow, reducing power loss by 88%. Disk rotation (from baseline dynamic flow of 4.4 L/min) resulted in a pressure increase of 0.03 mm Hg. A further increase in pressure of 5 to 20 mm Hg and 0 to 5 L/min flow was obtained with a vaned impeller at 0 to 7K rpm in a laboratory mock loop. Conclusions: A single viscous impeller pump stabilizes and augments cavopulmonary flow in 4 directions, in the desired pressure range, without venous pathway obstruction. A viscous impeller pump applies to the existing staged protocol as a temporary bridge-to-recovery or -transplant in established univentricular Fontan circulations and may enable compressed palliation of single ventricle without the need for intermediary surgical staging or use of a systemic-to-pulmonary arterial shunt. [ABSTRACT FROM AUTHOR]

Background: Resection and reconstruction of the superior vena cava (SVC) is occasionally required in the surgical treatment of intrathoracic neoplasms or symptomatic occlusion secondary to benign causes. We reviewed our institutional experience with SVC reconstruction using externally stented-polytetrafluoroethylene vascular prostheses. Methods: From 1991 to 2009, medical records of 38 patients who underwent SVC resection and reconstruction with externally stented-polytetrafluoroethylene vascular prostheses were reviewed. Indications for surgery were malignancy in 34 (89%) patients (germ cell, 13; thymoma, 10; lung cancer, 9; sarcoma, 2) and benign symptomatic occlusion in 4 (11%) patients. Results: Eighteen patients (47%) underwent right innominate vein to SVC interposition graft reconstruction, which became the favored approach during the study interval when resection of the innominate confluence was necessary. Eight patients (21%) had left innominate vein to SVC interposition grafts, earlier in the series or when the right innominate vein was unavailable. Nine patients (24%) received graft interposition of the proximal to distal SVC. The remaining 3 patients had a Y reconstruction. There were 2 perioperative mortalities. Follow-up averaged 15 months (range, 1 to 113 months), including 11 (29%) patients who died of disease. All patients demonstrated minimal to no brachiocephalic swelling at last follow-up. Twenty (53%) patients underwent imaging after an average of 24 months (range, 1 to 113 months) with only two grafts demonstrating complete occlusion. Conclusions: Although several SVC reconstructive techniques have been described, externally stented-polytetrafluoroethylene vascular prostheses are readily available for off-the-shelf use. In our experience, patency rates are high, and patients who do demonstrate graft thrombosis have minimal to no symptoms. [Copyright &y& Elsevier]

Objective: We evaluated focused training in coronary artery anastomosis with a porcine heart model and portable task station. Methods: At “Boot Camp,” 33 first-year cardiothoracic surgical residents participated in 4-hour coronary anastomosis sessions (6–7 attending surgeons per group of 8–9 residents). At beginning, midpoint, and session end, anastomosis components were assessed on a 3-point rating scale (1 good, 2 average, 3 below average). Performances were video recorded and reviewed by 3 surgeons in a blinded fashion. Participants completed questionnaires at session end, with follow-up surveys at 6 months. Results: Ten to 18 end-to-side anastomoses with porcine model and task station were performed. Initial assessments ranged from 2.11 ± 0.58 (forceps use) to 2.44 ± 0.48 (needle angles). Midpoint scores ranged from 1.76 ± 0.63 (forceps use) to 1.91 ± 0.49 (needle angles). Session end scores ranged from 1.29 ± 0.45 (needle holder use) to 1.58 ± 0.50 (needle transfer and suture management and tension; P < .001). Video recordings confirmed improved performance (interrater reliability >0.5). All respondents agreed that task station and porcine model were good methods of training. At 6 months, respondents noted that the anastomosis session provided a basis for training; however, only slightly more than half continued to practice outside the operating room. Conclusions: Four-hour focused training with porcine model and task station resulted in improved ability to perform anastomoses. Boot Camp may be useful in preparing residents for coronary anastomosis in the clinical setting, but emphasis on simulation development and deliberate practice is necessary. [Copyright &y& Elsevier]

Background: We evaluated left heart bypass (LHB) for spinal cord protection during aortic coarctation repair in patients with mild (primary, postsurgical, or intervention) and complex coarctation. Methods: Between 1990 and 2008, 19 patients (mean age, 21 years; weight, 70 ± 16 kg) using LHB were compared with 27 patients (mean age, 16 years; weight, 65 ± 8 kg) undergoing coarctation repair without LHB (non-LHB). Follow-up was similar (LHB, 5 ± 4 vs non-LHB 4 ± 3 years; p = 0.81). Results: Cohorts were similar in age and body surface area. No non-LHB patient lost somatosensory evoked potential or had a femoral artery pressure below 45 mm Hg with test clamping. LHB more often allowed graft interposition (18 of 19 [95%] vs non-LHB, 7 of 27 [26%]; p < 0.003) and a longer clamp time (LHB 44 ± 16 vs non-LHB 31 ± 12 minutes p < 0.003) without spinal cord ischemia. Two non-LHB patients had temporary spinal cord paresis. No early or late deaths occurred. Reintervention (LHB, 2 of 19 [11%] vs non-LHB, 2 of 27 [7%]; p = 0.82) and antihypertensive requirements were similar (LHB, 9 of 19 [40%] vs non-LHB, 8 of 27 [30%]; p = 0.35). The late peak transcoarctation gradient was 8 ± 6 mm Hg in the LBH cohort vs 18 ± 11 mm Hg in non-LBH patients (p= 0.001). Conclusions: Although the adequacy of spinal cord collateral assessment in coarctation repair is imperfect, no spinal cord ischemia occurred with coarctation repair and LHB. We recommend LHB in patients with mild or complex coarctation. [Copyright &y& Elsevier]

Objective: This study compared clinical outcomes of patients receiving CryoValve SG decellularized pulmonary valves with those of patients receiving conventionally processed CryoValve pulmonary valves. Methods: All consecutive patients undergoing Ross procedures and right ventricular outflow tract reconstructions with SG valves at 7 institutions (February 2000–November 2005) were assessed retrospectively (193 Ross procedures, 149 right ventricular outflow tract reconstructions). Patient, procedural, and outcome data were compared with those from 1246 conventional implants (665 Ross procedures, 581 right ventricular outflow tract reconstructions). Hemodynamic function was assessed at latest follow-up. Results: Follow-up was complete for 99% in SG group and 94% in conventional group, with mean follow-ups of 4.0 years (range, 0–6.7 years) for SG and 3.7 years (range, 0–6.7 years) for conventional. Five-year cumulative survivals and freedoms from adverse events were comparable between SG and conventional valves. Among patients undergoing Ross procedures, peak gradient at last follow-up was lower with SG valves (P < .01); no difference was observed in the right ventricular outflow tract reconstruction population. Pulmonary insufficiency was significantly reduced with SG valves in patients undergoing both Ross procedures (P < .01) and right ventricular outflow tract reconstructions (P < .01). Valve type was not a significant predictor of valve-related failure in propensity-adjusted analysis of either procedure. Conclusions: CryoValve SG decellularized pulmonary valves have acceptable clinical outcomes and favorably compare with conventionally processed valves. Improved hemodynamic function observed with SG valves could signify improved long-term outcomes and may be due to the decreased antigenicity of these valves. [Copyright &y& Elsevier]

Background: Construction of a total cavopulmonary anastomosis using an intra-atrial lateral tunnel Fontan (LTF) is known to yield good early and midterm results. Given the current controversy regarding indications for a total extracardiac conduit Fontan, we reviewed the long-term outcomes after a LTF operation and compared them with recently published series using one or both techniques. Methods: Between 1992 and 2008, 220 of 280 patients (median age, 2.5 years; range, 1 to 45) with a wide range of underlying diagnoses underwent a fenestrated or nonfenestrated LTF operation at our institution. Current follow-up information was available for 215 patients (98%; mean follow-up, 6.7 ± 3.9 years). Risk factor analysis included patient-related and procedure-related variables, with death, failure (takedown or transplantation), and bradyarrhythmia or tachyarrhythmia as outcome variables. Results: There was 1 early death, 10 late deaths, 3 takedown operations, and 1 heart transplantation. Kaplan-Meier estimated survival was 96% at 5 years and 95% at 10 and 15 years, and freedom from failure was 94% at 5 years and 93% at 10 years. Freedom from new supraventricular tachyarrhythmia was 98% at 5 years and 95% at 10 years; freedom from new bradyarrhythmia was 97% at 5 years and 96% at 10 years. Six patients have protein-losing enteropathy, and 2 of 6 have had Fontan takedown. Multivariable risk factors for development of supraventricular tachyarrhythmia included atrioventricular valve abnormalities (p = 0.02), and preoperative bradyarrhythmia (p = 0.01). Risk factors for bradyarrhythmia included the need for early postoperative pacing (p = 0.001). None of the patient-related variables significantly influenced survival. Conclusions: The LTF operation results in excellent midterm outcome even when used in patients with complex anatomy. The incidence of postoperative atrial tachyarrhythmia is low and depends largely on the underlying cardiac morphology and incidence of preoperative arrhythmia. The good midterm outcome after a LTF operation should serve as a basis for comparison with other surgical alternatives to complete the Fontan circulation. [Copyright &y& Elsevier]

Objective: Although several techniques have been described for the treatment of tracheal stenosis, including slide tracheoplasty, tracheal autograft, rib grafting, and use of a pericardial patch, the optimal repair remains controversial because of a lack of long-term follow-up data. The purpose of this study is to examine the long-term results of anterior pericardial tracheoplasty. Methods: To assess the long-term outcomes of patients who underwent repair of tracheal stenosis with anterior pericardial tracheoplasty, we reviewed the case histories of 26 consecutive patients (1984–present). All but 5 had long-segment tracheal stenosis with more than 10 complete tracheal rings. Twenty-one had significant cardiac disease, and 10 had their cardiac lesions repaired at the time of their tracheoplasty. The median age was 6 months (range, 2 days–25 years). All patients underwent anterior pericardial tracheoplasty through a median sternotomy during normothermic cardiopulmonary bypass. We have previously described our tracheoplasty technique. An average of 14 tracheal rings (range, 5–22) was divided anteriorly, and a patch of fresh autologous pericardium was used to enlarge the trachea to 1.5 times the predicted diameter for age and weight. Results: There were 3 hospital deaths (at 1, 2, and 7 months, respectively) and 2 late deaths (at 2 and 13 years postoperatively, respectively). No deaths were related to airway obstruction. Two survivors required tracheostomy postoperatively, one after formation of granulation tissue and stenosis and the other after failure to wean from mechanical ventilation. All survivors remain asymptomatic, with minimal to no evidence of airway obstruction. Median follow-up is 11 years (range, 3 months–22 years). Conclusion: Anterior pericardial tracheoplasty for tracheal stenosis provides excellent results in the majority of patients at long-term follow-up. [Copyright &y& Elsevier]

Background: Persistence or recurrence of stenosis is a complication of coarctation repair and is associated with major long-term morbidity. The rate of recurrence varies significantly, depending on the age of the patient, technique at initial repair, and the arch anatomy. We reviewed our experience with surgical repair of recurrent coarctation of the aorta and compared it with our institutional experience with balloon aortoplasty. Methods: We retrospectively reviewed our experience with 1,012 patients undergoing initial repair of coarctation between 1960 and 2008. During that time, 103 patients (10%) required reintervention. Median age at reintervention was 6.5 years (range, 2 weeks to 44 years) and median weight was 12 kg (range, 1.9 to 94 kg). Fifty-nine patients with recoarctation had surgical repair, and 44 patients were treated with balloon aortoplasty with or without stent placement. Results: Ninety-five percent of patients have been followed up (median time, 14.2 years; range, 2 months to 42 years). There were 5 late deaths. Actuarial survival was 98% at 15 and 40 years in patients with surgical reintervention, and it was 91% (p = 0.001) at 15 years in patients with balloon aortoplasty reintervention. A second redo coarctation of the aorta reintervention was performed in 12 patients: 8 patients after percutaneous intervention (nonsurgical) and 4 patients after surgical recoarctation repair. The median interval between first and second reintervention was 3.5 years (range, 1 month to 14 years). One patient who had two dilations underwent a third and fourth reintervention: patch enlargement and pseudoaneurysm resection. Freedom from reintervention in the surgical group was 96% at 15 years and 94% at 40 years, which was compared with actuarial freedom from reintervention for patients with percutaneous intervention (balloon/stent) at 15 years (82%; p < 0.001). Conclusions: Our study demonstrates that surgical repair of recurrent coarctation of the aorta can be performed safely and with excellent results. The recurrence after surgical reintervention is low, and most patients to date have not required further intervention. Balloon aortoplasty as an alternative method of managing recoarctation is efficient and less invasive than surgery; however, well-described complications may occur. Recurrence rates with angioplasty are significantly higher than with surgery. [Copyright &y& Elsevier]

Abstract: Perivascular epithelioid cell tumors (PEComas) are rare, soft tissue tumors characterized by epithelioid cells with clear or eosinophilic cytoplasm and a perivascular disbursement. We compiled the treatment and follow-up results from an extensive collection of reported gynecologic PEComa cases and statistically analyzed their respective therapy modalities and corresponding patient outcomes. In the group of patients with PEComa who received surgical management alone, there was a tendency for them to exhibit a lower disease recurrence rate. Conversely, patients with PEComa who initially received surgery and chemotherapy or radiation therapy were associated with a higher disease recurrence rate (P =.024). Metastatic involvement was related to higher patient mortality rates (P =.0001), although this finding was unrelated to treatment type. Surgical management alone may suffice for nonaggressive lesions, but chemotherapy and radiotherapy appear necessary for patients who present with high-risk histologic condition or metastatic disease. Because PEComas exhibit varying biologic behavior and an ill-defined presentation, the treatment for these lesions necessitates further evaluation. [Copyright &y& Elsevier]

Background: We reviewed our institutional midterm experience to assess autograft and homograft hemodynamics and reoperative frequency after Ross aortic valve replacement. Methods: Between June 1993 and January 2009, 212 consecutive patients (mean age, 24.8 ± 15.5 years; range, 1 month to 67 years) underwent Ross aortic valve replacement; 49% were younger than 19 years old. One hundred forty-two additional procedures were required in 101 of the 212 patients (48%) at the time of the Ross aortic valve replacement. One hundred ninety-three patients had isolated aortic valve disease, and 19 pediatric patients had more complex, multilevel left ventricular outflow tract obstruction. Results: There were 2 early (1%) and 2 late deaths (1%), with a mean follow-up of 7.9 ± 4.2 years (range, 1 month to 15 years). Actuarial survival at 15 years was 98%. To date 28 patients (13%) have required reoperation. At 15 years, freedom from autograft sinus or ascending aortic dilatation was 79%, autograft dysfunction, 91%, autograft reoperation, 89%, and autograft replacement, 96%. Freedom from pulmonary allograft replacement was 96% at 15 years. Conclusions: The Ross aortic valve replacement can be performed in children and adults with good midterm results. The late complications of autograft regurgitation, sinus or ascending aortic dilatation, can usually be corrected with a valve-sparing root replacement. These complications can often be prevented by (1) aggressive treatment of postoperative systemic hypertension, (2) replacement of a dilated ascending aorta at the initial Ross procedure, or (3) external fixation of the autograft annulus or sinotubular junction. The potential of late autograft insufficiency, ascending aortic and sinus dilatation, or homograft stenosis and insufficiency warrants annual follow-up. [Copyright &y& Elsevier]

Abstract: Objective: Anomalies of the aortic arch (vascular rings) are uncommon anomalies in which preferred strategies for diagnosis and treatment may vary among institutions. In this study, we report a description of our approach and review of our 38-year experience in patients surgically treated for vascular rings. Methods: A retrospective review was conducted of all patients with/without symptomatic tracheoesophageal compression secondary to anomalies of the aortic arch and great vessels diagnosed from 1970 to 2008. A total of 183 patients underwent surgical repair. Median age at the time of the operation was 5 months (range, 3 days to 30 years). Patients were classified into 5 major subtypes based on their surgical anatomy as follows: right aortic arch-left ligamentum (n = 77), double aortic arch (n = 67), aberrant (retroesophageal) right subclavian artery (n = 30), pulmonary sling (n = 8), and innominate artery compression (n = 1). Six patients (3%) had an associated Kommerell diverticulum. In patients with a double aortic arch, 82% had a dominant right arch and 18% had a dominant left arch. Preoperatively, 80 patients (44%) had stridor, and 86 patients (47%) had recurrent upper respiratory tract infection. Results: Associated cardiac diagnosis were present in 54 (30%) of 183 of all patients with vascular rings. Left thoracotomy was a common operative approach in all patients except pulmonary artery sling patients where a median sternotomy was the preferred approach. There were 3 early and 5 late deaths (all patients had complex cardiac anomalies) with a median follow-up of 6 years. Overall survival was 96% at 35 years. Postoperative complication occurred in 3 patients (2%) as follows: tracheostomy because of severe distal tracheal compression (n = 2) and left true vocal cord paralysis (n = 1). None of the patients showed any evidence of recurrent vascular ring anomalies at last follow-up. Of the children, 75% (135/180) were free from compressive symptoms within 1 year of the operation. Conclusions: Vascular anomalies with/without tracheoesophageal compression present symptomatically in a variety of ways, and noninvasive methods are used to identify the specific lesion and associated cardiac defects. Surgical repair is associated with low or no mortality in patients with uncomplicated complex of vascular anomalies. [Copyright &y& Elsevier]

Background: Previous studies have reported an 11% to 75% incidence of postoperative cognitive decline among cardiac surgery patients. The INVOS Cerebral Oximeter (Somanetics Corp, Troy, MI) is a Food and Drug Administration approved device that measures regional cerebral oxygen (rSo 2) saturation. The purpose of this study is to examine whether decreased rSo 2 predicts cognitive decline and prolonged hospital stay after coronary artery bypass grafting (CABG). Methods: The rSo 2 was monitored intraoperatively in a cohort of primary CABG patients. Patients were prospectively randomized to a blinded control group or an unblinded intervention group. Cognitive function was assessed preoperatively, postoperatively, and at 3 months using a battery of standardized neurocognitive tests. Cognitive decline was defined as a decrease of one standard deviation or more in performance on at least one neurocognitive measure. The rSo 2 desaturation score was calculated by multiplying rSo 2 below 50% by time (seconds). Multivariate logistic regression models were used to assess cognitive decline and hospital stay. The change in cognitive performance was also assessed using a multivariate linear regression model. Results: Patients with rSo 2 desaturation score greater than 3,000%-second had a significantly higher risk of early postoperative cognitive decline [p = 0.024]. Patients with rSo 2 desaturation score greater than 3,000%-second also had a near threefold increased risk of prolonged hospital stay (>6 days) [p = 0.007]. Conclusions: Intraoperative cerebral oxygen desaturation is significantly associated with an increased risk of cognitive decline and prolonged hospital stay after CABG. [Copyright &y& Elsevier]

Transverse aortic arch hypoplasia involving some or all segments of the arch (tubular hypoplasia) may exist in association with intra-cardiac anomalies of varying severity. Surgical repair of the distal transverse aortic arch and isthmus are adequately managed by an extended end-to-end coarctation repair in most infants via a left thoracotomy. The surgical management and timing of proximal aortic arch obstruction is controversial but almost always requires an approach via sternotomy using cardiopulmonary bypass. [Copyright &y& Elsevier]

The article discusses the clinical benefits of monocusp right ventricular outflow tract (RVOT) restoration procedure to patients with inborn cardiac deficiencies. It observes that the use of polytetrafluoroethylene (PTFE) monocusp valve has established to be an easy and reproducible procedure indicating exceptional early postoperative function with least pulmonary deficiency. Another benefit cited is the right ventricular outflow tract (ROVT) advantage for mid-term right ventricular remodeling procedure.

Background: In the Ross aortic valve replacement (AVR) procedure, the right ventricular outflow tract (RVOT) conduit is inserted in an orthotopic position. In complex congenital RVOT obstruction, the right ventricular-pulmonary artery (RV-PA) conduit is placed in a more heterotopic position. We hypothesized that durability of homograft RVOT reconstruction in the Ross AVR is improved secondary to orthotopic positioning and the ability to oversize the RV-PA homograft conduit in the Ross AVR. Methods: Between June 1993 and May 2007, 183 consecutive patients (mean age, 23.3 ± 15.2; median, 22; range, 1 month to 61 years) underwent Ross AVR with RVOT reconstruction using a cryopreserved pulmonary homograft (n = 156), decellularized pulmonary homograft (n = 22), and bovine jugular vein conduit (n = 5). Results: Three patients died (2 early, 1 late; mean follow-up, 5.7 ± 3.3 years). Twenty-four patients (13%) had a peak systolic RVOT gradient exceeding 20 mm Hg, 5 (3%) had a gradient exceeding 40 mm Hg, and 7 (4%) had more than 2+ RVOT insufficiency. Eight patients (4%) underwent conduit replacement for RV dysfunction. Freedom from RVOT reoperation at 10 years is 96%. Freedom from RV failure and dysfunction are 98% and 96% at 5 years and 96% and 93% at 10 years, respectively. Independent predictors of pulmonary homograft RV-PA conduit dysfunction are smaller (< 14 mm) RVOT conduit size ( p = 0.03) and follow-up exceeding 5 years ( p = 0.05). Conclusions: Stenosis and regurgitation of the RV-PA conduit in adults and children following Ross AVR is infrequent. The most logical reasons for the superior performance of the homograft in Ross patients are: (1) orthotopic positioning, (2) older age of implant, and (3) the ability to significantly oversize the homograft in Ross patients. [Copyright &y& Elsevier]

Purpose: We hypothesized that a propeller pump design would function optimally to provide cavopulmonary assist in a univentricular Fontan circulation. Description: The hydraulic and hemolysis performance of a rigid three-bladed propeller prototype (similar to a folding propeller design) was characterized. Pressure and flow measurements were taken for flow rates of 0.5 to 3 liters per minute (LPM) for 5,000 to 7,000 revolutions per minute (RPM) using a blood analog fluid. Hemolysis testing was performed using fresh bovine blood for 2 LPM at 6,000 RPM for a 6-hour duration. Evaluation: The prototype performed well over the design operating range by producing a pressure rise of 5 to 50 mm Hg. Plasma free hemoglobin concentration remained less than 15 mg/dL. The normalized index of hemolysis peaked during the first hour, and then remained less than 10 mg/dL thereafter. Conclusions: A propeller pump has the pressure-flow characteristics and minimal risk of hemolysis and venous pathway obstruction which make it ideal for temporary cavopulmonary assist. This type of device has the potential to provide a new therapeutic option for patients with failing univentricular Fontan physiology as a bridge-to-recovery or transplantation. [Copyright &y& Elsevier]

Background: Anomalous origin of the left coronary artery from the pulmonary artery causes severe myocardial ischemia, global left ventricular dysfunction, and annular dilatation producing varying degrees of mitral regurgitation. Mitral regurgitation secondary to the left ventricular or papillary muscle dysfunction in infants will usually improve in the absence of ongoing ischemia. The aim of this study is to determine the influence of the degree of preoperative mitral regurgitation on the early and late outcomes of patients with anomalous origin of the left coronary artery from the pulmonary artery who underwent coronary reimplantation. Methods: Twenty-five patients underwent coronary reimplantation and 1 early patient had ligation during a 30-year period (median age, 4 months; range, 1 month to 16 years), with a median follow-up of 7 years (range, 4 months to 25 years). Before repair, 7 infants (27%) presented in extremis requiring ventilatory and inotropic support, and 17 patients (65%) presented with heart failure. Mitral regurgitation was present in all patients: trivial in 6 patients, mild in 12 patients, moderate in 5 patients, and severe in 3 patients. No patient underwent mitral valve repair or replacement at the time of anomalous origin of the left coronary artery from the pulmonary artery repair. Results: Hospital survival was 92%. Three patients underwent mitral valve repair or replacement at the mean time of 3.5 years (all with severe preoperative mitral regurgitation). The degree of mitral regurgitation gradually improved in all remaining patients with preoperative mild and moderate mitral regurgitation. Echocardiographic studies demonstrated improvement in left ventricular function in all children. None of the patients showed any evidence of supravalvar pulmonary stenosis as a result of their pulmonary artery reconstruction. Conclusion: Long-term clinical outcome and left ventricular function are good despite severe left ventricular dysfunction at presentation. Mitral valve repair or replacement is generally not necessary at the time of anomalous origin of the left coronary artery from the pulmonary artery repair. Significant residual mitral regurgitation is present in some patients and can usually be managed surgically at a later date depending on its degree of severity. [Copyright &y& Elsevier]