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Objectives: Surgical management of mitral valve disease is challenging in infants <1 year old. We aimed at reviewing the French experience with Melody mitral valve replacement in critically ill infants., Methods: A retrospective cohort study reporting the French experience with Melody mitral valve replacement., Results: Seven symptomatic infants [complete atrioventricular septal defect (n = 4, Down syndrome: n = 3), hammock valve (n = 3)] underwent Melody mitral valve replacement [age: 3 months (28 days to 8 months), weight: 4.3 kg (3.2-6.4 kg)] because of severe mitral valve regurgitation (6) or mixed valve disease (1) and 14 mm (11-16 mm) mitral valve annulus. In 2 patients whose valve was felt irreparable, Melody mitral valve replacement was performed straightaway. The others underwent 2 (1-3) previous attempts of valve repair; 3 were on extracorporeal membrane oxygenation. Melody mitral valve replacement led to competent valve and low gradient [3 mmHg, (1-4 mmHg)]. One patient died 3 days post-implant from extracorporeal membrane oxygenation-related stroke. Of the 6 discharged home patients, 3 (50%) were readmitted for a definite diagnosis (1) or high suspicion (2) of infective endocarditis, of which 2 died. Over the follow-up, 1 underwent balloon expansions of the valve at 9- and 16-months post-implant, and mechanical mitral valve replacement at 2 years; another is currently planned for transcatheter Melody valve dilation., Conclusions: Melody mitral valve replacement may be considered in selected infants with small mitral valve annulus as an alternative to mechanical mitral valve replacement. Our experience highlights a high-risk of late infective endocarditis that deserves further consideration., (© The Author(s) 2024. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery.)

American football players and other individuals exposed to repetitive head impacts can exhibit a constellation of later-life cognitive and neuropsychiatric symptoms. While tau-based diseases such as chronic traumatic encephalopathy can underpin certain symptoms, contributions from non-tau pathologies from repetitive head impacts are increasingly recognized. We examined cross-sectional associations between myelin integrity using immunoassays for myelin-associated glycoprotein and proteolipid protein 1 with risk factors and clinical outcomes in brain donors exposed to repetitive head impacts from American football. Immunoassays for myelin-associated glycoprotein and proteolipid protein 1 were conducted on dorsolateral frontal white matter tissue samples of 205 male brain donors. Proxies of exposure to repetitive head impacts included years of exposure and age of first exposure to American football play. Informants completed the Functional Activities Questionnaire, Behavior Rating Inventory of Executive Function-Adult Version (Behavioral Regulation Index), and Barratt Impulsiveness Scale-11. Associations between myelin-associated glycoprotein and proteolipid protein 1 with exposure proxies and clinical scales were tested. Of the 205 male brain donors who played amateur and professional football, the mean age was 67.17 (SD = 16.78), and 75.9% ( n = 126) were reported by informants to be functionally impaired prior to death. Myelin-associated glycoprotein and proteolipid protein 1 correlated with the ischaemic injury scale score, a global indicator of cerebrovascular disease ( r = -0.23 and -0.20, respectively, P s < 0.01). Chronic traumatic encephalopathy was the most common neurodegenerative disease ( n = 151, 73.7%). Myelin-associated glycoprotein and proteolipid protein 1 were not associated with chronic traumatic encephalopathy status, but lower proteolipid protein 1 was associated with more severe chronic traumatic encephalopathy ( P = 0.03). Myelin-associated glycoprotein and proteolipid protein 1 were not associated with other neurodegenerative disease pathologies. More years of football play was associated with lower proteolipid protein 1 [beta = -2.45, 95% confidence interval (CI) [-4.52, -0.38]] and compared with those who played <11 years of football ( n = 78), those who played 11 or more years ( n = 128) had lower myelin-associated glycoprotein (mean difference = 46.00, 95% CI [5.32, 86.69]) and proteolipid protein 1 (mean difference = 24.72, 95% CI [2.40, 47.05]). Younger age of first exposure corresponded to lower proteolipid protein 1 (beta = 4.35, 95% CI [0.25, 8.45]). Among brain donors who were aged 50 or older ( n = 144), lower proteolipid protein 1 (beta = -0.02, 95% CI [-0.047, -0.001]) and myelin-associated glycoprotein (beta = -0.01, 95% CI [-0.03, -0.002]) were associated with higher Functional Activities Questionnaire scores. Lower myelin-associated glycoprotein correlated with higher Barratt Impulsiveness Scale-11 scores (beta = -0.02, 95% CI [-0.04, -0.0003]). Results suggest that decreased myelin may represent a late effect of repetitive head impacts that contributes to the manifestation of cognitive symptoms and impulsivity. Clinical-pathological correlation studies with prospective objective clinical assessments are needed to confirm our findings., (© The Author(s) 2023. Published by Oxford University Press on behalf of the Guarantors of Brain.)

Objectives: Musculoskeletal disorders (MSDs) account for the greatest burden of years lived with disability globally. To prevent disability, good-quality services need to be commissioned, appropriate for local need. We analysed data collected systematically from a new musculoskeletal service serving 70% of the population of Scotland to evaluate: age- and sex-specific occurrence; anatomical distribution; and impact and effect on work ability., Methods: A new centralized telephone-based triage for people with musculoskeletal disorders was set up in Scotland in 2015. Available to most of the population aged >16 years (>3 million people), data were collected systematically into a database detailing: anatomical site, nature of onset, duration, impact/risk (modified STarT score), deprivation level and, for those in employment, sickness absence., Results: Data were available from 219 314 new callers, 2015-18. Calls were more frequently from women (60%), increased with age until the eighth decade, and 66% reported symptoms that had been present for >6 weeks. Callers were more likely to be living in more deprived areas in each age band between 20 and 64 years and tended to have higher-impact symptoms. The majority (53%) of callers were in employment, and 19% of these were off sick because of their symptoms. Sickness absence was more common among those with highest impact/risk scores from deprived areas with more acute symptoms., Discussion: Large-scale systematic data collection for MSDs emphasizes the size and impact of the burden among adults aged >16 years. A socio-economic gradient is evident in terms of prevalence and impact of MSDs, particularly for sickness absence., (© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology.)

Summary: Our increasing ability to resolve fine details using light microscopy is matched by an increasing need to quantify images in order to detect and measure phenotypes. Despite their central role in cell biology, many image analysis tools require a financial investment, are released as proprietary software, or are implemented in languages not friendly for beginners, and thus are used as black boxes. To overcome these limitations, we have developed PyJAMAS, an open-source tool for image processing and analysis written in Python. PyJAMAS provides a variety of segmentation tools, including watershed and machine learning-based methods; takes advantage of Jupyter notebooks for the display and reproducibility of data analyses; and can be used through a cross-platform graphical user interface or as part of Python scripts via a comprehensive application programming interface., Availability and Implementation: PyJAMAS is open-source and available at https://bitbucket.org/rfg&#95;lab/pyjamas., Supplementary Information: Supplementary data are available at Bioinformatics online., (© The Author(s) 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Objectives: Pseudomonas aeruginosa is a leading cause of community- and hospital-acquired infections. Successful treatment is hampered by its remarkable ability to rapidly develop resistance to antimicrobial agents, primarily through mutation. In response, WHO listed carbapenem-resistant P. aeruginosa as a Priority 1 (Critical) pathogen for research and development of new treatments. A key resource in developing effective countermeasures is access to diverse and clinically relevant strains for testing. Herein we describe a panel of 100 diverse P. aeruginosa strains to support this endeavour., Methods: WGS was performed on 3785 P. aeruginosa isolates in our repository. Isolates were cultured from clinical samples collected from healthcare facilities around the world between 2003 and 2017. Core-genome MLST and high-resolution SNP-based phylogenetic analyses were used to select a panel of 100 strains that captured the genetic diversity of this collection. Antibiotic susceptibility testing was also performed using 14 clinically relevant antibiotics., Results: This 100-strain diversity panel contained representative strains from 91 different STs, including genetically distinct strains from major epidemic clones ST-111, ST-235, ST-244 and ST-253. Seventy-one distinct antibiotic susceptibility profiles were identified ranging from pan-susceptible to pan-resistant. Known resistance alleles as well as the most prevalent mutations underlying the antibiotic susceptibilities were characterized for all isolates., Conclusions: This panel provides a diverse and comprehensive set of P. aeruginosa strains for use in developing solutions to antibiotic resistance. The isolates and available metadata, including genome sequences, are available to industry, academia, federal and other laboratories at no additional cost., (Published by Oxford University Press on behalf of the British Society for Antimicrobial Chemotherapy 2021. This work is written by a US Government employee and is in the public domain in the US.)

Lessons Learned: The fibrolamellar carcinoma-associated DNAJB1-PRKACA gene fusion transcript RNA codes for the catalytic domain of protein kinase A and, thus, overexpression of Aurora kinase A. ENMD-2076 showed a favorable toxicity profile. The limited results, one patient (3%) with a partial response and 57% of patients with stable disease, do not support further evaluation of ENMD-2076 as single agent. Future studies will depend on the simultaneous targeting approach of DNAJB1-PRKACA and the critical downstream components., Background: Fibrolamellar carcinoma (FLC) represents approximately 0.85% of liver cancers. The associated DNAJB1-PRKACA gene fusion transcript RNA codes for the catalytic domain of protein kinase A and overexpression of Aurora kinase A (AURKA). ENMD-2076 is a selective anti-AURKA inhibitor., Methods: Patients aged >12 years with pathologically confirmed incurable FLC, with measurable disease, Eastern Cooperative Oncology Group performance status 0-2 or Lansky 70-100, and adequate organ function were eligible. Patients were prescribed ENMD-2076 based on body surface area. The primary endpoint was overall objective response rate by RECIST v1.1, with a null hypothesis of true response rate of 2% versus one-sided alternative of 15%. Secondary endpoints included 6-month progression-free survival (PFS) rate (Fig. 1), median PFS, time to progression (TTP), and overall survival (OS). Safety was evaluated throughout the study., Results: Of 35 patients who enrolled and received treatment, 1 (3%) had a partial response (PR) and 20 (57%) had stable disease (SD). Median TTP, PFS, and OS were 5, 3.9, and 19 months, respectively. The most frequently reported drug-related serious adverse event was hypertension in three patients. Three deaths were reported on-study-two due to disease progression and one due to pulmonary embolism not related to ENMD-2076., Conclusion: The study provided no rationale for further studying ENMD-2076 as a single agent in FLC., (© AlphaMed Press; the data published online to support this summary is the property of the authors.)

Lessons Learned: FLC is a complex cancer with many implicated oncogenic pathways. Single or dual targeting does not appear to alter the natural history of the cancer, and novel therapeutics are needed. Estrogen deprivation therapy with letrozole and leuprolide, alone or in combination with the mTOR inhibitor, everolimus, did not demonstrate clinical activity in advanced fibrolamellar carcinoma. The study drugs were well tolerated when administered as single agents or in combination in this patient population. This study demonstrates that, despite the rarity of FLC, multicenter therapeutic clinical trials are feasible and support the value of this consortium., Background: Fibrolamellar carcinoma (FLC) is an uncommon malignancy in young people and is sometimes associated with pregnancy and oral contraceptive use. Immunohistochemical staining and genetic profiling of FLC tumor specimens have revealed aromatase overexpression. The overexpression of mTOR and S6 kinase has been noted in 25% of FLC. On the basis of interaction between estrogen and the PI3K/Akt/mTOR pathway, we hypothesized that suppression of estrogen and mTOR signaling could have antineoplastic activity in FLC., Methods: Patients were randomized to arm A (everolimus), arm B (letrozole/leuprolide; estrogen deprivation therapy [EDT]), or arm C (everolimus/letrozole/leuprolide). Upon disease progression, patients in arm A or B could proceed to part 2 (everolimus/letrozole/leuprolide). The primary endpoint was progression-free survival (PFS) at 6 months (PFS6) assessed using a Simon's minimax two-stage design, hypothesizing an improvement in PFS6 from 40% to 64% with the study regimen., Results: Twenty-eight patients were enrolled. An unplanned analysis was performed because of perceived concern for lack of efficacy. Stable disease was observed in 9 of 26 evaluable patients (35%). PFS6 was 0%. Median overall survival (OS) was 12.4 months (95% confidence interval [CI], 7.4-20.9) for the whole study cohort. Grade 3 adverse events in ≥10% of patients were nausea (11%), vomiting (11%), anemia (11%), elevated aspartate transaminase (AST; 32%), alanine transaminase (ALT; 36%), and alkaline phosphatase (14%). All 28 patients experienced an event for PFS outcome, and four deaths were due to disease progression., Conclusion: Neither EDT nor mTOR inhibition improved outcomes in FLC. Other treatment strategies are needed., (© AlphaMed Press; the data published online to support this summary are the property of the authors.)

Objectives: Long-term outcomes after the arterial switch operation (ASO) for complex transposition of the great arteries (TGA) should be clarified., Methods: A retrospective study was conducted in patients operated on between 1982 and 1998. Overall 220 postoperative survivors, 79.1% with a ventricular septal defect, 13.2% with multiple ventricular septal defects, and 29.1% with aortic arch obstruction, were followed for 17 years (0-28 years)., Results: The conditional survival rate was 96.7% [95% confidence interval (CI): 94.4-99.1] at 25 years. Late sudden death occurred in 2 asymptomatic patients. The cumulative incidence rate of death or reinterventions was 3.8% (95% CI: 2.9-4.8) at 25 years, with age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. The cumulative incidence rate of neoaortic regurgitation was 41.6% (95% CI: 20.5-62.8) at 25 years with an aorto-pulmonary diameter mismatch at the time of the ASO, age at ASO <10 days and aortic regurgitation at discharge identified as independent risk factors. At the last follow-up, 53 patients (24.1%) had neoaortic root dilatation with an aortic sinus z-score ≥3 and 6 of them had a Bentall operation at a median delay of 14.1 years since the ASO. The only independent factors for neoaortic root dilatation were male sex and an aorto-pulmonary diameter mismatch at the time of the ASO., Conclusions: Despite a continual rate of reinterventions, long-term survival and cardiovascular outcome are excellent after ASO for complex TGA. Dilatation of the neoaortic root and neoaortic regurgitation may be observed with time and 2 late sudden deaths occurred, justifying a close follow-up in all patients., (© The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: The aim was to describe the early and mid-term outcome after atrio-ventricular valve (AVV) repair in patients with univentricular hearts (UVHs) and to identify risk factors for AVV reoperation and death., Methods: This study is a retrospective review of patients undergoing valve repair for AVV regurgitation at any stage of univentricular palliation from 1998 to 2014. Patient- and procedure-related variables were analysed., Results: A total of 31 consecutive patients underwent 38 procedures for ≥ moderate AVV regurgitation at a median age of 3.6 years. Thirty-two percent of patients had a common AVV, 26% had two AVVs, 22% had a dominant tricuspid valve and 19% had a dominant mitral valve. All patients underwent valve repair as a first procedure without early mortality. At discharge, patients preserved their ventricular function (fractional shortening <30%: preoperative 16% vs postoperative 22.5%, NS). In 19% (n = 6) of patients, the procedure was considered as failed because of significant residual regurgitation. There were three late deaths [median delay: 1 year (range 0.7-13.6)] and three heart transplantations. Six patients underwent seven AVV reoperations [median delay: 2 years (range 0.2-7.6)]. Longer intensive care stay (P = 0.022), longer total postoperative hospital stay (P = 0.039), higher total number of surgeries (P = 0.039), lower body mass index (P = 0.042) and higher preoperative mean pulmonary pressure (P = 0.047) were univariate risk factors for death/transplantation. Failed first AVV repair (P = 0.01), higher total number of surgeries (P = 0.026), lower body mass index (P = 0.031), male gender (P = 0.031) and need for valve repair before bidirectional cavopulmonary connection (P = 0.036) were univariate risk factors for AVV reoperation. In multivariate analysis, no univariate risk factor reached statistical significance. Freedom from death/transplantation was 84% (CI 95%: 70%-98%) at 5 and 10 years. Survival free from AVV reoperation was 72% (CI 95%: 52%-92%) at 5 years and 62% at 10 years (CI 95%: 36%-88%). Mean follow-up of survivors was 4.7 years (SD ± 4.3; range 0.2-15.6). At last visit, 96% of survivors were in NYHA Class I-II. Ninety-two percent had a ≤ mild residual regurgitation., Conclusions: In patients with a UVH and ≥ moderate AVV regurgitation, AVV repair is feasible without postoperative deterioration of their ventricular function. Nevertheless, these patients remain at increased risk for death/transplantation and AVV reoperation., (© The Author 2015. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved.)

Objectives: Postoperative left atrioventricular valve (LAVV) dysfunction is known to be the principal risk factor influencing outcome after repair of all types of atrioventricular septal defect (AVSD). The purpose of the present study was to identify the risk factors for reoperation and to assess the outcomes after reoperation for LAVV dysfunction., Methods: Records of 412 patients who underwent anatomical repair for different types of AVSD from January 2000 to July 2012 were reviewed. The study group (n = 60) included 13 additional patients for whom repair ± LAVV reoperation was performed in a primary institution. Outcomes, independent risk factors, reoperation and death were analysed., Results: There were 7 early, (1.7%) and 1 late death. Forty-seven (11.4%) required 64 reoperations for LAVV dysfunction. The median delay for the first LAVV reoperation was 3.5 months (range: 5 days to 10.0 years). Unbalanced ventricles with small left ventricle [odds ratio (OR) = 4.06, 95% confidence interval (CI): 1.58-10.44, P = 0.004], double-orifice LAVV (OR = 5.04, 95% CI: 1.39-18.27, P = 0.014), prior palliative surgery (OR = 3.5, 95% CI: 1.14-10.8, P = 0.029) and discharge echocardiography documenting LAVV regurgitation grade >2 (OR = 21.96, 95% CI: 8.91-54.09, P < 0.001) were found to be independent risk factors for LAVV reoperation. Twelve-year survival and freedom from LAVV reoperation rates were, respectively, 96.1% (95% CI: 94.1-98.1) and 85.8% (95% CI: 81.3-90.3). Survival was significantly worse in patients who underwent LAVV reoperation (P < 0.001) and in those who underwent valve replacement vs valve repair (P = 0.020)., Conclusion: After AVSD repair, LAVV dysfunction appears to be the principal factor that influences outcome. It can usually be managed by repair. Need for multiple reoperations is not uncommon. Long-term outcome in patients with repaired LAVV is favourable.

In sub-Saharan Africa, newborns and children continue to suffer from insufficient access to early diagnosis and antiretroviral (ARV) treatments. A survey had been conducted in Burkina Faso, Ghana and Ivory Coast, from January 2010 to February 2011 to identify the major challenges regarding HIV prophylaxis and treatment of children in western Africa. The results of this survey highlight that only a small proportion of HIV-exposed newborns receive ARV prophylaxis. However, this problem is often not perceived at the national level. The problem could be faced by improving the communication process between the peripheral health services and the national procurement system. Moreover, supporting the development of local pharmaceutical industries could facilitate the availability of child-sized drugs, contextualized to the socio-cultural needs of such area, adequate not only in terms of efficacy, safety and tolerability, but also in terms of palatability, storage, distribution and cost.

Objectives: The objectives were to determine in patients with Tetralogy of Fallot (ToF) and abnormal coronary artery (ACA): the long-term outcomes of different surgical strategies; the risk factors for right ventricular outflow tract (RVOT) obstruction, reoperation, heart failure and mortality. To date, the surgical strategies and prognostic factors for repair of ToF with an ACA, crossing the RVOT and avoiding a classic repair, have not been evaluated in a large series using a multivariate analysis., Methods: A retrospective study (1986-2011) included 72 patients. The mean follow-up was 9.6 ± 6.8 years. Median age at surgery was 1.5 years (0.2-11.3). The main surgical techniques were 'tailored' right ventriculotomy and patch of the RVOT (63%; n = 45), implantation of a conduit between the right ventricle (RV) and the pulmonary artery (PA; 25%; n = 18) and a transatrial ± transpulmonary approach (11%; n = 8). Univariate and multivariate logistic regression analyses were performed., Results: Intrahospital mortality was 2.7%. Actuarial freedom from reoperation and actuarial survival at 15 years were 77% (confidence interval [CI]: 70-83%) and 94% (CI: 90-97%), respectively. Reoperations occurred more frequently after conduit implantation (50%) than after patch reconstruction (17%) or transatrial ± transpulmonary approach (0%; P = 0.002). The transatrial ± transpulmonary approach was significantly less complicated, with a long-term RVOT obstruction of 0% compared with the other surgical techniques (45.4%; P = 0.03). Implantation of a RV-PA conduit was an independent risk factor for RVOT obstruction (odds ratio [OR]: 31; P < 0.001) and reoperation (OR: 20; P = 0.02). An immediate postoperative right ventricle/left ventricle (RV/LV) pressure ratio >0.5 was independently associated with a long-term RV/LV pressure ratio >0.5 (OR: 14; P = 0.001), but was not a risk factor for reoperation (P = 0.8). Postoperative electric ischaemic signs independently increased the risk of long-term heart failure (OR: 22; P = 0.04)., Conclusions: The transatrial ± transpulmonary approach displays the best long-term outcomes, by reducing the risks for RVOT obstruction and reoperation, but does not improve the patient survival. A RV-PA conduit was an independent risk factor for RVOT obstruction and reoperation. An immediate postoperative RV/LV pressure ratio >0.5 was not a risk factor for reoperation. The transatrial ± transpulmonary approach should be preferred to the implantation of a conduit or a tailored right ventriculotomy whenever possible.

Objective: Heterotaxy syndrome (HS) is generally associated with complex congenital cardiac defects and has a high morbidity and mortality despite recent surgical progress. Only few reports deal with an overall surgical population., Methods: Between 1989 and 2008, 139 patients with HS entered a programme of surgical repair. Fifty-one patients were suitable for univentricular heart repair (UVR) and 88 for biventricular repair (BVR). Among those tracked for UVR, two were switched to BVR and 11 from BVR to UVR. Median age at first surgery was 4.4 months (range: 3 days to 43 years] of whom 34 were neonates. The mean number of surgical procedure per patient was 1.99. Primary BVR was performed in 37 patients. Re-operation was required in 22 patients, 15 after BVR and seven after UVR (p>0.05)., Results: The overall mortality was 20.8%. It was 7.2% after the first surgery, 6.6% after the second and 11.5% after the third. The overall mortality in patients with univentricular physiology was 25.5% and 18.2% in the biventricular group (p<0.05). According to the surgical track, in the UVR group, mortality was 18% and 15.6% in the BVR group (p=NS). This rate was 40% in patients with long-lasting palliation (p<0.05 vs both other groups). Median follow-up was 127 months (range: 1 month to 19 years). The overall survival rate at 15 years was 70.6%. When considering ventricular anatomy, survival rates at 15 years were 69% for the univentricular group and 74.2% for the biventricular group (p>0.05). According to the type of surgical approach, at 15 years they were 85.1% for UVR and 77% for BVR (p=NS). For the palliation group, it was 15% only at 15 years (p<0.05 vs both other groups). Risk factors for overall mortality were neonatal surgery, long-standing palliation, total anomalous pulmonary vein return (TAPVR) and right ventricular outflow tract obstruction (RVOTO). At the last visit, all survivors were in the New York Heart Association (NYHA) class I to II and only two presented with supraventricular arrhythmias., Conclusions: HS remains a difficult situation with high morbidity and mortality. An aggressive approach to repair TAPVR when present should be considered. Early decision to track the patient in either uni- or biventricular repair programme should avoid long-lasting deleterious palliation., (Copyright © 2010 European Association for Cardio-Thoracic Surgery. Published by Elsevier B.V. All rights reserved.)

Background: Congenitally corrected TGA (CC-TGA) is characterized by discordant atrioventricular and ventriculo arterial connections. In absence of right ventricular outflow tract obstruction (RVOTO), repair by atrial and arterial switches remains a challenging procedure for which long term follow-up is uncertain., Methods: From 1995 to 2007, 20 patients (median age: 26 months) with CC-TGA had double switch procedure. Segmental anatomy was {SLL} in all patients, dextrocardia in two patients, mesocardia in two patients. Ventricular septal defect was present in 17 patients, aortic coarctation in 2 patients and interrupted aortic arch (IAoA) in 1 patient. Five patients had tricuspid valve regurgitation. Six patients had AV blocks, 4 patients had pacemaker implantation prior to repair. Pulmonary artery banding was performed in 17 patients, for congestive heart failure (14 patients) or left ventricular retraining (3 patients). Three patients, including one patient with IAoA had primary repair. After LV retraining, repair was performed when indexed LV mass to LV volume ratio was above 1.5. A median follow-up of 60 months was achieved in all., Results: There were no deaths. Postoperative pacemaker implantation was required in four patients. Reoperation for Senning obstruction was necessary in one patient, and pacemaker battery replacement in another patient. One patient had mild neoaortic insufficiency, two had mild tricuspid regurgitation and two had mild mitral regurgitation. All were in NYHA I-II. Actuarial survival at 10 years was 100% and freedom from reoperation at 5 and 10 years were 93% and 77.4%, respectively., Conclusion: Double switch for CC-TGA without RVOTO can be performed with no mortality and low morbidity. Since these results seem to last for several years, it should be considered as the optimal procedure.

Objective: Postoperative left ventricular outflow tract obstruction (LVOTO) after arterial switch operation (ASO) is rare. In this retrospective study, we reviewed the cases of 10 patients with LVOTOs post-ASO and analyzed the options used for the LVOTOs corrections and the patients' outcomes., Methods: From December 1982 to December 2006, 1689 consecutive ASO were performed. Ten patients presented with postoperative LVOTOs (0.59% of all ASO and 7.62% of ASO for Taussig-Bing anomaly (TBA)) leading to reoperations. Before ASO they presented with significant subaortic gradients (n=4) or predisposing anatomical features (n=9) such as: conal septum malalignment (7/9), abnormal tricuspid cords insertions (4/9), muscular bundle hypertrophy (3/9) and mitral accessory tissue (2/9)., Results: No patient was lost in the follow-up: 117+/-33 months. Subaortic gradients had developed between 5 months and 14 years after ASO (mean 41+/-35 months) leading to reoperations. Most LVOTOs post-ASO were due to fibrotic membranes or fibro-muscular hypertrophy, removed at first reoperation. After their first reoperations, seven patients (70%) had non-significant or low gradients and were not reoperated for LVOTO. Three patients underwent iterative surgical procedures for LVOTOs leading to two aortic valve replacements associated with LVOTO release or Konno procedure., Conclusion: Postoperative LVOTO after ASO is rare but happens more frequently in TBA. Most of them can benefit from resections of subaortic obstacles or septal plasties. In more complex cases iterative surgical procedures may lead to complications such as block or severe aortic valve regurgitation.

Objective: In presence of adequate pulmonary blood flow, patients presenting with unoperated or palliated pulmonary atresia with ventricular septal defect (PA/VSD) can reach adult age. However, they remain symptomatic with a limited life expectancy., Methods: Since 1993, 27 patients underwent surgery for unrepaired PA/VSD. Median age was 20 (range: 15-43) years. Nineteen patients had 33 previous palliative procedures while eight were unoperated survivors. Major aortopulmonary collateral artery (MAPCA) had been observed in all but 2 and were still patent in 23. All bronchopulmonary segments were connected to the native pulmonary arteries (NPA) in 4 (type A), to both NPA and MAPCA in 18 (type B) and only to MAPCA in 5 (type C). The biventricular repair was performed in 17 patients: 3 type A, 12 type B and 2 type C. Ten patients underwent palliative procedure: eight aortopulmonary shunt, with unifocalisation in two and one right ventricle to NPA restrictive conduit., Results: One (4%) hospital death occurred following the failure of a palliative procedure. No clinical improvement was observed in seven patients including one repaired and six palliated survivors. Two late cardiac death occurred 1 and 7 years after repair. At last visit, 15 of 16 repaired survivors were in NYHA class I or II. Only one patient awaits septation, while eight other with subsequent palliation were considered not repairable., Conclusion: The outcome was encouraging in patients who were eligible for completed biventricular repair. Although considered as unique alternative to cardiopulmonary transplantation, the justification for palliative surgery to improve pulmonary blood flow remains to be established.

CD31 is a specific and sensitive marker of endothelial differentiation. Previous reports have described its immunoreactivity in large series of soft tissue neoplasms, as well as its comparison with other available and commonly used endothelial markers. CD31 reactivity in carcinomas or mesotheliomas has been incompletely addressed, however. Hence, we applied anti-CD31 (JC70/A, DAKO, Carpinteria, Calif) to 290 previously characterized neoplasms by using a modified avidin-biotin-peroxidase complex technique following microwave epitope retrieval. Seven carcinomas showed plasmalemmal-based immunoreactivity (2 papillary thyroid carcinomas, 2 mucoepidermoid salivary gland carcinomas, 1 cutaneous adnexal tumor, 1 cutaneous squamous cell carcinoma, and 1 esophageal squamous cell carcinoma); the remaining 283 lesions were negative for this marker. We conclude that anti-CD31 immunostaining in carcinomas and mesotheliomas is rare. These findings support the concept that CD31 is a reliable marker of endothelial differentiation and should be included in diagnostic immunohistochemical panels when vascular tumors enter the differential diagnosis.