Outcome for Conservative Surgery for the Correction of Severe Mitral Valve Regurgitation in Children: A Single-Center Experience.

Evolving reconstructive techniques have progressively become the preferred approach for treatment of pediatric mitral valve regurgitation. We present our experience in a cohort of patients undergoing surgical correction for severe mitral regurgitation. Fifty-five patients (age 1 month-18 years; median 5 years) were included in the present analysis. Different surgical techniques were used (posterior leaflet augmentation in 25, isolated cleft closure in 12, Alfieri-type procedure in 10, annuloplasty in 5, with artificial chordae in 2, and quadrangular resection with chordal transposition in 1). Follow-up time ranged from 1 to 192 months (median 38[IQR 12-54] months). Operative and follow-up mortality was 0%. Reintervention in the whole population occurred in 31% of patients. However, when first surgery was performed under 2 years of age (no = 17), reintervention reached nearly 50%. The degree of residual mitral regurgitation at follow-up remained stable after surgery, while a significant increase in mean transmitral gradient was observed over time (paired t test = 0.03). In multivariable Cox-regression analysis, post-surgical transmitral gradient was the only independent predictor for reintervention (p = 0.017; HR 2.4; 95%CI 1.2-5.1), after correcting for differences in age at surgery, type of reintervention, mitral annulus dimension, and BSA at the first surgery. ROC curve demonstrated that a post-surgical transmitral mean gradient value > 5 mmHg, was predictive for reintervention (AUC = 0.89; Youden index = 0.44). Our study suggests that the use of conservative technique strategy achieves satisfactory functional results in infants and children with severe MR, although the rate of reoperation in younger patients remains substantial. Post-operative moderate mitral stenosis was the strongest predictor for reoperation.

Atrial septal defect morphology and stenting in hypoplastic left heart syndrome after hybrid palliation.

Aims The aim of this study was to describe atrial septal defect morphology in hypoplastic left heart syndrome, to report the incidence of restrictiveness and its relationship with defect morphology, to correlate restriction with midterm outcome, and to describe our interventional approach to restrictive defect. Methods and results From 2011 to 2015, 31 neonates with hypoplastic left heart syndrome underwent hybrid procedure with pulmonary artery banding and ductal stenting at our Institution. Restrictive physiology of the atrial septal defect was based on Doppler gradient >6 mmHg through the defect and on clinical signs of pulmonary hypertension. The mean gradient was then measured invasively. Restrictive defect occurred in 11/27 patients (40%). The restrictive group showed three ostium secundum defects (27%) and eight complex morphologies (73%). Conversely, in the non-restrictive group, we observed 11 ostium secundum defects (69%) and five complex morphologies (31%). Early balloon atrioseptostomy was required in three cases. Late restriction occurred in eight patients and was dealt with balloon dilation, stenting, or atrioseptectomy. There was no significant difference between restrictive and non-restrictive groups in terms of early or 12-month survival. CONCLUSIONS: Complex morphologies were more frequently related to restrictiveness. Stenting technique has a crucial role, as the procedure carries a significant risk for stent migration. Effective treatment of restrictive atrial septal defect is related to a better outcome, as it leads to equalisation of survival between patients with and those without restrictive atrial septal defect.

Early hybrid approach and enteral feeding algorithm could reduce the incidence of necrotising enterocolitis in neonates with ductus-dependent systemic circulation.

BACKGROUND: The reported incidence of necrotising enterocolitis in neonates with complex CHD with ductus-dependent systemic circulation ranges from 6.8 to 13% despite surgical treatment; the overall mortality is between 25 and 97%. The incidence of gastrointestinal complications after hybrid palliation for neonates with ductus-dependent systemic circulation still has to be defined, but seems comparable with that following the Norwood procedure. METHODS: We reviewed the incidence of gastrointestinal complications in a series of 42 consecutive neonates with ductus-dependent systemic circulation, who received early hybrid palliation associated with a standardised feeding protocol. RESULTS: The median age and birth weight at the time of surgery were 3 days (with a range from 1 to 10 days) and 3.07 kg (with a range from 1.5 to 4.5 kg), respectively. The median ICU length of stay was 7 days (1-70 days), and the median hospital length of stay was 16 days (6-70 days). The median duration of mechanical ventilation was 3 days. Hospital mortality was 16% (7/42). In the postoperative period, 26% of patients were subjected to early extubation, and all of them received treatment with systemic vasodilatory agents. Feeding was started 6 hours after extubation according to a dedicated feeding protocol. After treatment, none of our patients experienced any grade of necrotising enterocolitis or major gastrointestinal adverse events. CONCLUSIONS: Our experience indicates that the combination of an "early hybrid approach", systemic vasodilator therapy, and dedicated feeding protocol adherence could reduce the incidence of gastrointestinal complications in this group of neonates. Fast weaning from ventilatory support, which represents a part of our treatment strategy, could be associated with low incidence of necrotising enterocolitis.

Sporadic isolation of sabin-like polioviruses and high-level detection of non-polio enteroviruses during sewage surveillance in seven Italian cities, after several years of inactivated poliovirus vaccination.

Sewage surveillance in seven Italian cities between 2005 and 2008, after the introduction of inactivated poliovirus vaccination (IPV) in 2002, showed rare polioviruses, none that were wild-type or circulating vaccine-derived poliovirus (cVDPV), and many other enteroviruses among 1,392 samples analyzed. Two of five polioviruses (PV) detected were Sabin-like PV2 and three PV3, based on enzyme-linked immunosorbent assay (ELISA) and PCR results. Neurovirulence-related mutations were found in the 5'noncoding region (5'NCR) of all strains and, for a PV2, also in VP1 region 143 (Ile>Thr). Intertypic recombination in the 3D region was detected in a second PV2 (Sabin 2/Sabin 1) and a PV3 (Sabin 3/Sabin 2). The low mutation rate in VP1 for all PVs suggests limited interhuman virus passages, consistent with efficient polio immunization in Italy. Nonetheless, these findings highlight the risk of wild or Sabin poliovirus reintroduction from abroad. Non-polio enteroviruses (NPEVs) were detected, 448 of which were coxsackievirus B (CVB) and 294 of which were echoviruses (Echo). Fifty-six NPEVs failing serological typing were characterized by sequencing the VP1 region (nucleotides [nt] 2628 to 2976). A total of 448 CVB and 294 Echo strains were identified; among those strains, CVB2, CVB5, and Echo 11 predominated. Environmental CVB5 and CVB2 strains from this study showed high sequence identity with GenBank global strains. The high similarity between environmental NPEVs and clinical strains from the same areas of Italy and the same periods indicates that environmental strains reflect the viruses circulating in the population and highlights the potential risk of inefficient wastewater treatments. This study confirmed that sewage surveillance can be more sensitive than acute flaccid paralysis (AFP) surveillance in monitoring silent poliovirus circulation in the population as well as the suitability of molecular approaches to enterovirus typing.

Detection and distribution of culturable Human Enteroviruses through environmental surveillance in Milan, Italy.

AIMS: Human Enteroviruses (HEVs) infections have a significant impact on public health, being implicated in outbreaks of meningitis, encephalitis, hand-foot-mouth disease and other acute and chronic manifestation. In the strategic plan for poliomyelitis eradication, the environmental surveillance of poliovirus (PV) has been identified by the World Health Organization (WHO) as an activity that can complement the surveillance of polio. Having wastewater samples available for PV surveillance allows us to study nonpolio enteroviruses (NPEVs) circulating in the study population, which are widely spread. METHODS AND RESULTS: This study was carried out according to the WHO guidelines for environmental surveillance of PV and analysed the circulation of PV and NPEVs through the isolation of viruses in cell cultures in Milan area; from 2006 to 2010, 321 wastewater samples were collected, regularly over time, at the inlet of three diverse waste water treatment plants (WWTPs). Culturable HEVs were isolated in 80% of sewage samples: all isolates belonged to the HEV-B group and those circulating more intensely were CVB5 and Echo 6, while CVB4 was the predominant serotype found in 2010. In this study, two type 2 PVs were isolated, both characterized as Sabin like. CONCLUSION: Environmental monitoring of HEVs in Milan has proved to be an interesting tool to investigate the circulation and distribution of viruses. SIGNIFICANCE AND IMPACT OF THE STUDY: The detection of PV and other NPEV could be predictive of possible re-emergence of these viruses with an impact on public health. NPEV monitoring could also be a powerful public health tool to investigate the possible role of NPEV in different clinical manifestations.

[Exposure to asbestos in buildings in areas of Basilicata characterized by the presence of rocks containing tremolite]. / Esposizione ad amianto in edilizia in aree della Basilicata caratterizzate dalla presenza di rocce contaminate da tremolite.

Lucania, in southern Italy, is characterized by areas with natural outcrops of rocks containing tremolite. The study aims to assess the risk of exposure to asbestos in the building workers in these areas through environmental sampling near sites for implementation of safety of roads built on serpentinite rocks and personal sampling in a group of persons who work in the building industry and a group of residents engaged in activities without contact with the ground. Near road sites was found the presence of airborne tremolite in 66% of environmental samples with peaks up to 31 ff/l. The analysis of personal samples showed the presence of tremolite in doses higher than the natural background in 100% of the building workers, while there were no fibers in the samples of residents employed in activities without soil disturbance. The study shows that the building in areas with naturally occurring asbestos determines a condition of significant occupational exposure to asbestos. Adequate safety measures for workers exposed are needed.

Linguistic correlates of self in deceptive oral autobiographical narratives.

The current study collected orally-delivered autobiographical narratives from a sample of 44 undergraduate students. Participants were asked to produce both deceptive and non-deceptive versions of their narrative to two specific autobiographical question prompts while standing in front of a video camera. Narratives were then analyzed with Coh-Metrix software on 33 indices of linguistic cohesion. Following a Bonferroni correction for the large number of linguistic variables (p<.002), results indicated that the deceptive narratives contained more explicit action verbs, less linguistic complexity, and less referential coherence (sentences being cohesive with each other). The results support a theory that, in deceptive narratives, there is greater narrative distance between the self that narrates and the self that is narrated about. This suggests that narrative selves are constituted not as autonomous selves, but are subject to processes (e.g., psychological, linguistic, social) that are likely operating on a subconscious level.

Use of carotid artery cannulation during redo sternotomy in congenital cardiac surgery: a single-centre experience.

OBJECTIVES: Management of resternotomy is often a difficult challenge in patients with congenital diseases who have undergone multiple sternotomies. Our goal was to report our single-centre experience with carotid cannulation using a graft interposed during redo sternotomy in complex cardiac reintervention procedures. METHODS: We performed a retrospective review of all patients who had undergone complex redo sternotomies between January 2019 and May 2020 utilizing a cervical cannulation technique with a Gore-Tex graft interposed on the carotid artery. We classified our population study on the basis of the primary diagnosis and the type of surgery. The primary outcomes of our analysis were the evaluation of the safety of the procedure in terms of survival and freedom from neurological events. RESULTS: We analysed 22 patients who had undergone previous complex operations. The median age and weight at the time of reintervention were 130.35 (range 0.46-435) months and 31.5 (range 2.2-85) kg, respectively. Composite graft carotid cannulation provided adequate arterial flow in all patients with a median arterial flow of 3.5 l/min/m2 (range 0.6-6). One major cardiac injury occurred during sternotomy when emergency cardiopulmonary bypass (CPB) was initiated. Moreover, during their hospital stays, all patients had an uneventful recovery without neurological or vascular complications and no cervical wound infections. CONCLUSIONS: Carotid cannulation using interposition of a side graft on the common carotid artery for arterial inflow is a reliable and safe method for initiation of CPB in complex redo surgeries in patients with congenital disease. Complications directly associated with this type of cannulation are uncommon and allow surgical re-entry with overall low risks.

Formation of lipoxins and leukotrienes during receptor-mediated interactions of human platelets and recombinant human granulocyte/macrophage colony-stimulating factor-primed neutrophils.

The generation of lipoxygenase products of arachidonic acid is considered an important event in inflammation. This study demonstrates the levels of both lipoxins and leukotrienes (LTC4, LTD4, LTB4, and omega-oxidized LTB4) generated from endogenous sources of arachidonate by PMN primed with recombinant human granulocyte/macrophage colony-stimulating factor and in coincubations with platelets (1:1 to 1:100 ratio). Upon exposure to receptor-mediated stimuli (FMLP and thrombin), the levels of lipoxins generated were within the range of both LTB4 and LTC4. Co-incubation of [1-14C]arachidonate-labeled platelets with primed polymorphonuclear leukocytes (PMN) followed by addition of thrombin and FMLP led to the formation of both 5- and 15-LO products that carried 14C label. Thus, in addition to the transcellular conversion of LTA4 to platelet-derived lipoxins and LTC4, PMN can use platelet-derived arachidonate to generate lipoxygenase products. These results are the first to document the relationship between the levels of lipoxins and leukotrienes generated by receptor-mediated activation of cytokineprimed PMN interacting with platelets. Moreover, they indicate that PMN-platelet interactions utilize bidirectional transcellular routes to contribute to lipoxin formation.

Identification of a human cDNA encoding a functional high affinity lipoxin A4 receptor.

Lipoxin A4 (LXA4) triggers selective responses with human neutrophils that are pertussis toxin sensitive and binds to high affinity receptors (Kd = 0.5 +/- 0.3 nM) that are modulated by stable analogues of guanosine 5'-triphosphate (GTP). Here, we characterized [11,12-(3)]LXA4 specific binding with neutrophil granule and plasma membranes, which each display high affinity binding sites (Kd = 0.7 +/- 0.1 nM) that were regulated by GTP gamma S. Since functional LXA4 receptors are inducible in HL-60 cells, we tested orphan cDNAs encoding 7-transmembrane region receptors cloned from these cells for their ability to bind and signal with LXA4. Chinese hamster ovary (CHO) cells transfected with the orphan receptor cDNA (pINF114) displayed specific 3H-LXA4 high affinity binding (1.7 nM). When displacement of LXA4 binding with pINF114-transfected CHO cells was tested with other eicosanoids, including LXB4, leukotriene D4 (LTD4), LTB4, or prostaglandin E2, only LTD4 competed with LXA4, giving a Ki of 80 nM. In transfected CHO cells, LXA4 also stimulated GTPase activity and provoked the release of esterified arachidonate, which proved to be pertussis toxin sensitive. These results indicate that pINF114 cDNA encodes a 7-transmembrane region-containing protein that displays high affinity for 3H-LXA4 and transmits LXA4-induced signals. Together, they suggest that the encoded protein is a candidate for a LXA4 receptor in myeloid cells.

Aspirin-triggered 15-epi-lipoxin A4 (LXA4) and LXA4 stable analogues are potent inhibitors of acute inflammation: evidence for anti-inflammatory receptors.

Lipoxins are bioactive eicosanoids that are immunomodulators. In human myeloid cells, lipoxin (LX) A4 actions are mediated by interaction with a G protein-coupled receptor. To explore functions of LXA4 and aspirin-triggered 5(S),6(R),15(R)-trihydroxy-7,9,13-trans-11-cis-eicosatetraenoic acid (15-epi-LXA4) in vivo, we cloned and characterized a mouse LXA4 receptor (LXA4R). When expressed in Chinese hamster ovary cells, the mouse LXA4R showed specific binding to [3H]LXA4 (K(d) approximately 1.5 nM), and with LXA4 activated GTP hydrolysis. Mouse LXA4R mRNA was most abundant in neutrophils. In addition to LXA4 and 15-epi-LXA4, bioactive LX stable analogues competed with both [3H]LXA4 and [3H]leukotriene D4 (LTD4)-specific binding in vitro to neutrophils and endothelial cells, respectively. Topical application of LXA4 analogues and novel aspirin-triggered 15-epi-LXA4 stable analogues to mouse ears markedly inhibited neutrophil infiltration in vivo as assessed by both light microscopy and reduced myeloperoxidase activity in skin biopsies. The 15(R)-16-phenoxy-17,18, 19,20-tetranor-LXA4 methyl ester (15-epi-16-phenoxy-LXA4), an analogue of aspirin triggered 15-epi-LXA4, and 15(S)-16-phenoxy-17,18,19,20-tetranor-LXA4 methyl ester (16-phenoxy-LXA4) were each as potent as equimolar applications of the anti-inflammatory, dexamethasone. Thus, we identified murine LXA4R, which is highly expressed on murine neutrophils, and showed that both LXA4 and 15-epi-LXA4 stable analogues inhibit neutrophil infiltration in the mouse ear model of inflammation. These findings provide direct in vivo evidence for an anti-inflammatory action for both aspirin-triggered LXA4 and LXA4 stable analogues and their site of action in vivo.

Mercury speciation in the colloidal fraction of a soil polluted by a chlor-alkali plant: a case study in the South of Italy.

Mercury (Hg) speciation in different size fractions of a soil sample collected near an industrial area located in the South of Italy, which had been polluted by the dumping of Hg-containing wastes from a chlor-alkali plant, was investigated by XANES spectroscopy. In particular, a special procedure has been developed to study the soil colloidal fraction, both for sample preparation and for XANES data collection. In this soil, Hg was speciated in quite insoluble inorganic forms such as cinnabar (alpha-HgS), metacinnabar (beta-HgS), corderoite (Hg(3)S(2)Cl(2)), and some amorphous Hg, S and Cl-containing species, all derived from the land-disposal of K106 Hg-containing wastes. The contribution of the above-mentioned chemical forms to Hg speciation changed as a function of particle size. For the fraction <2 mm the speciation was: amorphous Hg-S-Cl (34%) > corderoite (26%) > cinnabar (20%) = metacinnabar (20%); for the fraction <2 microm: amorphous Hg-S-Cl (40%) > metacinnabar (24%) > corderoite (20%) > cinnabar (16%); and for the fraction 430-650 nm, where most of the colloidal Hg was concentrated: amorphous Hg-S-Cl (56%) > metacinnabar (33%) > corderoite (6%) > cinnabar (5%). From these data it emerged that, even if Hg was speciated in quite insoluble forms, the colloidal fraction, which is the most mobile and thus the most dangerous, was enriched in relatively more soluble species (i.e. amorphous Hg-S-Cl and metacinnabar), as compared with cinnabar. This aspect should be seriously taken into account when planning environmental risk assessment, since the small particle size in which Hg is concentrated and the changing speciation passing from millimetre to nanometre size could turn apparently safe conditions into more hazardous ones.

Italian consensus statement on paediatric HIV infection.

The objective of this document is to identify and reinforce current recommendations concerning the management of HIV infection in infants and children in the context of good resource availability. All recommendations were graded according to the strength and quality of the evidence and were voted on by the 57 participants attending the first Italian Consensus on Paediatric HIV, held in Siracusa in 2008. Paediatricians and HIV/AIDS care specialists were requested to agree on different statements summarizing key issues in the management of paediatric HIV. The comprehensive approach on preventing mother-to-child transmission (PMTCT) has clearly reduced the number of children acquiring the infection in Italy. Although further reduction of MTCT should be attempted, efforts to personalize intervention to specific cases are now required in order to optimise the treatment and care of HIV-infected children. The prompt initiation of treatment and careful selection of first-line regimen, taking into consideration potency and tolerance, remain central. In addition, opportunistic infection prevention, adherence to treatment, and long-term psychosocial consequences are becoming increasingly relevant in the era of effective antiretroviral combination therapies (ART). The increasing proportion of infected children achieving adulthood highlights the need for multidisciplinary strategies to facilitate transition to adult care and maintain strategies specific to perinatally acquired HIV infection.

Effects of levetiracetam on chronic pain in multiple sclerosis: results of a pilot, randomized, placebo-controlled study.

BACKGROUND AND PURPOSE: Central neuropathic pain (CNP) is a prevalent and distressing symptom in patients with multiple sclerosis (MS). The anticonvulsant levetiracetam (LEV) has been shown to be efficacious in some types of CNP, but its efficacy in MS-related CNP has not been confirmed. METHODS: To investigate the tolerability and potential effects of LEV against CNP in MS subjects, we performed a single-center, prospective, randomized, single-blind, placebo-controlled study in twenty patients with MS and CNP. Outcomes before and during the 3-month study were assessed using validated measures of pain, depression, disability and quality of life. RESULTS: The medication was well tolerated and analysis revealed a significant difference between the LEV and placebo arm in all study outcomes related to pain (mean pain intensity score, mean pain difference, percentage of patients with a clinically significant pain reduction). Furthermore, the individual quality of life rating improved in treated patients, showing a significant correlation with pain reduction. CONCLUSIONS: These findings suggest that further studies with larger samples of patients be carried out in order to confirm the efficacy of LEV in MS-related CNP population.

Women facing HIV. Key question on women with HIV infection: Italian consensus workshop.

A panel of leading Italian specialists in infectious diseases, obstetrics and gynaecology met in a national consensus workshop on women facing HIV to review critical aspects and discuss recommendations for selected key questions on four issues: (1) women and highly active antiretroviral therapy (HAART): access to care and adherence to therapy, side effects and drug-drug interaction; (2) HIV-infected pregnant women: prevention of mother to child transmission; (3) desire for children among women living with HIV: assisted reproduction; (4) sexually transmitted diseases and genital disturbances. The method of a nominal group meeting was used, and recommendations were graded for their strength and quality of evidence using a system based on the one adopted by the Infectious Diseases Society of America. Main conclusions are summarized and critically discussed, and some of the most recent data supporting recommendations are provided.

Hepatitis B or hepatitis C coinfection in HIV-infected pregnant women in Europe.

OBJECTIVES: The aim of the study was to investigate the prevalence of and risk factors for hepatitis C or B virus (HCV or HBV) coinfection among HIV-infected pregnant women, and to investigate their immunological and virological characteristics and antiretroviral therapy use. METHODS: Information on HBV surface antigen (HBsAg) positivity and HCV antibody (anti-HCV) was collected retrospectively from the antenatal records of HIV-infected women enrolled in the European Collaborative Study and linked to prospectively collected data. RESULTS: Of 1050 women, 4.9% [95% confidence interval (CI) 3.6-6.3] were HBsAg positive and 12.3% (95% CI 10.4-14.4) had anti-HCV antibody. Women with an injecting drug use(r) (IDU) history had the highest HCV-seropositivity prevalence (28%; 95% CI 22.8-35.7). Risk factors for HCV seropositivity included IDU history [adjusted odds ratio (AOR) 2.92; 95% CI 1.86-4.58], age (for > or =35 years vs. <25 years, AOR 3.45; 95% CI 1.66-7.20) and HBsAg carriage (AOR 5.80; 95% CI 2.78-12.1). HBsAg positivity was associated with African origin (AOR 2.74; 95% CI 1.20-6.26) and HCV seropositivity (AOR 6.44; 95% CI 3.08-13.5). Highly active antiretroviral therapy (HAART) use was less likely in HIV/HCV-seropositive than in HIV-monoinfected women (AOR 0.34; 95% CI 0.20-0.58). HCV seropositivity was associated with a higher adjusted HIV RNA level (+0.28 log(10) HIV-1 RNA copies/mL vs. HIV-monoinfected women; P=0.03). HIV/HCV-seropositive women were twice as likely to have detectable HIV in the third trimester/delivery as HIV-monoinfected women (AOR 1.95; P=0.049). CONCLUSIONS: Although HCV serostatus impacted on HAART use, the association between HCV seropositivity and uncontrolled HIV viraemia in late pregnancy was independent of HAART.

Reproductive assistance for infected couples with bloodborne viruses.

During the past 15 years Assisted Reproduction has been facing a new demand from patients requiring ART: couples at risk of partner to partner, and mother to child transmission of viral infections, mainly HIV-1, HCV and HIV-HCV co-infected partners. The general conditions and life expectancy of many patients with HIV infection are very good, and three-quarters of these individuals are in their reproductive years. For these reasons, a large number of young couples are expected to make future plans to have children. This desire is not easy to realize for serodiscordant couples, if we consider that, in order to avoid HIV virus transmission, it is necessary to encourage the condom use in vaginal and anal contacts. On the other hand infertile discordant HCV couples need to be included in protocols of controlled assisted reproduction procedures to avoid any risk of HCV transmission to the partner. In this paper we consider assisted reproduction in discordant couples for HIV or HCV-positive men.

Long-Term Outcomes After Extracardiac Fontan Takedown to an Intermediate Palliative Circulation.

BACKGROUND: Acute failure of the Fontan circulation is rare but remains associated with high morbidity and mortality rates. Little is known about the long-term outcome of patients who underwent Fontan takedown to an intermediate palliative circulation and their potential candidacy for redo Fontan completion. METHODS: Patients followed up at a single institution who underwent takedown of a Fontan circulation to an intermediate palliative circulation within 2 months of extracardiac Fontan completion were reviewed. RESULTS: Between October 1990 and December 2015, 18 patients underwent Fontan takedown to a superior cavopulmonary connection (with or without an additional shunt) at a median age of 3.3 years (range, 1.8 to 8.0) and median weight of 13.8 kg (range, 8.0 to 27.0 kg). Takedown was required during the Fontan procedure itself in 2 patients, and within the first 2 postoperative months in 16 patients (median time to takedown, 3 days). Seventeen patients survived the post-takedown period and 3 ultimately underwent successful redo Fontan. Four patients required heart transplantation, with 2 deaths. In patients with extended intermediate palliation, median arterial oxygen saturation was 84% (range, 76% to 92%) at a median follow-up of 6.3 years (range, 0.7 to 25.9). CONCLUSIONS: Takedown to a superior cavopulmonary connection is an effective treatment option and, in some patients, acts as a bridge to subsequent redo Fontan completion or heart transplantation. An extended intermediate palliative circulation is tolerated for several years with reasonable oxygen saturation levels at rest. In our experience, an early takedown strategy to a superior cavopulmonary connection is the treatment of choice for acute Fontan failure.

Isolated limb perfusion for the management limb threatening soft tissue sarcomas: The role of histological type on clinical outcomes.

BACKGROUND: Hyperthermic isolated limb perfusion (HILP) is an effective neoadjuvant treatment to avoid amputation in patients with locally advanced extremity soft tissue sarcomas (STS). We aimed to investigate whether STS histological type plays a role in predicting clinical outcomes. METHODS: This study reports a retrospective analysis of 125 patients with limb threatening STS (liposarcoma, n = 41; malignant peripheral nerve sheath tumor, n = 20; leiomyosarcoma, n = 20; miscellany, n = 44), who underwent HILP from 1990 through 2015 at our institution. The following endpoints were evaluated: tumor response (assessed by radiological imaging and histology), limb sparing rate, local progression-free survival (LPFS) and overall survival (OS). RESULTS: On average, overall (complete + partial) tumor response was significantly greater in patients affected with liposarcoma as compared to those with other histotypes (radiological response rate: 38/41, 92.7% vs 66/84, 78.6%, P-value: 0.048; mean histological necrosis: 83.6% vs 52.9%, P < 0.0001). Limb sparing rate was also higher among patients with liposarcoma as compared to other histotypes (39/41, 95.1% vs 62/84, 73.8%, P-value: 0.005). As regards survival, LPFS was similar across tumor types, whereas OS resulted significantly worse in patients with limb leiomyosarcoma (log-rank P-value: 0.009). CONCLUSIONS: HILP is a very effective treatment modality for limb threatening STS. In our series, liposarcoma appears to be the histological type most sensitive to HILP in terms of tumor response and thus limb sparing, which might help clinicians in the patient selection process.