Preservation of fertility in female patients with hematologic diseases.

Recent developments of assisted reproduction techniques turned possible to avoid the infertility consequences of oncologic treatments, but fertility preservation (FP) has been somewhat neglected in women with hematologic diseases undergoing gonadotoxic treatments. For these specific cases, the current options for FP include the cryopreservation of embryos, mature oocytes and ovarian tissue, and oocyte in-vitro maturation. We intend to make patients and clinicians aware of this important and relevant issue, and provide hematologists, assisted reproduction physicians and patients, with updated tools to guide decisions for FP. The physicians of the units responsible for female FP should always be available to decide on the best-individualized FP option in strict collaboration with hematologists. With a wide range of options for FP tailored to each case, a greater level of training and information is needed among clinicians, so that patients proposed to gonadotoxic treatments can be previously advised for FP techniques in hematological conditions. ABBREVIATED ABSTRACT: Recent developments of assisted reproduction techniques turned possible to preserve the fertility of women with hematologic diseases undergoing gonadotoxic treatments. Current options for fertility preservation in women with hematologic diseases are presented. It is imperative to offer fertility preservation to all women before starting any gonadotoxic treatment and in some cases after treatment. Fertility preservation methods enable to later achieve the desired pregnancy.

Characteristics and Outcomes of COVID-19 Cancer Patients Admitted to a Portuguese Intensive Care Unit: A Case-Control Study.

Cancer patients appear to be a vulnerable group in the COVID-19 pandemic. This study aims to compare clinical characteristics and outcomes of cancer and non-cancer patients with COVID-19 admitted to the ICU. All COVID-19 cancer patients (cases) admitted to a Portuguese ICU between March 2020 and January 2021 were included and matched on age, sex and comorbidities with COVID-19 non-cancer patients (controls); 29 cases and 29 controls were enrolled. Initial symptoms were similar between the two groups. Anemia was significantly superior among cases (76% vs. 45%; p = 0.031). Invasive mechanical ventilation (IMV) need at ICU admission was significantly higher among cases (48% vs. 7%; odds ratio (OR) = 12.600, 95% CI: 2.517-63.063, p = 0.002), but there were no differences for global need for IMV during all-length of ICU stay and mortality rates. In a multivariate model of logistic regression, the risk of IMV need at ICU admission among cases remained statistically significant (adjusted OR = 14.036, 95% CI: 1.337-153.111, p = 0.028). Therefore, compared to critical non-cancer patients, critical cancer patients with COVID-19 had an increased risk for IMV need at the moment of ICU admission, however, not for IMV need during all-length of ICU stay or death.

Modelling metabolic performance in paediatric obstructive sleep disordered breathing: A case-control study.

Paediatric obstructive sleep disordered breathing (OSDB) has a considerable impact on cardiovascular physiology, but the consequences on children's basal metabolism and response to exercise are far from being known. The objective was to propose model estimations for paediatric OSDB metabolism at rest and during exercise. A retrospective case-control analysis of data from children submitted to otorhinolaryngology surgery was performed. The heart rate (HR) was measured, while oxygen consumption (VO2 ) and energy expenditure (EE) at rest and during exercise were obtained using predictive equations. The results for the patients with OSDB were compared with controls. A total of 1256 children were included. A total of 449 (35.7%) had OSDB. The patients with OSDB showed a significantly higher resting heart rate (94.55 ± 15.061 bpm in OSDB vs. 92.41 ± 15.332 bpm in no-OSDB, p = 0.041). The children with OSDB showed a higher VO2 at rest (13.49 ± 6.02 mL min-1 kg-1 in OSDB vs. 11.55 ± 6.83 mL min-1 kg-1 in no-OSDB, p = 0.004) and a higher EE at rest (67.5 ± 30.10 cal min-1 kg-1 in OSDB vs. 57.8 + 34.15 cal min-1 kg-1 in no-OSDB, p = 0.004). At maximal exercise, patients with OSDB showed a lower VO2 max (33.25 ± 5.82 mL min-1 kg-1 in OSDB vs. 34.28 ± 6.71 in no-OSDB, p = 0.008) and a lower EE (166.3 ± 29.11 cal min-1 kg-1 in OSDB vs. 171.4 ± 33.53 cal min-1 kg-1 in no-OSDB, p = 0.008). The VO2 /EE increment with exercise (Δ VO2 and Δ EE) was lower in OSDB for all exercise intensities (p = 0.009). This model unveils the effect of paediatric OSDB on resting and exercise metabolism. Our findings support the higher basal metabolic rates, poorer fitness performance, and cardiovascular impairment found in children with OSDB.

Respiratory and non-respiratory outcomes of bronchopulmonary dysplasia in adolescents: A systematic review.

BACKGROUND: There is lack of evidence synthesis on the global consequences of bronchopulmonary dysplasia (BPD) in adolescence. AIM: Assess the impact of bronchopulmonary dysplasia on respiratory and non-respiratory outcomes in adolescents. METHODS: A systematic review of studies assessing the outcomes of adolescents aged 10 to 19 years-old with BPD was conducted. We independently screened studies published until 6th March 2023 in PubMed® and Scopus® databases. Data on methodologic design, sample descriptive and findings were extracted from each study. Risk of bias was assessed using quality assessment tools. RESULTS: Thirty-one studies were included. Adolescents with a history of BPD present with more respiratory symptoms (wheezing, respiratory exacerbations, need for respiratory medication) and twenty-five studies showed a reduction in pulmonary function, with varying impact according to BPD severity and no differences before and after the surfactant era. Spirometry evaluation throughout the years is not consensual, but methacholine and salbutamol response in BPD groups is increased compared to non-BPD groups. Markers of eosinophilic airway inflammation are not increased as in asthma patients. Exercise potential is identical, but data regarding physical capacity and activity are inconsistent. More frequent radiologic abnormalities translate into higher high-resolution computed tomography scores, with linear (72.2 %) and triangular subpleural opacities (58.3 %) as the most common findings. There is a higher risk for special needs in education, but quality of life seems to be equal to non-BPD adolescents. CONCLUSIONS: BPD negatively impacts both pulmonary and non-pulmonary outcomes in adolescents.

Ewing Sarcoma Developed at the Site of Previous Mast Cell Proliferation.

KIT gene mutations in Ewing sarcomas are rare; however, they are much more frequent in other neoplasms, namely mastocytosis. We describe a case of an adult male with a one-year duration of recurrent episodes of pain, swelling, and redness on the proximal phalanx of the third finger of his right hand. A core biopsy suggested a possible mastocytosis. After four years of recurrent episodes and worsening symptoms, an incisional biopsy revealed an Ewing sarcoma with a KIT gene mutation (M541L, on exon 10). KIT gene mutations with gain-of-function were identified in 2.6% of Ewing sarcomas. In this case, the detection of a KIT mutation in an Ewing sarcoma developed at the site of previous mast cell proliferation raises the hypothesis of a possible sarcomatous evolution of the original lesion. To the best of our knowledge, similar cases are not described in the current literature. This is also the first report describing the KIT M541L mutation (exon 10) in Ewing sarcoma.

The Role of ROS as a Double-Edged Sword in (In)Fertility: The Impact of Cancer Treatment.

Tumor cells are highly resistant to oxidative stress resulting from the imbalance between high reactive oxygen species (ROS) production and insufficient antioxidant defenses. However, when intracellular levels of ROS rise beyond a certain threshold, largely above cancer cells' capacity to reduce it, they may ultimately lead to apoptosis or necrosis. This is, in fact, one of the molecular mechanisms of anticancer drugs, as most chemotherapeutic treatments alter redox homeostasis by further elevation of intracellular ROS levels or inhibition of antioxidant pathways. In traditional chemotherapy, it is widely accepted that most therapeutic effects are due to ROS-mediated cell damage, but in targeted therapies, ROS-mediated effects are mostly unknown and data are still emerging. The increasing effectiveness of anticancer treatments has raised new challenges, especially in the field of reproduction. With cancer patients' life expectancy increasing, many aiming to become parents will be confronted with the adverse effects of treatments. Consequently, concerns about the impact of anticancer therapies on reproductive capacity are of particular interest. In this review, we begin with a short introduction on anticancer therapies, then address ROS physiological/pathophysiological roles in both male and female reproductive systems, and finish with ROS-mediated adverse effects of anticancer treatments in reproduction.

Use of recombinant human deoxyribonuclease in pediatric intensive care unit - a single-center experience.

OBJECTIVE: Dornase alfa (rhDNase) reduces the viscosity of purulent sputum in the lungs. The use in patients with cystic fibrosis (CF) is proven. However, the evidence of its applicability to other conditions is limited. This study aims to present the authors' experience with the use of rhDNase in non-CF patients admitted to the Pediatric Intensive Care Unit (PICU). At the study center, rhDNase was used during flexible bronchoscopies in 24 cases, of which 20 (83%) had atelectasis and seven (29%) were admitted to PICU. Four patients (57%) were on invasive mechanical ventilation (MV). CASE DESCRIPTION: Two cases of daily rhDNase administration at PICU are presented: patient A was an 8-year-old boy admitted with septic shock and acute respiratory distress syndrome (ARDS). The patient required mechanical ventilation with aggressive settings and experienced several clinical complications. On D50, he started rhDNase treatment with an improvement in FiO2, PaCO2 and PaO2/FiO2 ratio according to radiologic findings. He was extubated on D23 of treatment.Patient B was a 17-month-old girl admitted with a convulsive status epilepticus who experienced respiratory complications (infectious and barotrauma) with ARDS, requiring aggressive ventilation. She initiated rhDNase treatment on D60. During the treatment an improvement in FiO2, PaO2/FiO2 ratio and a tendency of PaCO2 decrease were found. She had radiological improvement. No complications were described. COMMENTS: RhDNase may be a helpful and safe tool to use in PICU prolonged intubated patients with ventilator-induced lung injury. Further studies are needed to assess and propose valid indications.

Gut microbiota changes after metabolic surgery in adult diabetic patients with mild obesity: a randomised controlled trial.

BACKGROUND: Roux-en-Y gastric bypass (RYGB) surgery is one of the most efficient procedures for the treatment of obesity, also improving metabolic and inflammatory status, in patients with mild obesity. The underlying mechanisms have not been fully understood, but gut microbiota is hypothesized to play a key role. Our aim was to evaluate the association between gut microbiota changes and anthropometric, metabolic and inflammatory profiles after metabolic surgery compared with medical therapy, in type 2 diabetic (T2DM) adults with mild obesity (BMI 30-35 kg/m2). METHODS: DM2 was an open-label, randomised controlled clinical trial (RCT: ISRCTN53984585) with 2 arms: (i) surgical, and (ii) medical. The main outcome was gut microbiota changes after: metabolic surgery (Roux-en-Y gastric bypass-RYGB) versus standard medical therapy. Secondary outcomes included anthropometric, metabolic and inflammatory profiles. Clinical visits, blood workup, and stool samples were collected at baseline and months (M)1, 3, 6, 12. Gut microbiota was profiled using 16S rRNA targeted sequencing. RESULTS: Twenty patients were included: 10 in surgical and 10 in medical arm. Anthropometric and metabolic comparative analysis favoured RYGB over medical arm. At M12, the percentage of weight loss was 25.5 vs. 4.9% (p < 0.001) and HbA1c was 6.2 vs. 7.7% (p < 0.001) respectively. We observed a continuous increase of genus richness after RYGB up until M12. In the medical arm, genus richness ended-up being significantly lower at M12. Composition analysis indicated significant changes of the overall microbial ecosystem (permanova p = 0.004, [R2 = 0.17]) during the follow-up period after RYGB. There was a strong association between improvement of anthropometric/metabolic/inflammatory biomarkers and increase in microbial richness and Proteobacterial lineages. CONCLUSIONS: This was the first RCT studying composite clinical, analytic, and microbiome changes in T2DM patients with class 1 obesity after RYGB versus standard medical therapy. The remarkable phenotypic improvement after surgery occurred concomitantly with changes in the gut microbiome, but at a lower level. TRIAL REGISTRATION: ISRCTN53984585.

COMPLICATED PNEUMONIA WITH EMPYEMA CAUSED BY STREPTOCOCCUS ANGINOSUS IN A CHILD.

OBJECTIVE: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. CASE: description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. COMMENTS: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.

Maxillary mesenchymal chondrosarcoma leading to a diagnosis of Li-Fraumeni syndrome.

Mesenchymal chondrosarcoma (MCS) is a rare histological variant of chondrosarcoma, with aggressive behaviour. Due to the unique nature of this disease, management strategies are not well established. Li-Fraumeni syndrome (LFS) is a rare cancer predisposition syndrome with a wide tumour spectrum, associated with TP53 germline mutations. We report a case of MCS of the maxilla, treated with surgical excision and adjuvant chemotherapy, in a patient with a past medical history of choroid plexus papilloma and a family history of early age first-degree cervical uterine cancer, that led to the clinical suspicion of a cancer predisposition syndrome and the subsequent diagnosis of LFS. This is the first MCS described in a LFS case. It demonstrates that adjuvant chemotherapy should be considered, in conjunction with surgical excision, in MCS and that cancer predisposition syndromes should be suspected in patients with multiple neoplasms and a strong family history of cancer.

Can Denosumab cure giant cell tumors of the spine? A case report and literature review.

BACKGROUND: Bone giant cell tumors, although benign, may be locally aggressive and cause severe morbidity; in some cases, they can also disseminate at distance and cause death. Denosumab has been approved to treat unresectable bone giant cell tumors or when surgery is likely to result in severe morbidity. Furthermore, its curative potential has been recently suggested. CASE: An 18-year-old girl presented with a spinal giant cell tumor at T9. Neo-adjuvant denosumab was administered for 9 months with great clinical and analytical tolerance. A posterior left T9 costo-transversectomy and vertebral body curettage was performed and the spine stabilized. Interestingly, histopathology examination of the surgical specimens found no evidence of tumoral cells. Denosumab was reinstated until completion of 12 months of treatment. CONCLUSION: Denosumab has an important but still limited role in the treatment of spinal giant cell tumors. Here, it resulted in complete histological resolution of the tumor, potentially widening its applicability from a strictly neo-adjuvant to a curative role.

Complicated pneumonia with empyema caused by streptococcus anginosus in a child / Complicated pneumonia with empyema caused by streptococcus anginosus in a child

ABSTRACT Objective: To highlight the pathogenicity of Streptococcus anginosus, which is rare in pediatric patients, but can cause severe infections that are known to have a better outcome when treated early with interventional procedures and prolonged antibiotic therapy. Case description: The patient is a 6-year-old boy with global developmental delay, examined in the emergency room due to fever and respiratory distress. The physical examination and diagnostic workout revealed complicated pneumonia with empyema of the left hemithorax; he started antibiotic therapy and underwent thoracic drainage. Pleural fluid cultures grew Streptococcus anginosus. On day 11, the child had a clinical deterioration with recurrence of fever, hypoxia, and respiratory distress. At this point, considering the causative agent, he was submitted to video-assisted thoracoscopic decortication, with good progress thereafter. Comments: Streptococcus anginosus is a commensal bacterium of the human oral cavity capable of causing severe systemic infections. Although reports of complicated thoracic infections with this agent are rare in the pediatric population, they have been increasing in adults. Streptococcus anginosus has a high capacity to form abscess and empyema, requiring different therapeutic approaches when compared to complicated pneumonia caused by other agents.

RESUMO Objetivo: Alertar para a patogenicidade do Streptococcus anginosus que, apesar de raro em pediatria, pode causar infeções graves que necessitam de tratamento invasivo e antibioterapia de longo curso para obter um melhor prognóstico. Descrição do caso: Criança de seis anos, com atraso do desenvolvimento psicomotor, avaliado no serviço de urgência por febre e dificuldade respiratória. O exame físico, juntamente com os exames complementares, revelou uma pneumonia complicada com empiema no hemitórax esquerdo, tendo iniciado antibioterapia e sido submetido à drenagem do líquido pleural. Foi identificado Streptococcus anginosus nesse líquido. No 11º dia de doença, a criança agravou o seu estado clínico, com recidiva da febre, hipoxemia e dificuldade respiratória. Considerando-se o microrganismo identificado, o paciente foi submetido à decorticação pulmonar por videotoracoscopia, com boa evolução clínica posterior. Comentários: Streptococcus anginosus é uma bactéria comensal da cavidade oral humana, que pode causar infecções sistêmicas graves. Apesar de serem raros os casos descritos em pediatria, têm sido cada vez mais descritas infecções torácicas complicadas em adultos. Esse microrganismo também tem a capacidade de formar abcessos e empiemas, que precisam de intervenções terapêuticas diferentes, quando comparados a pneumonias complicadas causadas por outros agentes.

Single Ventricle Palliation in a Developing Sub-Saharan African Country: What Should be Improved?

INTRODUCTION: Single ventricle physiology management is challenging, especially in low-income countries. OBJECTIVE: To report the palliation outcomes of single ventricle patients in a developing African country. METHODS: We retrospectively studied 83 consecutive patients subjected to single ventricle palliation in a single center between March 2011 and December 2017. Preoperative data, surgical factors, postoperative results, and survival outcomes were analyzed. The patients were divided by palliation stage: I (pulmonary artery banding [PAB] or Blalock-Taussig shunt [BTS]), II (Glenn procedure), or III (Fontan procedure). RESULTS: Of the 83 patients who underwent palliation (stages I-III), 38 deaths were observed (31 after stage I, six after stage II, and one after stage III) for an overall mortality of 45.7%. The main causes of operative mortality were multiple organ dysfunction due to sepsis, shunt occlusion, and cardiogenic shock. Twenty-eight survivors were lost to follow-up (22 after stage I, six after stage II). Thirteen stage II survivors are still waiting for stage III. The mean follow-up was 366 ± 369 days. Five-year survival was 28.4 % for PAB and 30.1% for BTS, while that for stage II and III was 49.8% and 57.1%, respectively. Age (hazard ratio, 0.61; 95% confidence interval: 0.47-0.7; P = .000) and weight at surgery (hazard ratio, 0.45; 95% confidence interval: 0.31-0.64; P = .002) impacted survival. CONCLUSION: A high-mortality rate was observed in this initial experience, mainly in stage I patients. A large number of patients were lost to follow-up. A task force to improve outcomes is urgently required.

Submitral aneurysm in children.

BACKGROUND: We report a surgical series of submitral aneurysm in children. METHODS: Between March 2011 and December 2015, eight consecutive patients less than 18 years old with submitral aneurysm underwent surgical correction. RESULTS: Six patients were female, the mean age was 7 ± 3.8 years old, and mean weight was 21.4 kg. Six patients were in NYHA functional class III or IV. Six patients underwent repair via a transatrial approach, another with a transatrial combined with transaneurysmal approach, and another with a transventricular approach. There were no in-hospital deaths but one 30-day mortality. One patient required reoperation. Two patients required mitral valve replacement. At discharge, one patient had severe and another had moderate mitral regurgitation. The mean follow-up time was 26.4 months and five patients were alive. No reintervention was required. CONCLUSIONS: Submitral aneurysm is not restricted to adults. Heart failure is the commonest clinical presentation in the pediatric age. The transatrial approach is feasible, safe, and associated with good short-term results. The mitral valve can be preserved in the majority of cases.

A Right Upper Lobe Mass in the Thorax After Modified Blalock-Taussig Shunt.

We report an incidental finding of pseudoaneurysm in a 10-month-old boy with tetralogy of Fallot and Down syndrome who had undergone placement of a modified Blalock-Taussig shunt at age four months. Computed tomography was a determinant exam for better assessment. The lesion was successfully resected with concomitant complete repair of tetralogy of Fallot in a single-stage. The child was asymptomatic at fourth month follow-up.

Anomalous Origin of One Pulmonary Artery From the Ascending Aorta: From Diagnosis to Treatment in Angola.

BACKGROUND: Anomalous origin of one pulmonary artery is a rare congenital heart disease in which one pulmonary artery branch originates from the ascending aorta. OBJECTIVE: To describe the experience of a cardiothoracic center in an African country to repair anomalous origin of one pulmonary artery in the context of Portugal-Angola collaboration. METHODS: Between March 2011 and March 2015, four consecutive patients with anomalous origin of pulmonary artery branch underwent surgical correction. The mean age was 1.6 months. The mean weight was 4 kg. All had right pulmonary artery branch originating from the ascending aorta. All patients underwent direct implantation of right pulmonary branch to main pulmonary artery. Two patents had patent ductus arteriosus and one had atrial septal defect. Two patients had pulmonary hypertension. RESULTS: There was no registration of death. The mean cardiopulmonary bypass time was 75.5 ± 4.5 minutes, mean aortic cross-clamping time was 40 ± 5.6 minutes, and mean duration of the postoperative intensive care unit stay was 6.8 ± 5.7 days. At discharge, one patient had residual gradient of 25 mm Hg, the remainder had no significant gradient. The mean follow-up time was 11 months (5-28 months). One week after discharge, one patient presented operative wound dehiscence. At the last follow-up, all patients were alive, and no significant residual gradient or stenosis at site of anastomosis was observed. No reintervention was required. CONCLUSION: Anomalous origin of one pulmonary artery is a rare but potentially treatable lesion if operated early in life. Direct implantation was a good technique with good short-term results.

Ebstein's anomaly in children: a single-center study in Angola.

INTRODUCTION AND OBJECTIVE: Ebstein's anomaly is a rare complex congenital heart defect of the tricuspid valve. We aimed to describe the frequency, clinical profile, and early and short-term post-operative results in patients under the age of 18 years operated for this anomaly in a tertiary center in Angola. METHODS: A retrospective cross-sectional study was conducted over a period of 37 months. We analyzed all patients diagnosed with congenital heart defects. RESULTS: Of the 1362 patients studied, eight (0.6%) had Ebstein's anomaly; six patients (75%) were female. Mean age was 69±59 months. Five patients were in NYHA functional class III or IV. Mean cardiothoracic index was 0.72. Seven patients (87.5%) had severe tricuspid regurgitation and five (62.5%) had another associated congenital heart defect. All patients were operated: two had complications and one (12.5%) died in the early post-operative period. The mean follow-up time was 1.22±0.6 years, and mortality during follow-up was 12.5% (n=1). At the end of the study, of the five patients in whom cone reconstruction was performed, four (80%) were in functional class I. Mean cardiothoracic index decreased to 0.64. Three patients had mild and two had moderate tricuspid regurgitation. The patient who underwent cone reconstruction and a Glenn procedure was in functional class I. CONCLUSION: The frequency of Ebstein's anomaly was similar to that in other centers. Cone reconstruction was viable in the majority of patients, with good early and short-term results.

Mitro-aortic aneurysms in children: single-centre experience and review of the literature.

OBJECTIVES: This publication aims to report the cases of four children with pseudoaneurysm of the mitral-aortic intervalvular fibrosa and carry out a review of the literature. BACKGROUND: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a very rare anomaly in children. It can be either congenital or acquired, namely, after bacterial endocarditis or cardiac trauma. This pathology does not usually cause specific symptoms but its outcome may be potentially fatal. METHODS: We report the cases of four patients presenting with pseudoaneurysm of the mitral-aortic intervalvular fibrosa, referred for treatment in a paediatric cardiology clinic. Patient clinical notes were retrospectively reviewed for aetiology, clinical presentation, diagnostic work-up, surgical treatment, and follow-up. Literature on the subject was extensively reviewed. RESULTS: In three patients, pseudoaneurysm of the mitral-aortic intervalvular fibrosa was acquired, being secondary to bacterial endocarditis in two cases and establishing after mitral surgery in another case. The remaining patient had a "congenital" aetiology - no other cause could be traced. The diagnosis was achieved by transthoracic echocardiography for all patients, and confirmed in all by trans-oesophageal echocardiography, to better define morphological details and to access flow into the aneurysmal formation. All patients were submitted to corrective cardiac surgery. Of the patients, three survived and were cured by surgery, staying asymptomatic, and one died after repeated interventions, for persistent endocarditis. CONCLUSIONS: Pseudoaneurysm of the mitral-aortic intervalvular fibrosa is a rare but potentially fatal anomaly. In our experience, surgical cure was achieved for the majority of the cases, except for a case for which infection could not be locally eradicated, leading to multiple reinterventions.

Acurácia e curva de aprendizado de observadores inexperientes para segmentação manual de eletromiogramas / Accuracy and learning curves of inexperienced observers for manual segmentation of electromyograms

INTRODUCTION: The shape-varying format of surface electromyograms introduces errors in the detection of contraction events. OBJECTIVE: To investigate the accuracy and learning curves of inexperienced observers to detect the quantity of contraction events in surface electromyograms. MATERIALS AND METHODS: Six observers performed manual segmentation in 1200 shape-varying waveforms simulated using a phenomenological model with variable events, smooth changes in amplitude, marked on-off timing, and variable signal-to-noise ratio (0-39 dB). Segmentation was organized in four sessions with 15 blocks of 20 signals each. Accuracy and learning curves were modeled per block by linear and power regression models and tested for difference among sessions. Cut-off values of signal-to-noise ratio for optimal manual segmentation were also estimated. RESULTS: The accuracy curve showed no significant linear trend throughout blocks and no difference among sessions 1-2-3-4 (87% [85; 89], 87% [85; 89], 87% [85; 89], 87% [81; 88]; p = 0.691). Accuracy was low for detection of 1 event (AUC = 0.40; sensitivity = 44%; specificity = 43%; cut-off = 12.9 dB) but was high and affected by the signal-to-noise ratio for detection of two events (AUC = 0.82; sensitivity = 77%; specificity = 76%; cut-off = 7.0 dB). The learning curve showed a significant power regression (p < 0.001) with decreasing values of learning percentages (time duration to complete the task) among sessions 1-2-3-4 (86.5% [68; 94], 76% [68; 91], 62% [38; 77], and 57% [52; 75]; p = 0.002). CONCLUSION: Inexperienced observers exhibit high, not trainable accuracy and a practice-dependent shortening in the time spent to detect the quantity of contraction events in simulated surface electromyograms.

INTRODUÇÃO: A forma de onda variável do eletromiograma de superfície introduz erros na detecção de eventos de contração. OBJETIVO: Investigar as curvas de acurácia e aprendizagem de observadores inexperientes para detectar a quantidade de eventos em eletromiogramas de superfície. MATERIAIS E MÉTODOS: Seis observadores realizaram segmentação manual em 1.200 sinais com formas de onda variável simulada usando modelo fenomenológico, com mudanças suaves em amplitude, tempos início-fim marcados, e relação sinal-ruído variável (0-39 dB). A segmentação foi organizada em quatro sessões, com 15 blocos de 20 sinais. As curvas de acurácia e aprendizagem foram modeladas por bloco utilizando modelos de regressão linear e de potência, e foram testados quanto à diferença entre as sessões. Valores de corte de relação sinal-ruído ideais para segmentação manual também foram estimadas. RESULTADOS: A curva de acurácia não mostrou tendência linear significativa e não houve diferença entre as sessões 1-2-3-4 (87% [85; 89], 87% [85; 89], 87% [85; 89], 87% [81; 88], p = 0,691). A acurácia foi baixa para a detecção de um evento (AUC = 0,40; sensibilidade = 44%; especificidade = 43%; limiar = 12,9 dB), mas foi elevada e afetada pela relação sinal-ruído para a detecção de dois eventos (AUC = 0,82; sensibilidade = 77%, especificidade = 76%; limiar = 7,0 dB). A curva de aprendizagem mostrou uma regressão de potência significativa (p < 0,001) com valores decrescentes de percentagens de aprendizagem (tempo de duração para completar a tarefa) entre sessões 1-2-3-4 (86,5% [68; 94], 76% [68; 91], 62% [38; 77] e 57% [52; 75], p = 0,002). CONCLUSÃO: Observadores inexperientes apresentam alta acurácia não treinável e uma redução prática-dependente do tempo gasto para detectar a quantidade de eventos de contração em eletromiogramas de superfície simulados.

Partial anomalous pulmonary venous connections: surgical management.

Partial anomalous pulmonary venous connections (PAPVCs) are a heterogeneous group of congenital heart lesions in which at least one pulmonary vein will drain into the systemic venous system. The consequences are a variable left-to-right hemodynamic shunt and more rarely pulmonary artery hypertension. Often, PAPVC occurs in association with other congenital cardiac malformations. Surgical correction is most often advisable and is generally straightforward and simple to achieve. Historically, some repairs have included incision across the junction of the superior vena cava with the right atrium, which can lead to late arrhythmias. The Warden technique avoids incision across the atriocaval junction. Neonates and infants with Scimitar syndrome represent the most challenging subset of patients with PAPVC.