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BACKGROUND: Pulmonary arterial hypertension (PAH) can have several consequences on sexual function, which can lead to worsened quality of life. AIM: The study sought to assess sexual function and its association with health functionality and quality of life in females with PAH. METHODS: A descriptive cross-sectional study was carried out in pulmonary circulation outpatient clinics from January 2022 to March 2023 in females diagnosed with pulmonary hypertension. Assessment was carried out through the application of the Female Sexual Function Index, the 36-item World Health Organization Disability Assessment Schedule, and the Medical Outcome Study 36-Item Short Form Survey. OUTCOMES: Data were analyzed using SPSS version 22.0 and JASP, and Spearman's correlation tests were applied between the instruments, with a P value <.05 considered significant. RESULTS: A total of 91 females were assessed. It was identified that 90.1% of females had sexual dysfunction, with worse scores in females with sexual dysfunction in the domains of satisfaction, arousal, and desire, with average health functionality and quality of life. There were significant correlations between the domains of mobility, getting along, life activities, and the overall functionality score with some domains of sexual function, especially arousal and satisfaction. We found significant correlations between some domains of quality-of-life assessment with the domains of desire, arousal, and satisfaction, and with the overall score of sexual function assessment, as well as strong correlations between health functionality and quality of life. CLINICAL IMPLICATION: The data reinforce the need for rehabilitation programs and social support for this population. STRENGTHS AND LIMITATIONS: This is one of the few studies to evaluate sexual function, quality of life, and health functionality in women with PAH. Due to limitations in data collection, we were unable to assess certain factors such as hormone levels and a history of sexual abuse. CONCLUSION: We identified a high prevalence of sexual dysfunction in females with PAH with mild functional impairment and a moderate quality-of-life score with correlations between sexual function, health functionality, and quality of life.

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INTRODUCTION: Blood transfusion is one of the most common medical practices worldwide. However, current scientific literature has shown that the immunomodulatory effects of blood transfusion are associated with an increased likelihood of infection, prolonged hospitalization, and morbimortality. Also, it means high costs for healthcare systems. METHODS: In this context, acknowledging that blood transfusions are essentially heterologous cell transplantations, the use of therapeutic options has gained strength and is collectively known as the patient blood management (PBM) program. PBM is an approach based on three main pillars: (1) treating anemias and coagulopathies in an optimized manner, especially in the preoperative period; (2) optimizing perioperative hemostasis and the use of blood recovery systems to avoid the loss of the patient's blood; (3) anemia tolerance, with improved oxygen delivery and reduced oxygen demand, particularly in the postoperative period. RESULTS: Current scientific evidence supports the effectiveness of PBM by reducing the need for blood transfusions, decreasing associated complications, and promoting more efficient and safer blood management. Thus, PBM not only improves clinical outcomes for patients but also contributes to the economic sustainability of healthcare systems. CONCLUSION: The aim of this review was to summarize PBM strategies in a comprehensive, evidence-based approach through a systematic and structured model for PBM implementation in tertiary hospitals. The recommendations proposed herein are from researchers and experts of a high-complexity university hospital in the network of the Sistema Único de Saúde, presenting itself as a strategy that can be followed as a guideline for PBM implementation in other settings.

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ABSTRACT Introduction: Blood transfusion is one of the most common medical practices worldwide. However, current scientific literature has shown that the immunomodulatory effects of blood transfusion are associated with an increased likelihood of infection, prolonged hospitalization, and morbimortality. Also, it means high costs for healthcare systems. Methods: In this context, acknowledging that blood transfusions are essentially heterologous cell transplantations, the use of therapeutic options has gained strength and is collectively known as the patient blood management (PBM) program. PBM is an approach based on three main pillars: (1) treating anemias and coagulopathies in an optimized manner, especially in the preoperative period; (2) optimizing perioperative hemostasis and the use of blood recovery systems to avoid the loss of the patient's blood; (3) anemia tolerance, with improved oxygen delivery and reduced oxygen demand, particularly in the postoperative period. Results: Current scientific evidence supports the effectiveness of PBM by reducing the need for blood transfusions, decreasing associated complications, and promoting more efficient and safer blood management. Thus, PBM not only improves clinical outcomes for patients but also contributes to the economic sustainability of healthcare systems. Conclusion: The aim of this review was to summarize PBM strategies in a comprehensive, evidence-based approach through a systematic and structured model for PBM implementation in tertiary hospitals. The recommendations proposed herein are from researchers and experts of a high-complexity university hospital in the network of the Sistema Único de Saúde, presenting itself as a strategy that can be followed as a guideline for PBM implementation in other settings.

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COVID-19's severity has been associated with a possible imbalance in the cross-regulation of cytokines and vascular mediators. Since the beginning of the pandemic, kidney transplant recipients (KTRs) have been identified as patients of high vulnerability to more severe diseases. Thus, aiming to describe the patterns of cytokines and vascular mediators and to trace patients' differences according to their KTR status, this prospective study enrolled 67 COVID-19 patients (20 KTRs) and 29 non-COVID-19 controls before vaccination. A panel comprising 17 circulating cytokines and vascular mediators was run on samples collected at different time points. The cytokine and mediator patterns were investigated via principal component analysis (PCA) and correlation-based network (CBN). In both groups, compared to their respective controls, COVID-19 was associated with higher levels of cytokines and vascular mediators. Differentiating between the KTRs and non-KTRs, the number of correlations was much higher in the non-KTRs (44 vs. 14), and the node analysis showed the highest interactions of NGAL and sVCAM-1 in the non-KTRs and KTRs (9 vs. 4), respectively. In the PCA, while the non-KTRs with COVID-19 were differentiated from their controls in their IL-10, IFN-α, and TNF-α, this pattern was marked in the NGAL, sVCAM-1, and IL-8 of the KTRs.

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OBJECTIVE: To identify factors associated with prolonged weaning and mortality in critically ill COVID-19 patients admitted to ICUs and under invasive mechanical ventilation. METHODS: Between March of 2020 and July of 2021, we retrospectively recorded clinical and ventilatory characteristics of critically ill COVID-19 patients from the day of intubation to the outcome. We classified the patients regarding the weaning period in accordance with established criteria. A logistic regression analysis was performed to identify variables associated with prolonged weaning and mortality. RESULTS: The study involved 303 patients, 100 of whom (33.0%) had a prolonged weaning period. Most of the patients were male (69.6%), 136 (44.8%) had more than 50% of pulmonary involvement on chest CT, and 93 (30.6%) had severe ARDS. Within the prolonged weaning group, 62% died within 60 days. Multivariate analysis revealed that lung involvement greater than 50% on CT and delay from intubation to the first separation attempt from mechanical ventilation were significantly associated with prolonged weaning, whereas age and prolonged weaning were significantly associated with mortality. CONCLUSIONS: Prolonged weaning can be used as a milestone in predicting mortality in critically ill COVID-19 patients. Lung involvement greater than 50% on CT and delay from intubation to the first separation attempt from mechanical ventilation were identified as significant predictors of prolonged weaning. These results might provide valuable information for healthcare professionals when making clinical decisions regarding the management of critically ill COVID-19 patients who are on mechanical ventilation.

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BACKGROUND: The neurological manifestations in COVID-19 adversely impact acute illness and post-disease quality of life. Limited data exist regarding the association of neurological symptoms and comorbid individuals. OBJECTIVE: To assess neurological symptoms in hospitalized patients with acute COVID-19 and multicomorbidities. METHODS: Between June 2020 and July 2020, inpatients aged 18 or older, with laboratory-confirmed COVID-19, admitted to the Hospital São Paulo (Federal University of São Paulo), a tertiary referral center for high complexity cases, were questioned about neurological symptoms. The Composite Autonomic Symptom Score 31 (COMPASS-31) questionnaire was used. The data were analyzed as a whole and whether subjective olfactory dysfunction was present or not. RESULTS: The mean age of the sample was 55 ± 15.12 years, and 58 patients were male. The neurological symptoms were mostly xerostomia (71%), ageusia/hypogeusia (50%), orthostatic intolerance (49%), anosmia/hyposmia (44%), myalgia (31%), dizziness (24%), xerophthalmia (20%), impaired consciousness (18%), and headache (16%). Furthermore, 91% of the patients had a premorbidity. The 44 patients with subjective olfactory dysfunction were more likely to have hypertension, diabetes, weakness, shortness of breath, ageusia/hypogeusia, dizziness, orthostatic intolerance, and xerophthalmia. The COMPASS-31 score was higher than that of previously published controls (14.85 ± 12.06 vs. 8.9 ± 8.7). The frequency of orthostatic intolerance was 49% in sample and 63.6% in those with subjective olfactory dysfunction (2.9-fold higher risk compared to those without). CONCLUSION: A total of 80% of inpatients with multimorbidity and acute COVID-19 had neurological symptoms. Chemical sense and autonomic symptoms stood out. Orthostatic intolerance occurred in around two-thirds of the patients with anosmia/hyposmia. Hypertension and diabetes were common, mainly in those with anosmia/hyposmia.

ANTECEDENTES: As manifestações neurológicas na COVID-19 impactam adversamente na enfermidade aguda e na qualidade de vida após a doença. Dados limitados existem em relação a associação de sintomas neurológicos e indivíduos com comorbidades. OBJETIVO: Avaliar os sintomas neurológicos em pacientes de hospitalizados com COVID-19 aguda e múltiplas comorbidades. MéTODOS: Entre junho e julho de 2020, pacientes de hospitais com idade 18 anos ou acima e COVID-19 laboratorialmente confirmada, admitidos no Hospital São Paulo (Universidade Federal de São Paulo), um centro de referência terciário para casos de alta complexidade, foram perguntados sobre sintomas neurológicos. O questionário Pontuação composta de sintoma autonômico (COMPASS-31) foi usado. Os dados foram analisados no geral e se a disfunção olfatória subjetiva estava presente ou não. RESULTADOS: A média de idade da amostra foi 55 ± 15.12 anos. 58 pacientes eram homens. Os sintomas neurológicos foram principalmente xerostomia (71%), ageusia/hipogeusia (50%), intolerância ortostática (49%), anosmia/hiposmia (44%), mialgia (31%), tontura (24%), xeroftalmia (20%), comprometimento na consciência (18%) e cefaleia (16%). Além disso, 91% dos pacientes tinham uma pré-morbidade. Os 44 pacientes com disfunção olfatória tinham maior chance de ter hipertensão, diabetes, fraqueza, falta de ar, ageusia/hipogeusia, tontura, intolerância ortostática e xeroftalmia. A pontuação do COMPASS-31 foi maior do que a de controles previamente publicados (14,85 ± 12,06 vs. 8,9 ± 8,7). A frequência de intolerância ortostática foi 49% na amostra e 63,6% naqueles com disfunção olfatória subjetiva (risco 2.9 vezes maior comparado com os sem). CONCLUSãO: Um total de 80% dos pacientes hospitalizados com múltiplas morbidades e COVID-19 aguda tinham sintomas neurológicos. Os sintomas do sentido químico e autonômicos se destacaram. A intolerância ortostática ocorreu em cerca de dois terços dos pacientes com anosmia/hiposmia. A hipertensão e o diabetes foram comuns, principalmente naqueles com anosmia/hiposmia.

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ABSTRACT Objective: To identify factors associated with prolonged weaning and mortality in critically ill COVID-19 patients admitted to ICUs and under invasive mechanical ventilation. Methods: Between March of 2020 and July of 2021, we retrospectively recorded clinical and ventilatory characteristics of critically ill COVID-19 patients from the day of intubation to the outcome. We classified the patients regarding the weaning period in accordance with established criteria. A logistic regression analysis was performed to identify variables associated with prolonged weaning and mortality. Results: The study involved 303 patients, 100 of whom (33.0%) had a prolonged weaning period. Most of the patients were male (69.6%), 136 (44.8%) had more than 50% of pulmonary involvement on chest CT, and 93 (30.6%) had severe ARDS. Within the prolonged weaning group, 62% died within 60 days. Multivariate analysis revealed that lung involvement greater than 50% on CT and delay from intubation to the first separation attempt from mechanical ventilation were significantly associated with prolonged weaning, whereas age and prolonged weaning were significantly associated with mortality. Conclusions: Prolonged weaning can be used as a milestone in predicting mortality in critically ill COVID-19 patients. Lung involvement greater than 50% on CT and delay from intubation to the first separation attempt from mechanical ventilation were identified as significant predictors of prolonged weaning. These results might provide valuable information for healthcare professionals when making clinical decisions regarding the management of critically ill COVID-19 patients who are on mechanical ventilation.

RESUMO Objetivo: Identificar fatores associados ao desmame prolongado e à mortalidade em pacientes críticos com COVID-19 admitidos em UTI e sob ventilação mecânica invasiva. Métodos: Entre março de 2020 e julho de 2021, registramos retrospectivamente as características clínicas e ventilatórias de pacientes críticos com COVID-19 desde o dia da intubação até o desfecho. Os pacientes foram classificados quanto ao período de desmame de acordo com critérios estabelecidos. Foi realizada análise de regressão logística para identificar variáveis associadas ao desmame prolongado e à mortalidade. Resultados: O estudo incluiu 303 pacientes, 100 dos quais (33,0%) apresentaram período de desmame prolongado. A maioria dos pacientes era do sexo masculino (69,6%), 136 (44,8%) apresentaram mais de 50% de acometimento pulmonar na TC de tórax, e 93 (30,6%) apresentaram SDRA grave. No grupo desmame prolongado, 62% foram a óbito em 60 dias. A análise multivariada revelou que o acometimento pulmonar maior que 50% na TC e a demora na primeira tentativa de retirada da ventilação mecânica após a intubação apresentaram associação significativa com o desmame prolongado, enquanto a idade e o desmame prolongado apresentaram associação significativa com a mortalidade. Conclusões: O desmame prolongado pode ser utilizado como marco na predição de mortalidade em pacientes críticos com COVID-19. O acometimento pulmonar maior que 50% na TC e a demora na primeira tentativa de retirada da ventilação mecânica após a intubação foram identificados como preditores significativos de desmame prolongado. Esses resultados podem fornecer informações valiosas para os profissionais de saúde na tomada de decisões clínicas sobre o manejo de pacientes críticos com COVID-19 e em ventilação mecânica.

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Abstract Background The neurological manifestations in COVID-19 adversely impact acute illness and post-disease quality of life. Limited data exist regarding the association of neurological symptoms and comorbid individuals. Objective To assess neurological symptoms in hospitalized patients with acute COVID-19 and multicomorbidities. Methods Between June 2020 and July 2020, inpatients aged 18 or older, with laboratory-confirmed COVID-19, admitted to the Hospital São Paulo (Federal University of São Paulo), a tertiary referral center for high complexity cases, were questioned about neurological symptoms. The Composite Autonomic Symptom Score 31 (COMPASS-31) questionnaire was used. The data were analyzed as a whole and whether subjective olfactory dysfunction was present or not. Results The mean age of the sample was 55 ± 15.12 years, and 58 patients were male. The neurological symptoms were mostly xerostomia (71%), ageusia/hypogeusia (50%), orthostatic intolerance (49%), anosmia/hyposmia (44%), myalgia (31%), dizziness (24%), xerophthalmia (20%), impaired consciousness (18%), and headache (16%). Furthermore, 91% of the patients had a premorbidity. The 44 patients with subjective olfactory dysfunction were more likely to have hypertension, diabetes, weakness, shortness of breath, ageusia/hypogeusia, dizziness, orthostatic intolerance, and xerophthalmia. The COMPASS-31 score was higher than that of previously published controls (14.85 ± 12.06 vs. 8.9 ± 8.7). The frequency of orthostatic intolerance was 49% in sample and 63.6% in those with subjective olfactory dysfunction (2.9-fold higher risk compared to those without). Conclusion A total of 80% of inpatients with multimorbidity and acute COVID-19 had neurological symptoms. Chemical sense and autonomic symptoms stood out. Orthostatic intolerance occurred in around two-thirds of the patients with anosmia/hyposmia. Hypertension and diabetes were common, mainly in those with anosmia/hyposmia.

Resumo Antecedentes As manifestações neurológicas na COVID-19 impactam adversamente na enfermidade aguda e na qualidade de vida após a doença. Dados limitados existem em relação a associação de sintomas neurológicos e indivíduos com comorbidades. Objetivo Avaliar os sintomas neurológicos em pacientes de hospitalizados com COVID-19 aguda e múltiplas comorbidades. Métodos Entre junho e julho de 2020, pacientes de hospitais com idade 18 anos ou acima e COVID-19 laboratorialmente confirmada, admitidos no Hospital São Paulo (Universidade Federal de São Paulo), um centro de referência terciário para casos de alta complexidade, foram perguntados sobre sintomas neurológicos. O questionário Pontuação composta de sintoma autonômico (COMPASS-31) foi usado. Os dados foram analisados no geral e se a disfunção olfatória subjetiva estava presente ou não. Resultados A média de idade da amostra foi 55 ± 15.12 anos. 58 pacientes eram homens. Os sintomas neurológicos foram principalmente xerostomia (71%), ageusia/hipogeusia (50%), intolerância ortostática (49%), anosmia/hiposmia (44%), mialgia (31%), tontura (24%), xeroftalmia (20%), comprometimento na consciência (18%) e cefaleia (16%). Além disso, 91 % dos pacientes tinham uma pré-morbidade. Os 44 pacientes com disfunção olfatória tinham maior chance de ter hipertensão, diabetes, fraqueza, falta de ar, ageusia/hipogeusia, tontura, intolerância ortostática e xeroftalmia. A pontuação do COMPASS-31 foi maior do que a de controles previamente publicados (14,85 ± 12,06 vs. 8,9 ± 8,7). A frequência de intolerância ortostática foi 49% na amostra e 63,6% naqueles com disfunção olfatória subjetiva (risco 2.9 vezes maior comparado com os sem). Conclusão Um total de 80% dos pacientes hospitalizados com múltiplas morbidades e COVID-19 aguda tinham sintomas neurológicos. Os sintomas do sentido químico e autonômicos se destacaram. A intolerância ortostática ocorreu em cerca de dois terços dos pacientes com anosmia/hiposmia. A hipertensão e o diabetes foram comuns, principalmente naqueles com anosmia/hiposmia.

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A machine learning approach is a useful tool for risk-stratifying patients with respiratory symptoms during the COVID-19 pandemic, as it is still evolving. We aimed to verify the predictive capacity of a gradient boosting decision trees (XGboost) algorithm to select the most important predictors including clinical and demographic parameters in patients who sought medical support due to respiratory signs and symptoms (RAPID RISK COVID-19). A total of 7336 patients were enrolled in the study, including 6596 patients that did not require hospitalization and 740 that required hospitalization. We identified that patients with respiratory signs and symptoms, in particular, lower oxyhemoglobin saturation by pulse oximetry (SpO2) and higher respiratory rate, fever, higher heart rate, and lower levels of blood pressure, associated with age, male sex, and the underlying conditions of diabetes mellitus and hypertension, required hospitalization more often. The predictive model yielded a ROC curve with an area under the curve (AUC) of 0.9181 (95% CI, 0.9001 to 0.9361). In conclusion, our model had a high discriminatory value which enabled the identification of a clinical and demographic profile predictive, preventive, and personalized of COVID-19 severity symptoms.

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Abstract Background: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis. Objective: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS. Methods: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05). Results: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09). Conclusion: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

Resumo Fundamento: A síndrome hepatopulmonar (SHP), presente em pacientes cirróticos, é pouco estudada na esquistossomose hepatoesplênica (EHE) e inclui a ocorrência de dilatações vasculares intrapulmonares (DVP). O ecocardiograma transesofágico com contraste (ETEc) de microbolhas é mais sensível que o ecocardiograma transtorácico com contraste (ETTc) de microbolhas na identificação de DVP na cirrose. Objetivo: Avaliar o desempenho do ETEc comparado ao ETTc na identificação de DVP para diagnóstico de SHP em pacientes com EHE. Métodos: Incluímos 22 pacientes com EHE submetidos a ETEc e ETTc para pesquisa de DVP, além de exames laboratoriais. Os ETEc e ETTc foram realizados empregando-se solução salina agitada, injetada em veia periférica. A visualização tardia das microbolhas em câmaras esquerdas indicava presença de DVP. Os resultados foram comparados entre os dois métodos pelos testes t de Stu dent e qui-quadrado (significância p < 0,05). Resultados: Todos os 22 pacientes realizaram ETEc sem intercorrências. Foram excluídos três pela presença de forame oval patente (FOP), e a análise final foi realizada nos outros 19. A DVP esteve presente ao ETEc em 13 pacientes (68%) e em apenas seis ao ETTc (32%, p < 0,01). Não houve diferenças significativas nos dados clínicos e laboratoriais entre os grupos com e sem DVP, incluindo a diferença alveoloarterial de oxigênio. O diagnóstico de SHP (presença de DVP com alterações gasométricas) ocorreu em cinco pacientes pelo ETEc e em apenas um pelo ETTc (p = 0,09). Conclusão: Em pacientes com EHE, o ETEc foi seguro e superior ao ETTc na detecção de DVP não identificada ao ETTc, o que possibilitou adicionalmente excluir FOP.

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BACKGROUND: Hepatopulmonary syndrome (HPS), found in cirrhotic patients, has been little studied in hepatosplenic schistosomiasis (HSS) and includes the occurrence of intrapulmonary vascular dilatations (IPVD). Contrast transesophageal echocardiography (cTEE) with microbubbles is more sensitive than contrast transthoracic echocardiography (cTTE) with microbubbles in the detection of IPVD in cirrhosis. OBJECTIVE: To assess the performance of the cTEE, compared with that of cTTE, in detecting IPVD for the diagnosis of HPS in patients with HSS. METHODS: cTEE and cTTE for investigation of IPVD and laboratory tests were performed in 22 patients with HSS. Agitated saline solution was injected in peripheral vein during the cTEE and cTTE procedures. Late appearance of the microbubbles in the left chambers indicated the presence of IPVD. Results of the two methods were compared by the Student's t-test and the chi-square test (p < 0.05). RESULTS: cTEE was performed in all patients without complications. Three patients were excluded due to the presence of patent foramen ovale (PFO). The presence of IPVD was confirmed in 13 (68%) of 19 patients according to the cTEE and in only six (32%, p < 0.01) according to the cTTE. No significant differences in clinical or laboratory data were found between the groups with and without IPVD, including the alveolar-arterial gradient. The diagnosis of HPS (presence of IPVD with changes in the arterial blood gas analysis) was made in five patients by the cTEE and in only one by the cTTE (p = 0.09). CONCLUSION: In HSS patients, cTEE was safe and superior to cTTE in detecting IPVD and allowed the exclusion of PFO.

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A hipertensão pulmonar é um estado hemodinâmico e fisiopatológico que pode ser encontrado em diversas condições clínicas e quando presente, está associada ao aumento da morbimortalidade. Somados à etiologia diversa estão os achados clínicos inespecíficos. Esses dois aspectos contribuem para o atraso no diagnóstico e, por conseguinte, no início do tratamento. Daí a importância da sistematização da avaliação diagnóstica aliada à suspeição clínica na investigação de dispneia, sobretudo nas populações de risco, visando o diagnóstico etiológico correto e a instituição de medidas terapêuticas adequadas. O seguimento desses pacientes deverá ser feito também de forma sistemática, associado a ferramentas complementares que permitam a avaliação adequada da gravidade da doença...

Pulmonary hypertension is a hemodynamic and pathophysiological state that can be find in various clinical conditions and is associated with increased morbidity and mortality. As symptoms are nonspecific and the condition is uncommon, usually the diagnosis is delayed and, consequently, the start of treatment.Hence, the importance of systematization of diagnostic evaluation combined with clinical suspicion in dyspnea research, especially in at-risk populations, in order to achieve the correct etiologic diagnosis and institution of appropriate therapeutic measures. The follow-up of these patients also should be do systematically, together with complementary tools that allow the proper assessment of disease severity...

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Grandes avanços têm sido observados no conhecimento da patogenia da Hipertensão Arterial Pulmonar. A vasoconstrição, a disfunção endotelial, a proliferação da camada íntima e a hiperplasia e/ou hipertrofia da camada média muscular, assim como a trombose in situ, são os principais mecanismos aceitos na gênese da HAP. Novas drogas surgiram nos últimos anos, mudando a evolução natural desta condição. Atualmente, existem 10 medicamentos autorizados pelas agências regulatórias mundiais de saúde para o tratamento da HAP: os derivados da prostaciclina (beraprost, epoprostenol, iloprost e treprostinil); os antagonistas dos receptores da endotelina (ambrisentana, bosentana e macitentan); os inibidores da fosfodiesterase-5 (sildenafil e tadalafila) e o estimulador da guanilato ciclase solúvel (riociguate). Resultados de metanálises e de estudos clínicos controlados e randomizados sugerem que estes medicamentos reduziram a mortalidade em aproximadamente 43% nos grupos tratados em relação ao placebo. Porém, numa coorte de pacientes com HAP incluídos no Registro Francês de Hipertensão Pulmonar no período de 2002 a 2003 e acompanhados por 3 anos, observou-se que a doença ainda é progressiva, ressaltando as limitações existentes no manejo atual do paciente com hipertensão pulmonar...

Great advances has been observed in understanding the pathogenesis of Pulmonary Arterial Hypertension. Vasoconstriction, endothelial dysfunction, proliferation and intimal hyperplasia and / or hypertrophy of muscle middle layer, and in situ thrombosis are major mechanisms in the accepted genesis of HAP. New drugs have emerged in recent years, changing the natural history of this condition. Currently, there are 10 drugs approved by worldwide regulatory health agencies for the treatment of PAH: derived prostacyclin (beraprost, epoprostenol, iloprost and treprostinil); antagonists of endothelin receptors (ambrisentan, bosentan and macitentan); phosphodiesterase-5 inhibitors (sildenafil and tadalafil) and the stimulator of soluble guanylate cyclase (riociguate). Results of metanalysis and randomized controlled trials suggest that these drugs have reduced mortality in about 43% in the treated groups compared to placebo groups. But a cohort of patients with PAH included in the French Registration Pulmonary Hypertension between 2002 and 2003 followed for 3 years, showed that the disease still is progressive, emphasizing the limitations in the current management of the patient with PAH....

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Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

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As doenças pulmonares intersticiais (DPIs) são afecções heterogêneas, envolvendo um elevado número de condições, cuja abordagem ainda é um grande desafio para o pneumologista. As Diretrizes de DPIs da Sociedade Brasileira de Pneumologia e Tisiologia, publicadas em 2012, foram estabelecidas com o intuito de fornecer aos pneumologistas brasileiros um instrumento que possa facilitar a abordagem dos pacientes com DPIs, padronizando-se os critérios utilizados para a definição diagnóstica das diferentes condições, além de orientar sobre o melhor tratamento nas diferentes situações. Esse artigo teve como objetivo descrever resumidamente os principais destaques dessas diretrizes.

Interstitial lung diseases (ILDs) are heterogeneous disorders, involving a large number of conditions, the approach to which continues to pose an enormous challenge for pulmonologists. The 2012 Brazilian Thoracic Association ILD Guidelines were established in order to provide Brazilian pulmonologists with an instrument that can facilitate the management of patients with ILDs, standardizing the criteria used for the diagnosis of different conditions and offering guidance on the best treatment in various situations. The objective of this article was to briefly describe the highlights of those guidelines.

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Hipertensão arterial pulmonar (HAP) é uma doença rara causada pela proliferação vascular e remodelamento, resultando no aumento progressivo da resistência vascular pulmonar e disfunção ventricular direita. Apesar de recentes avanços terapêuticos, essa doença é ainda grave e rapidamente progressiva. Existem, atualmente, três classes principais de drogas que podem ser utilizadas para o tratamento da HAP: prostanoides, antagonistas dos receptores de endotelina e inibidores da fosfodiesterase-5. Nessa revisão, discutiremos o tratamento de suporte nessa população de doentes, assim como as drogas específicas atualmente disponíveis.

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A tromboembolia pulmonar constitui, juntamente com a trombose venosa profunda, a condição denominada tromboembolismo venoso. Apesar dos avanços, a morbidade e a mortalidade atribuídas a essa doença ainda são elevadas, pois os pacientes apresentam doenças mais complexas, são submetidos a um maior número de procedimentos invasivos e sobrevivem por mais tempo. Embora existam inúmeras diretrizes internacionais disponíveis, optou-se por redigir estas recomendações para sua aplicação na prática médica nacional, embasadas nas melhores evidências na literatura e na opinião do grupo de consultores. Este documento é apenas uma ferramenta para o atendimento dos pacientes, e, embora possa ser aplicado na maioria das situações, o médico deve adaptar as informações a sua realidade local e ao caso específico. O diagnóstico de tromboembolia pulmonar é realizado através da combinação da probabilidade clínica pré-teste (escores) com o resultado dos exames de imagem, sendo atualmente o método de eleição a angiotomografia computadorizada. É fundamental a estratificação do risco de desfecho desfavorável, sendo a instabilidade hemodinâmica o preditor mais importante. Pacientes de baixo risco devem ser tratados com heparina, comumente as de baixo peso molecular. Pacientes de alto risco requerem vigilância intensiva e uso de trombolíticos em alguns casos. A longo prazo, os pacientes devem receber anticoagulantes por no mínimo três meses, sendo sua manutenção decidida pela presença de fatores de risco para a recorrência e a probabilidade de sangramento. A profilaxia é altamente eficaz e deve ser amplamente utilizada, tanto em pacientes clínicos como cirúrgicos, conforme os grupos de risco. Finalmente, são feitas recomendações relacionadas ao diagnóstico, tratamento e prevenção da tromboembolia pulmonar.

Pulmonary thromboembolism and deep vein thrombosis together constitute a condition designated venous thromboembolism. Despite the advances, the morbidity and the mortality attributed to this condition are still high, because the patients present with more complex diseases, are submitted to a greater number of invasive procedures and survive longer. Although there are various international guidelines available, we decided to write these recommendations for their application in medical practice in Brazil. These recommendations are based on the best evidence in the literature and the opinion of the advisory committee. This document is only a tool for use in the management of patients. Although the recommendations it contains can be applied to most situations, physicians should adapt its content depending on their local context and on a case-by-case basis. Pulmonary thromboembolism is diagnosed by evaluating pre-test clinical probability (scores) together with the results of imaging studies, the current method of choice being CT angiography. Stratification of the risk for an unfavorable outcome is fundamental. Hemodynamic instability is the most important predictor. Low-risk patients should be treated with heparin, commonly low-molecular-weight heparins. High-risk patients require intensive monitoring and, in some cases, thrombolytic therapy. In the long term, patients should receive anticoagulants for at least three months. The decision to prolong this treatment is made based on the presence of risk factors for the recurrence of the condition and the probability of bleeding. Prophylaxis is highly effective and should be widely used in clinical and surgical patients alike, according to their risk group. Finally, we include recommendations regarding the prevention, diagnosis and treatment of pulmonary thromboembolism.

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Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia with airflow obstruction is a rare form of lung injury. All of the reported cases have been diagnosed by surgical lung biopsy. Only three of the reported cases presented with diffuse interstitial lung opacities on HRCT scans. We report three additional cases of this entity. All of the patients were female and presented with mild-to-moderate airflow obstruction. In the first case, transbronchial biopsy and imaging data were sufficient to make the diagnosis. Although the HRCT scans of all three cases revealed a mosaic pattern, that of the third patient also revealed diffuse interstitial infiltrate. In extremely rare cases, HRCT findings can simulate those seen in other interstitial lung diseases.

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