Loss of response to calcium channel blockers after long-term follow-up treatment in patients with idiopathic pulmonary arterial hypertension / Perda de resposta a bloqueadores dos canais de cálcio após acompanhamento prolongado em pacientes com hipertensão arterial pulmonar idiopática

ABSTRACT Idiopathic pulmonary arterial hypertension (PAH) patients with a positive response to acute vasodilator challenge and a clinical response to calcium channel blockers (CCBs) for at least one year are traditionally designated true responders. Nevertheless, little is known about a sustained response to CCBs over longer periods of time. We evaluated the loss of response to CCBs after long-term treatment in a cohort of idiopathic PAH patients previously classified as being true responders. Our data suggest that idiopathic PAH patients can lose clinical response to CCBs even after one year of clinical stability, reinforcing the need for constant multidimensional reevaluation to assess the need for targeted PAH therapies and to classify these patients correctly.

RESUMO Pacientes com hipertensão arterial pulmonar (HAP) idiopática com resposta positiva ao teste de vasorreatividade aguda e resposta clínica a bloqueadores dos canais de cálcio (BCC) durante no mínimo um ano são tradicionalmente denominados "respondedores verdadeiros". No entanto, pouco se sabe sobre a manutenção da resposta a BCC durante períodos mais longos. Avaliamos a perda de resposta a BCC após tratamento prolongado em uma coorte de pacientes com HAP idiopática previamente considerados respondedores verdadeiros. Nossos dados sugerem que pacientes com HAP idiopática podem deixar de apresentar resposta clínica a BCC mesmo depois de um ano de estabilidade clínica, reforçando a necessidade de reavaliação multidimensional constante para avaliar a necessidade de terapias específicas para HAP e classificar esses pacientes corretamente.

Brazilian Thoracic Society recommendations for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.

Is there a precise correlation between the lengths of the peritoneal reflection and the middle rectal valve?

Introduction: Pelvic anatomy remains a challenge, and thorough knowledge of its intricate landmarks has major clinical and surgical implications in several medical specialties. The peritoneal reflection is an important landmark in intraluminal surgery, rectal trauma, impalement, and rectal adenocarcinoma. Objectives: To investigate the correlation between the lengths of the middle rectal valve and of the peritoneal reflection determined with rigid sigmoidoscopy and to determine whether there are any differences in the location of the peritoneal reflection between the genders and in relation to body mass index (BMI) and parity. Design: We prospectively investigated the location of the middle rectal valve and of the peritoneal reflection via intraoperative rigid sigmoidoscopy in colorectal cancer patients undergoing elective colorectal surgery. Results: We evaluated 38 patients with a mean age of 55.5 years old (57.5% males) who underwent colorectal surgery at the coloproctology service of the Hospital Santa Marcelina, São Paulo, state of São Paulo, Brazil. There was substantial agreement between the lengths of the middle rectal valve and of the peritoneal reflection (Kappa = 0.66). In addition, the peritoneal reflection was significantly lower in overweight patients (p = 0.013 for women and p < 0.005 for men) and in women with > 2 vaginal deliveries (p = 0.009), but there was no significant difference in the length of the peritoneal reflection between genders (p = 0.32). Conclusion: There was substantial agreement between the lengths of the peritoneal reflection and of the middle rectal valve, and the peritoneal reflection was significantly lower in overweight patients and in women with more than two vaginal deliveries. (AU)

Is fatigue associated with balance in Parkinson's disease?

Abstract Background: Fatigue is a disabling symptom in the spectrum of Parkinson's disease (PD), affecting from 30% to 70% of the persons. Even though it is a common symptom, with negative repercussions for PD individuals, its correlation with balance is not established. Aim: The aims of this study were to verify the correlation between fatigue and balance in individuals with PD and to compare balance in individuals with PD that presents low fatigue or high fatigue. Methods: This study included 37 individuals with PD, who were divided into two groups: low fatigue (n = 25) and high fatigue (n = 12). Fatigue was evaluated using the Parkinson's disease Fatigue Scale and a force platform was used to assess four balance tasks: bipedal, tandem with eyes open/closed, and tandem with the dual-task, in three parameters: (1) 95% confidence ellipse area of the center of pressure (COP) (2) mean velocity (3) root mean square of COP. To verify the correlation between fatigue and balance, the Spearman rank-order correlation coefficient was assessed. Comparison of medians between the groups was analyzed using the Mann-Whitney test. Results: There was no significant correlation between fatigue and balance. There was no difference between the groups with low and high fatigue. Conclusion: This study's findings, together with those reported in the literature, suggest there is no correlation between fatigue and balance, and even though individuals with PD report fatigue or experience situations of fatigue, they do not present greater posture instability than individuals with PD who do not report fatigue. Fatigue is a disabling symptom in the spectrum of Parkinson's disease (PD), affecting from

Atualização no Tratamento da Hipertensão Arterial Pulmonar / Update on the Treatment of Pulmonary Arterial Hypertension

Resumo Muitos avanços ocorreram nas últimas décadas na terapêutica da hipertensão arterial pulmonar (HAP), uma doença grave, progressiva, incurável e potencialmente fatal. Para seu tratamento adequado, são fundamentais o diagnóstico hemodinâmico e a classificação de sua etiologia, em que várias delas (colagenoses, hipertensão portal, cardiopatia congênitas, esquistossomose) requerem medidas específicas, além do tratamento farmacológico característico para HAP. O tratamento com fármacos-alvo para HAP baseia-se em produtos farmacêuticos que interferem em três vias fisiopatológicas moleculares: da prostaciclina, da endotelina e do óxido nítrico. Tais fármacos apresentam múltiplas apresentações (oral, endovenosa, subcutânea e inalatória) e mudaram a história da HAP. Essas medicações e suas estratégias de uso, assim como particularidades das diferentes formas de HAP, são o foco desta revisão.

Abstract In the last decades, important advances have been made in the treatment of pulmonary arterial hypertension (PAH), a severe, progressive, incurable, and potentially fatal disease. For an adequate therapy, correct hemodynamic diagnosis and etiology classification are fundamental. Many etiologies - rheumatic disease, portal hypertension, congenital heart diseases, schistosomiasis - require specific measures, in addition to drug therapy for PAH. The specific therapy for PAH is based on medications that act on three pathophysiological pathways - prostacyclin, endothelin, and nitric oxide pathways. These drugs have multiple presentations (oral, intravenous, subcutaneous, and inhaled) and have changed the history of PAH. This review presents an overview of drug therapy strategies and different forms and peculiarities of PAH.

Testes morphology and the identification of transcripts of the hormonal pathways of the velvetbean caterpillar Anticarsia gemmatalis Hübner, 1818 (Lepidoptera: Erebidae).

Anticarsia gemmatalis is one of the main defoliating pests of soybeans in Brazil. In the current study, we characterized the histomorphology of the testes and the spermatogenesis process in A. gemmatalis. We also identified transcripts involved in the biosynthesis, metabolism, and signaling of juvenile and ecdysteroid hormones, in order to provide information about potential mechanisms of regulation of hormonal pathways in this species. Our analyses revealed that the A. gemmatalis larvae have a pair of kidney-shaped testicles. These are divided into four testicular follicles, where there are germ cell cysts at different stages of development. In the pupal stage, the testicles are fused, so adults have a single spherical testis, with a variable number of follicles. The A. gemmatalis has centripetal spermatogenesis and exhibits spermatic dimorphism. We identified 31 transcripts that encode proteins involved in juvenile hormone and ecdysteroid pathways, such as mevalonate kinase, CYP14A1, ecdysone receptor, among others. Our results on the morphology of the testes and spermatogenesis process, as well as identification of the genes involved in hormonal pathways in A. gemmatalis, provide important data for understanding the biology of this agricultural pest, which can be used as a basis for further research in other economically important lepidopterans.

Antioxidant activity and chemical composition of meat from broilers fed diets containing different essential oils.

BACKGROUND AND AIM: Broiler meat is susceptible to lipid oxidation due to its high content of unsaturated fatty acids, especially when stored for a long time. Concerning with that problem, we can add natural compounds to the animal feed, as the plant essential oils, which have antioxidant, antimicrobial, and antifungal activities. The objective of this study was to evaluate the antioxidant activity, fatty acid profile, and chemical composition of the meat of broilers fed with diets containing different essential oil, including lemongrass (Cymbopogon flexuosus) and pedestrian tea (Lippia aff. rotundifolia). MATERIALS AND METHODS: The experimental design used was a 4×4 factorial scheme (storage time×diets), and each treatment was repeated thrice. The treatments were applied as negative control (without antimicrobial); positive control (ration supplemented with 10 ppm of enramycin and 42 ppm of salinomycin), lemongrass (ration with 120 mg of essential oil of lemongrass for each kilogram of live weight of the animals), and pedestrian tea (ration with 120 mg of essential oil of pedestrian tea for each kilogram of live weight of the animals). Storage was performed after slaughter and evaluated monthly for 4 months. RESULTS: The inclusion of lemongrass oil and pedestrian tea in the broiler diet did not change the ether extract content of the meat sample obtained from thigh and drumstick. The storage time promotes an increase in the moisture loss of the meat from the 2nd month in all treatments. There was an interaction between the additive and storage time for moisture loss. In the 2nd month of storage, the meat from broilers fed the antibiotic-containing feed had a lower moisture loss than those from broilers in the other treatment groups. The fatty acid profile did not differ statistically between the different treatment groups. The inclusion of additives in the food dropped the lipid oxidation at the 2nd month of storage. From the 3rd month, however, only the essential oils showed antioxidant effect. CONCLUSION: Antioxidant activity was positive for treatments that included essential oils. The lemongrass oil and pedestrian tea can be used as antioxidant additives in broiler diets to improve the oxidative stability of thigh-drumstick meat during storage. The results of the study suggest a good prospective for further research with profitability of usage essentials oils examined, for their economical application as feed components in broiler nutrition.

New detection of the occurrence of trypanosomiasis in a bovine herd in a rural property in the state of Bahia/BA ­ case report / Nova detecção da ocorrência de tripanossomíase em rebanho bovino em propriedade rural no estado da Bahia/BA ­ relato de caso

This report aims to discuss the occurrence of protozoan infestation in a Girolanda cattle herd in the state of Bahia / BA. Bovine trypanosomiasis is a severe disease that is harmful to cattle, inducing large productive and economic losses. The animals are affected by protozoa of the genus Trypanosoma spp., giving rise to infestations in the vast majority of them in a silent and lasting way. In the Bahian rural property described, 10% of adult cows died and the rest of the animals showed clinical signs of anemia, drop in milk production, low body score, apathy, among others. Among the information obtained through the farm manager, who helped in the diagnosis and in ordering tests for the respective disease were: purchase of cattle from the region of the state of Minas Gerais and dubious management of oxytocin for improvements in milk production (suspected shared needles). Faced with the suspicion and in conjunction with the information obtained, laboratory tests and molecular tests were carried out to confirm the suspicion. After the examinations, it was found that the animals were affected by protozoa of the genus Trypanossoma vivax. The animals were treated with volume replenishers and drugs such as isometamidium chloride in a single dose calculated according to the body weight.

O presente relato tem por objetivo discorrer sobre a ocorrência da infestação protozoária em um rebanho bovino da raça Girolanda no estado da Bahia/BA. A tripanossomíase bovina é uma doença severa e prejudicial aos bovinos, induzindo-os a grandes perdas produtivas e econômicas. Os animais são afetados por protozoários do gênero Trypanosoma spp., dandoorigem a infestações em sua grande maioria de forma silenciosa e duradoura. Na propriedade rural baiana descrita, 10% das vacas adultas morreram e o restante dos animais apresentava sinais clínicos de anemia, queda da produção leiteira, baixo escore corpóreo, apatia, prostração, entre outros. Dentre as informações obtidas através do gerente da fazenda, que auxiliaram no diagnóstico e na requisição de exames para a respectiva doença estavam: compra de bovinos da região do estado de Minas Gerais e manejo duvidoso de ocitocina para melhorias na produção leiteira (suspeita de agulhas compartilhadas). Diante da suspeita e em conjunto com informações obtidas, foram realizados exames laboratoriais e testes moleculares para confirmar a suspeita. Após a realização dos exames, constatou-se que os animais estavam acometidos por protozoários do gênero Trypanossoma vivax. Os animais foram tratados com repositores de volume e fármacos como o cloreto de isometamidium em uma única dose calculada de acordo com o peso corpóreo.

Association between pulmonary artery to aorta diameter ratio with pulmonary hypertension and outcomes in diffuse cystic lung diseases.

ABSTRACT: To investigate the importance of pulmonary vascular measurements on computed tomography (CT) in predicting pulmonary hypertension (PH) and worse outcomes in diffuse cystic lung diseases (DCLDs).We conducted a cross-sectional study of patients with DCLDs. Patients underwent pulmonary function tests, a six-minute walk test (6MWT), chest CT, transthoracic echocardiography, and right heart catheterization. Pulmonary artery (PA) diameter and PA-ascending aorta ratio (PA-Ao ratio) were obtained from CT. Mean pulmonary artery pressure (mPAP) from right heart catheterization was correlated with tomographic, functional, and echocardiographic variables. The association between the PA-Ao ratio with outcomes was determined by Kaplan-Meier curves.Thirty-four patients were included (18 with pulmonary Langerhans cell histiocytosis and 16 with lymphangioleiomyomatosis, mean age 46 ±â€Š9 years). Forced expiratory volume in the first second and lung diffusing capacity for carbon monoxide were 47 ±â€Š20% and 38 ±â€Š21% predicted, respectively. PA diameter and PA-Ao ratio were 29 ±â€Š6 mm and 0.95 ±â€Š0.24, respectively. PA-Ao ratio > 1 occurred in 38.2% of patients. PA-Ao ratio was a good predictor of PH. mPAP correlated best with PA-Ao ratio, PA diameter, oxygen desaturation during six-minute walk test, and echocardiographic variables. Patients with PA-Ao ratio > 1 had greater mPAP, and a higher risk of death or lung transplantation (log-rank, P < .001) than those with PA-Ao ratio ≤ 1.The PA-Ao ratio measured on CT scan has a potential role as a non-invasive tool to predict the presence of PH and as a prognostic parameter in patients with DCLDs.

Performance of brazilian university handball players in the vertical jump after maximum strength training

This study aimed to analyze the performance of the vertical jump with counter movement (CMJ), in university handball athletes, immediately after the maximum strength training in multiple series and after 10 and 15min of rest. Twelve male athletes participated, age 20.9±2.4 years old, height 1.78±0.05m and Body Mass Index (BMI) 28.74±8.1kg/m2. After the tests and retests of 1 repetition maximum (1RM) for the dominant and non-dominant sides, in the leg extension, the volunteers performed crossover after 48 hours of the training sessions. CMJ measurements were collected before and immediately after each training protocol and after 10 and 15min of rest. Passive recovery between sets was three minutes. The intervals between training protocols were at least 45min. There were no statistical differences for the CMJ between the moments pre-, post-training session, 10 and 15min of rest in the different protocols, but progressive increases in the CMJ variables were observed, extending up to the 15th minute of rest, in all training protocols when comparing the results of the means of the CMJ variables of the moments post-training session, 10 and 15min of rest with the averages of the variables from the pre-training session, being more evident in the alternate unilateral protocol, which, unlike the other protocols of the study, performed series with the two lower limbs. It was evidenced that the training protocols of short duration and with high dynamic efforts, previously performed, influenced the performance of the CMJ, favoring for the transient improvement of the explosive muscular strength of the athletes.

Medical students with performance difficulties need wide support: Initial results of an academic tutoring program

BACKGROUND: Even students with previous academic success may face challenges that affect their academic performance. Many medical schools offer programs to students at the risk of academic failure, to ensure that they succeed in the course. OBJECTIVE AND METHODS: In this report we describe a pioneering academic tutoring program developed at a Brazilian medical school and discuss the initial results of the program based on the feedback from tutors and data regarding the progression of students in the medical course. RESULTS: In 2018, 33 students enrolled into the program. Students' performance difficulties were mainly associated with mental health problems and socioeconomic vulnerability. Of the 33 students, 27 (81.8%) were assisted by the Mental Health Support Service and 16 (48.5%) were assisted by the Social Assistance Service. In addition to the planning academic activity class load, tutors were able to assist students in solving socioeconomic issues, carrying out personal support interventions with the promotion of self-esteem, and presenting suggestions for behavioral changes in their routine. For most students (72%), the action plan proposed by the tutors was successful. Eight of the 14 (57%) students in the fourth year progressed to the final two years of in-hospital practical training (internship). CONCLUSIONS: The Academic Tutoring Program showed positive results for most of the students. Close monitoring and tutor intervention allowed students with poor academic performance to overcome the low performance cycle. These important tasks demand time and energy from tutors, and institutional recognition of these professionals is essential for the successful maintenance of the program.

Association between NDO-LID and PGL-1 for leprosy and class I and II human leukocyte antigen alleles in an indigenous community in Southwest Amazon

The frequencies of the Human leukocyte antigen (HLA) alleles in the Puyanawa indigenous reserve population and their association with the NDO-LID and ELISA PGL-1 rapid serological test was assessed. This was a cross-sectional study with an epidemiological clinical design conducted in two indigenous communities in the state of Acre, Brazil. Blood was collected in a tube with EDTA to identify HLA alleles and perform serological tests. DNA was obtained using the salting out procedure. The LabType™ technique (One-Lambda-USA) was used for HLA class I (loci A*, B* and C*) and II (loci DRB1*, DQA1* and DQB1*) typing. Allele frequency was obtained by direct count, and the chi-square test was used to assess the association with the NDO-LID and PGL-1 tests. The most frequent alleles in the two communities were: HLA-A*02:01, HLA-B*40:02, HLA-DRB1*16:02, HLA-DQA1*05:05 and HLA-DQB1*03:01. The allele HLA-C*04:01 was the most common in the Barão community, and the allele HLA-C*07:01 in Ipiranga. Among individuals who presented seropositivity to the NDO-LID test, the association with alleles HLA-A*02 (43.18% vs 24.8%, p = 0.03, OR = 2.35) and HLA-B*53 (6.83% vs 0.0%, p = 0.03, OR = 8.95) was observed in the Barão community. HLA-B*15 was associated with non-seroconversion to the NDO-LID test in Ipiranga. In both communities, HLA-B*40 and HLA-C*03 were associated with positive serological response to ELISA PGL-1. The HLA class I and II alleles most frequently found in this study have already been described among Terena indigenous groups, and HLA class I contributes to seroconversion to NDO-LID and PGL-1 tests in inhabitants of the Barão and Ipiranga communities(AU).

Mechanisms of Exercise Limitation and Prevalence of Pulmonary Hypertension in Pulmonary Langerhans Cell Histiocytosis.

BACKGROUND: Pulmonary Langerhans cell histiocytosis (PLCH) determines reduced exercise capacity. The speculated mechanisms of exercise impairment in PLCH are ventilatory and cardiocirculatory limitations, including pulmonary hypertension (PH). RESEARCH QUESTION: What are the mechanisms of exercise limitation, the exercise capacity, and the prevalence of dynamic hyperinflation (DH) and PH in PLCH? STUDY DESIGN AND METHODS: In a cross-sectional study, patients with PLCH underwent an incremental treadmill cardiopulmonary exercise test with an evaluation of DH, pulmonary function tests, and transthoracic echocardiography. Those patients with lung diffusing capacity for carbon monoxide (Dlco) < 40% predicted and/or transthoracic echocardiogram with tricuspid regurgitation velocity > 2.5 m/s and/or with indirect PH signs underwent right heart catheterization. RESULTS: Thirty-five patients were included (68% women; mean age, 47 ± 11 years). Ventilatory and cardiocirculatory limitations, impairment suggestive of PH, and impaired gas exchange occurred in 88%, 67%, 29%, and 88% of patients, respectively. The limitation was multifactorial in 71%, exercise capacity was reduced in 71%, and DH occurred in 68% of patients. FEV1 and Dlco were 64 ± 22% predicted and 56 ± 21% predicted. Reduction in Dlco, an obstructive pattern, and air trapping occurred in 80%, 77%, and 37% of patients. FEV1 and Dlco were good predictors of exercise capacity. The prevalence of PH was 41%, predominantly with a precapillary pattern, and mean pulmonary artery pressure correlated best with FEV1 and tricuspid regurgitation velocity. INTERPRETATION: PH is frequent and exercise impairment is common and multifactorial in PLCH. The most prevalent mechanisms are ventilatory, cardiocirculatory, and suggestive of PH limitations. CLINICAL TRIAL REGISTRATION: ClinicalTrials.gov; No.: NCT02665546; URL: www.clinicaltrials.gov.

Use of medical therapies before pulmonary endarterectomy in chronic thromboembolic pulmonary hypertension patients with severe hemodynamic impairment.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare complication of acute pulmonary embolism, characterized by non-resolving fibro-thrombotic obstructions of large pulmonary arteries. Pulmonary endarterectomy (PEA) is the treatment of choice for the disease, significantly improving survival. Patients with worse hemodynamic profile have worse prognosis after surgery, raising the question of whether the use of medical therapy prior to surgery to optimize hemodynamics could improve outcomes. The aim of this study was to evaluate the role of medical therapy pre-PEA, according to the hemodynamic profile at the diagnosis. We retrospectively analyzed all patients submitted to PEA, from January 2013 to December 2017. Functional, clinical and hemodynamic data were collected to evaluate the main prognostic determinants. Patients were stratified according to the hemodynamic severity and use of targeted therapies prior to surgery. A total of 108 patients were included. Thirty-five patients (32,4%) used targeted therapy pre-PEA. The use of medical therapy delayed the surgical procedure by about 7 months. There was no difference in overall survival between patients that received targeted therapy and those treated only with supportive therapy (87.8% vs 80.3%, respectively, p = 0.426). Nevertheless, when analyzing the group of patients with severe hemodynamic impairment, defined by low cardiac output(<3.7L/min) at baseline, patients treated with targeted therapies presented a significantly better one-year survival. In higher-risk CTEPH patients, characterized by the presence of low cardiac output, the use of targeted therapies prior to PEA was associated with better outcome, suggesting a potential role for pre-operative use of medical treatment in this particular subgroup.

Lung Cavities in Chronic Thromboembolic Pulmonary Hypertension.

OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.

Brazilian Thoracic Society recommendations for the diagnosis and treatment of chronic thromboembolic pulmonary hypertension / Recomendações para o diagnóstico e tratamento da hipertensão pulmonar tromboembólica crônica da Sociedade Brasileira de Pneumologia e Tisiologia

ABSTRACT Chronic thromboembolic pulmonary hypertension (CTEPH) is a serious and debilitating disease caused by occlusion of the pulmonary arterial bed by hematic emboli and by the resulting fibrous material. Such occlusion increases vascular resistance and, consequently, the pressure in the region of the pulmonary artery, which is the definition of pulmonary hypertension. The increased load imposed on the right ventricle leads to its progressive dysfunction and, finally, to death. However, CTEPH has a highly significant feature that distinguishes it from other forms of pulmonary hypertension: the fact that it can be cured through treatment with pulmonary thromboendarterectomy. Therefore, the primary objective of the management of CTEPH should be the assessment of patient fitness for surgery at a referral center, given that not all patients are good candidates. For the patients who are not good candidates for pulmonary thromboendarterectomy, the viable therapeutic alternatives include pulmonary artery angioplasty and pharmacological treatment. In these recommendations, the pathophysiological bases for the onset of CTEPH, such as acute pulmonary embolism and the clinical condition of the patient, will be discussed, as will the diagnostic algorithm to be followed and the therapeutic alternatives currently available.

RESUMO A hipertensão pulmonar tromboembólica crônica (HPTEC) é uma doença grave e debilitante, causada pela oclusão do leito arterial pulmonar por êmbolos hemáticos e por material fibroso induzido pela presença desses êmbolos. Essa oclusão eleva a resistência vascular e, por consequência, a pressão do território arterial pulmonar, caracterizando a presença de hipertensão pulmonar. Esse aumento da carga imposta ao ventrículo direito leva a progressiva insuficiência do mesmo e, finalmente, ao óbito. No entanto, ao contrário das outras formas de hipertensão pulmonar, a HPTEC possui uma particularidade muito significativa: a existência de tratamento potencialmente curativo através da tromboendarterectomia pulmonar. Dessa forma, o objetivo primordial do manejo deve ser a avaliação do potencial cirúrgico do paciente em um centro de referência em HPTEC. Entretanto, nem todos os pacientes podem ser submetidos à cirurgia. Para esses pacientes outras alternativas terapêuticas viáveis são a angioplastia de artérias pulmonares e o tratamento farmacológico. Nestas recomendações, discutir-se-ão as bases fisiopatológicas para o surgimento de HPTEC, a partir da embolia pulmonar aguda, bem como o quadro clínico apresentado pelo paciente, o algoritmo diagnóstico a ser seguido e as alternativas terapêuticas disponíveis.

Lung cavities in chronic thromboembolic pulmonary hypertension

OBJECTIVES: Chronic thromboembolic pulmonary hypertension (CTEPH) is a unique form of pulmonary hypertension (PH) that arises from obstruction of the pulmonary vessels by recanalized thromboembolic material. CTEPH has a wide range of radiologic presentations. Commonly, it presents as main pulmonary artery enlargement, peripheral vascular obstructions, bronchial artery dilations, and mosaic attenuation patterns. Nevertheless, other uncommon presentations have been described, such as lung cavities. These lesions may be solely related to chronic lung parenchyma ischemia but may also be a consequence of concomitant chronic infectious conditions. The objective of this study was to evaluate the different etiologies that cause lung cavities in CTEPH patients. METHODS: A retrospective data analysis of the medical records of CTEPH patients in a single reference PH center that contained or mentioned lung cavities was conducted between 2013 and 2016. RESULTS: Seven CTEPH patients with lung cavities were identified. The cavities had different sizes, locations, and wall thicknesses. In two patients, the cavities were attributed to pulmonary infarction; in 5 patients, an infectious etiology was identified. CONCLUSION: Despite the possibility of being solely associated with chronic lung parenchyma ischemia, most cases of lung cavities in CTEPH patients were associated with chronic granulomatous diseases, reinforcing the need for active investigation of infectious agents in this setting.

Pulmonary Hypertension in General Cardiology Practice / Hipertensão Pulmonar na Prática do Cardiologista

Abstract The finding of pulmonary hypertension (PH) by echocardiography is common and of concern. However, echocardiography is just a suggestive and non-diagnostic assessment of PH. When direct involvement of pulmonary circulation is suspected, invasive hemodynamic monitoring is recommended to establish the diagnosis. This assessent provides, in addition to the diagnostic confirmation, the correct identification of the vascular territory predominantly involved (arterial pulmonary or postcapillary). Treatment with specific medication for PH (phosphodiesterase type 5 inhibitors, endothelin receptor antagonists and prostacyclin analogues) has been proven effective in patients with pulmonary arterial hypertension, but its use in patients with PH due to left heart disease can even be damaging. In this review, we discuss the diagnosis criteria, how etiological investigation should be carried out, the clinical classification and, finally, the therapeutic recommendations for PH.

Resumo O achado de hipertensão pulmonar (HP) em avaliação ecocardiográfica é frequente e preocupante. No entanto, o ecocardiograma é apenas um exame sugestivo e não diagnóstico de HP. Quando se suspeita de acometimento direto da circulação pulmonar, está indicada medida hemodinâmica invasiva para estabelecer o diagnóstico. Essa avaliação permite, além da confirmação diagnóstica, a correta identificação do território vascular predominantemente acometido (arterial pulmonar ou pós-capilar). O tratamento com as medicações específicas de HP (inibidores da fosfodiestarese 5, antagonistas do receptor de endotelina, análogos da prostaciclina e estimulador da guanilil ciclase solúvel) é comprovadamente eficaz para pacientes com hipertensão arterial pulmonar, mas seu uso em pacientes com HP decorrente de doença cardíaca de câmaras esquerdas pode até mesmo ser prejudicial. Discutiremos nesta revisão o critério diagnóstico, a maneira de proceder a investigação etiológica, a classificação clínica e, finalmente, as recomendações terapêuticas na HP.