El péptido Pro-BNP como predictor de daño cardíaco en pacientes con esclerosis sistémica / Pro-BNP Peptide as a Predictor of Cardiac Damage in Systemic Sclerosis Patients

Introducción: La esclerosis sistémica es una enfermedad crónica del tejido conectivo de carácter autoinmune, de causa desconocida, que produce exceso de colágeno provocando fibrosis en la piel, con afectación de órganos internos. Los anticuerpos frecuentes son: antitopoisomerasa 1 y anticentrómero. Las formas clínicas son la cutánea difusa y cutánea limitada. La prevalencia de la afectación cardíaca varía entre un 8-28 por ciento y en fases tardías la presencia de signos y síntomas cardiovasculares es de mal pronóstico y una de las principales causas de mortalidad. Objetivo: Determinar la asociación entre afectación cardíaca y las formas clínicas, el pro-péptido natriurético cerebral N-terminal (NT-proBNP) y los autoanticuerpos en la esclerosis sistémica. Métodos: Se realizó un estudio descriptivo transversal de un universo de 140 pacientes, la muestra fue de 54 pacientes. Se le realizó ecocardiograma, niveles de NT-proBNP, anti-scl 70, anticentrómero y determinación de formas clínicas a todos los pacientes que cumplieron criterios de inclusión y que fueron atendidos en el Hospital: Hermanos Ameijeiras Habana-Cuba, entre julio de 2016 a diciembre de 2017. Resultados: La edad media fue 51,76 ± 12,82. Sexo femenino en un 96,3 por ciento. El 72,2 por ciento era piel blanca. La afectación cardíaca de la EScd fue de 77,5 por ciento, hormona NT-proBNP tuvo niveles elevados en un 55,0 por ciento. Los anti-scl-70 estuvieron negativos en el 70 por ciento (n = 28) de los pacientes con afectación cardíaca. El anti-centrómero estuvo negativo en el 95,0 por ciento (n = 38). Conclusiones: Se determinó que la afectación cardíaca en pacientes con esclerosis sistémica, es independiente de las formas clínicas y de la presencia de autoanticuerpos. Los pacientes que tuvieron los niveles séricos de NT-proBNP elevados presentaron afectación cardíaca(AU)

Introduction: Systemic sclerosis is a chronic autoimmune connective tissue disease of unknown cause, which produces excess collagen causing fibrosis in the skin, affecting internal organs. Common antibodies are antitopoisomerase 1 and anticentromere. The clinical forms are diffuse cutaneous and limited cutaneous. The prevalence of cardiac involvement varies between 8-28percent and in late stages the presence of cardiovascular signs and symptoms have poor prognosis and one of the main causes of mortality. Objective: To determine the association between cardiac involvement and clinical forms, N-terminal pro-brain natriuretic peptide (NT-proBNP) and autoantibodies in SSc. Methods: A cross-sectional descriptive study of a universe of 140 patients was carried out. Fifty four patients made up the sample. An echocardiogram, NT-proBNP, Anti-scl 70, anticentromere levels and determination of clinical forms were performed on all patients who met the inclusion criteria and who were treated at Hermanos Ameijeiras hospital in Havana, Cuba, from July 2016 to December 2017. Results: The mean age was 51.76 ± 12.82. Female sex accounted 96.3percent. 72.2percent were white skinned. Cardiac involvement of EScd was 77.5percent, NT-proBNP hormone had high levels in 55.0percent. Anti-scl-70 were negative in 70percent (n=28) of patients with cardiac involvement. Anti-centromere (ACT) was negative in 95.0percent (n=38). Conclusions: Cardiac involvement in patients with SS is independent of the clinical forms and the presence of autoantibodies. Patients with elevated NT-proBNP serum levels had cardiac involvement(AU)

Disfunción crónica de órganos en pacientes con drepanocitosis. Parte I: manifestaciones cardiorrespiratorias / Chronic organ dysfunction in patients with sickle cell anemia. Part I: cardiorespiratory manifestations

Introducción: En la actualidad la mayoría de los pacientes con anemia falciforme alcanzan la adultez. La disfunción crónica de órganos constituye la causa primaria de muerte y representa un desafío en el manejo de estos enfermos. Objetivo: Analizar los aspectos generales de la disfunción orgánica crónica en pacientes con drepanocitosis y profundizar en las alteraciones cardiorrespiratorias. Métodos: Se realizó una revisión de los artículos publicados en los últimos diez años con el uso de los buscadores PubMed, SciELO y Google Académico. Los términos de búsqueda fueron: anemia de células falciformes, disfunción orgánica, mortalidad, hipertensión pulmonar, enfermedad pulmonar crónica, asma, apnea obstructiva del sueño. Análisis y síntesis de la información: Con el aumento de la expectativa de vida, en el manejo de los pacientes con drepanocitosis, gana en relevancia el diagnóstico precoz y el tratamiento oportuno de la disfunción crónica de órganos. Este es un proceso que comienza en la infancia, pero se hace más evidente en la etapa adulta. Las manifestaciones cardiorrespiratorias más comunes y con impacto en la morbilidad y en la mortalidad son: la hipertensión pulmonar y la enfermedad pulmonar crónica. Se describen aspectos relacionado con la prevalencia, diagnóstico, implicaciones en la evolución de los enfermos y aspectos relacionados con su tratamiento. Conclusiones: La hipertensión pulmonar y la enfermedad pulmonar crónica son frecuentes en pacientes con drepanocitosis. Ambas complicaciones tienen un impacto negativo en la evolución de estos enfermos y se asocian a aumento de la mortalidad. La detección temprana de estas afecciones, permite tomar acciones terapéuticas para disminuir sus consecuencias(AU)

Introduction: Currently, most patients with sickle cell disease reach adulthood. Chronic organ dysfunction constitutes the primary cause of death and represents a challenge in the management of these patients. Objectives: To analyze the general aspects of chronic organic dysfunction in patients with sickle cell disease and to delve into the cardiorespiratory ones. Methods: A review of the articles published in the last ten years was carried out using the PubMed, SciELO and Google Scholar search engines. The search terms were: sickle cell anemia, organ dysfunction, mortality, pulmonary hypertension, chronic lung disease, asthma, obstructive sleep apnea. Information analysis and synthesis: With the increase in life expectancy, in the management of patients with sickle cell disease, early diagnosis and timely treatment of chronic organ dysfunction gain relevance. This is a process that begins in childhood, but becomes more apparent in adulthood. The most common cardiorespiratory manifestations with an impact on morbidity and mortality are: pulmonary hypertension and chronic lung disease. Aspects related to prevalence, diagnosis, implications in the evolution of patients and aspects related to their treatment are described. Conclusions: Pulmonary hypertension and chronic lung disease are frequent in patients with sickle cell disease. Both complications have a negative impact on the evolution of these patients and are associated with increased mortality. Early detection of these conditions allows therapeutic actions to be taken to reduce their consequences(AU)

Frecuencia de hipertensión pulmonar en pacientes con enfermedad pulmonar obstructiva crónica / Frequency of pulmonary hypertension in patients with chronic obstructive pulmonary disease

Introducción: La enfermedad pulmonar obstructiva crónica es uno de los padecimientos pulmonares más frecuente a nivel mundial. Tiene repercusión sobre la mortalidad, causa importante discapacidad y afecta un número considerable de sujetos en edad productiva. La hipertensión pulmonar es una complicación usual de las enfermedades respiratorias crónicas y en particular de la pulmonar obstructiva crónica. Objetivo: Caracterizar a pacientes con enfermedad pulmonar obstructiva crónica y sospecha de hipertensión pulmonar. Métodos: Se realizó un estudio descriptivo, prospectivo en 50 pacientes atendidos en el Hospital Neumológico Benéfico Jurídico desde noviembre 2016 a enero 2018. Se analizaron variables tales como la edad, el sexo, la prueba de función ventilatoria, la radiografía de tórax, el electrocardiograma y el ecocardiograma. Resultados: De 700 pacientes portadores de enfermedad pulmonar crónica, se sospechó hipertensión pulmonar en 7 por ciento y fue confirmada en 34. Las frecuencias por sexo fueron similares y primaron los mayores de 60 años. El 41,6 por ciento de los pacientes tuvo afectación respiratoria severa, 70,6 por ciento presentó hipertensión pulmonar ligera y 64,7 por ciento tenía disnea grado 3. Los índices kappa entre las técnicas utilizadas para el diagnóstico de la hipertensión pulmonar superaron la cifra de 0,74. Conclusiones: La frecuencia de hipertensión pulmonar en pacientes con enfermedad pulmonar obstructiva crónica fue elevada. Primaron pacientes con afectación severa de la función respiratoria e hipertensión pulmonar ligera. El grado de enfermedad pulmonar obstructiva crónica no determinó la severidad de hipertensión pulmonar. Los mayores valores de concordancia entre las técnicas diagnósticas se obtuvieron para la radiografía y el ecocardiograma(AU)

Introduction: Chronic obstructive pulmonary disease is one of the most frequent, worldwide. It has impact on mortality, causing significant disability and affecting a considerable number of subjects of productive age. Pulmonary hypertension is a common complication of chronic respiratory diseases, particularly chronic obstructive pulmonary disease. Objective: To describe patients with chronic obstructive pulmonary disease and suspected pulmonary hypertension. Methods: A descriptive, prospective study was carried out in 50 patients treated at Benéfico Jurídico Pneumological Hospital from November 2016 to January 2018. Variables such as age, sex, ventilatory function test, chest X-ray, electrocardiogram and the echocardiogram were analyzed. Results: Out of 700 patients with chronic lung disease, pulmonary hypertension was suspected in 7 por ciento and confirmed in 34. The frequencies by sex were similar and those older than 60 years prevailed. 41.6 por ciento of the patients had severe respiratory involvement, 70.6 por ciento had mild pulmonary hypertension and 64.7 por ciento had grade 3 dyspnea. The kappa indices between the techniques used for the diagnosis of pulmonary hypertension exceeded the figure of 0.74. Conclusions: The frequency of pulmonary hypertension in patients with chronic obstructive pulmonary disease was high. Patients with severe impairment of respiratory function and mild pulmonary hypertension predominated. The degree of chronic obstructive pulmonary disease did not determine the severity of pulmonary hypertension. The highest concordance values between diagnostic techniques were obtained for radiography and echocardiography(AU)

Remisión a largo plazo de la hipertensión portopulmonar mediante trasplante hepático en paciente con cirrosis asociada a hepatitis autoinmune / Long term remission of portopulmonary hypertension with liver transplantation in a patient with cirrhosis associated to autoimmune hepatitis

La hipertensión portopulmonar (HTPP) es una complicación infrecuente de la hipertensión portal, que sigue un curso progresivo con un pronóstico sombrío. Los reportes en pacientes pediátricos son escasos y con períodos de seguimiento cortos. Se describe una paciente con cirrosis descompensada que desarrolló HTPP resuelta mediante trasplante hepático, que permanece asintomática tras diez años de seguimiento.

Portopulmonary hypertension is an uncommon complication of portal hypertension, running a progressive course with a negative prognosis. Reports in pediatric patients are scarce with short follow up. We describe the case of decompensated cirrhosis who developed PoPH and resolved with liver transplantation, remaining asymptomatic after ten years of follow up.

El impacto de CoViD-19 en la hipertensión arterial pulmonar

La enfermedad por coronavirus 2019 (CoViD-19) está causada por el virus del síndrome respiratorio agudo severo por coronavirus 2 (SARS-CoV-2), siendo particularmente perjudicial para los pacientes con enfermedad cardiovascular subyacente, y provocando una causa de morbilidad y mortalidad significativas en todo el mundo. Este virus lleva a una neumopatía, al tiempo que causa lesiones agudas de miocardio y daño crónico al sistema cardiovascular. Como consecuencia del daño del parénquima pulmonar y de la circulación pulmonar alterada, puede desarrollarse hipertensión pulmonar (HP), con su respectiva consecuencia. La fisiopatología de este tipo de HP es compleja y multifactorial, considerándose factores potenciales para las alteraciones de la circulación pulmonar. En estudios recientes, la prevalencia evidenciada de HP en pacientes con CoViD-19 es de alrededor del 12%, pero su evolución aún no está clara. La pandemia de CoViD-19 ha tenido un impacto significativo en todos los aspectos de la HP, desde el diagnóstico y manejo hasta la observación de un mayor riesgo de muerte en pacientes con hipertensión arterial pulmonar (HAP). En una encuesta de 77 centros de atención médica integral de HAP, la incidencia de infección por CoViD-19 fue de 2,1 casos por cada 1000 pacientes con HAP, similar a la incidencia de infección por CoViD-19 en la población general. Si bien, esta pandemia ha alterado el estándar de atención médica de rutina y de manejo agudo, particularmente, en aquellos pacientes con HAP, los riesgos asociados con CoViD-19 son significativos, presentándose nuevos desafíos en el cuidado de pacientes con HP. Dado que los pacientes con HAP han demostrado tener peores resultados en el ámbito de esta pandemia, es esencial trabajar de manera proactiva para disminuir el riesgo de infección por CoViD-19, mientras se continúa brindando un alto nivel de atención médica. El impacto de CoViD-19 en la prestación de atención médica y en la sociedad en general requirió que se establecieran nuevos protocolos para el tratamiento de HAP para disminuir el riesgo de exposición o transmisión de CoViD-19. De manera similar, ha habido una disminución en las pruebas de pacientes estables. Actualmente, la forma en que brindamos la atención médica se evidencia en un aumento de las visitas de telemedicina, una menor exposición a los entornos de atención médica para los pacientes y los profesionales de la salud, ayudando a nuestra necesidad continua de brindar servicios a los pacientes dentro del entorno de CoViD-19 y adaptándonos a una forma diferente de interactuar, ampliando nuestra comprensión de la mejor manera de cuidar a nuestros pacientes.

Coronavirus disease 2019 (CoViD-19) causes severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), being particularly harmful for patients with underlying cardiovascular disease, and causing a cause of significant morbidity and mortality throughout the world. This virus leads to lung disease, while causing acute myocardial injury and chronic damage to the cardiovascular system. As a consequence of the damage to the lung parenchyma and altered pulmonary circulation, pulmonary hypertension (PH) can develop, with its respective consequence. The pathophysiology of this type of PH is complex and multifactorial, considering potential factors for alterations in pulmonary circulation. In recent studies, the evidenced prevalence of PH in patients with CoViD-19 is around 12%, but its evolution is not yet clear. The CoViD-19 pandemic has had a significant impact on all aspects of PH, from diagnosis and management to observing an increased risk of death in patients with pulmonary arterial hypertension (PAH). In a survey of 77 comprehensive PAH healthcare centers, the incidence of CoViD-19 infection was 2.1 cases per 1,000 PAH patients, similar to the incidence of CoViD-19 infection in the general population. Although this pandemic has altered the standard of routine medical care and acute management, particularly in those patients with PAH, the risks associated with CoViD-19 are significant, presenting new challenges in the care of patients with PH. Since PAH patients have been shown to have worse outcomes in the setting of this pandemic, it is essential to work proactively to decrease the risk of CoViD-19 infection, while continuing to provide a high level of medical care. The impact of CoViD-19 on the provision of health care and on society in general required that new protocols be established for the treatment of PAH to reduce the risk of exposure or transmission of CoViD-19. Similarly, there has been a decline in stable patient testing. Currently, the way we provide healthcare is evidenced by an increase in telemedicine visits, less exposure to healthcare settings for patients and healthcare professionals, aiding our continued need to provide services to patients. patients within the CoViD-19 environment and adapting to a different way of interacting, broadening our understanding of the best way to care for our patients

A doença coronavírus 2019 (CoViD-19) causa síndrome respiratória aguda grave coronavírus 2 (SARS-CoV-2), sendo particularmente prejudicial para pacientes com doença cardiovascular subjacente e causando uma importante morbidade e mortalidade em todo o mundo. Este vírus leva à doença pulmonar, enquanto causa lesão aguda do miocárdio e dano crônico ao sistema cardiovascular. Como consequência do dano ao parênquima pulmonar e da circulação pulmonar alterada, pode ocorrer hipertensão pulmonar (HP), com suas respectivas consequências. A fisiopatologia desse tipo de HP é complexa e multifatorial, considerando fatores potenciais para alterações da circulação pulmonar. Em estudos recentes, a prevalência de HP evidenciada em pacientes com CoViD-19 gira em torno de 12%, mas sua evolução ainda não está clara. A pandemia CoViD-19 teve um impacto significativo em todos os aspectos da HP, desde o diagnóstico e tratamento até a observação de um risco aumentado de morte em pacientes com hipertensão arterial pulmonar (HAP). Em uma pesquisa com 77 centros de saúde com HAP abrangentes, a incidência de infecção por CoViD-19 foi de 2,1 casos por 1.000 pacientes com HAP, semelhante à incidência de infecção por CoViD-19 na população em geral. Embora essa pandemia tenha alterado o padrão de cuidados médicos de rotina e tratamento agudo, particularmente em pacientes com HAP, os riscos associados ao CoViD-19 são significativos, apresentando novos desafios no cuidado de pacientes com HP. Como os pacientes com HAP demonstraram ter resultados piores no cenário dessa pandemia, é essencial trabalhar proativamente para diminuir o risco de infecção por CoViD-19, enquanto continua a fornecer um alto nível de cuidados médicos. O impacto do CoViD-19 na prestação de cuidados de saúde e na sociedade em geral exigiu o estabelecimento de novos protocolos para o tratamento da HAP para reduzir o risco de exposição ou transmissão do CoViD-19. Da mesma forma, houve um declínio nos testes de pacientes estáveis. Atualmente, a forma como prestamos serviços de saúde é evidenciada por um aumento nas visitas de telemedicina, menos exposição aos ambientes de saúde para pacientes e profissionais de saúde, auxiliando nossa necessidade contínua de fornecer serviços aos pacientes. Pacientes dentro do ambiente CoViD-19 e adaptando-se de uma maneira diferente de interagir, ampliando nosso entendimento sobre a melhor forma de cuidar de nossos pacientes.

Chronic thromboembolic pulmonary hypertension: the impact of advances in perioperative techniques in patient outcomes / Hipertensão pulmonar tromboembólica crônica: o impacto dos avanços nas técnicas perioperatórias nos resultados dos pacientes

ABSTRACT Objectives Pulmonary endarterectomy (PEA) is the gold standard treatment for chronic thromboembolic pulmonary hypertension (CTEPH). This study aimed at reporting outcomes of CTEPH patients undergoing PEA within 10 years, focusing on advances in anesthetic and surgical techniques. Methods We evaluated 102 patients who underwent PEA between January 2007 and May 2016 at the Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Changes in techniques included longer cardiopulmonary bypass, heating, and cooling times and mean time of deep hypothermic circulatory arrest and shortened reperfusion time. Patients were stratified according to temporal changes in anesthetic and surgical techniques: group 1 (January 2007-December 2012), group 2 (January 2013-March 2015), and group 3 (April 2015-May 2016). Clinical outcomes were any occurrence of complications during hospitalization. Results Groups 1, 2, and 3 included 38, 35, and 29 patients, respectively. Overall, 62.8% were women (mean age, 49.1 years), and 65.7% were in New York Heart Association functional class III-IV. Postoperative complications were less frequent in group 3 than in groups 1 and 2: surgical complications (10.3% vs. 34.2% vs. 31.4%, p=0.035), bleeding (10.3% vs. 31.5% vs. 25.7%, p=0.047), and stroke (0 vs. 13.2% vs. 0, p=0.01). Between 3 and 6 months post-discharge, 85% were in NYHA class I-II. Conclusion Improvements in anesthetic and surgical procedures were associated with better outcomes in CTEPH patients undergoing PEA during the 10-year period.

RESUMO Objetivo A endarterectomia pulmonar (EAP) é o tratamento padrão ouro para hipertensão pulmonar tromboembólica crônica (HPTEC). O objetivo deste estudo foi relatar a evolução de pacientes com HPTEC submetidos a EAP em 10 anos, com foco nos avanços nas técnicas anestésicas e cirúrgicas. Métodos Foram avaliados 102 pacientes submetidos à EAP entre janeiro de 2007 e maio de 2016 no Instituto do Coração do Hospital das Clínicas da Universidade de São Paulo. Mudanças nas técnicas incluíram circulação extracorpórea, tempos de aquecimento e resfriamento mais longos e tempo médio de hipotermia profunda com parada circulatória e tempo de reperfusão reduzido. Os pacientes foram estratificados de acordo com as mudanças temporais nas técnicas anestésicas e cirúrgicas: grupo 1 (janeiro de 2007 a dezembro de 2012), grupo 2 (janeiro de 2013 a março de 2015) e grupo 3 (abril de 2015 a maio de 2016). Os desfechos clínicos foram qualquer ocorrência de complicações durante a hospitalização. Resultados Os grupos 1, 2 e 3 incluíram 38, 35 e 29 pacientes, respectivamente. No geral, 62,8% eram mulheres (idade média, 49,1 anos) e 65,7% estavam em classe funcional III-IV da New York Heart Association. As complicações pós-operatórias foram menos frequentes no grupo 3 do que nos grupos 1 e 2: complicações cirúrgicas (10,3% vs. 34,2% vs. 31,4%, p=0,035), sangramento (10,3% vs. 31,5% vs. 25,7%, p=0,047) e acidente vascular cerebral (0 vs. 13,2% vs. 0, p=0,01). Entre 3 e 6 meses após a alta, 85% estavam na classe I-II da NYHA. Conclusão Melhorias nos procedimentos anestésicos e cirúrgicos foram associadas a melhores resultados em pacientes com HPTEC submetidos a EAP durante o período de 10 anos.

Echocardiographic features of pre- and postcapillary pulmonary hypertension with a focus on the right ventricle in dogs / Características ecocardiográficas da hipertensão pulmonar pré e pós-capilar em cães com foco no ventrículo direito

Pulmonary hypertension (PH) in dogs is a syndrome that can occur secondary to several causes, including left heart disease (postcapillary) and chronic respiratory disease (precapillary). This study evaluates morphological and functional consequences in the right ventricle (RV) of dogs with pre- and postcapillary PH through echocardiography, and also considers the severity of PH (mild, moderate or severe). Echocardiography was performed on 66 dogs of various breeds and weights (age >3 years old) which were assigned to three groups: postcapillary PH, which included mitral valve disease/endocardiosis, precapillary PH, which included chronic respiratory diseases (bronchitis, collapse of the trachea and primary lung cancer or metastasis), and finally, a healthy group of controls. The parameters for RV morphology were RV1, RV2, and RV3 for systole and diastole. The following measurements were used to assess RV systolic function: tricuspid annular plane systolic excursion (TAPSE), TAPSE:Ao (aorta), maximum velocity of the tricuspid systolic wave obtained by tissue Doppler (S'), S':Ao, right ventricle end-diastolic area (RVEDA); RVEDA:BSA (body surface area); flow velocity integral (FVI) and FVI:Ao. The variables were assessed using ANOVA. The results showed that RV3d, RV1s, S':Ao, S', and FVI were able to distinguish cases of pre- and postcapillary PH in this study. Remodeling of the RV of dogs with PH was observed, which can be influenced by the pre- or postcapillary origin of the PH, with dilation in dogs with postcapillary PH and severe PH. The results for RV systolic function were similar, with FVI and FVI:Ao showing that RV ejection function is reduced in dogs with postcapillary PH and with severe PH.(AU)

A hipertensão pulmonar (HP) em cães é uma síndrome que pode ocorrer secundária às diversas causas, dentre elas, a doença cardíaca esquerda (pós-capilar) e a doença respiratória crônica (pré-capilar). Essa é uma condição importante que motivou os objetivos do estudo: avaliar ecocardiograficamente as consequências morfológicas e funcionais no ventrículo direito (VD) dos cães acometidos com HP pré- e pós-capilar, considerando também o estágio de severidade da HP (leve, moderada ou severa). A ecocardiografia foi realizada em 66 cães de diversas raças e pesos, com pelo menos quatro anos de idade, que compuseram um grupo com doença valvar mitral, ou endocardiose, outro com doença respiratória crônica (bronquite, colapso de traqueia e neoplasia pulmonar primária ou metástase), e por último, um grupo saudável. Os parâmetros para a morfologia do VD foram RV1, RV2 e RV3 na sístole e na diástole. Para a avaliação da função sistólica do VD foram mensurados: excursão sistólica do plano anular tricúspide (TAPSE), TAPSE:Ao (aorta), velocidade máxima da onda sistólica da tricúspide obtida pelo doppler tecidual (S'), S':Ao, área do ventrículo direito no final da diástole (RVEDA); RVEDA:BSA (body superficie area); integral tempo velocidade (FVI) e FVI:Ao. As variáveis foram avaliadas por meio da ANOVA. Os resultados mostraram que RV3d, RV1s, S':Ao, S' e FVI foram capazes de distinguir casos de HP pré e pós-capilar neste estudo. Observou-se que há remodelamento do VD de cães com HP e este pode ser influenciado pela origem pré ou pós-capilar da HP, encontrando-se dilatado em cães com HP pós capilar e com HP severa. Os resultados para a função sistólica do VD foram similares, uma vez que o FVI e FVI:Ao mostraram que a capacidade de ejeção do VD está reduzida nos cães com HP pós-capilar e com HP severa.(AU)

Echocardiographic features of pre- and postcapillary pulmonary hypertension with a focus on the right ventricle in dogs

ABSTRACT: Pulmonary hypertension (PH) in dogs is a syndrome that can occur secondary to several causes, including left heart disease (postcapillary) and chronic respiratory disease (precapillary). This study evaluates morphological and functional consequences in the right ventricle (RV) of dogs with pre- and postcapillary PH through echocardiography, and also considers the severity of PH (mild, moderate or severe). Echocardiography was performed on 66 dogs of various breeds and weights (age >3 years old) which were assigned to three groups: postcapillary PH, which included mitral valve disease/endocardiosis, precapillary PH, which included chronic respiratory diseases (bronchitis, collapse of the trachea and primary lung cancer or metastasis), and finally, a healthy group of controls. The parameters for RV morphology were RV1, RV2, and RV3 for systole and diastole. The following measurements were used to assess RV systolic function: tricuspid annular plane systolic excursion (TAPSE), TAPSE:Ao (aorta), maximum velocity of the tricuspid systolic wave obtained by tissue Doppler (S), S:Ao, right ventricle end-diastolic area (RVEDA); RVEDA:BSA (body surface area); flow velocity integral (FVI) and FVI:Ao. The variables were assessed using ANOVA. The results showed that RV3d, RV1s, S:Ao, S, and FVI were able to distinguish cases of pre- and postcapillary PH in this study. Remodeling of the RV of dogs with PH was observed, which can be influenced by the pre- or postcapillary origin of the PH, with dilation in dogs with postcapillary PH and severe PH. The results for RV systolic function were similar, with FVI and FVI:Ao showing that RV ejection function is reduced in dogs with postcapillary PH and with severe PH.

RESUMO: A hipertensão pulmonar (HP) em cães é uma síndrome que pode ocorrer secundária às diversas causas, dentre elas, a doença cardíaca esquerda (pós-capilar) e a doença respiratória crônica (pré-capilar). Essa é uma condição importante que motivou os objetivos do estudo: avaliar ecocardiograficamente as consequências morfológicas e funcionais no ventrículo direito (VD) dos cães acometidos com HP pré- e pós-capilar, considerando também o estágio de severidade da HP (leve, moderada ou severa). A ecocardiografia foi realizada em 66 cães de diversas raças e pesos, com pelo menos quatro anos de idade, que compuseram um grupo com doença valvar mitral, ou endocardiose, outro com doença respiratória crônica (bronquite, colapso de traqueia e neoplasia pulmonar primária ou metástase), e por último, um grupo saudável. Os parâmetros para a morfologia do VD foram RV1, RV2 e RV3 na sístole e na diástole. Para a avaliação da função sistólica do VD foram mensurados: excursão sistólica do plano anular tricúspide (TAPSE), TAPSE:Ao (aorta), velocidade máxima da onda sistólica da tricúspide obtida pelo doppler tecidual (S), S:Ao, área do ventrículo direito no final da diástole (RVEDA); RVEDA:BSA (body superficie area); integral tempo velocidade (FVI) e FVI:Ao. As variáveis foram avaliadas por meio da ANOVA. Os resultados mostraram que RV3d, RV1s, S:Ao, S e FVI foram capazes de distinguir casos de HP pré e pós-capilar neste estudo. Observou-se que há remodelamento do VD de cães com HP e este pode ser influenciado pela origem pré ou pós-capilar da HP, encontrando-se dilatado em cães com HP pós capilar e com HP severa. Os resultados para a função sistólica do VD foram similares, uma vez que o FVI e FVI:Ao mostraram que a capacidade de ejeção do VD está reduzida nos cães com HP pós-capilar e com HP severa.

Gut microbiota changes in airway diseases: a systematic review / Alterações da microbiota intestinal em doenças das vias aéreas: uma revisão sistemática

Introduction: studies have highlighted the importance of gut microbiota (GM) to the host immune defenses, influencing the host development and physiology. Changes in the composition and diversity of GM have been detected in some disease and could be implicated in the pathophysiological mechanisms of them. Objective: the purpose of this study was to show an overview of the current knowledge about the GM of patients with airway diseases (AD). Methodology: the literature search was performed in four databases, using a combination of the descriptors: "Gastrointestinal Microbiome", "Gut Microbiome", "Gut Microbiota", "Cystic Fibrosis" (CF), "Asthma", "Pulmonary Hypertension" (HP) and/or "Chronic Obstructive Pulmonary Disease" (COPD). Results: fifteen studies were herein included: ten of CF and five of asthma. No study about other AD matched the inclusion criteria. In all studies about CF, changes were detected in GM, particularly quantitative and qualitative microbial changes. For asthma, data showed changes in GM also including a reduction of microbial richness, evenness and diversity and in the Bacteroidetes/Firmicutes ratio. Conclusions: the current data indicate the existence of GM changes in AD. However, due to the few studies for asthma and the lack of investigations on HP and COPD, it was not possible to confirm whether these GM changes are observed in other AD. Furthermore, this review shows the necessity of more studies in this area to characterize dysbiosis and which alterations are more frequent observed in AD patients.

Introdução: estudos têm destacado a importância da microbiota intestinal (GM) para as defesas imunológicas do hospedeiro, influenciando o desenvolvimento e a fisiologia do hospedeiro. Mudanças na composição e diversidade da GM foram detectadas em algumas doenças e podem estar implicadas nos mecanismos fisiopatológicos delas. Objetivo: o objetivo desta revisão foi avaliar estudos sobre a microbiota intestinal (MI) de pacientes com doenças das vias aéreas (DA). Metodologia: esta pesquisa bibliográfica foi realizada em quatro bases de dados, utilizando a combinação dos descritores: "Microbioma Gastrointestinal", "Microbioma Intestinal", "Microbiota Intestinal", "Fibrose Cística" (CF), "Asma", "Hipertensão Pulmonar" (HP), "Doença Pulmonar Obstrutiva Crônica" (DPOC). Resultados: quinze estudos foram incluídos: dez de FC e cinco de asma. Nenhum estudo sobre outra DA correspondeu aos critérios de inclusão. Em todos os estudos sobre FC, foram detectadas alterações na MI, particularmente alterações microbianas qualitativas e quantitativas. Para a asma, os dados mostraram mudanças na MI, incluindo também uma redução da quantidade, uniformidade e diversidade microbiana e na razão Bacteroidetes/Firmicutes. Conclusão: os dados atuais indicam a existência de alterações na MI nas DA. No entanto, devido aos poucos estudos para asma e à falta de investigações para HP e DPOC, não foi possível confirmar se essas alterações na MI são observadas em outras DA também. Além disso, esta revisão mostra a necessidade de mais estudos nessa área para caracterizar a disbiose e quais alterações são mais frequentes em pacientes com DA.

Demographic and clinical characteristics of pulmonary arterial hypertension caused by schistosomiasis are indistinguishable from other etiologies

Abstract INTRODUCTION: Pulmonary arterial hypertension (PAH) is a serious pulmonary circulation disease caused by several etiologies, including schistosomiasis. The present study retrospectively evaluated the clinical and hemodynamic characteristics of patients with schistosomal PAH (PAH-Sch) compared to those of non-Sch PAH patients (non-Sch PAH). METHODS: Patients treated at the Pronto-Socorro Cardiológico de Pernambuco and diagnosed by right cardiac catheterization were divided into PAH-Sch and non-Sch PAH groups. Their socio-demographic and clinical characteristics, N-terminal-pro B-type natriuretic peptide (NT-proBNP), and echocardiography and hemodynamic parameters were retrospectively reviewed. RESULTS: Among the included 98 patients (mean age, 45 ± 14 years; 68 women [69.4%]), we found 56 PAH-Sch and 42 non-Sch PAH. The age distribution was heterogeneous in the PAH-Sch group, with patients predominantly ranging from 50-59 (p <0.004). Dyspnea was the most common symptom, reported by 92 patients (93.8%), and commonly present for over two years prior to diagnosis. Clinical symptoms were similar in both groups, with no differences in functional class, pulmonary artery systolic pressure (p = 0.102), 6-minute walk test score (p = 0.234), NT-proBNP serum levels (p = 0.081), or hemodynamic parameters. CONCLUSIONS: Patients with PAH-Sch present clinical, laboratory, and hemodynamic profiles similar to those with PAH resulting from other etiologies of poor prognosis. PAH is an important manifestation of schistosomiasis in endemic regions that is often diagnosed late.

Várices esofágicas superiores ( Downhill varices ). Presentación de casos y revisión de la literatura / Upper esophageal varices (Downhill varices): Case presentation and literature review

Resumen Se describen 21 casos con hallazgo incidental de várices esofágicas superiores (Downhill varices) en endoscopia de las vías digestivas altas. Además, se detallan algunos datos relevantes de la historia clínica y de los hallazgos endoscópicos. La relación hombres/mujeres fue 1:1 y la edad promedio fue de 67,9 años. La comorbilidad más frecuente fue la enfermedad pulmonar obstructiva crónica (EPOC), seguida de las neumoconiosis, la obesidad y el síndrome de apnea-hipopnea, todas con hipertensión pulmonar (HTP) asociada. Las tres últimas causas no han sido descritas previamente en la literatura sobre várices esofágicas superiores. Ningún paciente tenía antecedente de hemorragia variceal.

Abstract We describe 21 cases with incidental endoscopic findings of upper esophageal varices (downhill varices) and relevant data from patients' clinical histories and endoscopic findings. The male/female ratio was 1:1 and patients' average age was 67.9 years. The most frequent comorbidity was chronic obstructive pulmonary disease, followed by pneumoconiosis, obesity and apnea-hypopnea syndrome. All comorbidities had associated pulmonary hypertension. The last three causes have not been previously described in the literature on upper esophageal varices. None of the patients had histories of variceal hemorrhaging.

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.

Hipertensión pulmonar tromboembólica crónica: Del diagnóstico al pronóstico

La hipertensión pulmonar tromboembólica crónica (HPTC) es una enfermedad que remodela de manera obstructiva a las arterias pulmonares de gran y pequeño calibre como consecuencia de un tromboembolismo pulmonar (TEP) importante. Se cree que presenta una incidencia acumulada del 0,1 al 9,1% en los primeros 2 años después de un evento sintomático de TEP. El gran margen de error entre los autores que ofrecen estos números se debe probablemente al sesgo de referencia, a la escasez de síntomas tempranos y a la dificultad de diferenciar al TEP agudo de los síntomas de HPTC preexistente. Aunque su prevalencia exacta y la incidencia anual son desconocidas, algunos datos sugieren que esta condición puede ocurrir en aproximadamente 5 individuos por millón de habitantes por año. En el diagnóstico diferencial de la misma, se debe tener en cuenta distintas condiciones muy diferentes a los fenómenos trombóticos. Estas afecciones incluyen el sarcoma de arteria pulmonar, embolia de células tumorales, parásitos (como el quiste hidatídico), embolia de cuerpo extraño y estenosis congénita o adquirida de la arteria pulmonar. Todo esto hace que el diagnóstico se torne dificultoso y afecte consecutivamente al pronóstico de esta enfermedad. Esta revisión pretende interpretar la metodología diagnóstica usada actualmente en la HPTC y definir las diferentes situaciones pronósticas de la misma.

Chronic thromboembolic pulmonary hypertension (CTEPH) is a disease that obstructively remodels the pulmonary arteries of large and small caliber as a consequence of an important pulmonary embolism (PE). It is believed to have an accumulated incidence of 0.1 to 9.1% in the first 2 years after a symptomatic event of PE. The large margin of error among the authors offering these numbers is probably due to the reference bias, the shortage of early symptoms, and the difficulty of differentiating acute PE from the symptoms of pre-existing CTEPH. Although its exact prevalence and annual incidence are unknown, some data suggest that this condition can occur in approximately 5 individuals per million inhabitants per year. In the differential diagnosis of the same, must take into account different conditions very different from the thrombotic phenomena. These conditions include pulmonary artery sarcoma, tumor cell embolism, parasites (such as hydatid cyst), foreign body embolism, and congenital or acquired pulmonary artery stenosis. All this makes the diagnosis difficult and affects consecutively the prognosis of this disease. This review aims to interpret the diagnostic methodology currently used in the CTEPH and define the different prognostic situations of it.

A hipertensão pulmonar tromboembólica crônica (HPTC) é uma doença que remodela de forma obstrutiva as artérias pulmonares de grande e pequeno calibre, como resultado de uma embolia pulmonar (EP) significativa. Acredita-se que tenha uma incidência acumulada de 0,1 a 9,1% nos primeiros 2 anos após um evento sintomático de EP. A grande margem de erro entre os autores que oferecem esses números provavelmente se deve ao viés de referência, à falta de sintomas precoces e à dificuldade de diferenciar a EP aguda dos sintomas de HPTC pré-existente. Embora sua prevalência exata e incidência anual sejam desconhecidas, alguns dados sugerem que essa condição pode ocorrer em aproximadamente 5 indivíduos por milhão de habitantes por ano. No diagnóstico diferencial do mesma, deve levar em conta condições distintas muito diferentes dos fenômenos trombóticos. Essas condições incluem sarcoma de artéria pulmonar, embolia de células tumorais, parasitas (como cisto hidático), embolia de corpo estranho e estenose da artéria pulmonar congênita ou adquirida. Tudo isso dificulta o diagnóstico e afeta consecutivamente o prognóstico dessa doença. Esta revisão tem como objetivo interpretar a metodologia diagnóstica atualmente utilizada na HPTC e definir as diferentes situações prognósticas da mesma.

Disability and its clinical correlates in pulmonary hypertension measured through the World Health Organization Disability Assessment Schedule 2 0: a prospective, observational study / Deficiência e seus correlatos clínicos na hipertensão pulmonar medidos pelo World Health Organization Disability Assessment Schedule 2 0: um estudo prospectivo e observacional

ABSTRACT Objective: To characterise the degree of disability in pulmonary hypertension (PH) patients based on the World Health Organisation Disability Assessment Schedule 2.0 (WHODAS 2.0). Method: A prospective and observational study of patients with documented PH (N = 46). Patients completed the WHODAS 2.0 questionnaire during a scheduled routine clinical visit, and their demographic and clinical characteristics were retrieved from electronic medical records (EMR). In subsequent visits, selected clinical variables were registered to assess disease progression. Results: WHODAS 2.0 scores were indicative of mild to moderate disability for the domains of mobility (22.0 ± 23.2), life activities (23.7 ± 25.5), and participation in society (17.2 ± 15.9), as well as total WHODAS 2.0 score (15.3 ± 15.2). For the domains of cognition (9.1 ± 14.1), self-care (8.3 ± 14.4), and interpersonal relationships (11.7 ± 15.7), scores were lower. Disability scores were, generally, proportional to the PH severity. The main baseline correlates of disability were World Health Organisation (WHO) functional class, fatigue, dyspnoea, 6-minute walking distance (6MWD), and N-terminal pro b-type natriuretic peptide (NTproBNP). Baseline WHODAS 2.0 scores showed significant associations with disease progression. However, this effect was not transversal to all domains, with only a few domains significantly associated with disease progression variables. Conclusions: This PH population shows mild disability, with higher degree of disability in the domains of mobility and life activities. This study is the first one to assess disability in PH using WHODAS 2.0. Further studies should apply this scale to larger PH populations with suitable representations of more severe PH forms.

RESUMO Objetivo: Caracterizar o nível de deficiência em pacientes com hipertensão pulmonar (HP) com base no World Health Organization Disability Assessment Schedule 2.0 (WHODAS 2.0). Método: Estudo prospectivo e observacional em pacientes com HP documentada (N = 46). Os pacientes completaram o questionário WHODAS 2.0 durante uma consulta de rotina, e seus dados demográficos e clínicos foram retirados de prontuários médicos eletrônicos. Nas visitas subsequentes, variáveis clínicas específicas foram registradas para avaliar a progressão da doença. Resultados: a pontuação no WHODAS 2.0 indicaram deficiência leve a moderada para os domínios de mobilidade (22,0 ± 23,2), atividades da vida (23,7 ± 25,5) e participação em sociedade (17,2 ± 15,9), assim como a pontuação total do WHODAS 2.0 (15,3 ± 15,2). Para os domínios de cognição (9,1 ± 14,1), autocuidado (8,3 ± 14,4) e relações interpessoais (11,7 ± 15,7), a pontuação foi mais baixa. Os pontos referentes à deficiência foram, em geral, proporcionais à gravidade da HP. Os principais parâmetros da deficiência foram a classificação de funcionalidade da Organização Mundial da Saúde (OMS), fatiga, dispneia, teste de caminhada de 6 minutos (TC6M), e a porção N-terminal do pró-hormônio do peptídeo natriurético do tipo B (NT-proBNP). Os valores de parâmetro do WHODAS 2.0 mostraram associações significativas com a progressão da doença. Porém, este efeito não foi transversal a todos os domínios; apenas alguns estiveram significativamente associados às variáveis da progressão da doença. Conclusão: Esta população com HP mostra deficiência leve, com níveis mais altos nos domínios de mobilidade e atividades da vida diária. Este estudo é o primeiro a avaliar a deficiência em HP usando o WHODAS 2.0. Outros estudos devem aplicar esta escala em populações maiores com HP, com representações de formas mais graves da HP.

Angioplastia percutánea con balón en hipertensión pulmonar tromboembólica crónica / Percutaneous balloon angioplasty for treatment of chronic thromboembolic pulmonary hypertension

La hipertensión pulmonar tromboembólica crónica se caracteriza por la presencia de material trombótico organizado dentro de las arterias pulmonares que genera elevación de la resistencia vascular pulmonar, insuficiencia cardíaca derecha y, eventualmente, la muerte. El tratamiento de elección es la tromboendarterectomía pulmonar, que suele ser curativa si la obstrucción es proximal. En algunos casos este tratamiento no es posible y surge como alternativa la angioplastia pulmonar con balón (APB), que está generando creciente interés. Se presentan tres casos de pacientes con hipertensión pulmonar tromboembólica a los que por diferentes circunstancias no pudo tratarse con tromboendarterectomía y se realizó APB comprobándose, en los tres casos, mejoría de la clase funcional, prueba de la caminata de seis minutos, además de parámetros hemodinámicos y angiográficos.

Chronic thromboembolic pulmonary hypertension is characterized by the presence of organized thrombotic material in the pulmonary arteries which causes elevation of the pulmonary vascular resistance, right heart failure, and death if not treated. Pulmonary thromboendarterectomy is the treatment of choice and can be curative when the obstruction is proximal. There are cases in which this therapy is not possible, and pulmonary angioplasty is a therapeutic alternative of growing interest. We present our experience with three patients diagnosed with chronic thromboembolic pulmonary hypertension in whom pulmonary endarterectomy was not possible and pulmonary angioplasty was performed. All patients showed improvement of functional class, six-minute walk distance, and hemodynamic as well as angiographic parameters.

Use of direct oral anticoagulants for chronic thromboembolic pulmonary hypertension

OBJECTIVES: Chronic thromboembolic pulmonary hypertension is one of the most prevalent forms of pulmonary hypertension and is a major complication of acute pulmonary embolism. One mainstay of chronic thromboembolic pulmonary hypertension treatment is lifelong anticoagulation. The recent advent of direct oral anticoagulants for acute pulmonary embolism treatment has provided a viable and effective alternative for treating this condition. However, little is known about the efficacy of this new class of drugs for treating chronic thromboembolic pulmonary hypertension. We aimed to evaluate the safety and efficacy of direct oral anticoagulants in the treatment of chronic thromboembolic pulmonary hypertension. METHODS: A cohort of chronic thromboembolic pulmonary hypertension patients who initiated treatment with direct oral anticoagulants between June 2015 and November 2016 were enrolled in this study. RESULTS: Sixteen patients used rivaroxaban, three used dabigatran and one used apixaban for a mean follow-up of 20.9 months. The mean age was 51 years, and eighteen patients were classified as functional class II/III. Eight patients underwent a pulmonary endarterectomy and exhibited clinical, hemodynamic and functional improvement and currently continue to use direct oral anticoagulants. No episode of venous thromboembolism recurrence was identified during the follow-up period, but there was one episode of major bleeding after a traumatic fall. CONCLUSIONS: Although direct oral anticoagulants appear to be a safe and effective alternative for treating chronic thromboembolic pulmonary hypertension, larger studies are needed to support their routine use.

De la tromboembolia pulmonar aguda a la hipertensión pulmonar tromboembólica crónica: implicaciones en la patobiología y fisiopatología / From acute pulmonary embolism to chronic thromboembolic pulmonary hypertension: Pathobiology and pathophysiology

Resumen: La hipertensión pulmonar tromboembólica crónica (HPTEC) es un subtipo de hipertensión pulmonar (HP) caracterizada por la obstrucción mecánica de las arterias pulmonares causada por una tromboembolia pulmonar. Sin tratamiento es una enfermedad progresiva y devastadora, y es el único subgrupo de HP potencialmente curable mediante la endarterectomía pulmonar. La magnitud, así como la recurrencia de la embolia pulmonar, son determinantes y contribuyen al desarrollo de la HPTEC aun cuando está asociada solo a algunos factores trombofílicos. Una hipótesis es que la enfermedad es consecuencia de la resolución incompleta y la organización del trombo, favorecido por fenómenos de inflamación, inmunitarios y/o genéticos que promueven el desarrollo de estenosis de tipo fibroso que culminan con el remodelado vascular oclusivo de vasos proximales y distales. Los mecanismos involucrados en la fallida resolución del trombo aún no están esclarecidos. Los pacientes con HPTEC con frecuencia exhiben HP severa que no puede ser explicada por el grado de obstrucción vascular demostrada en estudios por imagen. En tales casos la arteriopatía pulmonar y las lesiones trombóticas obstructivas, distales al nivel subsegmentario, pueden contribuir al incremento fuera de proporción de las resistencias vasculares pulmonares. Los procesos que llevan al desarrollo de la arteriopatía pulmonar y los cambios microvasculares que ocurren en la HPTEC explican el comportamiento progresivo de la HP y el deterioro clínico gradual con pobre pronóstico para los pacientes, así como también la falta de correlación entre la magnitud de la obstrucción vascular y la medición de parámetros hemodinámicos, aun en ausencia de tromboembolismo venoso recurrente. Esta revisión resume los aspectos más relevantes y actuales de la patobiología y fisiopatología de la HPTEC.

Abstract: Chronic thromboembolic pulmonary hypertension (CTEPH) represents a unique subtype of pulmonary hypertension characterized by the presence of mechanical obstruction of the major pulmonary vessels caused by venous thromboembolism. CTEPH is a progressive and devastating disease if not treated, and is the only subset of PH potentially curable by a surgical procedure known as pulmonary endarterectomy. The clot burden and pulmonary embolism recurrence may contribute to the development of CTEPH however only few thrombophilic factors have been found to be associated. A current hypothesis is that CTEPH results from the incomplete resolution and organization of thrombus modified by inflammatory, immunologic and genetic mechanisms, leading to the development of fibrotic stenosis and adaptive vascular remodeling of resistance vessels. The causes of thrombus non-resolution have yet to be fully clarified. CTEPH patients often display severe PH that cannot be fully explained by the degree of pulmonary vascular obstruction apparent on imaging studies. In such cases, the small vessel disease and distal obstructive thrombotic lesions beyond the sub-segmental level may contribute for out of proportion elevated PVR. The processes implicated in the development of arteriopathy and microvascular changes might explain the progressive nature of PH and gradual clinical deterioration with poor prognosis, as well as lack of correlation between measurable hemodynamic parameters and vascular obstruction even in the absence of recurrent venous thromboembolism. This review summarizes the most relevant up-to-date aspects on pathobiology and pathophysiology of CTEPH.

Mexican registry of pulmonary hypertension REMEHIP / Registro Mexicano de Hipertensión Pulmonar REMEHIP

Abstract: Objective: REMEHIP is a prospective, multicentre registry on pulmonary hypertension. The main objective will be to identify the clinical profile, medical care, therapeutic trends and outcomes in adult and pediatric Mexican patients with well-characterized pulmonary hypertension. Methods: REMEHIP a multicenter registry began in 2015 with a planned recruitment time of 12 months and a 4-year follow-up. The study population will comprise a longitudinal cohort study, collecting data on patients with prevalent and incident pulmonary hypertension. Will be included patients of age >2 years and diagnosis of pulmonary hypertension by right heart catheterization within Group 1 and Group 4 of the World Health Organization classification. The structure, data collection and data analysis will be based on quality current recommendations for registries. The protocol has been approved by institutional ethics committees in all participant centers. All patients will sign an informed consent form. Currently in Mexico, there is a need of observational registries that include patients with treatment in the everyday clinical practice so the data could be validated and additional information could be obtained versus the one from the clinical trials. In this way, REMEHIP emerges as a link among randomized clinical trials developed by experts and previous Mexican experience.

Resumen: Objetivo: REMEHIP es un registro prospectivo, multicéntrico en hipertensión pulmonar. El objetivo principal será identificar el perfil clínico, atención médica, tendencias terapéuticas y evolución en pacientes mexicanos adultos y pediátricos con hipertensión pulmonar bien caracterizada. Métodos: El REMEHIP comenzó en el año de 2015 y se planea un reclutamiento de 12 meses con un seguimiento de 4 años. La población en estudio será una cohorte longitudinal y se obtendrán datos de pacientes prevalentes e incidentes con hipertensión pulmonar. Se incluirán pacientes con edad > 2 años con diagnóstico de hipertensión pulmonar demostrado por cateterismo cardiaco derecho del Grupo 1 y Grupo 4 de la clasificación de la Organización Mundial de la Salud. La estructura, colección de datos y el análisis se establecerá a través de las recomendaciones actuales de calidad para los registros. El protocolo ha sido aprobado por los comités de ética de todos los centros participantes. Todos los pacientes firmarán un consentimiento informado. Actualmente en México existe una necesidad de registros observacionales que incluyan a pacientes con tratamiento en la práctica clínica cotidiana, de tal forma que los datos obtenidos podrían validarse y el resto de la información podría compararse con la derivada de los estudios clínicos. De esta forma REMEHIP surge como un vínculo entre los estudios clínicos aleatorizados conducidos por expertos y la experiencia mexicana previa.

Evaluating respiratory musculature, quality of life, anxiety, and depression among patients with indeterminate chronic Chagas disease and symptoms of pulmonary hypertension

Abstract INTRODUCTION: Chagas disease (CD) is progressive and incapacitating, especially when cardiopulmonary function is affected. For example, respiratory muscle weakness can cause dyspnea upon exertion and fatigue, which may be exacerbated when it is associated with pulmonary hypertension (PH). The present study aimed to evaluate respiratory musculature, quality of life, anxiety, and depression among patients with indeterminate chronic CD and symptoms of PH. METHODS: All individuals completed a clinical evaluation, spirometry, a 6-min walking test, respiratory musculature testing using maximum inspiratory pressure (PImax) and maximum expiratory pressure (PEmax), the Hospital Anxiety and Depression Scale, and the SF-36 questionnaire. RESULTS: We evaluated 107 patients who were assigned to a control group with only CD (G1, 8 patients), a group with CD and possible PH (G2, 93 patients), and a group with CD and echocardiography evidence of PH (G3, 6 patients). The three groups had similar values for PImax and PEmax. Compared to the G1 and G2 groups, the G3 group covered significantly less distance during the 6-min walking test and had a significantly shorter predicted distance (p < 0.05 vs. the G1 group). All three groups had similar values for their spirometry results, Hospital Anxiety and Depression Scale scores, and SF-36 questionnaire results. CONCLUSIONS: Patients with indeterminate chronic CD and symptoms of PH did not experience significant impairment in the studied variables, with the exception of the 6-min walking test, which suggests a low exercise tolerance.

Prevalencia de hipertensión pulmonar y factores asociados en adultos con insuficiencia mitral de una institución prestadora de servicios de salud de tercer nivel de la ciudad de Tunja / Prevalence of pulmonary hypertension and associated factors in adults with mitral regurgitation of a third level health services provider institution of Tunja city / Prevalência de hipertensão pulmonar e fatores associados em adultos com regurgitação mitral de uma instituição prestadora de serviços de saúde de terceiro nível na cidade de Tunja

Introducción. La hipertensión pulmonar es el aumento de la presión en las arterias pulmonares. Cuando la presión media en la arteria pulmonar sobrepasa los 25 mm Hg, se considera que existe hipertensión arterial pulmonar. Objetivo. Determinar la prevalencia y los factores asociados de la hipertensión pulmonar en adultos con insuficiencia mitral. Métodos. Se llevó a cabo un estudio de corte transversal que incluyó 676 pacientes mediante mues-treo no probabilístico de tipo censo, con diagnóstico de insuficiencia mitral comprobada mediante ecocardiografía, atendidos en un periodo de un año en una institución de tercer nivel de complejidad de Tunja. Se contó con autorización para el acceso y la revisión de las historias clínicas y los ecocar-diogramas. Resultados. La prevalencia de hipertensión pulmonar en pacientes con insuficiencia mitral fue de 42,9% El promedio de edad de los pacientes fue de 73,7 años y la proporción por sexo fue similar, con 50,7 % de mujeres y 49,3 % de hombres. Se encontró una asociación estadísticamente significativa de la variable enfermedad pulmonar obstructiva crónica con la de hipertensión pulmonar (OR ajustada de 1,82) (IC95% 1,2-2,7) y con la de falla cardiaca (OR ajustada de 3,01) (IC95% 1,7-5,3). Conclusiones. En los pacientes con insuficiencia mitral, se encontró una alta prevalencia de hiper-tensión pulmonar, situación que, según elementos clínicos, puede empeorar su pronóstico. Se halló una asociación estadísticamente significativa entre hipertensión pulmonar, y enfermedad pulmonar obstructiva crónica y falla cardiaca.

Introduction: Pulmonary hypertension is the increase in the pressure of the pulmonary arteries. When the average pressure in the pulmonary artery exceeds 25 mm Hg, it is considered to be associated with pulmonary arterial hypertension. Objective: To determine the prevalence and associated factors of pulmonary hypertension in adults with mitral insufficiency. Methods: A cross-sectional study of 676 patients with mitral insufficiency determined by echocar-diography performed in the one-year period in a third-level complexity institution of Tunja city was carried out. Access and review of clinical records and echocardiograms was authorized. A total of 676 patients was included using non-probabilistic sensorial type sampling. Results: A 42.9% prevalence of pulmonary hypertension was found in patients with mitral insuffi-ciency with a mean age of 73.7 years, the proportion of gender was similar, 50.7% in females and 49.3% in the male gender. A statistically significant association was found between chronic obstructi-ve pulmonary disease and pulmonary hypertension with an adjusted OR of 1.82 [95% CI: 1.24-2.66]; likewise, with heart failure with an adjusted OR of 3.01 [95% CI: 1.70-5.33]. Conclusions: A high prevalence of pulmonary hypertension was found in patients with mitral insu-fficiency, which according to clinical elements may worsen the prognosis of patients with this type of pathology. A statistically significant association was found in the population among pulmonary hypertension with chronic obstructive pulmonary disease or heart failure

Introdução. Hipertensão pulmonar é o aumento da pressão nas artérias pulmonares. Quando a pressão média na artéria pulmonar excede 25 mm Hg, considera-se a presencia da hipertensão arterial pulmonar. Objetivo. Determinar a prevalência e fatores associados à hipertensão pulmonar em adultos com regurgitação mitral. Métodos. Foi levado a cabo um estudo transversal que incluiu 676 pacientes com amostragem não probabilística de tipo censo, com diagnóstico de regurgitação mitral comprovada por ecocardiografia, atendido em um período de um ano em uma instituição de complexidade de terceiro nível em Tunja. Houve autorização para acesso e revisão de prontuários e ecocardiogramas. Resultados. A prevalência de hipertensão pulmonar em pacientes com regurgitação mitral foi de 42,9%. A média de idade dos pacientes foi de 73,7 anos e a proporção por sexo foi semelhante, com 50,7% das mulheres e 49,3% dos homens. Encontrou-se associação estatisticamente significativa entre a variável doença pulmonar obstrutiva crônica e hipertensão pulmonar (OR ajustada de 1,82) (IC95% 1,2-2,7) e com insuficiência cardíaca (OR ajustada de 3,01). (IC95% 1,7-5,3). Conclusões. Nos pacientes com regurgitação mitral, foi encontrada alta prevalência de hipertensão pulmonar, situação que, segundo os elementos clínicos, pode piorar seu prognóstico. Uma associação estatisticamente significativa foi encontrada entre hipertensão pulmonar, doença pulmonar obstrutiva crônica e insuficiência cardíaca.