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La hipertensión arterial pulmonar se caracteriza por una presión arterial pulmonar media y resistencia vascular pulmonar elevadas y remodelado patológico de las arterias pulmonares. La entrada de calcio desde el espacio extracelular al intracelular a través de canales dependientes e independientes de voltaje juega un rol fundamental en el aumento de la contractilidad de las arterias pulmonares y la pérdida de regulación del comportamiento proliferativo de las células de las distintas capas de la pared de las arterias pulmonares. De esta manera, estos canales contribuyen con la vasoconstricción exacerbada de las arterias pulmonares y a su remodelado patológico. El objetivo de esta revisión es recapitular la evidencia obtenida desde modelos celulares y animales respecto a la contribución de los principales canales de calcio de membrana plasmática en estos mecanismos fisiopatológicos claves en el desarrollo de la hipertensión pulmonar, discutiendo su valor potencial como diana farmacológica para terapias presentes y futuras.

Pulmonary arterial hypertension is characterized by increased mean pulmonary arterial pressure, resistance, and pathological remodeling of pulmonary arteries. Calcium entry from the extracellular to the intracellular space through voltage-dependent and -independent channels play a major role in the increase of contractility of pulmonary arteries and in the loss of regulation of the proliferative behavior of the cells from the different layers of the pulmonary arterial wall. In doing so, these channels contribute to enhanced vasoconstriction of pulmonary arteries and their pathological remodeling. This review aims to summarize the evidence obtained from animal and cellular models regarding the involvement of the main plasma membrane calcium channels in these key pathophysiological processes for pulmonary arterial hypertension, discussing the potential value as pharmacological targets for therapies in the present and the future.

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Resumo Fundamento O ecocardiograma transtorácico (ETT) tem um papel de triagem no algoritmo diagnóstico da hipertensão pulmonar (HP). Estudos demonstraram uma discordância significativa entre as medições do ETT da pressão arterial pulmonar sistólica (PAPs) e da pressão atrial direita (PAD) e as obtidas pelo cateterismo do coração direito (CCD). Objetivo Comparar as medições do ETT da PAPs e da PAD com as obtidas pelo CCD em pacientes com suspeita de HP. Métodos Pacientes encaminhados a um centro de referência com probabilidade alta ou intermediária de PH ao ETT na admissão hospitalar passaram por CCD. A concordância entre a PAPs e a PAD em ambos os procedimentos foi avaliada pelo teste de Bland-Altman. Diferenças de até 10 mmHg na PAPs e de até 5 mmHg na PAD foram consideradas dentro da variabilidade do teste. A curva de característica de operação do receptor (ROC) foi construída para determinar os valores mais precisos de PAPs e VRT associados ao diagnóstico de HP pelo CCD. O nível de significância estatística adotado foi 5%. Resultados Foram incluídos noventa e cinco pacientes. A análise de Bland-Altman análise revelou um viés de 8,03 mmHg (IC 95%: -34,9 a 50,9) na PAPs e -3,30 mmHg (IC 95%: -15,9 a 9,3) na PAD. AUC da PAPs e VRT medidas pelo ETT para a discriminação de provável HP foram de 0,936 (IC 95%: 0,836 a 1,0) e 0,919 (IC 95%: 0,837 a 1,0), respectivamente. Entretanto, apenas 33,4% da estimativa ecocardiográfica da PAPs e 55,1% da PAD foram precisas, em comparação às medições obtidas pelo CCD. Conclusão O ETT tem um alto poder discriminatório como método diagnóstico de triagem para HP, apesar de apresentar discordâncias entre os valores absolutos de PAPs e PAD, em comparação às medições por CCD.

Abstract Background The transthoracic echocardiogram (TTE) plays a screening role in the diagnostic algorithm of pulmonary hypertension (PH). Studies have shown a significant disagreement between TTE measurements of the systolic pulmonary artery pressure (sPAP) and right atrial pressure (RAP) and those obtained by right heart catheterization (RHC). Objective To compare TTE measurements of sPAP and RAP with those obtained by RHC in patients being investigated for PH. Methods Patients referred to a PH reference center with a high or intermediate TTE probability of PH upon admission were submitted to RHC. The agreement between sPAP and RAP from both procedures was assessed through the Bland-Altman test. Differences of up to 10 mmHg for sPAP and 5 mmHg for RAP were considered within the variability of the test. Receiver Operating Characteristic (ROC) curve was constructed to determine the most accurate sPAP and Tricuspid regurgitation maximal velocity (TRV)values associated with the diagnosis of PH by RHC. The adopted level of statistical significance was 5%. Results Ninety-five patients were included. The Bland-Altman analysis showed a bias of 8.03 mmHg (95% CI:-34.9-50.9) for sPAP and -3.30 mmHg (95% CI:-15.9-9.3) for RAP. AUC for sPAP and TRV measured by TTE for discrimination of probable PH were 0.936 (95% CI: 0.836-1.0) and 0.919 (95% CI: 0.837-1.0), respectively. However, only 33.4% of the echocardiographic estimate of sPAP and 55.1% of RAP were accurate, as compared to the measurements obtained by RHC. Conclusion TTE has a high discriminatory power as a screening diagnostic method for PH despite presenting disagreements between sPAP and RAP absolute values when compared to RHC measurements.

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Systemic sclerosis is an autoimmune disease whose etiology remains unknown. Some patients prove refractory and require other therapies. Recently, the use of mesenchymal stem cells (MSC) for the treatment of disease refractory to conventional treatments has been considered. We present a case of refractory systemic sclerosis; Wharton's jelly mesenchymal stem cell was given in response. Decrease in perioral wrinkles, reduced telangiectasia and decrease in modified Rodnan skin score were observed two years later. A decrease in brain natriuretic peptide and improved pulmonary function were also found. And improvement of pulmonary fibrosis on high resolution tomography and capillaroscopy changes. In conclusion, MSC infusion seems to be effective and safe treatment of refractory scleroderma

La esclerosis sistémica es una enfermedad autoinmune de etiología desconocida y difícil manejo. Algunos casos que se tornan refractarios requieren terapias alternativas, como las células madre mesenquimales (MSC). Presentamos un caso de esclerosis sistémica refractaria que se llevó a terapia con MSC de gelatina de Wharton. Tras dos años, se observó ∗ Corresponding disminución en arrugas peribucales, aumento en apertura bucal, reducción de telangiectasias y en Rodnan modificado. También hubo disminución del péptido natriurético cerebral y mejora de pruebas de función pulmonar desde los seis meses de seguimiento, con mejoría en fibrosis pulmonar en tomografía de alta resolución y cambios en la capilaroscopia. En conclusión, el tratamiento con infusión de MSC parece efectivo y seguro en esclerosis sistémica refractaria.

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Background: The phenomenon of deformation of devices for closure of intracardiac defects in catheterization has been reported, but not of devices for closure of patent ductus arteriosus. Objective: to report a case of deformation of a relatively new type of device for closure of patent ductus arteriosus. Methods: report an adult with hypertensive patent ductus arteriosus and a positive balloon occlusion test will be presented, in whom the occlusion was attempted with an Occlutech®-PDA device. Results: Upon delivery, the device took the shape of a "horn" instead of a "champagne cork" (usual appearance), so it was extracted prior to it is release and the procedure was carried out with another device. Conclusion: To our knowledge, no deformation during the delivery transcatheter of the Occlutech®-PDA device has been published. Adverse events related to new technologies, as in our case, should be reported as soon as possible, since it can help manufacturers to improve their quality and provide greater safety to patients.

Introducción: Existen comunicados sobre el fenómeno de la deformación de dispositivos oclusores de defectos intracardíacos durante el cateterismo, pero no acerca de los dispositivos oclusores para el conducto arterioso persistente. Objetivo: comunicar un caso de deformación de un relativamente nuevo tipo de dispositivo oclusor de conducto arterioso persistente. Método: se presenta un paciente adulto con conducto arterioso persistente hipertensivo y prueba de oclusión con balón positiva, en quien se intentó ocluirle con un dispositivo Occlutech®-PDA. Resultado: al momento de la entrega, el dispositivo adoptó la "forma de claxon" en lugar de "corcho de champagne" (apariencia habitual), motivo por el que fue retirado previo a su liberación y el procedimiento fue llevado a cabo con otro dispositivo. Conclusión: hasta donde conocemos, no hay comunicados acerca de deformación del dispositivo Occlutech®-PDA durante su implante mediante cateterismo. Los eventos adversos relacionados a nuevas tecnologías, como el presentado en este caso, deben ser comunicados tan pronto como sea posible, ya que esto puede ayudar a los fabricantes a mejorar su calidad y proveer mayor seguridad a los pacientes.

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Background: Usually hemoglobin and hematocrit are obtained from peripheral venous blood samples processed in equipment for hematic biometry. These parameters can also be determined from arterial samples processed on blood gas equipment. Its variability when using arterial samples and this equipment is unknown, in addition, if the lack of knowledge of this variability affects clinical decisions. Objective: To know the variability of hemoglobin and hematocrit from arterial blood samples processed in blood gas equipment. Material and methods: Analytic cross design. Consecutive subjects with stable cardiopulmonary disease were blindly studied. The samples were obtained at the same time for analysis in both equipments. Variability was analyzed with Bland-Altman descriptive statistic and its magnitude with the intraclass correlation coefficient. Results: Two hundred of blood samples from 50 patients were studied, 31 (62%) women, age 62 ± 14 years old, body mass index 30.11 ± 5.69 Kg/m2. The main comparison was peripheral venous blood sample processed in laboratory equipment (SYSMEX) vs. arterial sample on blood gas equipment (GEM). The mean difference (bias) and intraclass correlation coefficient for hemoglobin were: 0.12 (-1.45, 1.23) and 0.95 (0.91, 0.97); for the hematocrit -4.4 (-0.4, 8.54), and 0.72 (0.51, 0.84). Conclusions: The mean difference and bias for hemoglobin was close to 0; the hematocrit was higher. Hemoglobin from arterial samples processed in blood gas equipment can be used to make clinical decisions.

Introducción: usualmente, la hemoglobina y el hematocrito se obtienen de sangre venosa periférica procesada en equipo convencional para biometría hemática, también pueden ser determinados de muestras arteriales procesadas en equipos para gases sanguíneos. Se desconoce su variabilidad al utilizar muestras arteriales y equipos para gases sanguíneos, además, si esta variabilidad pudiera afectar las decisiones clínicas. Objetivo: conocer la variabilidad de la hemoglobina y hematocrito de muestras sanguíneas arteriales procesadas en equipo de gases sanguíneos. Material y métodos: diseño transversal analítico. Se estudiaron de forma ciega sujetos consecutivos con enfermedad cardiopulmonar estable. Las muestras sanguíneas se adquirieron al mismo tiempo para su análisis en ambos equipos. La variabilidad se analizó con el estadístico descriptivo de Bland-Altman y su magnitud con el coeficiente de correlación intraclase. Resultados: se analizaron 200 muestras sanguíneas de 50 pacientes, 31 (62%) mujeres, edad grupal 62 ± 14 años, índice de masa corporal 30.11 ± 5.69 Kg/m2. La principal comparación: muestra sanguínea venosa periférica procesada en equipo del laboratorio (SYSMEX) frente a muestra arterial en equipo de gases sanguíneos (GEM). La diferencia media (sesgo) y el coeficiente de correlación intraclase de la hemoglobina fueron: 0.12 (-1.45, 1.23) y 0.95 (0.91, 0.97); hematocrito -4.4 (-0.4, 8.54) y 0.72 (0.51, 0.84). Conclusiones: la diferencia media y sesgo de la hemoglobina fue cercana a 0; la del hematocrito fue mayor. La hemoglobina de muestras arteriales procesadas en equipos de gases sanguíneos son útiles para tomar decisiones clínicas.

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Resumo Fundamento A hipertensão pulmonar (HP) é uma doença rara e complexa com prognóstico ruim, que exige tratamento pela vida toda. Objetivo Descrever dados de 3 anos de acompanhamento da vida real sobre o tratamento com estimuladores de guanilato ciclase solúvel (Riociguate) de pacientes com HP, medindo parâmetros atuais de avaliação de risco. Métodos Coletamos dados clínicos e epidemiológicos retrospectivamente de pacientes com HP do grupo 1 (hipertensão arterial pulmonar) e do grupo 4 (HP tromboembólica crônica). Parâmetros não invasivos e invasivos correspondentes à avaliação de risco foram analisados na linha de base e no acompanhamento. Foram realizadas análises estatísticas usando o software SPSS 18.0, e os p-valores <0,050 foram considerados estatisticamente significativos. Resultados No total, 41 pacientes tratados com riociguate foram incluídos no estudo. Entre eles, 31 já concluíram 3 anos de tratamento e foram selecionados para a seguinte análise. Na linha de base, 70,7% dos pacientes estavam nas classes funcionais III ou IV da OMS. Depois de 3 anos de tratamento, a classe funcional da OMS melhorou significativamente em todos os pacientes. Além disso, a mediana do teste de caminhada de 6 minutos (TC6M) aumentou significativamente de 394 ± 91 m na linha de base para 458 ± 100 m após 3 anos de acompanhamento (p= 0,014). O índice de sobrevida após três anos foi de 96,7%. Conclusão Em nossa coorte de vida real, a maioria dos pacientes com HP tratados com riociguate demonstraram parâmetros de risco estáveis ou melhores, especialmente no TC6M, aos 3 anos de acompanhamento.

Abstract Background Pulmonary hypertension (PH) is a rare and complex disease with poor prognosis, which requires lifelong treatment. Objective To describe 3-year follow-up real-life data on treatment with soluble guanylate cyclase stimulators (Riociguat) of patients with PH, measuring current risk assessment parameters. Methods This study retrospectively collected clinical and epidemiological data of patients with PH of group 1 (pulmonary arterial hypertension) and group 4 (chronic thromboembolic PH). Non-invasive and invasive parameters corresponding to the risk assessment were analyzed at baseline and follow-up. Statistical analyses were performed using the SPSS 18.0 software, and p-values < 0.050 were considered statistically significant. Results In total, 41 patients receiving riociguat were included in the study. Of them, 31 had already completed 3 years of treatment and were selected for the following analysis. At baseline, 70.7% of patients were in WHO functional class III or IV. After 3 years of treatment, the WHO functional class significantly improved in all patients. In addition, the median of the 6-minute walk test (6MWT) significantly increased from 394 ± 91 m at baseline to 458 ± 100 m after 3 years of follow-up (p= 0.014). The three-year survival rate was 96.7%. Conclusion In our real-life cohort, most patients with PH treated with riociguat showed stable or improved risk parameters, especially in the 6MWT, at 3 years of follow-up.

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BACKGROUND: Pulmonary thromboendarterectomy is the current treatment of choice in patients with chronic thromboembolic pulmonary hypertension. The objective of the present study was to analyze the clinical and hemodynamic outcomes and the risk factors for mortality in a cardiovascular center in Colombia. METHODS: Cohort study, conducted between 2001 and 2019. All operated patients were included in the study. Risk factors associated with mortality were established by means of a multivariate regression using the COX method and survival was established using the Kaplan-Meier method. p < 0.05 was considered statistically significant. RESULTS: Seventy-three patients were operated. Median age was 51 years, 55% of females, 79% had functional Class III and IV. The mean pulmonary arterial pressure was 50 mmHg and 640 dyn.s.cm-5 for pulmonary vascular resistance (PVR). After the intervention, there was a decrease in mean pulmonary artery pressure (p ≤ 0.001) and in PVR (p = 0.357); 21% had evidence of residual pulmonary hypertension. Only 8% and 6% continued with functional Class III and IV at 6 and 12 months, respectively. There were 15 deaths (19.1%; 12% at 30 days). The factors associated with mortality were the diastolic diameter of the right ventricle measured postoperatively (hazard ratio [HR] 10.88 95% confidence interval [CI] 1.97-62, p = 0.007), time of invasive mechanical ventilation (HR 1.06 95% CI 1.02-1.09 p = 0.004), and the presence of complications during the surgical procedure (HR 5.62 95% CI 1.94-16.22 p = 0.001). CONCLUSIONS: Pulmonary thromboendarterectomy is associated with excellent clinical and hemodynamic outcomes. The mortality risk factors found are not those usually described in the literature.

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Introdução: Hipertensão Pulmonar (HP), uma condição clínica grave, pode levar à disfunção sistólica do ventrículo direto (DSVD), com implicações prognósticas. Pacientes com suspeita de HP devem ser submetidos ao ecocardiograma transtorácico (ECOTT) para diagnóstico e avaliação, colocando-o como o principal exame de triagem e acompanhamento. Objetivo: Verificar a associação e a concordância das medidas referentes à pressão média no átrio direito (AD) e à disfunção sistólica do ventrículo direto (DSVD) ao (ECOTT) e ao cateterismo de câmaras direitas (CCD) em pacientes com (HP). Métodos: Foram incluídos indivíduos com diagnóstico de (HP). Todos os pacientes foram submetidos ao ECOTT e CCD. Avaliou-se pelo ECOTT: área do átrio direito (AAD), pressão média do átrio direito (AD) através por meio do diâmetro e da colapsabilidade da veia cava inferior (PMADECOTT ), strain AD (SAD), TAPSE (excursão sistólica do plano anular tricúspide), MAF (mudança da área fracional), SPLVD (strain da parede livre do VD) e onda s´ tricuspídea. Pelo CCD avaliaram-se pressão média do (PMADCCD ) e índice cardíaco (IC). Resultados: Dos 16 pacientes, 13 eram do sexo feminino. A idade média foi de 44,4 anos (±14,9). Constataram-se associação entre pressão média do átrio direito PMADCCD com área do átrio direito, PMADECOTT pressão média do átrio direito e SAD strain do átrio direito (r=0,845, r=0,621 e r=-0,523, respectivamente; p< 0,05). Verificou-se associação entre as categorias de risco de mortalidade, mensuradas pelas medidas AAD da área do átrio direito e pressão média do átrio direito PMADCCD (X2=10,42; p=0,003), com concordância moderada (k=0,44; p=0,012). DSVD A disfunção sistólica do ventrículo direto estava presente em dez pacientes. Houve associação entre disfunção sistólica do ventrículo direto DSVD (presente ou ausente) e índice cardíaco IC (r=0,522; p=0,04), com concordância moderada (k=0,43; p=0,037). Conclusão: As medidas do ecocardiograma transtorácico (ECOTT) e cateterismo de câmara direita (CCD) demostraram associação na avaliação da pressão média do átrio direito com melhor associação entre área do átrio direito AAD e pressão média do átrio direito (PMADCCD) . Houve associação com concordância moderada quanto à disfunção sistólica do ventrículo direto (DSVD) entre métodos. (AU)

Introduction: Pulmonary hypertension (PH), a serious clinical condition, can lead to right ventricular systolic dysfunction (RVSD) with prognostic implications. Patients with suspected PH should undergo transthoracic echocardiography (TTE) for diagnosis and evaluation as the main screening and follow-up exam. Objective: To verify the associations of and agreement between measurements of mean pressure in the right atrium (RA) and RVSD with TTE Method: Individuals diagnosed with PH were included. All patients underwent TTE and RCC. The following were evaluated by TTE: right atrial area (RAA), mean right atrial pressure through the diameter and collapsibility of the inferior vena cava (RMAPTTE), RA strain (RAS), tricuspid annular plane systolic excursion, fractional area change, RV free wall strain, and tricuspid s' wave. Mean RA pressure (RMAPRCC) and cardiac index (CI) were evaluated through the RCC. Results: Of the 16 patients, 13 were female. The mean patient age was 44.4 (±14.9) years. An association was found between RMAPRCC and AAD, RMAPTTE, and RAS (r=0.845, r=0.621, and r=-0.523, respectively; p<0.05). There was an association between the mortality risk categories measured by the RAA and RMAPRCC measures (X2=10.42; p=0.003), with moderate agreement (k=0.44; p=0.012). RVSDJ was present in 10 patients. There was an association between RVSD (present or absent) and CI (r=0.522; p=0.04) with moderate agreement (k=0.43; p=0.037). Conclusion: The TTE and RCC measurements showed an association in the assessment of mean right atrial pressure, especially between RAA and RMAPRCC. An association with RVSD and moderate agreement between methods were also noted. (AU)

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Abstract Patients with pulmonary hypertension present limited tolerance to exercise and aerobic capacity; which is reflected in the reduction in peak oxygen consumption and their functional performance. This intolerance to exercise has traditionally been attributed to cardiac and respiratory dysfunction. However, as it happens in other chronic diseases, lower-limb and respiratory muscle alterations are also involved in patients with pulmonary hypertension. Increasing evidence suggests that physical exercise training is an efficient and safe strategy. The recommendation for light and moderate-intensity exercise is made within the framework of pulmonary rehabilitation, and its benefits have been previously described. In diseases such as COPD, lower-limb muscle function, exercise tolerance and quality of life improve following exercise training. And just as with COPD, it is necessary to promote evidence-based interventions with pulmonary hypertension. This narrative review focuses on the evaluation, safety and efficacy of training the respiratory muscles and the muscles of upper and lower extremities in patients with pulmonary hypertension.

Resumen Los pacientes con hipertensión pulmonar presentan tolerancia limitada al ejercicio y disminución de la capacidad aeróbica; lo que se refleja en la reducción del consumo pico de oxígeno y su desempeño funcional. Esta intolerancia al ejercicio se ha atribuido tradicionalmente a disfunciones cardíacas y respiratorias. Sin embargo, al igual que ocurre en otras enfermedades crónicas, las alteraciones de los músculos respiratorios y de los miembros inferiores y superiores también están implicadas en los pacientes con hipertensión pulmonar. La creciente evidencia sugiere que el entrenamiento físico es una estrategia eficiente y segura. La recomendación de ejercicio de intensidad ligera y moderada se realiza en el marco de la rehabilitación pulmonar, y sus beneficios han sido descritos previamente. En enfermedades como la EPOC, la función muscular de las extremidades inferiores, la tolerancia al ejercicio y la calidad de vida mejoran después del entrenamiento físico. Y al igual que con la EPOC, es necesario promover intervenciones basadas en la evidencia con la hipertensión pulmonar. Esta revisión narrativa se centra en la evaluación, seguridad y eficacia del entrenamiento de los músculos respiratorios y de las extremidades superiores e inferiores en pacientes con hipertensión pulmonar.

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Aortopulmonary window (APW) is a rare congenital heart defect with abnormal communication between the ascending aorta and the pulmonary trunk with two separate semilunar valves. We present an autopsy case report wherein a young primigravida woman presented with progressive breathlessness and central cyanosis at 21 weeks of gestation. Echocardiography performed in the emergency room revealed elevated right-sided cardiac pressures suggestive of severe pulmonary hypertension; however, no structural cardiac defect was discernible. The patient succumbed to congestive cardiac failure and progressive hypoxia within 5 days of hospitalization. The autopsy revealed a Type I aortopulmonary window (2 cm) with patent ductus arteriosus. The lungs showed changes of severe pulmonary hypertension with superadded bronchopneumonia. This report underscores a rare presentation of APW, undiagnosed until pregnancy, leading to the Eisenmenger syndrome and death.

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ABSTRACT BACKGROUND: The mechanism of exercise limitation in idiopathic pulmonary arterial hypertension (IPAH) is not fully understood. The role of hemodynamic alterations is well recognized, but mechanical, ventilatory and gasometric factors may also contribute to reduction of exercise capacity in these individuals. OBJECTIVE: To investigate whether there is an association between ventilatory pattern and stress Doppler echocardiography (SDE) variables in IPAH patients. DESIGN AND SETTING: Single-center prospective study conducted in a Brazilian university hospital. METHODS: We included 14 stable IPAH patients and 14 age and sex-matched controls. Volumetric capnography (VCap), spirometry, six-minute walk test and SDE were performed on both the patients and the control subjects. Arterial blood gases were collected only from the patients. The IPAH patients and control subjects were compared with regard to the abovementioned variables. RESULTS: The mean age of the patients was 38.4 years, and 78.6% were women. The patients showed hypocapnia, and in spirometry 42.9% presented forced vital capacity (FVC) below the lower limit of normality. In VCap, IPAH patients had higher respiratory rates (RR) and lower elimination of CO2 in each breath. There was a significant correlation between reduced FVC and the magnitude of increases in tricuspid regurgitation velocity (TRV). In IPAH patients, VCap showed similar tidal volumes and a higher RR, which at least partially explained the hypocapnia. CONCLUSIONS: The patients with IPAH showed hypocapnia, probably related to their higher respiratory rate with preserved tidal volumes; FVC was reduced and this reduction was positively correlated with cardiac output.

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Resumo Fundamento A resistência vascular pulmonar elevada ainda é um grande problema na seleção de candidatos ao transplante cardíaco. Objetivo Nosso objetivo foi avaliar o efeito da administração de sildenafila pré-transplante cardíaco em pacientes com hipertensão pulmonar fixa. Métodos O estudo retrospectivo, de centro único, incluiu 300 candidatos a transplante cardíaco consecutivos tratados entre 2003 e 2013. Destes, 95 pacientes tinham hipertensão pulmonar fixa e, dentre eles, 30 pacientes foram tratados com sildenafila e acabaram passando pelo transplante, formando o Grupo A. O Grupo B incluiu 205 pacientes sem hipertensão pulmonar que passaram pelo transplante cardíaco. A hemodinâmica pulmonar foi avaliada antes do transplante, 1 semana e 1 ano após o transplante. A taxa de sobrevivência foi comparada entre os grupos. Neste estudo, um P valor < 0,05 foi considerado estatisticamente significativo. Resultados Após o tratamento com sildenafila, mas antes do TxC, a RVP (-39%) e a PAPs (-10%) diminuíram significativamente. A PAPs diminuiu após o TxC em ambos os grupos, mas permaneceu significativamente alta no grupo A em relação ao grupo B (40,3 ± 8,0 mmHg versus 36,5 ± 11,5 mmHg, P=0,022). Um ano após o TxC, a PAPs era 32,4 ± 6,3 mmHg no Grupo A versus 30,5 ± 8,2 mmHg no Grupo B (P=0,274). O índice de sobrevivência após o TxC 30 dias (97% no grupo A versus 96% no grupo B), 6 meses (87% versus 93%) e um ano (80% versus 91%) após o TxC não foi estatisticamente significativo (Log-rank P=0,063). Depois do primeiro ano, o índice de mortalidade era similar entre os dois grupos (sobrevivência condicional após 1 ano, Log-rank p=0,321). Conclusão Nos pacientes com HP pré-tratados com sildenafila, a hemodinâmica pós-operatória inicial e o prognóstico são numericamente piores em pacientes sem HP, mas depois de 1 ano, a mortalidade em médio e longo prazo são semelhantes. (Arq Bras Cardiol. 2021; 116(2):219-226)

Abstract Background Elevated pulmonary vascular resistance remains a major problem for heart transplant (HT) candidate selection. Objective This study sought at assess the effect of pre-HT sildenafil administration in patients with fixed pulmonary hypertension. Methods This retrospective, single-center study included 300 consecutive, HT candidates treated between 2003 and 2013, in which 95 patients had fixed PH, and of these, 30 patients were treated with sildenafil and eventually received a transplant, forming Group A. Group B included 205 patients without PH who underwent HT. Pulmonary hemodynamics were evaluated before HT, as well as 1 week after and 1 year after HT. Survival was compared between the groups. In this study, a p value < 0.05 was considered statistically significant. Results After treatment with sildenafil but before HT, PVR (-39%) and sPAP (-10%) decreased significantly. sPAP decreased after HT in both groups, but it remained significantly higher in group A vs. group B (40.3 ± 8.0 mmHg vs 36.5 ± 11.5 mmHg, p=0.022). One year after HT, sPAP was 32.4 ± 6.3 mmHg in group A vs 30.5 ± 8.2 mmHg in group B (p=0.274). The survival rate after HT at 30 days (97% in group A versus 96% in group B), at 6 months (87% versus 93%) and at one year (80% vs 91%) were not statistically significant (Log-rank p=0.063). After this first year, the attrition rate was similar among both groups (conditional survival after 1 year, Log-rank p=0.321). Conclusion In patients with severe PH pre-treated with sildenafil, early post-operative hemodynamics and prognosis are numerically worse than in patients without PH, but after 1 year, the medium to long-term mortality proved to be similar. (Arq Bras Cardiol. 2021; 116(2):219-226)

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RESUMO

Patients with pulmonary hypertension present limited tolerance to exercise and aerobic capacity; which is reflected in the reduction in peak oxygen consumption and their functional performance. This intolerance to exercise has traditionally been attributed to cardiac and respiratory dysfunction. However, as it happens in other chronic diseases, lower-limb and respiratory muscle alterations are also involved in patients with pulmonary hypertension. Increasing evidence suggests that physical exercise training is an efficient and safe strategy. The recommendation for light and moderate-intensity exercise is made within the framework of pulmonary rehabilitation, and its benefits have been previously described. In diseases such as COPD, lower-limb muscle function, exercise tolerance and quality of life improve following exercise training. And just as with COPD, it is necessary to promote evidence-based interventions with pulmonary hypertension. This narrative review focuses on the evaluation, safety and efficacy of training the respiratory muscles and the muscles of upper and lower extremities in patients with pulmonary hypertension.

Los pacientes con hipertensión pulmonar presentan tolerancia limitada al ejercicio y disminución de la capacidad aeróbica; lo que se refleja en la reducción del consumo pico de oxígeno y su desempeño funcional. Esta intolerancia al ejercicio se ha atribuido tradicionalmente a disfunciones cardíacas y respiratorias. Sin embargo, al igual que ocurre en otras enfermedades crónicas, las alteraciones de los músculos respiratorios y de los miembros inferiores y superiores también están implicadas en los pacientes con hipertensión pulmonar.La creciente evidencia sugiere que el entrenamiento físico es una estrategia eficiente y segura. La recomendación de ejercicio de intensidad ligera y moderada se realiza en el marco de la rehabilitación pulmonar, y sus beneficios han sido descritos previamente.En enfermedades como la EPOC, la función muscular de las extremidades inferiores, la tolerancia al ejercicio y la calidad de vida mejoran después del entrenamiento físico. Y al igual que con la EPOC, es necesario promover intervenciones basadas en la evidencia con la hipertensión pulmonar. Esta revisión narrativa se centra en la evaluación, seguridad y eficacia del entrenamiento de los músculos respiratorios y de las extremidades superiores e inferiores en pacientes con hipertensión pulmonar.

Assuntos

RESUMO

Clinical monitoring of pulmonary arterial hypertension in our country, in which patients come from different altitudes above sea level, forces us to rule out pulmonary hypertension in relation to chronic exposure to high heights, described in third group of international classification. When reviewing the hemodynamic variations in pulmonary pressure with exercise at altitude with respect to sea level in healthy patients, this is greater in height, this would explain that the patient with pulmonary arterial hypertension is more symptomatic while living at a higher altitude above sea level.