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1.
Arq. bras. cardiol ; 119(4): 574-584, Oct. 2022. tab, graf
Article in Portuguese | LILACS-Express | LILACS | ID: biblio-1403371

ABSTRACT

Resumo Fundamento A hipertrofia e a dilatação do ventrículo direito observadas na hipertensão arterial pulmonar (HAP) prejudicam a dinâmica do ventrículo esquerdo (VE) achatando o septo interventricular. Objetivo Investigar se o treinamento físico resistido (TFR) de intensidade baixa a moderada é benéfico para funções contráteis do VE e de cardiomiócitos em ratos durante o desenvolvimento de HAP induzida por monocrotalina (MCT). Métodos Foram usados ratos Wistar machos (Peso corporal: ~ 200 g). Para avaliar o tempo até o possível surgimento de insuficiência cardíaca (ou seja, ponto de desfecho), os ratos foram divididos em dois grupos, hipertensão com sedentarismo até a insuficiência (HSI, n=6) e hipertensão com treinamento até a insuficiência (HTI, n=6). Para testar os efeitos do TFR, os ratos foram divididos entre grupos de controle sedentários (CS, n=7), hipertensão com sedentarismo (HS, n=7) e hipertensão com treinamento (HT, n=7). A HAP foi induzida por duas injeções de MCT (20 mg/kg, com um intervalo de 7 dias). Os grupos com treinamento foram submetidos a um protocolo de TFR (subir escadas; 55-65% da máxima carga carregada), 5 dias por semana. A significância estatística foi definida em p <0,05. Resultados O TFR prolongou o ponto de desfecho (~25%), melhorou a tolerância ao esforço físico (~55%) e atenuou as disfunções de contratilidade de VE e de cardiomiócitos promovidas pela MCT preservando a fração de ejeção e o encurtamento fracional, a amplitude do encurtamento, e as velocidades de contração e relaxamento nos cardiomiócitos. O TFR também preveniu os aumentos de fibrose e colágeno tipo I no ventrículo esquerdo causados pela MCT, além de manter as dimensões de miócitos e colágeno tipo III reduzidas por MCT. Conclusão O TFR de intensidade baixa a moderada é benéfico para funções contráteis de VE e cardiomiócitos em ratos durante o desenvolvimento de HAP induzida por MCT.


Abstract Background The right ventricular hypertrophy and dilation observed in pulmonary artery hypertension (PAH) damages the left ventricle (LV) dynamics by flattening the interventricular septum. Objective To investigate whether low- to moderate-intensity resistance exercise training (RT) is beneficial to LV and cardiomyocyte contractile functions in rats during the development of monocrotaline (MCT)-induced PAH. Methods Male Wistar rats (Body weight: ~ 200 g) were used. To assess the time to potential heart failure onset (i.e., end point), rats were divided into sedentary hypertension until failure (SHF, n=6) and exercise hypertension until failure (EHF, n=6) groups. To test RT effects, rats were divided into sedentary control (SC, n = 7), sedentary hypertension (SH, n=7), and exercise hypertension (EH, n=7) groups. PAH was induced by two MCT injections (20 mg/kg, with 7 days interval). Exercise groups were submitted to an RT protocol (Ladder climbing; 55-65% of carrying maximal load), 5 times/week. Statistical significance was assumed at P < 0.05. Results RT prolonged the end point (~25 %), enhanced the physical effort tolerance (~ 55%), and mitigated the LV and cardiomyocyte contractility dysfunctions promoted by MCT by preserving the ejection fraction and fractional shortening, the amplitude of shortening, and the velocities of contraction and relaxation in cardiomyocytes. RT also prevented increases in left ventricle fibrosis and type I collagen caused by MCT, and maintained the type III collagen and myocyte dimensions reduced by MCT. Conclusion Low- to moderate-intensity RT benefits LV and cardiomyocyte contractile functions in rats during the development of MCT-induced PAH.

2.
Arch. cardiol. Méx ; 92(3): 312-319, jul.-sep. 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1393825

ABSTRACT

Abstract 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.


Resumen Antecedentes: La tromboendarterectomía pulmonar es el tratamiento de elección actual en pacientes con hipertensión pulmonar tromboembólica crónica. El objetivo del presente estudio fue analizar los resultados clínicos y hemodinámicos y los factores de riesgo de mortalidad en un centro cardiovascular de Colombia. Métodos: Estudio de cohorte entre 2001 y 2019. Se incluyeron todos los pacientes operados. Los factores de riesgo asociados a la mortalidad se establecieron mediante una regresión multivariante mediante el método COX y la supervivencia se estableció mediante el método de Kaplan-Meier. Los valores de p < 0.05 se consideraron estadísticamente significativos. Resultados: se operaron 73 pacientes. La mediana de edad fue de 51 años, 55% mujeres, 79% tenían clase funcional III y IV. La presión arterial pulmonar media fue de 50 mmHg y 640 dyn.s.cm−5 para la resistencia vascular pulmonar. Después de la intervención, hubo una disminución en la presión arterial pulmonar media (p ≤ 0.001) y en la resistencia vascular pulmonar (p = 0.357). El 21% tenía evidencia de hipertensión pulmonar residual. Solo el 8% y el 6% continuaron con clase funcional III y IV a los 6 y 12 meses respectivamente. Hubo 15 muertes (19.1%; 12% a los 30 días). Los factores asociados con la mortalidad fueron el diámetro diastólico del ventrículo derecho medido en el postoperatorio (HR 10.88 IC 95% 1.97-62, p = 0.007), el tiempo de ventilación mecánica invasiva (HR 1.06 IC 95% 1.02-1.09 p = 0.004) y el presencia de complicaciones durante el procedimiento quirúrgico (HR 5.62 IC 95% 1.94-16.22 p = 0.001). Conclusiones: La tromboendartectomía pulmonar se asocia con excelentes resultados clínicos y hemodinámicos. Los factores de riesgo de mortalidad encontrados no son los habitualmente descritos en la literatura.

3.
Rev. bras. cir. cardiovasc ; 37(4): 554-565, Jul.-Aug. 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1394719

ABSTRACT

ABSTRACT Introduction: Pulmonary artery denervation (PADN) can reduce the sympathetic nervous system (SNS) activity, reduce pulmonary artery pressure (PAP), and improve the quality of life in patients with pulmonary hypertension (PH). We conducted a systematic meta-analysis of the effectiveness of PADN in the treatment of PH patients. Methods: This is a comprehensive literature search including all public clinical trials investigating the effects of PADN on PH. Outcomes were mean pulmonary artery pressure (mPAP), pulmonary vascular resistance (PVR), cardiac output (CO), right ventricular (RV) Tei index, 6-minute walk distance (6MWD), and New York Heart Association (NYHA) cardiac function grading. Results: A total of eight clinical studies with 213 PH patients who underwent PADN were included. Meta-analysis showed that after PADN, mPAP (mean difference [MD] -12.51, 95% confidence interval [CI] -17.74 to -7.27, P<0.00001) (mmHg) and PVR (MD -5.17, 95% CI -7.70 to -2.65, P<0.0001) (Wood unit) decreased significantly, CO (MD 0.59, 95% CI 0.32 to 0.86, P<0.0001) (L/min) and 6MWD (MD 107.75, 95% CI 65.64 to 149.86, P<0.00001) (meter) increased significantly, and RV Tei index (MD -0.05, 95% CI -0.28 to 0.17, P=0.63) did not change significantly. Also after PADN, the proportion of NYHA cardiac function grading (risk ratio 0.23, 95% CI 0.14 to 0.37, P<0.00001) III and IV decreased significantly. Conclusion: This meta-analysis supports PADN as a potential new treatment for PH. Further high-quality randomized controlled studies are needed.

4.
Rev. bras. cir. cardiovasc ; 37(3): 394-400, May-June 2022. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1376546

ABSTRACT

Abstract Introduction: Here we describe our technique and results of beating heart pulmonary thromboendarterectomy (PTE) with cardiopulmonary bypass (CPB) in four patients for treatment of chronic thromboembolic pulmonary hypertension (CTEPH). Methods: Retrospective analysis of data from patients who underwent PTE for CTEPH between January 2019 and September 2020. Patients were followed up with clinical assessment, 2D echocardiography, and computed tomography pulmonary angiogram. Results: Four patients were operated for CTEPH using our technique. Moderate tricuspid regurgitation (TR) and severe TR were found in two patients each. Severe right ventricular (RV) dysfunction was found in all cases. Thrombi were classified as Jamieson type II in three cases and type I in one case. Postoperative median direct manometric pulmonary artery (PA) pressures decreased (from 46.5 mmHg to 23.5 mmHg), median CPB time was 126 minutes, and median temperature was 33.35 °C. Mechanical ventilation was for a median of 19.5 hours. There was one re-exploration. Median intensive care unit stay was 7.5 days. There was no mortality. Postoperative 2D echocardiography revealed decrease in median PA systolic pressures (from 85 mmHg to 33 mmHg), improvement in RV function by tricuspid annular plane systolic excursion (median 14 mm vs. 16 mm), and improved postoperative oxygen saturations (88.5% vs. 99%). In follow-up (ranging between 2-15 months), all patients reported improvement in quality of life and were in New York Heart Association class I. Conclusion: With our described simple modifications, advances in perfusion, and blood conservation technologies, one can avoid the need for deep hypothermic circulatory arrest during PTE.

6.
Rev. urug. cardiol ; 37(1): e402, jun. 2022. ilus, tab
Article in Spanish | LILACS, BNUY, UY-BNMED | ID: biblio-1390040

ABSTRACT

Introducción: la hipertensión pulmonar asociada a cardiopatías congénitas del adulto implica un amplio espectro de situaciones clínicas complejas de difícil manejo para el cardiólogo clínico. Revisión: se repasarán los principales aspectos fisiopatológicos relacionados con el desarrollo de hipertensión pulmonar en los diferentes grupos de cardiopatías congénitas, así como los criterios para establecer el diagnóstico. Se discutirá el tratamiento guiado por perfil de riesgo y los grupos farmacológicos disponibles en nuestro medio. Por último, se discutirán grupos especiales, como el síndrome de Eisenmenger y la embarazada con cardiopatía e hipertensión pulmonar. Conclusión: es importante tener presente de todas formas que la hipertensión pulmonar en este escenario puede tener un mal pronóstico (como en el síndrome de Eisenmenger), por lo cual se deben realizar los mayores esfuerzos para hacer un diagnóstico precoz y tratamiento oportuno de estos pacientes.


Introduction: pulmonary hypertension associated with congenital heart disease in adults involves a wide spectrum of complex clinical situations that are difficult to manage for the clinical cardiologist. Review: the main pathophysiological aspects related to the development of pulmonary hypertension in the different groups of congenital heart disease will be reviewed, as well as the criteria to establish the diagnosis. Treatment guided by risk profile and the pharmacological groups available in our setting will be discussed. Finally, special groups such as Eisenmenger syndrome and pregnant women with heart disease and pulmonary hypertension will be discussed. Conclusion: it is important to keep in mind, however, that pulmonary hypertension in this scenario could have a poor prognosis (for example in Eisenmenger syndrome), which is why every effort should be made to make an early diagnosis and timely treatment of these patients.


Introdução: a hipertensão pulmonar associada à cardiopatia congênita em adultos envolve um amplo espectro de situações clínicas complexas e de difícil manejo para o cardiologista clínico. Revisão: serão revistos os principais aspectos fisiopatológicos relacionados ao desenvolvimento da hipertensão pulmonar nos diferentes grupos de cardiopatias congênitas, bem como os critérios para estabelecer o diagnóstico. Serão discutidos o tratamento guiado pelo perfil de risco e os grupos farmacológicos disponíveis em nosso meio. Por fim, serão discutidos grupos especiais como síndrome de Eisenmenger e gestantes com cardiopatia e hipertensão pulmonar. Conclusão: de qualquer forma, é importante ter em mente que a hipertensão pulmonar nesse cenário pode ter um prognóstico ruim (como na síndrome de Eisenmenger), razão pela qual todo esforço deve ser feito para o diagnóstico precoce e tratamento oportuno desses pacientes.


Subject(s)
Humans , Adult , Heart Defects, Congenital/complications , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/therapy
8.
Rev. colomb. cardiol ; 29(2): 209-214, ene.-abr. 2022. graf
Article in English | LILACS-Express | LILACS | ID: biblio-1376879

ABSTRACT

Abstract Introduction: Scimitar syndrome is a congenital malformation with an incidence of 1-3 per 100,000 live births, consisting of abnormal drainage of the right pulmonary veins. The age of diagnosis varies according to the severity of the pathology, which depends on the degree of cardiac and pulmonary malformation. The case of a patient with a late diagnosis of the syndrome due to a silent course is reported despite severe anatomical alterations. Clinical Case: A 76-year-old female was admitted due to a 6-month clinical picture of progressive dyspnea to minimal efforts. A transthoracic echocardiogram was performed showing severe pulmonary hypertension of 96 mmHg with abnormal communication between the right pulmonary veins and the inferior vena cava, confirmed by CT angiography. Discussion: The late clinical course in relation to severe anatomical malformations has not been described in the scientific literature due to its high mortality in childhood.


Resumen Introducción: El síndrome de la cimitarra es una malformación congénita con una incidencia de 1 a 3 por cada 100,000 nacidos vivos, que consiste en un drenaje anormal de las venas pulmonares derechas. La edad en el momento del diagnóstico varía según la severidad de la patología, la cual depende del grado de malformación cardíaca y pulmonar. Se reporta el caso de una paciente con diagnóstico tardío del síndrome dado por un curso silencioso a pesar de tener alteraciones anatómicas severas. Caso clínico: mujer de 76 años de edad quien se hospitaliza por un cuadro clínico de seis meses de evolución consistente en disnea progresiva de mínimos esfuerzos. Se le practica un ecocardiograma transtorácico evidenciando hipertensión pulmonar severa de 96 mmHG con una comunicación anormal entre las venas pulmonares derechas y la vena cava inferior, confirmada por angiografía por TC. Discusión: El curso clínico tardío con relación a las malformaciones anatómicas severas no se ha descrito en la literatura científica dada su alta mortalidad en la infancia.

9.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1408434

ABSTRACT

RESUMEN 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.


ABSTRACT 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.

10.
Rev. cuba. med ; 61(1)mar. 2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1408973

ABSTRACT

RESUMEN 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 % y fue confirmada en 34. Las frecuencias por sexo fueron similares y primaron los mayores de 60 años. El 41,6 % de los pacientes tuvo afectación respiratoria severa, 70,6 % presentó hipertensión pulmonar ligera y 64,7 % 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.


ABSTRACT 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% and confirmed in 34. The frequencies by sex were similar and those older than 60 years prevailed. 41.6% of the patients had severe respiratory involvement, 70.6% had mild pulmonary hypertension and 64.7% 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.

11.
Rev. cuba. med ; 61(1)mar. 2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1408982

ABSTRACT

RESUMEN Desde las perspectivas actuales, la hipertensión pulmonar es considerada un importante problema sanitario. El objetivo del artículo fue identificar las características epidemiológicas e historia natural de la hipertensión pulmonar en el contexto internacional y principalmente en la región americana. El conocimiento de la epidemiología de la hipertensión pulmonar ha experimentado un notable desarrollo con los resultados de los registros americanos, franceses, suizos, entre otros. Reportes iniciales consideraban que la enfermedad afectaba a pacientes jóvenes (edad promedio 36 años), siendo casi dos veces más frecuente en mujeres que en varones (1,7:1) y con una incidencia de 1-2 casos/1 000 000 habitantes/año y afecta a todos los grupos etarios. Las estimaciones actuales sugieren una prevalencia alrededor del 1 % de la población mundial y aumenta hasta el 10 % en los mayores de 65 años. La enfermedad auricular o ventricular izquierda y las enfermedades pulmonares son la causa más frecuente de hipertensión pulmonar. En Cuba no hay datos epidemiológicos disponibles sobre esta entidad. Los programas que ayuden a su conocimiento por la población médica se deben reforzar e impulsar un registro único de datos.


ABSTRACT From current perspectives, pulmonary hypertension is considered a major health problem. The present work was carried out to identify the epidemiological characteristics and natural history of pulmonary hypertension in the international context and mainly in the Americas. Knowledge of the epidemiology of pulmonary hypertension has undergone a remarkable development with the results of the American, French, and Swiss registries, among others. Initial reports considered that the disease affected young patients (average age 36 years), being almost twice more frequent in women than in men (1.7:1) and with an incidence of 1-2 cases/1,000,000 inhabitants/ year and affects all age groups. Current estimates suggest a prevalence around 1% of the world population and increases to 10% in those over 65 years of age. Left atrial or left ventricular disease and pulmonary diseases are the most common cause of pulmonary hypertension. In Cuba there are no epidemiological data available on this entity. The programs that help their knowledge by the medical population must be reinforced and promote a single data registry.

12.
Ars méd ; 47(1): 9-15, mar. 2022.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1380724

ABSTRACT

Introducción:la eritrocitosis excesiva afecta a los habitantes de grandes alturas como La Paz, Bolivia, a 3600 msnm. El objetivo del pre-sente trabajo es determinar la existencia de hipertensión arterial pulmonar en pacientes fumadores con eritrocitosis excesiva, residentes de la gran altura entre 20 y 60 años de edad, casos estudiados en el Instituto Boliviano de Biología de Altura (IBBA) a lo largo de tres décadas.Materiales y métodos:estudio retrospectivo en el que revisamos sistemáticamente expedientes clínicos del IBBA. Se hallan 540 expedientes clínicos de la unidad de fisiología y fisiopatología respiratoria, de los cuales el 7% (30) fueron incluidos. Se buscaron correlaciones lineales entre edad, tabaquismo, hemoglobina sérica, altitud de residencia, presión arterial pulmonar y pruebas de fun-ción pulmonar. Resultados: existe correlación entre presión arterial pulmonar sistólica y hemoglobina sérica con R=0,579 (p=0,005). El índice de Tiffeneau (TIFF.O) tiene una correlación con la presión arterial media sistémica (PAM) (p=0,006). Conclusión: en pacientes con eritrocitosis excesiva fumadores residentes de grandes alturas, existe una correlación positiva entre presión arterial pulmonar sistólica y concentración de hemoglobina sin existir correlación con valores espirométricos que sugieran patrones obstructivos.


Introduction: Excessive erythrocytosis affects some people that live at high altitudes, such as in La Paz ­ Bolivia, 3600 meters above sea level. The objective of this paper is to determine the presence of pulmonary arterial hypertension in smokers that were diagnosed with excessive erythrocytosis. These smokers live at high altitudes. The Bolivian Institute of Altitude Biology (IBBA ­ Instituto Boliviano de Bio-logía de Altura) has studied this population for three decades. Materials and methods: Retrospective study in which we systematically reviewed IBBA's clinical records. We found 540 clinical records from the respiratory physiology and pathophysiology unit, from which we included 30 (7%). We also sought correlations between serum hemoglobin, pulmonary arterial pressure, and respiratory function tests. Results: we found that the correlation between systolic pulmonary artery pressure and serum hemoglobin is R = 0.579 (p = 0.005). We also found a strong correlation between the Tiffeneau index (TIFF.O) and the mean systemic arterial pressure (p = 0.005). Conclusion: in smokers diagnosed with excessive erythrocytosis that live at high altitudes, there is a correlation between systolic pulmonary arterial pressure and hemoglobin concentration, without any of them correlating with spirometry values that suggest an obstructive pattern.

13.
Arch. argent. pediatr ; 120(1): e17-e20, feb 2022. tab, ilus
Article in English, Spanish | LILACS, BINACIS | ID: biblio-1353739

ABSTRACT

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.


Subject(s)
Humans , Female , Child , Liver Transplantation , Hepatitis, Autoimmune/complications , Hypertension, Portal/complications , Hypertension, Pulmonary/etiology , Liver Cirrhosis/complications
14.
China Pharmacy ; (12): 2040-2043, 2022.
Article in Chinese | WPRIM | ID: wpr-936986

ABSTRACT

Selexipag i s a kind of oral highly selective prostacyclin (IP)receptor agonist ,which can inhibit the contraction and proliferation of pulmonary artery smooth muscle cells. Because of its good patient compliance and high receptor affinity ,it is currently used in the treatment of pulmonary hypertension (PH). This article reviews the mechanism of action ,pharmacokinetics/ pharmacodynamics and application of selexipag in the treatment of PH. The results show that selexipag alone or in combination with endothelin receptor antagonists and (or)phosphodiesterase inhibitors can effectively reduce the risk of worsening/death events , delay disease progression ,and improve patients ’life quality in patients with adult pulmonary arterial hypertension (PAH)of WHO cardiac function Ⅱ-Ⅲ grade. However ,its application in children with PAH and patients with chronic thromboembolic PH needs further exploration.

15.
Article in Chinese | WPRIM | ID: wpr-936368

ABSTRACT

OBJECTIVE@#To explore the therapeutic mechanism of tanshinone IIA in the treatment of pulmonary arterial hypertension (PAH) in rats.@*METHODS@#A total of 100 male SD rats were randomized into 5 groups (n=20), and except for those in the control group with saline injection, all the rats were injected with monocrotaline (MCT) on the back of the neck to establish models of pulmonary hypertension. Two weeks after the injection, the rat models received intraperitoneal injections of tanshinone IIA (10 mg/kg), phosphatidylinositol 3 kinase (PI3K) inhibitor (1 mg/kg), both tanshinone IIA and PI3K inhibitor, or saline (model group) on a daily basis. After 2 weeks of treatment, HE staining and α-SMA immunofluorescence staining were used to evaluate the morphology of the pulmonary vessels of the rats. The phosphorylation levels of PI3K, protein kinase B (PKB/Akt) and endothelial nitric oxide synthase (eNOS) in the lung tissue were determined with Western blotting; the levels of eNOS and NO were measured using enzyme-linked immunosorbent assay (ELISA).@*RESULTS@#The results of HE staining and α-SMA immunofluorescence staining showed that tanshinone IIA effectively inhibited MCT-induced pulmonary artery intimamedia thickening and muscularization of the pulmonary arterioles (P < 0.01). The results of Western blotting showed that treatment with tanshinone IIA significantly increased the phosphorylation levels of PI3K, Akt and eNOS proteins in the lung tissue of PAH rats; ELISA results showed that the levels of eNOS and NO were significantly decreased in the rat models after tanshinone IIA treatment (P < 0.01).@*CONCLUSION@#Treatment with tanshinone IIA can improve MCT-induced pulmonary hypertension in rats through the PI3K/Akt-eNOS signaling pathway.


Subject(s)
Abietanes , Animals , Hypertension, Pulmonary/drug therapy , Male , Monocrotaline/toxicity , Nitric Oxide Synthase Type III/therapeutic use , Phosphatidylinositol 3-Kinase/pharmacology , Phosphatidylinositol 3-Kinases/metabolism , Proto-Oncogene Proteins c-akt/metabolism , Pulmonary Artery , Rats , Rats, Sprague-Dawley , Signal Transduction
16.
China Pharmacy ; (12): 1754-1758, 2022.
Article in Chinese | WPRIM | ID: wpr-934960

ABSTRACT

OBJECTIVE To observe th e efficacy and safety of intermittently repeated application of levosimendan in the treatment of right heart failure of pulmonary hypertension. METHODS A total of 70 cases of right heart failure of pulmonary hypertension were collected from the First Affiliated Hospital of Guangxi Medical University during Jan. 2019-May 2021,and then randomly divided into control group and observation group ,with 35 cases in each group.In control group ,the patients with moderate risk of classification of pulmonary hypertension were given Tadalafil tablets and Ambrisentan tablets ,while the patients with high risk were given Tadalafil tablets ,Ambrisentan tablets and Treprostinil injection ;both were given anti-right heart failure drugs at the same time. On the basis of treatment in the control group ,patients in the observation group were additionally intravenously pumped with levosimendan injection 12.5 mg,once a month ,at a rate of 0.05-0.1 μg/(kg·min),3 months in total. Response rate of therapy ,improvement of risk stratification of pulmonary hypertension after treatment ,and average pulmonary artery pressure ,six-minute walk distance (6MWD),serum level of N-terminal pro brain natriuretic peptide (NT-proBNP),right atrial pressure (RAP),oxygen saturation in mixed venous blood (SvO2),cardiac index (CI),right ventricular ejection fraction (RVEF),early diastolic tricuspid inflow velocity/early diastolic tricuspid annular velocity (E/Ea),tricuspid annular plane systolic excursion(TAPSE),right ventricular end-diastolic diameter (RVEDD),right ventricular end-systolic diameter (RVESD)before and after treatment ,and the occurrence of adverse reactions were observed in 2 groups. RESULTS The total response rate of observation group was significantly higher than control group after treatment (P<0.05). After treatment ,average pulmonary artery pressure,NT-proBNP level ,RAP,E/Ea,RVEDD and RVESD of 2 groups were significantly lower than before treatment ,while 6MWD,SvO2, CI, TAPSE and RVEF were significantly higher than before treatment ;NT-proBNP level and E/Ea of observation group were significantly lower than control group , while 6MWD,CI,TAPSE and RVEF were significantly higher than control group (P<0.05 or P<0.01). There was no significant difference in average pulmonary artery pressure , risk stratification improvement cases of pulmonary hypertension , RAP, SvO2, RVEDD, RVESD and the incidence of asymptomatic hypotension between 2 groups(P>0.05). CONCLUSIONS Intermittently repeated application of levosimendan can improve the function of right heart of pulmonary hypertension complicated with right heart failure and has good safety.

17.
Article in Chinese | WPRIM | ID: wpr-933687

ABSTRACT

Objective:To compare the effect of extracorporeal membrane oxygenation(ECMO)on pulmonary transplantation(LTx)in patients with idiopathic pulmonary fibrosis(IPF)complicated with pulmonary hypertension(PH).Methods:From January 2017 to December 2020, clinical data were retrospectively reviewed for 112 IPF patients complicated with PH undergoing LTx assisted by venous ECMO(VV-ECMO group, n=68)or venous arterial ECMO(VA-ECMO group, n=44). Gender, age, mechanical ventilation time, oxygenation index, cold ischemic time, preoperative gender, age, smoking history, PO 2, PCO 2, PH degree, NYHA cardiac function grade, right cardiac function, ejection fraction(EF)and complications(hypertension & diabetes)of two groups were compared. Intraoperative approach, operative duration, ECMO transfer time, blood loss, blood transfusion, urine volume, postoperative blood transfusion, mechanical ventilation time, ICU stay time, re-thoracotomy, pulmonary infection, primary graft dysfunction(PGD)and renal insufficiency were recorded. And the effects of two different diversion modes on early postoperative complications and short-term outcomes of LTx were further analyzed by multiple factors. Cox proportional risk model was employed for comparing VV-ECMO and VA-ECMO flow patterns with factors related to recipient survival after transplantation. Results:The preoperative PO 2 of 58.3(51.3, 72.0)mmHg was significantly lower in VV-ECMO bypass group than that of 73.2(63.3, 96.8)mmHg in VA-ECMO group and the difference was statistically significant( P<0.006). Compared with VV-ECMO group, 24(54.5%), 15(34.1%)and 22(50.0%)had NYHA class Ⅲ, severe PH and preoperative right heart enlargement in VA-ECMO group respectively and the differences were statistically significant compared with 17(25.0%), 6(8.8%)and 16(23.5%)in VV-ECMO group( P<0.05 for all). No significant inter-group differences existed in postoperative PGD, postoperative mechanical ventilation time(≥3 d), pulmonary infection, postoperative thoracotomy ratio, postoperative renal insufficiency, ICU stay, hospital stay and other aspects( P>0.05). And 6-month postoperative survival rates of VV-ECMO and VA-ECMO groups were 80.9% and 61.4%, respectively and no significant inter-group difference existed in short-term survival rate(6 months)after adjustment by multivariate Cox regression model( P>0.05). Multivariate statistics indicated that the risk of delayed postoperative withdrawal was 14.452-fold higher in VV-ECMO group than in VA-ECMO group and the inter-group difference was statistically significant(95% CI: 2.448-85.323, P=0.03). Conclusions:No differences exist in postoperative complications or short-term survival rate between IPF recipients with mild PH on VV-ECMO mode and IPF recipients with severe PH on VA-ECMO mode. VV-ECMO flow reversal can delay the transplant back-off time.

18.
Article in Chinese | WPRIM | ID: wpr-933682

ABSTRACT

Objective:To explore the feasibility and advantages of planned initiation of extracorporeal membrane oxygenation(ECMO)prior to liver transplantation.Methods:From November 2017 to July 2021, clinical data were retrospectively reviewed for 3 liver transplantation recipients assisted by ECMO.There were such preoperative symptoms of right ventricular dysfunction as fatigue, chest tightness and palpitations.In the first case, right heart catheterization was not performed due to patient refusal; another two patients were screened by transthoracic Doppler echocardiography(TDE)and diagnosed through right heart catheterization as portopulmonary hypertension(POPH)and pulmonary hypertension.Results:Three recipients with pulmonary hypertension received catheterization in right femoral artery and vein.After freeing of diseased liver and before blocking inferior vena cava, V-A ECMO support was performed.The dose of heparin was adjusted according to activated clotting time(ACT)and perioperative vital signs remained stable.They were ventilated for 54, 12 and 62 hours and supported by ECMO for 27, 61 and 14 hours.All were smoothly discharged.During a mean follow-up period of 26(9-22)months, liver functions were normal.Conclusions:Patients with end-stage liver disease with pulmonary hypertension should undergo routine TDE examinations during waiting period before liver transplantation.Those with pulmonary hypertension should undergo further right heart catheterization to confirm the diagnosis and severity of the disease.Planned application of ECMO through multidisciplinary consultations can expand surgical indications for liver transplantation, maintain intraoperative hemodynamic stability and facilitate smooth liver transplantation and postoperative patient recovery.

19.
Article in Chinese | WPRIM | ID: wpr-932397

ABSTRACT

Objective:To assess the configuration and systolic function of the left ventricle in patients with chronic thromboembolic pulmonary hypertension (CTEPH) by routine ultrasound, two-dimensional speckle tracking imaging and three-dimensional echocardiography, and to observe the recovery after pulmonary endarterectomy (PEA).Methods:The patients who were diagnosed with CTEPH, underwent PEA and had no left heart disease were enrolled as the CTEPH group ( n=30) in the China-Japan Friendship Hospital from November 2016 and June 2021. The right heart catheterization data before and after surgery were recorded. In the meantime, gender- and age-matched healthy individuals who sought for physical examination during the same period were included as the control group ( n=23). Echocardiography findings before and after PEA were comparatively analyzed and compared between the two groups, including left ventricular end-diastolic diameter (LVEDd), right and left ventricular cross-section ratio (RVd/LVd), left ventricular global longitudinal strain (LVGLS), left ventricular end-diastolic/systolic volume index (LVEDVi/LVESVi), left ventricular ejection fraction (LVEF) and left ventricular stroke volume (LVSV). Associations between the mean pulmonary arterial pressure (mPAP)/pulmonary vascular resistance (PVR) and left ventricular function were discussed. Results:When compared with the control group, the LVEDd, LVEDVi, LVESVi, LVSV, LVGLS and the mitral early to late diastolic flow velocity ratio (E/A) in the CTEPH group were lower (all P<0.05). There were no significant differences between the two groups regarding LVEF, cardiac output (CO), and cardiac index (CI) (all P>0.05). There were no statistical differences of the left ventricular volume and LVSV between PEA group and the control group (both P>0.05), while the LVGLS and E/A remained lower (both P<0.05). Correlation analysis showed negative associations between mPAP and LVSV as well as E/A ( r=-0.490, -0.455; both P<0.05). Conclusions:There are changes in left ventricular configuration with abnormal filling pattern and potential systolic dysfunction in CTEPH patients. The PEA surgery could lead to recovery of the left ventricular configuration and volume, but the filling pattern and LVGLS at follow-up can not recover completely.

20.
Acta Pharmaceutica Sinica B ; (6): 2315-2329, 2022.
Article in English | WPRIM | ID: wpr-929379

ABSTRACT

Pulmonary hypertension (PH) is a life-threatening disease characterized by pulmonary vascular remodeling, in which hyperproliferation of pulmonary artery smooth muscle cells (PASMCs) plays an important role. The cysteine 674 (C674) in the sarcoplasmic/endoplasmic reticulum Ca2+ ATPase 2 (SERCA2) is the critical redox regulatory cysteine to regulate SERCA2 activity. Heterozygous SERCA2 C674S knock-in mice (SKI), where one copy of C674 was substituted by serine to represent partial C674 oxidative inactivation, developed significant pulmonary vascular remodeling resembling human PH, and their right ventricular systolic pressure modestly increased with age. In PASMCs, substitution of C674 activated inositol requiring enzyme 1 alpha (IRE1α) and spliced X-box binding protein 1 (XBP1s) pathway, accelerated cell cycle and cell proliferation, which reversed by IRE1α/XBP1s pathway inhibitor 4μ8C. In addition, suppressing the IRE1α/XBP1s pathway prevented pulmonary vascular remodeling caused by substitution of C674. Similar to SERCA2a, SERCA2b is also important to restrict the proliferation of PASMCs. Our study articulates the causal effect of C674 oxidative inactivation on the development of pulmonary vascular remodeling and PH, emphasizing the importance of C674 in restricting PASMC proliferation to maintain pulmonary vascular homeostasis. Moreover, the IRE1α/XBP1s pathway and SERCA2 might be potential targets for PH therapy.

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