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1.
Article in English | WPRIM | ID: wpr-929043

ABSTRACT

Molecular hydrogen exerts biological effects on nearly all organs. It has anti-oxidative, anti-inflammatory, and anti-aging effects and contributes to the regulation of autophagy and cell death. As the primary organ for gas exchange, the lungs are constantly exposed to various harmful environmental irritants. Short- or long-term exposure to these harmful substances often results in lung injury, causing respiratory and lung diseases. Acute and chronic respiratory diseases have high rates of morbidity and mortality and have become a major public health concern worldwide. For example, coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has become a global pandemic. An increasing number of studies have revealed that hydrogen may protect the lungs from diverse diseases, including acute lung injury, chronic obstructive pulmonary disease, asthma, lung cancer, pulmonary arterial hypertension, and pulmonary fibrosis. In this review, we highlight the multiple functions of hydrogen and the mechanisms underlying its protective effects in various lung diseases, with a focus on its roles in disease pathogenesis and clinical significance.


Subject(s)
Acute Lung Injury , Aging , Animals , Anti-Inflammatory Agents , Antioxidants/chemistry , Asthma/therapy , Autophagy , COVID-19/therapy , Humans , Hydrogen/therapeutic use , Hypertension, Pulmonary/therapy , Inflammation , Lung Diseases/therapy , Lung Neoplasms/therapy , Mice , Oxidative Stress , Pulmonary Disease, Chronic Obstructive/therapy , Pulmonary Fibrosis/therapy , Pyroptosis , Reactive Oxygen Species
2.
Rev. medica electron ; 43(5): 1345-1359, 2021. tab, graf
Article in Spanish | LILACS | ID: biblio-1352115

ABSTRACT

RESUMEN Introducción: la hipertensión pulmonar es un hallazgo frecuente en la insuficiencia cardíaca. El uso del sildenafilo en estos casos es una práctica habitual, pero aún controversial por lo limitado de los estudios realizados. Objetivo: comparar las variables ecocardiográficas de hemodinamia pulmonar, en pacientes con disfunción sistólica ventricular izquierda e hipertensión pulmonar secundaria severa, antes y después del uso del sildenafilo. Materiales y métodos: se realizó un estudio de cohorte, donde se incluyeron 19 pacientes; se realizó un seguimiento de dos años. Se analizaron variables clínicas, de laboratorio y ecocardiográficas. Se evaluaron las principales variables de hemodinamia pulmonar antes del uso del sildenafilo y a las doce semanas de su indicación. Se realizó una curva de supervivencia al concluir el seguimiento. El nivel de significación estadístico empleado fue de p < 0,05. Resultados: la edad promedio fue de 56,16 ± 15,77 años y predominó el sexo masculino, con un 73,7 %. La supervivencia al término del seguimiento fue de 78,9 %. Las principales variables ecocardiográficas de hemodinamia pulmonar mostraron una reducción significativa a las doce semanas del tratamiento con sildenafilo. La supervivencia de los pacientes con una reducción del 25 % de las presiones pulmonares en el ecocardiograma realizado a las doce semanas del tratamiento, fue mayor al terminar el estudio (100 % vs 33 %, log-rank test p = 0,001). Conclusiones: posterior al uso del sildenafilo se encontró una reducción significativa de las variables de hemodinamia pulmonar en el ecocardiograma evolutivo. La sobrevida fue mayor en los pacientes que presentaron dicha reducción (AU).


ABSTRACT Introduction: pulmonary hypertension is a common finding in heart failure. The use of sildenafil in these cases is a common practice, but still controversial due to the limited number of studies carried out. Objective: to compare echocardiographic variables of pulmonary hemodynamics, in patients with left ventricular systolic dysfunction and severe secondary pulmonary hypertension, before and after the use of sildenafil. Materials and methods: a cohort study was led, including 19 patients; a two-year follow-up was carried out. Clinical, laboratory and echocardiographic variables were analyzed. The main pulmonary hemodynamics variables were evaluated before the use of sildenafil and 12 weeks after its indication. A survival curve was performed at the end of the follow-up. The statistical significance level used was p < 0.05. Results: the average age was 56.16 ± 15.77 years, and male sex predominated with 73.3 %. Survival at the end of the follow up was 78.9 %. The main echocardiographic variables of pulmonary hemodinamics showed a significant reduction at 12 weeks of treatment with sildenafil. The survival of patients with a 25 % reduction in pulmonary pressures in the echocardiogram performed at 12 weeks of treatment was greater at the end of the study (100 % vs 33 %, log-rank test p = 0.001). Conclusions: after using sildenafil, a significant reduction of pulmonary hemodynamics variables was found in the evolutionary echocardiogram. Survival was higher in patients who had this reduction (AU).


Subject(s)
Humans , Male , Female , Ventricular Dysfunction, Left/drug therapy , Hypertension, Pulmonary/drug therapy , Patients , Ventricular Dysfunction, Left/diagnosis , Ventricular Dysfunction, Left/therapy , Sildenafil Citrate/supply & distribution , Sildenafil Citrate/therapeutic use , Sildenafil Citrate/pharmacology , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy
3.
Medicina (B.Aires) ; 81(2): 180-190, June 2021. graf
Article in English | LILACS | ID: biblio-1287269

ABSTRACT

Abstract The epidemiology of pulmonary hypertension (PH), especially pulmonary arterial hypertension (PAH), has not been evaluated in our country, therefore there is no reference parameter to establishing the representativeness of this information in the national order. This registry represents the first collaborative effort to provide a knowledge base of this disease, including 5 scientific societies that represent different specialties (pediatrics, rheumatology, pulmonology and cardiology) with data from 23 Argentine provinces. These efforts involved five societies of various adult (cardiology, rheumatology, and pulmonology) and pediatric (cardiology) specialties. Subjects were grouped (1-5) in accord with the 2013 Nice classification. A total of 627 patients (mean age, 50.8±18 years; women, 69.2%) were recruited. Incident cases accounted for 53%. Functional class III-IV accounted for 69% at time of diagnosis and 33.4% at time of inclusion. Distributions in groups 1-5 were 63.6%, 15.9%, 8.3%, 9.7%, and 2.4%, respectively. Treatment consisted of diuretics (51.2%), mineralocorticoid receptor antagonists (44.7%), digoxin (16.6%), anticoagulants (39.2%), renin-angiotensin antagonists (15.5%), beta blockers (15.6%), and calcium channel blockers (8%). Rates of specific therapies usage in PAH vs. non-PAH group were 80.5% vs. 40.8% (phosphodiesterase-5 inhibitors: 71% vs. 38.6%; endothelin receptor antagonists: 54.4% vs. 14.5%; prostanoids: 14.3 vs. 3.1%; all p < 0.001). Three-year survival in PAH and non-PAH differed significantly (82.8% vs. 73.3%; p = 0.001). In the Argentine RECOPILAR registry, the clinic-epidemiologic profile was that of advanced-stage disease. Diagnostic workups and therapeutics interventions, including use of specific therapy for PAH, were consistent with current recommendations. Despite delays in diagnosis, survival was aligned with other contemporary registries.


Resumen La epidemiología de la hipertensión pulmonar (HP), especialmente la arterial (HAP), no ha sido evaluada en nuestro país, por lo cual no existe un parámetro de referencia para establecer la representatividad de esta información en el orden nacional. El presente registro representa el primer esfuerzo colaborativo para una base de conocimiento de esta enfermedad, incluyendo 5 sociedades científicas que representan a distintas especiali dades médicas (pediatría, reumatología, neumonología y cardiología) con datos de 23 provincias argentinas. Los sujetos se agruparon (1-5) de acuerdo con la clasificación de Niza de 2013. El seguimiento se completó en 583 pacientes (93%) un año después del final de la inscripción. Se incluyeron 627 pacientes (edad media, 50.8 ± 18 años; mujeres, 69.2%). Los casos incidentes representaron el 53%. La clase funcional III-IV representaba 69% en el momento del diagnóstico y 33.4% en el momento de la inclusión. Las manifestaciones clínicas fueron disnea (81.8%), fatiga (54.1%), síncope (10.8%), dolor torácico (14.7%), palpitaciones (20.9%) e insuficiencia cardíaca (20.4%). Las tasas de uso de terapias específicas en la hipertensión arterial pulmonar (HAP) frente al grupo sin HAP fueron del 80.5% frente al 40.8%. La supervivencia a tres años en los subconjuntos de HAP y no HAP difirió significativamente (82.8% vs. 73.3%; p = 0.001). En el registro RECOPILAR argentino, que aborda principalmente la HAP, el perfil clínico-epidemiológico fue el d e una enfermedad en estadios avanzados. El diag nóstico y las intervenciones terapéuticas, incluido el uso de terapia específica para la HAP, fueron consistentes con las recomendaciones actuales.


Subject(s)
Humans , Female , Child , Adult , Middle Aged , Aged , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Hypertension, Pulmonary/epidemiology , Argentina/epidemiology , Registries , Endothelin Receptor Antagonists , Anticoagulants
5.
Clinics ; 75: e1373, 2020. tab, graf
Article in English | LILACS | ID: biblio-1055875

ABSTRACT

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.


Subject(s)
Humans , Male , Female , Pulmonary Embolism/diagnosis , Thromboembolism/etiology , Granulomatous Disease, Chronic/pathology , Hypertension, Pulmonary/diagnosis , Pulmonary Embolism/complications , Pulmonary Embolism/therapy , Angiography/methods , Tomography, X-Ray Computed/methods , Chronic Disease , Retrospective Studies , Treatment Outcome , Perfusion Imaging , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Lung/blood supply , Anticoagulants/therapeutic use
6.
Ann. afr. méd. (En ligne) ; 13(4): 3829-3839, 2020. tab
Article in French | AIM | ID: biblio-1259096

ABSTRACT

Contexte et objectif. L'hypertension pulmonaire (HTP) entraîne des symptômes qui altèrent la qualité de vie des patients. En plus, le diagnostic tardif et le traitement inefficace de l'HTP réduisent considérablement la durée de vie des malades. Peu d'études sur cette maladie ont été publié en Afrique subsaharienne et pratiquement aucune en république démocratique du Congo. Le présent travail a pour objectif de déterminer la prévalence et les étiologies de l'HTP à l'hôpital provincial Général de Référence de Bukavu (HPGRB) dans le Sud-Kivu, une province de l'Est de la République Démocratique du Congo (RD Congo). Méthodes. La présente étude mono centrique s'est déroulée dans le Département de Médecine interne de l'HPGRB. C'est une étude de descriptive rétrospective qui s'est réalisée entre le 1er octobre 2014 et le 1er octobre 2019. La population de la présente étude était constituée de tout patient reçus dans le département de Médecine interne et chez qui il a été diagnostiqué une hypertension pulmonaire. Il s'agissait d'un échantillonnage exhaustif. Résultats. La prévalence de l'hypertension pulmonaire était de 3,7 %. Le sexe féminin est majoritaire avec un sex ratio de 1,49 : 1. Dans notre étude, la majorité des patients (61,3 %) avait une hypertension pulmonaire due à des cardiopathies gauches. L'hypertension pulmonaire des maladies respiratoires concernait un quart des patients (25,5 %). L'hypertension pulmonaire post embolique chronique était retrouvée chez 10,2 % des patients. Conclusion. L'hypertension pulmonaire est une pathologie assez fréquente au Sud-Kivu. Son diagnostic est possible à Bukavu et sa prise en charge demande un suivi spécialisé


Subject(s)
Democratic Republic of the Congo , Disease Management , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/therapy , Quality of Life
7.
Neumol. pediátr. (En línea) ; 14(3): 145-153, sept. 2019. tab
Article in Spanish | LILACS | ID: biblio-1087577

ABSTRACT

Pulmonary Hypertension is not a disease but a hemodynamic condition, caused by various etiologies, which are different in children and adults not only in terms of the age of onset but also as for incidence and prognosis. In the pediatric population a multifactorial etiology is typical. As this condition is rare and caused by different etiologies both a high clinical suspicion and a complete diagnostic algorithm are necessary for proper diagnosis and adequate staging to choose the best treatment option. In this manuscript the definition, classification and recommendations of treatment of PH in children will be presented, taking into account the updates based on results of studies and opinions of the experts that are exposed in the World Symposium of Pulmonary Hypertension (WSPH) that takes every 5 years.


La Hipertensión Pulmonar (HP) no es una enfermedad, sino una condición hemodinámica. Se produce por variadas etiologías, que difieren en los niños respecto de los adultos no solo en la edad de presentación, sino también en su incidencia y pronóstico, siendo una característica en la edad pediátrica la etiología multifactorial. Debido a lo poco frecuente de esta afección y a la multiplicidad de etiologías, su diagnóstico requiere de alta sospecha clínica, un completo algoritmo diagnóstico para llegar a la causa y una adecuada categorización del paciente según su riesgo para ofrecerle el tratamiento oportuno. En este trabajo se expondrán la definición, clasificación y recomendaciones de tratamiento de la HP en niños, teniendo en cuenta las actualizaciones basadas en resultados de estudios y opiniones de los expertos que se exponen en el Simposio Mundial de Hipertensión Pulmonar (WSPH) que se lleva acabo cada 5 años.


Subject(s)
Humans , Child , Hypertension, Pulmonary/classification , Hypertension, Pulmonary/diagnosis , Severity of Illness Index , Algorithms , Hypertension, Pulmonary/therapy
8.
Arq. bras. cardiol ; 113(3): 419-428, Sept. 2019. tab, graf
Article in English | LILACS | ID: biblio-1038559

ABSTRACT

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


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


Subject(s)
Humans , Hypertension, Pulmonary/diagnostic imaging , Echocardiography , Pulmonary Circulation , Risk Assessment , Pulmonary Disease, Chronic Obstructive/complications , Heart Diseases/complications , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/therapy
10.
Med. infant ; 26(2): 168-176, Junio 2019. Tab, ilus
Article in Spanish | LILACS | ID: biblio-1016785

ABSTRACT

La hipertensión arterial pulmonar (HAP) en pediatría comparte características comunes de la enfermedad en adultos, pero está asociada con varios trastornos y desafíos adicionales que requieren enfoques diferentes. En este artículo se analizan los avances recientes, los desafíos actuales y los distintos enfoques para el cuidado de niños con HAP. Se actualizan la definición, epidemiología, clasificación, diagnóstico y tratamiento. Se plantea el uso del cateterismo cardíaco como diagnóstico y las definiciones hemodinámicas de HAP, incluido el test de vasorreactividad. Se proporcionan actualizaciones sobre los enfoques pediátricos específicos del manejo médico e intervencionista de la HAP (incluyendo la derivación de Potts). Aunque la falta de datos de ensayos clínicos para el uso de la terapia dirigida a la HAP, los datos emergentes están mejorando la identificación de objetivos adecuados para la terapia orientada a objetivos en niños.(AU)


Pulmonary arterial hypertension (PAH) in children shares the typical features of the disease in adults, but is associated with different disorders and additional challenges that require different approaches. This article discusses recent developments, current challenges, and different approaches to PAH care in children. Definition, epidemiology, classification, diagnosis, and treatment are updated. The use of cardiac catheterization as a diagnostic tool and hemodynamic definitions of PAH are proposed, including the vasoreactivity test. Updates are provided on specific pediatric approaches to the medical and interventional management of PAH (including Potts shunt). In spite of the lack of clinical trial data for the use of PAH-targeted therapy, emerging data are improving the identification of appropriate targets for therapy in children (AU)


Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Hypertension, Pulmonary/classification , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/therapy , Hypertension, Pulmonary/epidemiology , Cardiac Catheterization
11.
Rev. méd. Chile ; 147(4): 426-436, abr. 2019. tab, graf
Article in Spanish | LILACS | ID: biblio-1014243

ABSTRACT

Background: Balloon pulmonary angioplasty (BPA) is a therapeutic alternative for patients with inoperable chronic thromboembolic pulmonary hypertension (CTEPH). Aim: To report the initial experience with the "refined BPA technique" with the use of intravascular images. Patients and Methods: Between June 2015 and June 2016 we selected fourteen patients with CTEPH who were considered candidates for BPA. Lesions targeted for treatment were further analyzed using intravascular imaging with optical frequency domain imaging (OFDI). We report the immediate hemodynamic results and four weeks of follow-up of the first eight patients of this series. Results: We performed 16 BPA in eight patients aged 61 ± 14 years (88% women). Mean pulmonary artery pressure (PAPm) was 48.6 ± 5.8 mmHg. Success was achieved in seven patients (88%). A mean of 2.3 segments per patient were intervened in 11 sessions (1.6 sessions/ patient). Only one patient developed lung reperfusion injury. No mortality was associated with the procedure. After the last BPA session, PAPm decreased to 37.4 ± 8.6 mmHg (p=0.02). Pulmonary vascular resistance (RVP) decreased from 858,6 ± 377,0 at baseline to 516,6 ± 323,3 Dynes/sec/cm−5 (p<0.01) and the cardiac index increased from 2.4±0.6 at baseline to 2.8±0.3 L/min/m2 (p=0.01). At 4 weeks after the last BPA, WHO functional class improved from 3.3±0.5 to 2.5±0.5 (p<0,01) and six minutes walking distance from 331±92 to 451±149 m (p=0.01). Conclusions: BPA guided by OFDI for the treatment of inoperable CTEPH patients is a safe alternative with excellent immediate hemodynamic and clinical results.


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Pulmonary Embolism/therapy , Angioplasty, Balloon/methods , Hypertension, Pulmonary/therapy , Pulmonary Embolism/physiopathology , Pulmonary Embolism/diagnostic imaging , Time Factors , Angiography/methods , Chronic Disease , Reproducibility of Results , Treatment Outcome , Tomography, Optical Coherence/methods , Hemodynamics , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/diagnostic imaging
12.
Medicina (B.Aires) ; 78(6): 453-457, Dec. 2018. ilus, tab
Article in Spanish | LILACS | ID: biblio-976147

ABSTRACT

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.


Subject(s)
Humans , Female , Adult , Aged , Pulmonary Embolism/therapy , Angioplasty, Balloon/methods , Hypertension, Pulmonary/therapy , Pulmonary Embolism/diagnostic imaging , Angiography/methods , Chronic Disease , Treatment Outcome , Endarterectomy/methods , Hypertension, Pulmonary/diagnostic imaging
13.
Arch. cardiol. Méx ; 88(1): 25-38, ene.-mar. 2018. tab
Article in Spanish | LILACS | ID: biblio-1054985

ABSTRACT

Resumen: La hipertensión portopulmonar (HPP) es una entidad poco frecuente a nivel mundial, aunque se desconocen los datos epidemiológicos en México. Sin embargo, las enfermedades crónicas del hígado son muy prevalentes en mexicanos. La HPP es el 4.◦ subtipo en frecuencia del grupo de la hipertensión arterial pulmonar. Su diagnóstico está dentro de 2 escenarios: los pacientes con sospecha de hipertensión pulmonar y los candidatos a trasplante hepático ortotópico (THO). Tanto el ecocardiograma como el cateterismo cardiaco derecho son determinantes para el diagnóstico en ambos escenarios. La HPP es un reto para el THO, pues aumenta la mortalidad perioperatoria de manera importante. El uso de terapia específica es la piedra angular de este padecimiento, como una medida para poder mejorar el desenlace de los que llegan a ser candidatos a un THO con HPP moderada a grave. Es importante reconocer que la HPP puede llegar a ser una contraindicación para el THO. Hasta el momento el papel del trasplante combinado pulmón-hígado o corazón-pulmón-hígado como una medida de curación de la enfermedad vascular pulmonar en pacientes con HPP es incierto. © 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Publicado por Masson Doyma Me´xico S.A. Este es un art´ıculo Open Access bajo la licencia CC BY-NC-ND (https://creativecommons.org/licenses/by-nc-nd/4.0/).


Abstract: Portopulmonary hypertension (PPH) is a rare condition worldwide, although epidemiological data are unknown in Mexico. However, chronic liver diseases are very prevalent in Mexico. PPH is the 4th subtype in frequency in the group of pulmonary arterial hypertension. Its diagnosis is made within 2 scenarios: patients with suspected pulmonary hypertension and candidates for orthotopic liver transplantation (OLT). Both echocardiogram and a right cardiac catheterisation are crucial for diagnosis in both cases. PPH is a challenge for OLT, since it can significantly increase perioperative mortality. The use of specific therapy is the cornerstone of this disease, as a measure to improve the outcome of those who become candidates for OLT with moderate to severe PPH. It is important to recognise that PPH can be a contraindication to OLT. The role of lung-liver transplantation or heart-lung-liver transplantation as a measure to heal pulmonary vascular disease in patients with PPH is still uncertain.© 2016 Instituto Nacional de Cardiolog´ıa Ignacio Cha´vez. Published by Masson Doyma Me´xico S.A. This is an open access article under the CC BY-NC-ND license (http://creativecommons. org/licenses/by-nc-nd/4.0/).


Subject(s)
Humans , Hypertension, Portal/complications , Hypertension, Pulmonary/complications , Liver Transplantation , Hypertension, Portal/diagnosis , Hypertension, Portal/therapy , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy
14.
Arch. cardiol. Méx ; 88(5): 503-506, dic. 2018. graf
Article in Spanish | LILACS | ID: biblio-1142162

ABSTRACT

Resumen El desarrollo de hipertensión arterial pulmonar asociada al virus de inmunodeficiencia humana reduce la probabilidad de sobrevivencia en el paciente afectado en comparación con el que no presenta esta alteración cardiopulmonar. La fisiopatogenia aún es incierta. Existen varias líneas de investigación para asociar las diferentes proteínas del virus en la lesión endo- telial. Desde el punto de vista terapéutico, existen modalidades de tratamiento que permiten una expectativa de vida aceptable.


Abstract The development of pulmonary arterial hypertension associated with human immunodeficiency virus reduces the probability of survival in the patient affected compared to those without cardiopulmonary disease. The pathophysiology is uncertain. There are several lines of research to associate the different proteins of the virus in the endothelial lesion. From a therapeutic point of view there are treatment modalities that allow an acceptable life expectancy.


Subject(s)
Humans , Viral Proteins/metabolism , HIV Infections/complications , Hypertension, Pulmonary/etiology , HIV Infections/mortality , Life Expectancy , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/therapy
15.
Braz. j. med. biol. res ; 51(12): e7437, 2018. tab, graf
Article in English | LILACS | ID: biblio-974252

ABSTRACT

Pulmonary arterial hypertension (PAH), characterized by localized increased arterial blood pressure in the lungs, is a slow developing long-term disease that can be fatal. PAH is characterized by inflammation, vascular tone imbalance, pathological pulmonary vascular remodeling, and right-sided heart failure. Current treatments for PAH are palliative and development of new therapies is necessary. Recent and relevant studies have demonstrated that epigenetic processes may exert key influences on the pathogenesis of PAH and may be promising therapeutic targets in the prevention and/or cure of this condition. The aim of the present mini-review is to summarize the occurrence of epigenetic-based mechanisms in the context of PAH physiopathology, focusing on the roles of DNA methylation, histone post-translational modifications and non-coding RNAs. We also discuss the potential of epigenetic-based therapies for PAH.


Subject(s)
Humans , DNA Methylation/genetics , RNA, Untranslated/genetics , Epigenesis, Genetic/genetics , Histone Code/genetics , Hypertension, Pulmonary/physiopathology , Hypertension, Pulmonary/genetics , Pulmonary Artery/pathology , Down-Regulation/genetics , Up-Regulation/genetics , Ubiquitination/genetics , Molecular Targeted Therapy , Hypertension, Pulmonary/therapy
16.
Rev. chil. pediatr ; 88(6): 699-706, dic. 2017. graf
Article in Spanish | LILACS | ID: biblio-1042711

ABSTRACT

Resumen Hace 50 años Northway describió la Displasia Broncopulmonar (DBP), en nacidos de pretérmino expuestos a ventilación mecánica. Desde entonces, ha aumentado la sobrevida de ellos; sin embar go, ha aparecido una "nueva DBP" y la incidencia de esta no ha disminuido. Una de las caracte rísticas de esta patología es la remodelación vascular anómala, que en su expresión más severa se conoce como Hipertensión Pulmonar (HP); con una incidencia de 17%, que es proporcional a la severidad de la DBP (33% en DBP severa); y como un factor de mortalidad (hasta un 48% mortali dad a 2 años con HP por DBP). Debido a esto resulta importante conocer los métodos diagnósticos y alternativas terapéuticas, tema que se discute en esta revisión. Considerando la alta mortalidad de la asociación HP-DBP, adquiere importancia una estrategia de tamizaje en la población de riesgo. El gold standard para el diagnóstico de HP es el cateterismo cardíaco, sin embargo, el ecocardio-grama transtorácico es una herramienta útil para el tamizaje y diagnóstico de HP en pacientes dis-plásicos, con mediciones cuantitativas y cambios cualitativos en la evaluación diagnóstica. A nivel sanguíneo el péptido natriurético tipo B (BNP), ha mostrado ser útil en el seguimiento; en cuanto a imágenes, la tomografía computarizada se utiliza en casos severos. En cuanto a las terapias, se han propuesto el óxido nítrico inhalado como vasodilatador pulmonar, los inhibidores de la fosfodies-terasas -sildenafil-, los antagonistas de la endotelina -bosentán- y los análogos de prostaciclinas -iloprost-. Aún no se cuenta con evidencia de alta calidad para su uso, dosis y duración del trata miento, pero hay variadas experiencias clínicas. Además, es relevante el cuidado interdisciplinario, destacando optimizar la nutrición. El desafío es lograr una prevención efectiva de la DBP y de sus complicaciones. Un protocolo de tamizaje de HP debe asociarse a una estratificación de riesgo y directrices de tratamiento.


Abstract 50 years ago, Northway described Broncopulmonary Dysplasia (BPD) in preterm infants exposed to mechanical ventilation. Since then, their survival has increased, nevertheless a "new BPD" has appeared and its incidence has not diminished. One of the characteristics of this pathology is the the abnormal vascular remodeling, which in its most severe expression is known as Pulmonary Hyper tension (PH); with an incidence of 17% in patients with BPD, which is proportional to the severity of the disease (33% in severe BPD), and as mortality factor (up to 48% 2-year mortality in PH-BPD). Thereby, it is important to know the diagnostic methods and therapeutic alternatives, topics discus sed in this review. Considering the high mortality in BPD associated PH, screening strategies in at risk population become important. The gold standard is cardiac catheterization; however, transtho-rathic echocardiography is a useful tool for the screening and diagnosis of PH in displasic patients, using cuantitive measures and cualitative changes in the evaluation. Seric type-B natriuretic peptide has shown to be useful for follow-up; regarding images, CT scan is used in severe cases. In terms of therapy; inhaled Nitric Oxide as a pulmonary vasodilator, phosphodiesterase inhibitors -sildenafil-, endotelin antagonists -bosentan-, and prostacyclin analogues -iloprost-, have been proposed. Their use, dosis and treatment lenght still lack support of high quality evidence, but diverse clinical expe riences have been described. Interdisciplinary care is also important, highlighting to optimize nu trition. Therefore, the challenge is to effectively prevent BPD and its complications. A PH screening protocol should be associated with risk stratification and treatment guidelines.


Subject(s)
Humans , Infant, Newborn , Bronchopulmonary Dysplasia/complications , Hypertension, Pulmonary/etiology , Oxygen Inhalation Therapy , Respiration, Artificial , Complementary Therapies , Bronchodilator Agents/therapeutic use , Bronchopulmonary Dysplasia/diagnosis , Bronchopulmonary Dysplasia/metabolism , Bronchopulmonary Dysplasia/therapy , Infant, Premature , Biomarkers/metabolism , Tomography, X-Ray Computed , Combined Modality Therapy , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/metabolism , Hypertension, Pulmonary/therapy , Nitric Oxide/therapeutic use
17.
Arch. cardiol. Méx ; 87(1): 13-17, ene.-mar. 2017.
Article in English | LILACS | ID: biblio-887489

ABSTRACT

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.


Subject(s)
Humans , Registries , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Research Design , Prospective Studies , Mexico
18.
ABC., imagem cardiovasc ; 29(3): 84-91, jul.-set. 2016. ilus
Article in Portuguese | LILACS | ID: lil-789846

ABSTRACT

Introdução: A esquistossomose, parasitose tropical, pode provocar hipertensão pulmonar grave (HAPE), que leva a remodelamento e disfunção do ventrículo direito (VD), que pode ser detectada pela diminuição da excursão sistólica do anel tricúspide (TAPSE) e da variação de áreas do VD. No VD normal, rico em fibras longitudinais, predomina o strain longitudinal, sendo menor o strain transversal. Objetivo: Avaliar, com ecocardiografia convencional e com strain bidimensional do VD, pacientes portadores de HAPE, comparando os resultados com dados clínicos, hemodinâmicos e com parâmetros ecocardiográficos obtidos em controles sadios. Material: Trinta e dois pacientes com HAPE, média etária de 45 ± 12 anos. Vinte e três controles sadios, média etária de 48 ± 18 anos.Métodos: Foram avaliados os parâmetros de função do VD (variação de áreas e TAPSE) e o gradiente de refluxo tricúspide. Foi determinado o strain longitudinal e transversal do VD em pacientes com HAPE e em controles sadios. Resultados: Entre os pacientes com HAPE e os controles sadios, a variação das áreas foi, respectivamente, 28% e 46% (p = 0,0001), o TAPSE, 1,9 cm e 2,2 cm (p = 0,02); gradiente de regurgitação tricúspide, 76 mmHg e 28 mmHg (p = 0,0001); deformação longitudinal da parede lateral do VD -22% e -37% (p = 0,0001); e deformação transversal 39% e 21% (p = 0,001). Conclusão: Pacientes com HAPE modificaram o padrão de deformação do VD, com aumento do strain transversal, provavelmente por adaptação do VD à sobrecarga pressórica. O ecocardiograma convencional também foi útil paraavaliar a função do VD na HAPE.


Introduction: Schistosomiasis is a tropical parasitic disease may cause severe pulmonary hypertension (SIPH), which leads to right ventricular (RV) remodeling and dysfunction, which can be detected by decreased tricuspid annular plane systolic excursion (TAPSE) and variation of RV areas. In normal RV, rich in longitudinal fibers, longitudinal strain prevails, and transverse strain is smaller. Objective: To assess, using conventional echocardiography and two-dimensional RV strain, patients with SIPH, comparing the results with clinical and hemodynamic data and echocardiographic parameters obtained from healthy controls. Material: Thirty-two patients with SIPH, mean age 45 ± 12 years old. Twenty-three healthy controls, mean age 48 ± 18 years old. Methods: RV function parameters (range of areas and TAPSE) and the tricuspid regurgitation gradient were evaluated. Longitudinal and transverse RV strain RV were determined in patients with SIPH and in healthy controls. Results: Among SIPH patients and healthy controls, the variation of areas was 28% and 46%, respectively (p = 0.0001), TAPSE was 1.9 cm and 2.2 cm (p = 0.02); tricuspid regurgitation gradient was 76 mmHg and 28 mmHg (p = 0.0001); RV sidewall longitudinal strain -22% and -37%(p = 0.0001); and transverse strain of 39% and 21% (p = 0.001). Conclusion: Patients with SIPH changed the RV strain pattern with increased transverse strain, probably due to RV adaptation to pressure overload. Conventional echocardiography was also useful to assess RV function in SIPH.


Subject(s)
Humans , Male , Female , Middle Aged , Ventricular Dysfunction, Right/diagnosis , Ventricular Dysfunction, Right/therapy , Echocardiography/methods , Schistosomiasis mansoni/diagnosis , Schistosomiasis mansoni/therapy , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Patients , Analysis of Variance , Heart Atria , Parasitic Diseases/complications , Parasitic Diseases/diagnosis , Prospective Studies , Ventricular Remodeling , Heart Ventricles
19.
Bogotá; IETS; mayo 2016. tab.
Monography in Spanish | LILACS, BRISA | ID: biblio-846825

ABSTRACT

Tecnologías evaluadas: Nueva: ambrisentan (principal) + tadalafil (complementario) Nueva: iloprost (principal) + bosentan (complementario), Actual: bosentan (principal) + sildenafil (complementario). Población: Pacientes diagnosticados con hipertensión pulmonar grupo 1 (HAP idiopática-HAPI y asociada-HAPA) de clase funcional II, III y IV de la NYHA/WHO en Colombia. Perspectiva: La perspectiva del presente AIP corresponde al tercero pagador, que en este caso es el Sistema General de Seguridad Social en Salud (SGSSS) en Colombia. Horizonte Temporal: El horizonte temporal de este AIP en el caso base corresponde a un año. Adicionalmente se reportan las estimaciones del impacto presupuestal para los años 2 y 3, bajo el supuesto de la inclusión en el POS en el año 1. Costos incluidos: Costo promedio ponderado de l mg de a mbrisentán, tadalafil, bosentan y sildenafil y costo promedio del mcg del iloprost en Colombia; Costo de las dosis individuales y en terapia combinada de ambrisentan, tadalafil, bosentan, sildenafil e iloprost para la población objetivo del AIP Colombia. Fuentes de Costos: Precios de ambrisentan, tadalafil, bosentan, sildenafil e iloprost en sus diferentes CUM s reportados por SISMED. Escenarios: Escenario 1: la participación de mercado de iloprost se reduce significativamente mientras que ambrisentan gana un espacio importante y bosentan disminuye ligeramente su participación en el primer año y la conserva \r\nen los dos años siguientes debido a que i) iloprost no es más efectivo que bosentan o ambrisentan, ii) la terapia combinada ambrisentan + tadalafil reduce hospitalizaciones, iii) ambrisentan es más barata que iloprost y iv) el sistema de salud en Colombia viene ganando eficiencia. Además, en la práctica la mayoría \r\nde los pacientes inician con terapia combina da y con seguridad 100% de ellos hace tránsito a terapia combinada si comenzaron con monoterapia; Escenario 2: la participación de mercado de ambrisentan se incrementa de manera pausada y llega a un nivel no superior a la participación de mercado de iloprost, que mantiene un segmento importante del mercado por la fuerte formulación de ese medicamento. En este escenario bosentan conserva su participación de mercado inalterada dada su comprobada efectividad en el tratamiento de HAPG1 y a que es la tecnología más antigua y conocida en el mercado. Resultados:\r\nEl costo de la tecnología actual de la terapia combinada bosentan + sildenafil, es de 98.773 millones COP en el año base. Bajo el escenario 1, el costo de adopción de las terapias combinadas nuevas analizadas, ambrisentan + tadalafil e iloprost + bosentan, implica un esfuerzo financiero adicional de 12.318 millones COP, mientras que bajo el escenario 2 el esfuerzo adicional es de 40.645 millones COP, es decir, 3.3 veces más. Para el segundo año el es fuerzo adicional requerido decrece con relación al año 1 ubicándose en 2.734 y de 3.071 millones COP en los escenarios 1 y 2 respectivamente. En el tercer año el impacto adicional se incrementa en 3.215 y 7.055 millones COP en cada caso.(AU)


Subject(s)
Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy , Biomedical Technology , Colombia , Costs and Cost Analysis/methods , Drug Therapy, Combination , Endothelin Receptor Antagonists/therapeutic use , Health Evaluation/economics , Iloprost/therapeutic use , Sildenafil Citrate/therapeutic use , Tadalafil/therapeutic use
20.
Rev. chil. enferm. respir ; 32(1): 13-17, mar. 2016. graf, tab
Article in Spanish | LILACS | ID: lil-784857

ABSTRACT

Introduction: The presence ofpulmonary hypertension (PH) in patients with pulmonary fibrosis is a predictor of severity andpoor survival in patients awaiting lung transplantation. Little is known about the impact of PH on survival after lung transplantation. Objective: To evaluate the effect of PH in pulmonary fibrosis patient survival after lung transplantation. Methods: Retrospective study ofpatients diagnosed with pulmonary fibrosis subjected to lung transplantation at the Instituto Nacional del Tórax during the period of August 2010 to June 2015. Thresholds of > 25 and > 35 mm Hg were chosen for mean pulmonary artery pressure (PAmean) and systolic pulmonary artery pressure (PAsystolic), respectively as indicators of PH. Results: Out of a total of 63 patients undergoing lung transplantation during the 2010-2015 period, 42 patients were diagnosed with pulmonary fibrosis. 35 of these patients had histologic diagnosis of usual interstitial pneumonia (UIP) and 7 of extrinsic allergic alveolitis in fibrotic stage. Of the total 25 patients with pulmonary fibrosis (60 percent) had PH in the pre-transplant period. A total of 15 patients died during the follow-up. There was no significant difference in survival between patients with and without PH (p = 0.74). Conclusions: Similar to international studies, we observed that the presence of PH in patients with pulmonary fibrosis did not increase risk of death in post-transplant period.


Introducción: La presencia de hipertensión pulmonar (HTP) en pacientes con fibrosis pulmonar es un predictor de gravedad y pobre sobrevida en pacientes en espera de trasplante pulmonar. Poco se sabe del impacto de la HTP en la sobrevida de los pacientes en el período post trasplante. Objetivo: Evaluar el efecto de la HTP en la sobrevida de los pacientes con fibrosis pulmonar sometidos a trasplante pulmonar. Material y Método: Estudio retrospectivo de pacientes con diagnóstico de fibrosis pulmonar sometidos a trasplante pulmonar en el Instituto Nacional de Tórax durante el período de agosto de 2010 a junio de 2015. Los criterios diagnósticos de hipertensión pulmonar fueron: presión de arteria pulmonar media mayor o igual a 25 mmHg y/o presión sistólica de arteria pulmonar mayor o igual a 35 mmHg. Resultados: De un total de 63 pacientes sometidos a trasplante pulmonar durante el período 2010-2015 en el Hospital del Tórax, 42 pacientes tenían diagnóstico de fibrosis pulmonar. De estos, 35 pacientes tenían diagnóstico histológico de neumonía intersticial usual (UIP) y 7 de alveolitis alérgica extrínseca en etapa fibrótica. Del total de pacientes con fibrosis pulmonar, 25 (60 por ciento) presentaba HTP en el período pre trasplante. Un total de 15 pacientes fallecieron durante el seguimiento. Al comparar la sobrevida post trasplante de pacientes con HTP vs sin HTP no se observó diferencia significativa (p = 0,74). Conclusiones: Al igual que en estudios internacionales, no observamos que la presencia de HTP en pacientes con fibrosis pulmonar aumente el riesgo de muerte en el período post-trasplante.


Subject(s)
Humans , Male , Female , Middle Aged , Pulmonary Fibrosis , Lung Transplantation , Hypertension, Pulmonary/epidemiology , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/therapy
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