Hipertensión pulmonar y leiomiomas uterinos, ¿existe relación causal? Serie de casos y revisión fisiopatológica / Pulmonary Hypertension and Uterine Leiomyomas, Is there a Causal Relationship? Case Series and Pathophysiological Review

Introducción: Los leiomiomas uterinos son un tipo de neoplasia benigna de frecuente aparición en mujeres de edad reproductiva, relacionados con enfermedad tromboem- bólica venosa. Este vínculo surge del efecto producido por la compresión de fibromas que genera estasis venosa en la región pelviana. Sin embargo, este pareciera no ser el único factor que lo relaciona con el desarrollo posterior de hipertensión pulmonar, sino que su presencia es gatillo de una serie de fenómenos que influyen sobre la vasculatu - ra pulmonar y también a nivel sistémico. Método: Revisión de una serie de casos (seis) atendidos en nuestra unidad, seguido de una revisión sobre la relación entre leiomio- mas y distintas formas de hipertensión pulmonar con una revisión desde la fisiopatología. Resultado y conclusiones: Encontramos sustento bibliográfico en los múltiples caminos fisiopatológicos que relacionan los mediadores vasculares comunes, que parecieran ser el punto clave en la relación entre estas dos patologías.

Introduction: Uterine leiomyomas are a type of benign neoplasm that frequently appears in women of reproductive age, related to venous thromboembolic disease. This link arises from the effect produced by the compression of fibroids, which generates venous stasis in the pelvic region. However, this seems not to be the only factor that re- lates it to the subsequent development of pulmonary hypertension, but rather its presence is a trigger for a series of phenomena that influence the pulmonary vasculature and also at a systemic level. Method: Review of a series of cases (six) cared for in our unit, followed by a review on the relationship between leiomyomas and different forms of pulmonary hypertension with a review from the pathophysiology. Result and conclusions: We found bibliographic support in the multiple pathophysiological paths that relate the common vascular mediators, which appear to be the key point in the relationship between these two pathologies.

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

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

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

Asistencia del recién nacido con hernia diafragmática congénita / Care of newborns with congenital diaphragmatic hernia / Cuidados ao recém-nascido com hérnia diafragmática congênita

La hernia diafragmática congénita es un defecto en el diafragma que lleva a la herniación del contenido abdominal a la cavidad torácica durante el período intrauterino. La morbimortalidad está determinada por la asociación con otras malformaciones, el grado de hipoplasia pulmonar y la presencia de hipertensión pulmonar secundaria. Presenta una incidencia estimada de 1 cada 2.500-3.000 recién nacidos vivos, constituyendo en un 60% una malformación aislada. Es una patología evolutiva que puede ser diagnosticada a partir de la semana 20-24, la ubicación más habitual es la posterolateral izquierda. Se trata de una patología que requiere ingreso a cuidados intensivos al nacimiento y luego de lograda la estabilización del paciente es de sanción quirúrgica. Los objetivos de este trabajo son conocer las características generales de la patología para sistematizar el manejo logrando así un óptimo asesoramiento de los padres a nivel prenatal y seguimiento postnatal del recién nacido.

Congenital diaphragmatic hernia is a defect in the diaphragm that leads to herniation of theabdominal contents of the thoracic cavity during the intrauterine period. Morbidity and mortality are determined by the association with other malformations, the degree ofpulmonary hypoplasia and the presence of secondary pulmonary hypertension.It has an estimated incidence of 1 every 2,500-3,000 live newborns, and in 60% of the cases it is an isolated malformation. It is an evolutionary pathology that can be diagnosed from week 20-24; it is most commonly located in the left posterolateral. It is a pathology that requires intensive care at birth and after delivery and once the patient has been stabilized, surgical action is required. The objectives of this work are to understand the general characteristics of the pathology in order to refine its manipulation and achieve optimal counseling for parents at the newborn's prenatal and postnatal stages.

A hérnia diafragmática congênita é um defeito no diafragma que leva à herniação doconteúdo abdominal para a cavidade torácica durante o período intrauterino. A morbimortalidade é determinada pela associação com outras malformações, pelo grau de hipoplasia pulmonar e pela presença de hipertensão pulmonar secundária. Apresenta uma incidência estimada de 1 a cada 2.500-3.000 nascidos vivos, constituindo-se em 60% uma malformação isolada. É uma patologia evolutiva que pode ser diagnosticada a partir da semana 20-24 e a localização mais comum é o póstero-lateral esquerdo. É uma patologia que requer internação em terapia intensiva ao nascimento e após o parto. Uma vez que o paciente for estabilizado, é necessária ação cirúrgica. Os objetivos deste paper são conhecer as características gerais da patologia para melhorar o seu manejo, obtendo assim um aconselhamento ideal para os pais no nível pré-natal e no acompanhamento do crescimento pós-natal do recém-nascido.

Sildenafil in endotoxin-induced pulmonary hypertension: an experimental study

Abstract Background Sepsis and septic shock still represent great challenges in critical care medicine. Sildenafil has been largely used in the treatment of pulmonary arterial hypertension, but its effects in sepsis are unknown. The aim of this study was to investigate the hypothesis that sildenafil can attenuate endotoxin-induced pulmonary hypertension in a porcine model of endotoxemia. Methods Twenty pigs were randomly assigned to Control group (n = 10), which received saline solution; or to Sildenafil group (n = 10), which received sildenafil orally (100 mg). After 30 minutes, both groups were submitted to endotoxemia with intravenous bacterial lipopolysaccharide endotoxin (LPS) infusion (4 µg.kg-1.h-1) for 180 minutes. We evaluated hemodynamic and oxygenation functions, and also lung histology and plasma cytokine (TNFα, IL-1β, IL6, and IL10) and troponin I response. Results Significant hemodynamic alterations were observed after 30 minutes of LPS continuous infusion, mainly in pulmonary arterial pressure (from Baseline 19 ± 2 mmHg to LPS30 52 ± 4 mmHg, p< 0.05). There was also a significant decrease in PaO2/FiO2 (from Baseline 411 ± 29 to LPS180 334 ± 49, p< 0.05). Pulmonary arterial pressure was significantly lower in the Sildenafil group (35 ± 4 mmHg at LPS30, p< 0.05). The Sildenafil group also presented lower values of systemic arterial pressure. Sildenafil maintained oxygenation with higher PaO2/FiO2 and lower oxygen extraction rate than Control group but had no effect on intrapulmonary shunt. All cytokines and troponin increased after LPS infusion in both groups similarly. Conclusion Sildenafil attenuated endotoxin-induced pulmonary hypertension preserving the correct heart function without improving lung lesions or inflammation.

Multidisciplinary approach for the management of term pregnancy complicated by Eisenmenger syndrome / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

Pregnancy in patients with Eisenmenger syndrome (ES) is associated with high maternal mortality rates of 30%‒50%, or even up to 65% in the case of a cesarean section (Yuan, 2016). Here, we report a case of term pregnancy complicated with ES and severe pulmonary artery hypertension (PAH), which was managed by a multidisciplinary team (MDT) and resulted in an uncomplicated delivery via elective cesarean section. The goal of this study is to emphasize the importance of multidisciplinary approach in the management of pregnancy with ES, which can profoundly improve maternal and infant outcomes.

Analysis on missed diagnosis or misdiagnosis of anomalous origin of left coronary artery from pulmonary artery by echocardiography from one single medical center / 中华心血管病杂志

Objectives: To analyze the reasons of missed diagnosis or misdiagnosis on anomalous origin of left coronary artery from pulmonary artery (ALCAPA) by echocardiography. Methods: This is a retrospective study. Patients with ALCAPA who underwent surgical treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2008 to December 2021 were included. According to the results of preoperative echocardiography and surgical diagnosis, the patients were divided into confirmed group or missed diagnosis/misdiagnosis group. The results of preoperative echocardiography were collected, and the specific echocardiographic signs were analyzed. According to the experience of the doctors, the echocardiographic signs were divided into four types, namely clear displayed, vague/doubtful displayed, no display and no notice, and the display rate of each sign was calculated (display rate=number of clearly displayed cases/total number of cases×100%). By referring the surgical data, we analyzed and recorded the pathological anatomy and pathophysiological characteristics of the patients, and the rate of missed diagnosis/misdiagnosis of echocardiography in patients with different characteristics was compared. Results: A total of 21 patients were enrolled, including 11 males, aged 1.8 (0.8, 12.3) years (range 1 month to 47 years). Except for one patient with anomalous origin of left anterior descending artery, the others were all originated from the main left coronary artery (LCA). There were 13 cases of ALCAPA in infant and children, and 8 cases of adult ALCAPA. There were 15 cases in the confirmed group (diagnostic accuracy was 71.4% (15/21)), and 6 cases in the missed diagnosis/misdiagnosis group (three cases were misdiagnosed as primary endocardial fibroelastosis, two cases were misdiagnosed as coronary-pulmonary artery fistula; and one case was missed diagnosis). The working years of the physicians in the confirmed group were longer than those in the missed diagnosis/misdiagnosed group ((12.8±5.6) years vs. (8.3±4.7) years, P=0.045). In infants with ALCAPA, the detection rate of LCA-pulmonary shunt (8/10 vs. 0, P=0.035) and coronary collateral circulation (7/10 vs. 0, P=0.042) in confirmed group was higher than that in missed diagnosis/misdiagnosed group. In adult ALCAPA patients, the detection rate of LCA-pulmonary artery shunt was higher in confirmed group than that in missed diagnosis/misdiagnosed group (4/5 vs. 0, P=0.021). The missed diagnosis/misdiagnosis rate of adult type was higher than that of infant type (3/8 vs. 3/13, P=0.410). The rate of missed diagnosis/misdiagnosis was higher in patients with abnormal origin of branches than that of abnormal origin of main trunk (1/1 vs. 5/21, P=0.028). The rate of missed diagnosis/misdiagnosis in patients with LCA running between the main and pulmonary arteries was higher than that distant from the main pulmonary artery septum (4/7 vs. 2/14, P=0.064). The rate of missed diagnosis/misdiagnosis in patients with severe pulmonary hypertension was higher than that in patients without severe pulmonary hypertension (2/3 vs. 4/18, P=0.184). The reasons with an echocardiography missed diagnosis/misdiagnosis rate of≥50% included that (1) the proximal segment of LCA ran between the main and pulmonary arteries; (2) abnormal opening of LCA at the right posterior part of the pulmonary artery; (3) abnormal origin of LCA branches; (4) complicated with severe pulmonary hypertension. Conclusions: Echocardiography physicians' knowledge of ALCAPA and diagnostic vigilance are critical to the accuracy of diagnosis. Attention should be paid to the pediatric cases with no obvious precipitating factors of left ventricular enlargement, regardless of whether the left ventricular function is normal or not, the origin of coronary artery should be routinely explored.

Research progress on the mechanism of phenotypic transformation of pulmonary artery smooth muscle cells induced by hypoxia / 浙江大学学报·医学版

Phenotypic transformation of pulmonary artery smooth muscle cells (PASMCs) is a key factor in pulmonary vascular remodeling. Inhibiting or reversing phenotypic transformation can inhibit pulmonary vascular remodeling and control the progression of hypoxic pulmonary hypertension. Recent studies have shown that hypoxia causes intracellular peroxide metabolism to induce oxidative stress, induces multi-pathway signal transduction, including those related to autophagy, endoplasmic reticulum stress and mitochondrial dysfunction, and also induces non-coding RNA regulation of cell marker protein expression, resulting in PASMCs phenotypic transformation. This article reviews recent research progress on mechanisms of hypoxia-induced phenotypic transformation of PASMCs, which may be helpful for finding targets to inhibit phenotypic transformation and to improve pulmonary vascular remodeling diseases such as hypoxia-induced pulmonary hypertension.

Effect of platelet-derived growth factor-BB on pulmonary vascular remodeling in neonatal rats with hypoxic pulmonary hypertension and its mechanism / 中国当代儿科杂志

OBJECTIVES@#To study the effect of platelet-derived growth factor-BB (PDGF-BB) on pulmonary vascular remodeling in neonatal rats with hypoxic pulmonary hypertension (HPH).@*METHODS@#A total of 128 neonatal rats were randomly divided into four groups: PDGF-BB+HPH, HPH, PDGF-BB+normal oxygen, and normal oxygen (n=32 each). The rats in the PDGF-BB+HPH and PDGF-BB+normal oxygen groups were given an injection of 13 μL 6×1010 PFU/mL adenovirus with PDGF-BB genevia the caudal vein. After 24 hours of adenovirus transfection, the rats in the HPH and PDGF-BB+HPH groups were used to establish a neonatal rat model of HPH. Right ventricular systolic pressure (RVSP) was measured on days 3, 7, 14, and 21 of hypoxia. Hematoxylin-eosin staining was used to observe pulmonary vascular morphological changes under an optical microscope, and vascular remodeling parameters (MA% and MT%) were also measured. Immunohistochemistry was used to measure the expression levels of PDGF-BB and proliferating cell nuclear antigen (PCNA) in lung tissue.@*RESULTS@#The rats in the PDGF-BB+HPH and HPH groups had a significantly higher RVSP than those of the same age in the normal oxygen group at each time point (P<0.05). The rats in the PDGF-BB+HPH group showed vascular remodeling on day 3 of hypoxia, while those in the HPH showed vascular remodeling on day 7 of hypoxia. On day 3 of hypoxia, the PDGF-BB+HPH group had significantly higher MA% and MT% than the HPH, PDGF-BB+normal oxygen, and normal oxygen groups (P<0.05). On days 7, 14, and 21 of hypoxia, the PDGF-BB+HPH and HPH groups had significantly higher MA% and MT% than the PDGF-BB+normal oxygen and normal oxygen groups (P<0.05). The PDGF-BB+HPH and HPH groups had significantly higher expression levels of PDGF-BB and PCNA than the normal oxygen group at all time points (P<0.05). On days 3, 7, and 14 of hypoxia, the PDGF-BB+HPH group had significantly higher expression levels of PDGF-BB and PCNA than the HPH group (P<0.05), while the PDGF-BB+normal oxygen group had significantly higher expression levels of PDGF-BB and PCNA than the normal oxygen group (P<0.05).@*CONCLUSIONS@#Exogenous administration of PDGF-BB in neonatal rats with HPH may upregulate the expression of PCNA, promote pulmonary vascular remodeling, and increase pulmonary artery pressure.

Compound Tinglizi Decoction intervenes COPD-associated pulmonary hypertension through regulation of HMGB1-mediated pyroptosis and immune imbalance / 中国中药杂志

This paper aimed to investigate the effects of high mobility group box 1(HMGB1)-mediated pulmonary artery smooth muscle cell pyroptosis and immune imbalance on chronic obstructive pulmonary disease-associated pulmonary hypertension(COPD-PH) in rats and the intervening mechanism of Compound Tinglizi Decoction. Ninety rats were randomly divided into a normal group, a model group, low-dose, medium-dose, and high-dose Compound Tinglizi Decoction groups, and a simvastatin group. The rat model of COPD-PH was established by fumigation combined with lipopolysaccharide(LPS) intravascular infusion, which lasted 60 days. Rats in the low, medium, and high-dose Compound Tinglizi Decoction groups were given 4.93, 9.87, and 19.74 g·kg~(-1) Compound Tinglizi Decoction by gavage, respectively. Rats in the simvastatin group were given 1.50 mg·kg~(-1) simvastatin by gavage. After 14 days, the lung function, mean pulmonary artery pressure, and arterial blood gas of rats were analyzed. Lung tissues of rats were collected for hematoxylin-eosin(HE) staining to observe the pathological changes. Real-time fluorescent quantitative polymerase chain reaction(qRT-PCR) was used to determine the expression of related mRNA in lung tissues, Western blot(WB) was used to determine the expression of related proteins in lung tissues, and enzyme linked immunosorbent assay(ELISA) was used to determine the levels of inflammatory factors in the lung tissues of rats. The ultrastructure of lung cells was observed by transmission electron microscope. The forced vital capacity(FVC), forced expiratory volume in 0.3 second(FEV_(0.3)), FEV_(0.3)/FVC, peek expiratory flow(PEF), respiratory dynamic compliance(Cdyn), arterial partial pressure of oxygen(PaO_2), and arterial oxygen saturation(SaO_2) were increased, and resistance of expiration(Re), mean pulmonary arterial pressure(mPAP), right ventricular hypertrophy index(RVHI), and arterial partial pressure of carbon dioxide(PaCO_2) were decreased by Compound Tinglizi Decoction in rats with COPD-PH. Compound Tinglizi Decoction inhibited the protein expression of HMGB1, receptor for advanced glycation end products(RAGE), pro caspase-8, cleaved caspase-8, and gasdermin D(GSDMD) in lung tissues of rats with COPD-PH, as well as the mRNA expression of HMGB1, RAGE, and caspase-8. Pulmonary artery smooth muscle cell pyroptosis was inhibited by Compound Tinglizi Decoction. Interferon-γ(IFN-γ) and interleukin-17(IL-17) were reduced, and interleukin-4(IL-4) and interleukin-10(IL-10) were incresead by Compound Tinglizi Decoction in lung tissues of rats with COPD-PH. In addition, the lesion degree of trachea, alveoli, and pulmonary artery in lung tissues of rats with COPD-PH was improved by Compound Tinglizi Decoction. Compound Tinglizi Decoction had dose-dependent effects. The lung function, pulmonary artery pressure, arterial blood gas, inflammation, trachea, alveoli, and pulmonary artery disease have been improved by Compound Tinglizi Decoction, and its mechanism is related to HMGB1-mediated pulmonary artery smooth muscle cell pyroptosis and helper T cell 1(Th1)/helper T cell 2(Th2), helper T cell 17(Th17)/regulatory T cell(Treg) imbalance.

Hipertensión pulmonar en cardiopatías congénitas del adulto / Pulmonary hypertension in adult congenital heart disease / Hipertensão pulmonar na cardiopatia congênita do adulto

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.

Early reduction of pulmonary arterial hypertension in patients using a long-term mechanical ventricular assistance device: a cross-sectional study

ABSTRACT BACKGROUND: Severe pulmonary arterial hypertension (PAH) is a contraindication for heart transplantation (HT). It has been correlated with increased early and late mortality, mainly associated with right ventricular failure. Ventricular assistance devices (VADs) can promote reduction of intracardiac pressures and consequent reduction of PAH over the medium and long terms, thus enabling future candidature for HT. The diminution of early pulmonary pressure within this scenario remains unclear. OBJECTIVE: To evaluate the reduction of PAH and correlate data from right catheterization with the earliness of this reduction. DESIGN AND SETTING: Cross-sectional study in a general hospital in São Paulo, Brazil. METHODS: This was a retrospective analysis on the medical records of patients undergoing VAD implantation in a single hospital. Patients for whom VAD had been indicated as a bridge to candidature for HT due to their condition of constant PAH were selected. RESULTS: Four patients with VADs had constantly severe PAH. Their mean pulmonary artery systolic pressure (PASP) before VAD implantation was 66 mmHg. Over the 30-day period after the procedure, all the patients evolved with a drop in PASP to below 60 mmHg. Their new average was 36 mmHg, which was a drop of close to 50% from baseline values. The one-year survival of this sample was 100%. CONCLUSION: VAD implantation can reduce PAH levels. Early reduction occurred in all patients. Thus, use of VAD is an important bridge tool for enabling candidature for HT among patients with constantly severe PAH.

Results of percutaneous closure with device in congenital heart disease of the atrial septal defect type / Resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular

Congenital Interatrial Septal Defects (TEA) are among the most common Congenital Heart Diseases in the population, where Atrial Septal Defect stands out, which is produced by a left-right short circuit that provides greater survival and less morbidity and mortality. The objective was to determine the results of Percutaneous Closure with a Device in Congenital Heart Disease of the Atrial Communication type at the Carlos Andrade Marín Specialty Hospital (Quito) in the Hemodynamics service from January 2009 to July 2019. It is a retrospective descriptive cross-sectional study . With a population of 122 cases from the AS400 registry. Thus, it was determined: there is a female predominance in a ratio of 2:1, the ranges for defect intervention were 5.5 mm to 35 mm, post-procedure pulmonary artery pressures (systolic 18-25 mmHg and diastolic 6-10 mmHg) in 95% of patients, ventricular overload in 76% in pre-procedure patients. Concluding that 96% of our population has a significant post-procedure improvement.

Los defectos del tabique interauricular congénitos (TEA) se encuentran entre las cardiopatías congénitas más comunes en la población donde resalta la comunicación interauricular que es producido por un cortocircuito izquierda derecha que brinda mayor sobrevida y menor morbimortalidad. El objetivo fue determinar los resultados del cierre percutáneo con dispositivo en la cardiopatía congénita de tipo comunicación interauricular en el hospital de especialidades Carlos Andrade Marín (Quito) en el servicio de hemodinamia en el período enero 2009 a julio 2019. Es un estudio transversal descriptivo retrospectivo. Con una población 122 casos del registro de AS400. Encontramos un predominio en sexo femenino en una proporción de 2:1, los rangos para intervención del defecto fueron de 5,5 mm a 35 mm, las presiones de la arteria pulmonar pos-procedimiento (sistólica de 18-25 mmHg y diastólica de 6-10 mmHg) en un 95% de los pacientes, sobrecarga ventricular en un 76% en los pacientes pre-procedimiento. Concluyendo que el 96% de nuestra población tiene una mejoría significativa post-procedimiento.

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

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

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

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

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

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

La epidemiología de la hipertensión pulmonar / The epidemiology of pulmonary hypertension

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 por ciento de la población mundial y aumenta hasta el 10 por ciento 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(AU)

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 percent of the world population and increases to 10 percent 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(AU)

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

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

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

Relatório de recomendação final para adoção de um protocolo estadual no estado de Santa Catarina complementar ao protocolo clínico e diretrizes terapêuticas do ministério da saúde para tratamento farmacológico da hipertensão pulmonar no âmbito do SUS / Final recommendation report for the adoption of a state protocol in the state of Santa Catarina complementary to the clinical protocol and therapeutic guidelines of the Ministry of Health for pharmacological treatment of pulmonary hypertension within the scope of the SUS

Este relatório refere-se à análise crítica do documento "Diagnóstico e Tratamento de Hipertensão Pulmonar'', elaborado pela ACAPTI e enviado como proposta para elaboração de Protocolo Estadual de Hipertensão Pulmonar, contemplando o tratamento farmacológico de HP grupo 1 (HAP) e grupo 4 (HPTEC). No documento encaminhado pelo demandante consta uma breve introdução e contextualização da patologia, diagnóstico clínico e exames complementares, critérios de inclusão e exclusão, especialidades médicas, estratificação de risco e seguimento, tratamento medicamentoso, algoritmo de tratamento medicamentoso, acessos aos medicamentos e centros de referência. Os itens relacionados ao diagnóstico foram mantidos neste relatório, conforme o documento enviado pelo demandante. Este relatório visa avaliar e emitir um parecer técnico embasado em evidências científicas sobre a disponibilização do medicamento Selexipague, a disponibilização da terapia combinada (Ambrisentana, Bosentana, Sildenafila, Ilopros a e Selexipague) para o tratamento da HP grupo 1 (HAP), a disponibilização do medicamento Riociguate para tratamento de HP grupo 4 (HPTEC), algoritmo de tratamento medicamentoso e fluxo de acesso aos medicamentos, para posterior elaboração de um Protocolo Estadual para a patologia solicitada. O Protocolo Estadual será elaborado complementarmente ao protocolo do Ministério da Saúde, assim, caso os medicamentos englobados nele sejam incorporados para a patologia em questão pela CONITEC, o fornecimento dos mesmos passa a ser por meio do CEAF.

Perinatal Outcomes after Fetal Endoscopic Tracheal Occlusion for Isolated Congenital Diaphragmatic Hernia: Rapid Review / Resultados perinatais após oclusão traqueal endoscópica fetal por hérnia diafragmática congênita isolada: Revisão rápida

Abstract Objective To compare the perinatal outcomes of fetuses with isolated congenital diaphragmatic hernia after fetal endoscopic tracheal occlusion (FETO) and antenatal expectant management. Data sources In this rapid review, searches were conducted in the MEDLINE, PMC, EMBASE and CENTRAL databases between August 10th and September 4th, 2020. Randomized controlled trials (RCTs), quasi-RCTs or cluster-RCTs published in English in the past ten years were included. Study selection We retrieved 203 publications; 180 studies were screened by abstract. Full-text selection was performed for eight studies, and 1 single center RCTmet the inclusion criteria (41 randomized women; 20 in the FETO group, and 21 in the control group). Data collection Data collection was performed independently, by both authors, in two steps (title and abstract and full-text reading). Data synthesis There were no cases of maternal mortality. The mean gestational age at delivery was of 35.6±2.4 weeks in the intervention group, and of 37.4±1.9 weeks among the controls (p<0.01). Survival until 6 months of age was reported in 50% of the intervention group, and in 5.8% of the controls (p<0.01; relative risk: 10.5; 95% confidence interval [95%CI]: 1.5-74.7). Severe postnatal pulmonary hypertension was found in 50% of the infants in the intervention group, and in 85.7% of controls (p=0.02; relative risk: 0.6; 95%CI: 0.4-0.9). An analysis of the study indicated some concerns of risk of bias. The quality of evidence was considered moderate to low. Conclusion Current evidence is limited but suggests that FETO may be an effective intervention to improve perinatal outcomes.

Resumo Objetivo Comparar os resultados perinatais de fetos com hérnia diafragmática congênita após oclusão traqueal endoscópica fetal (OTEF) e conduta expectante pré-natal. Fontes dos dados Nesta revisão rápida, pesquisas foram conduzidas nas bases de dados MEDLINE, PMC, EMBASE e CENTRAL entre 10 de agosto de 2020 e 4 de setembro de 2020. Ensaios clínicos randomizados (ECRs), quase-ECRs e ECRs em cluster publicados em inglês nos últimos dez anos foram incluídos. Seleção dos estudos Foram recuperadas 203 publicações; 180 destas foram triadas pelo resumo. Fez-se a leitura do texto completo de 8 estudos, e 1 ECR cumpriu os critérios de inclusão (41 mulheres aleatorizadas; 20 no grupo OTEF e 21 no grupo de controle). Coleta de dados A coleta de dados realizada independentemente pelos dois autores, em duas etapas (título e resumo, e leitura do texto completo). Síntese dos dados Não houve casos de mortematerna. A idade gestacionalmédia no parto foi de 35,6±2,4 semanas no grupo de intervenção, e de 37,4±1,9 semanas entre os controles (p<0,01). A sobrevida até 6 meses de idade foi relatada em 50% do grupo de intervenção, e em 5,8% dos controles (p<0,01; risco relativo: 10,5; intervalo de confiança de 95% [IC95%]: 1,5-74,7). Hipertensão pulmonar grave ocorreu em 50% dos lactentes do grupo de intervenção, e em 85,7% dos controles (p = 0.02; risco relativo: 0,6; IC95%: 0,4-0,9). Uma análise do estudo indicou algumas preocupações quanto ao risco de viés. A qualidade da evidência foi considerada de moderada a baixa. Conclusão As evidências atuais são limitadas,mas sugeremque a OTEF pode ser uma intervenção eficaz para melhorar resultados perinatais.

Molecular hydrogen is a promising therapeutic agent for pulmonary disease / 浙江大学学报（英文版）（B辑：生物医学和生物技术）

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.