Prevalence of Clinical COPD Phenotypes in Patients Who Are Candidates for Lung Transplantation, Complications and Post-Transplant Survival.

Introduction: The prevalence of COPD phenotypes that are referred for assessment for lung transplantation is unknown, as well as whether specific phenotype influences post-transplant evolution in those patients who receive it. Material and methods: Ambispective observational study without intervention. The main objective was to know the prevalence of the different COPD phenotypes of the patients referred for the evaluation of a lung transplant. Secondary objective were to compare their clinical characteristics, to perform an analysis of post-transplant survival or complications according to their phenotype. Results: 502 patients were evaluated for lung transplantation, of which 173 met the study criteria. 31.21% of the patients were discarded for transplantation on a first visit. The final cohort of potential transplant candidates who completed the pre-transplant study was 119 (69%) and 47 finally received a lung transplant (39.5%). The most frequent COPD phenotype evaluated for lung transplantation was the exacerbator (59%), followed by the non-exacerbator (38%) and the Asthma COPD Overlap [ACO] (3%). 59.8% of the exacerbator-phenotype patients assessed did not complete the pre-transplant study. Exacerbator-phenotype patients have a lower post-transplant survival (1115.1 days [standard deviation-DE-587]) vs. ACO: 1432 days [DE 507.5] and Non-exacerbators: 1317.8 days [DE 544.7] p = 0.16), although this difference has not been statistically significant. Conclusions: The most frequent COPD phenotype assessed for lung transplantation is the exacerbator, although more than half of these patients fail to complete the pre-transplant study.

Introducción: Se desconoce la prevalencia de los fenotipos de EPOC que son remitidos para valorar el trasplante pulmonar, así como si un fenotipo específico tiene influencia en la evolución postrasplante en aquellos pacientes que lo reciben. Materiales y métodos: Estudio observacional ambispectivo, sin intervención. El objetivo principal fue conocer la prevalencia de los diferentes fenotipos de EPOC de los pacientes que fueron remitidos para valorar un trasplante de pulmón. Los objetivos secundarios fueron comparar sus características clínicas para realizar un análisis de la supervivencia postrasplante o de las complicaciones de acuerdo con el fenotipo. Resultados: Se valoró a 502 pacientes para la realización de un trasplante de pulmón, de los cuales 173 cumplían los criterios del estudio. El 31,21% de los pacientes fue descartado para el trasplante en la primera visita. La cohorte final de candidatos potenciales a trasplante que completaron el estudio pretrasplante fue de 119 pacientes (69%) y, finalmente, 47 recibieron el trasplante de pulmón (39,5%). El fenotipo de EPOC que se evaluó con mayor frecuencia para el trasplante de pulmón fue el agudizador (59%), seguido del no agudizador (38%) y el mixto EPOC-asma (3%). El 59,8% de los pacientes con fenotipo agudizador valorados no completó el estudio pretrasplante. Los pacientes con fenotipo agudizador presentan una supervivencia postrasplante más baja (1.115,1 días; desviación estándar [DE]: 587) frente a los mixto EPOC-asma (1.432 días; DE: 507,5) y los no agudizadores (1.317,8 días; DE: 544,7; p = 0,16), aunque esta diferencia no fue estadísticamente significativa. Conclusiones: El fenotipo más frecuentemente estudiado para el trasplante de pulmón es el agudizador, aunque más de la mitad de estos pacientes no completa el estudio pretrasplante.

Efficacy and safety of the combination of reduced duration prophylaxis followed by immuno-guided prophylaxis to prevent cytomegalovirus disease in lung transplant recipients (CYTOCOR STUDY): an open-label, randomised, non-inferiority clinical trial.

INTRODUCTION: Prolonged use of antivirals to prevent the development of cytomegalovirus (CMV) disease in lung transplant patients has been shown to have significant side effects, for which alternatives are being sought to reduce their use. The monitoring of cell immunity against CMV could be an alternative as it has shown to be useful in identifying transplant patients at low risk of infection, who could benefit from shorter prophylaxis. The aim of the CYTOCOR study is to demonstrate that the combination of a reduced prophylaxis strategy with subsequent CMV-specific immunological monitoring would allow CMV infection to be controlled in lung transplant patients as effectively as the usual strategy (prophylaxis followed by pre-emptive therapy), while reducing the side effects of antivirals due to the shorter duration of prophylaxis. METHODS AND ANALYSIS: Phase III randomised, open, multicentre, parallel, non-inferiority clinical trial to study the efficacy and safety of the combination of a prophylaxis strategy up to month +3 post-transplant followed by immuno-guided prophylaxis using the QuantiFERON-CMV technique up to month +12 post-transplant to prevent CMV disease in CMV-seropositive lung transplant recipients. This strategy will be compared with a combination of a usual prophylaxis strategy up to month +6 post-transplant followed by pre-emptive therapy up to month +12. To study the incidence of CMV disease, patients will be followed up to 18 months post-transplantation. A total of 150 patients are expected to be recruited for the study. ETHICS AND PUBLIC DISSEMINATION: The clinical trial has been approved by the Research Ethics Committees and authorised by the Spanish Agency of Medicines and Medical Devices (AEMPS).If the hypothesis of this clinical trial is verified, the dissemination of the results could change clinical practice by increasing knowledge about the safety and efficacy of discontinuing valganciclovir prophylaxis in lung transplant recipients. TRIAL REGISTRATION NUMBER: NCT03699254.

Clasificación de la hipertensión arterial pulmonar basada en el estudio genético y familiar / Classification of pulmonary arterial hypertension by genetic and familial testing

No disponible

Tratamiento con esteroides sistémicos en agudización grave de enfermedad pulmonar obstructiva crónica: empleo de pautas cortas en práctica clínica habitual y relación con la estancia hospitalaria / Treatment With Systemic Steroids in Severe Chronic Obstructive Pulmonary Disease Exacerbations: Use of Short Regimens in Routine Clinical Practice and Their Impact on Hospital Stay

Introducción: Se desconoce si en la práctica clínica habitual se siguen las recomendaciones de las guías de práctica clínica con respecto al tratamiento de las exacerbaciones de la EPOC con pautas cortas (PC) de corticoesteroides sistémicos (CS). Método: Estudio de cohortes, prospectivo y observacional en pacientes que ingresan por una agudización grave de su EPOC. La variable principal fue porcentaje de pacientes que recibían PC de CS como tratamiento en la exacerbación grave (dosis acumulada total de 200 a 300 mg y una duración de 5-6 días). Las variables secundarias fueron porcentaje de pacientes con duración o dosis corta, dosis en las primeras 24 horas, días de CS intravenosos y duración de la estancia hospitalaria (EH). Se realizó regresión lineal simple con días de estancia hospitalaria como variable dependiente y análisis multivariante con factores asociados a estancia hospitalaria. Resultados: Se evaluaron 158 pacientes; 4,4% (7) pacientes recibieron una PC de CS. El 8,7% recibió un tratamiento corto y el 15,8% una duración reducida. La mediana de dosis y duración de CS fue 602,5 mg (rango intercuartílico: 430-850) y 14 (rango intercuartílico: 4-36) días respectivamente. Observamos asociación entre más días de CS y una mayor EH (p < 0,001) y una mayor dosis de CS intrahospitalaria e incremento de EH (p < 0,001).Los factores asociados con EH fueron dosis de CS intrahospitalaria recibida (0,01 [IC 95%: 0,007-0,013]; p < 0,001), días de tratamiento esteroideo (0,14 [IC 95%: 0,03-0,25]; p = 0,009) y PAFI (cociente pO2/FiO2) al ingreso (-0,012 [IC 95%: -0,012 a -0,002]; p = 0,015). Conclusiones: Las pautas de CS empleadas en la práctica clínica habitual son más prolongadas y a una mayor dosis que las recomendadas, asociando una mayor estancia hospitalaria (AU)

Introduction: It is not known whether clinical practice guidelines for the treatment of COPD exacerbations with short coursesofsystemic corticosteroids(SC-SCS) are followed in clinical practice. Method: Prospective, observational cohort study in patients admitted due to severe COPD exacerbation. The primary endpoint was the percentage of patients who received SC-SCS as treatment for severe exacerbation (doses of 200-300 mg for 5-6 days). Secondary variables were percentageof patients with duration or reduced dose, dose in the first 2 4hours, days of intravenous systemic corticosteroids (SCS), and duration of hospital length of stay (LOS). Simple linear regression was performed with LOS as a dependent variable and multivariate analysis with factors associated with LOS. Results: 158 patients were evaluated. 4.4% (7) patients received SC-SCS, 8.7% received a reduced dose and duration was reduced in 15.8%. The median dose and duration of SCS were 602.5mg (200-1625) and 14 (4-36) days, respectively. We observed an association between days of SCSand LOS (P < .001) and doses of intrahospitalSCSand LOS (P < .001). Factors associated with LOS were doses of intrahospitalSCS received (.01 [95% CI:.007-.013]; P < 0.001), days of steroid treatment (.14 [95% CI .03-.25], P = .009) and PAFI (pO2/FiO2 ratio) at admission (-.012 [95% CI: -.012 to -.002], P = .015). Conclusions: The SCS schedules used in routine clinical practice are longer and administered at a higher dose than recommended, leading toa longer hospital stay (AU)

Discordance of physician clinical judgment vs. pneumonia severity index (PSI) score to admit patients with low risk community-acquired pneumonia: a prospective multicenter study.

BACKGROUND: The relationship between clinical judgment and the pneumonia severity index (PSI) score in deciding the site of care for patients with community-acquired pneumonia (CAP) has not been well investigated. The objective of the study was to determine the clinical factors that influence decision-making to hospitalize low-risk patients (PSI ≤2) with CAP. METHODS: An observational, prospective, multicenter study of consecutive CAP patients was performed at five hospitals in Spain. Patients admitted with CAP and a PSI ≤2 were identified. Admitting physicians completed a patient-specific survey to identify the clinical factors influencing the decision to admit a patient. The reason for admission was categorized into 1 of 6 categories. We also assessed whether the reason for admission was associated with poorer clinical outcomes [intensive care unit (ICU) admission, 30-day mortality or readmission]. RESULTS: One hundred and fifty-five hospitalized patients were enrolled. Two or more reasons for admission were seen in 94 patients (60.6%), including abnormal clinical test results (60%), signs of clinical deterioration (43.2%), comorbid conditions (28.4%), psychosocial factors (28.4%), suspected H1N1 pneumonia (20.6%), and recent visit to the emergency department (ED) in the past 2 weeks (7.7%). Signs of clinical deterioration and abnormal clinical test results were associated with poorer clinical outcomes (P<0.005). CONCLUSIONS: Low-risk patients with CAP and a PSI ≤2 are admitted to the hospital for multiple reasons. Abnormal clinical test results and signs of clinical deterioration are two specific reasons for admission that are associated with poorer clinical outcomes in low risk CAP patients.

Treatment With Systemic Steroids in Severe Chronic Obstructive Pulmonary Disease Exacerbations: Use of Short Regimens in Routine Clinical Practice and Their Impact on Hospital Stay. / Tratamiento con esteroides sistémicos en agudización grave de enfermedad pulmonar obstructiva crónica: empleo de pautas cortas en práctica clínica habitual y relación con la estancia hospitalaria.

INTRODUCTION: It is not known whether clinical practice guidelines for the treatment of COPD exacerbations with short coursesofsystemic corticosteroids(SC-SCS) are followed in clinical practice. METHOD: Prospective, observational cohort study in patients admitted due to severe COPD exacerbation. The primary endpoint was the percentage of patients who received SC-SCS as treatment for severe exacerbation (doses of 200-300mg for 5-6 days). Secondary variables were percentageof patients with duration or reduced dose, dose in the first 24hours, days of intravenous systemic corticosteroids (SCS), and duration of hospital length of stay (LOS). Simple linear regression was performed with LOS as a dependent variable and multivariate analysis with factors associated with LOS. RESULTS: 158 patients were evaluated. 4.4% (7) patients received SC-SCS, 8.7% received a reduced dose and duration was reduced in 15.8%. The median dose and duration of SCS were 602.5mg (200-1625) and 14 (4-36) days, respectively. We observed an association between days of SCSand LOS (P<.001) and doses of intrahospitalSCSand LOS (P<.001). Factors associated with LOS were doses of intrahospitalSCS received (.01 [95% CI:.007-.013]; P<0.001), days of steroid treatment (.14 [95% CI .03-.25], P=.009) and PAFI (pO2/FiO2 ratio) at admission (-.012 [95% CI: -.012 to -.002], P=.015). CONCLUSIONS: The SCS schedules used in routine clinical practice are longer and administered at a higher dose than recommended, leading toa longer hospital stay.

Acute eosinophilic pneumonia as a complication of influenza A (H1N1) pulmonary infection.

Acute eosinophilic pneumonia (AEP) is a rare disease characterized by its acute onset and a clinical presentation simulating a bacterial pneumonia. Although it can be idiopathic, it has been described related to drugs, toxic agents and infections, mostly parasitic. We describe the case of influenza A (H1N1) severe pneumonia complicated by an acute eosinophilic pneumonia. Patient presented with respiratory failure and diffuse ground-glass opacities at chest-computed tomography. Clinical suspicion for this complication and bronchoalveolar lavage with cellular count analysis is crucial.

Synchronized parallel deployment of vascular plugs for treating large aneurysms of pulmonary arteries.

We report an endovascular technique for the treatment of large neck pulmonary artery aneurysms. This technique consists of the synchronized parallel deployment of two vascular plugs.

[Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome]. / Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger.

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. METHODS: Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. RESULTS: Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. CONCLUSIONS: Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension.

[Clinical efficacy of sildenafil in patients with pulmonary hypertension in functional class II or III]. / Hipertensión pulmonar: eficacia clínica del sildenafilo en clases funcionales II-III.

OBJECTIVE: To assess the efficacy of treatment with sildenafil monotherapy in patients with pulmonary hypertension. PATIENTS AND METHODS: An observational study was undertaken in 11 patients with pulmonary hypertension in functional class II or III who received treatment with sildenafil (150 mg/day). Seven of the patients had inoperable chronic thromboembolic pulmonary hypertension and 4 had pulmonary arterial hypertension. To assess treatment response, the following parameters were assessed during follow-up at 3, 6, and 12 months: exercise tolerance in the 6-minute walk test, change in functional class, and systolic pulmonary arterial pressure measured by echocardiography. RESULTS: We observed a significant improvement in exercise tolerance, as shown by increased 6-minute walk distance after 3, 6, and 12 months of treatment (increases of 20, 67, and 95 m, respectively). All patients showed an improvement in functional class. The results of echocardiography did not reveal statistically significant differences in systolic pulmonary arterial pressure between baseline and 6 or 12 months of treatment. No significant adverse effects were observed, although sildenafil treatment was suspended in 1 patient due to persistent headache. CONCLUSIONS: The results of this study confirm that sildenafil is an effective drug for the management of pulmonary arterial hypertension and inoperable chronic thromboembolic pulmonary hypertension both in the short term and medium to long term, and that the drug is well tolerated and shows few side effects.

Hipertensión pulmonar: eficacia clínica del sildenafilo en clases funcionales II-III / Clinical efficacy of sildenafil in patients with pulmonary hypertension in functional class II or III

Objetivo: Valorar la eficacia del tratamiento con sildenafilo en monoterapia en pacientes con hipertensión pulmonar (HP). Pacientes y métodos: Se ha realizado un estudio observacional de 11 pacientes con HP en clases funcionales II y III, a quienes se administró tratamiento con sildenafilo (150 mg/día). Siete presentaban HP tromboembólica crónica y no quirúrgica y 4, hipertensión arterial pulmonar. Para valorar la respuesta analizamos a los 3, 6 y 12 meses los siguientes parámetros: tolerancia al esfuerzo mediante el test de la marcha de 6 min, cambio de clase funcional y ecocardiograma para valorar la presión sistólica en la arteria pulmonar. Resultados: Encontramos una mejoría significativa de la tolerancia al esfuerzo, con un incremento de la distancia caminada en 6 min, a los 3, 6 y 12 meses de tratamiento (+20, +67 y +95 m, respectivamente). Todos los pacientes mejoraron de clase funcional. El seguimiento ecocardiográfico no mostró diferencias estadísticamente significativas entre los valores basal y a los 6 y 12 meses de tratamiento de la presión sistólica en la arteria pulmonar. No observamos efectos adversos significativos, aun cuando se retiró el tratamiento con sildenafilo en un paciente por cefalea persistente. Conclusiones: Los hallazgos de este estudio confirman que el sildenafilo es un fármaco eficaz para el manejo de la hipertensión arterial pulmonar y la HP tromboembólica crónica y no quirúrgica, tanto a corto como a medio-largo plazo, con buena tolerancia y escasos efectos secundarios

Objective: To assess the efficacy of treatment with sildenafil monotherapy in patients with pulmonary hypertension. Patients and methods: An observational study was undertaken in 11 patients with pulmonary hypertension in functional class II or III who received treatment with sildenafil (150 mg/day). Seven of the patients had inoperable chronic thromboembolic pulmonary hypertension and 4 had pulmonary arterial hypertension. To assess treatment response, the following parameters were assessed during follow-up at 3, 6, and 12 months: exercise tolerance in the 6-minute walk test, change in functional class, and systolic pulmonary arterial pressure measured by echocardiography. Results: We observed a significant improvement in exercise tolerance, as shown by increased 6-minute walk distance after 3, 6, and 12 months of treatment (increases of 20, 67, and 95 m, respectively). All patients showed an improvement in functional class. The results of echocardiography did not reveal statistically significant differences in systolic pulmonary arterial pressure between baseline and 6 or 12 months of treatment. No significant adverse effects were observed, although sildenafil treatment was suspended in 1 patient due to persistent headache. Conclusions: The results of this study confirm that sildenafil is an effective drug for the management of pulmonary arterial hypertension and inoperable chronic thromboembolic pulmonary hypertension both in the short term and medium to long term, and that the drug is well tolerated and shows few side effects

Tratamiento con sildenafilo y/o bosentán en niños y jóvenes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger / Treatment with sildenafil, bosentan, or both in children and young people with idiopathic pulmonary arterial hypertension and Eisenmenger's Syndrome

Introducción y objetivos. La hipertensión arterial pulmonar tiene mal pronóstico en pacientes adultos y pediátricos. El conocimiento actual de los mecanismos que condicionan la hipertensión arterial pulmonar ha permitido descubrir medicamentos como los antagonistas de los receptores de la endotelina e inhibidores de la 5-fosfodiesterasa, administrables ambos por vía oral y generalmente bien tolerados. En este estudio se valora la capacidad funcional y la tolerancia al ejercicio en el tratamiento a largo plazo con sildenafilo o bosentán en pacientes con hipertensión arterial pulmonar idiopática y síndrome de Eisenmenger, y se comparan los resultados en ambos grupos de pacientes. Métodos. Siete pacientes fueron incluidos en el protocolo de estudio de la hipertensión arterial pulmonar, y en ellos se confirmó el diagnóstico de hipertensión arterial pulmonar idiopática. Se trató con sildenafilo a 5 pacientes y con bosentán a 2. Cinco pacientes con comunicación interventricular no restrictiva e hipertensión arterial pulmonar fueron tratados con sildenafilo. En uno de ellos se añadió bosentán al tratamiento con sildenafilo. Resultados. El sildenafilo y el bosentán mejoraron significativamente la capacidad de ejercicio en pacientes con hipertensión arterial pulmonar idiopática. El efecto fue menor en los pacientes con fisiología de síndrome de Eisenmenger. La mejoría en la clase funcional de la Organización Mundial de la Salud fue mayor en los pacientes con hipertensión arterial pulmonar idiopática, aunque fue significativa en ambos grupos. Conclusiones. El tratamiento a largo plazo con sildenafilo y bosentán mejora la capacidad de ejercicio y la clase funcional en la hipertensión arterial pulmonar idiopática y debida a cardiopatías congénitas. Los cambios son más llamativos en los pacientes con hipertensión arterial pulmonar idiopática (AU)

Introduction and objectives. Pulmonary arterial hypertension carries a poor prognosis in both adult and pediatric patients. Current understanding of the mechanisms underlying pulmonary arterial hypertension has enabled the rapid development of appropriate drugs, such as endothelin receptor antagonists and 5-phosphodieste-rase inhibitors, that can be administered orally and which are generally well tolerated. The aims of the present study were to evaluate functional class and exercise capacity following long-term treatment with sildenafil or bosentan in patients with idiopathic pulmonary arterial hypertension and Eisenmenger's syndrome and to compare results in the two groups. Methods. Seven patients were included in the pulmonary arterial hypertension study, and diagnoses of idiopathic pulmonary arterial hypertension were confirmed. Five patients were treated with sildenafil, while two received bosentan. The five patients with a non-restrictive ventricular septal defect and pulmonary arterial hypertension were treated with sildenafil. In one patient, bosentan was added to the sildenafil. Results. Both sildenafil and bosentan significantly improved exercise capacity in patients with idiopathic pulmonary arterial hypertension. The treatment effect was less in those with Eisenmenger physiology. Although the improvement in World Health Organization functional class was greater in patients with idiopathic pulmonary arterial hypertension, it was significant in both groups. Conclusions. Long-term treatment with sildenafil and bosentan improved both exercise capacity and functional class in patients with idiopathic pulmonary arterial hypertension and in those with hypertension due to congenital heart disease. The changes were more marked in patients with idiopathic pulmonary arterial hypertension (AU)