Pulmonary vascular disease in chronic lung diseases: cause or comorbidity?

PURPOSE OF REVIEW: To provide timely and relevant insights into the complex relationship between pulmonary vascular disease (PVD) and chronic lung disease (CLD), focusing on the causative and consequential dynamics between these conditions. RECENT FINDINGS: There are shared pathogenic mechanisms between pulmonary arterial hypertension (PAH) and group 3 pulmonary hypertension, including altered expression of mediators and growth factors implicated in both conditions. Factors such as hypoxia, hypoxemia, and hypercapnia also contribute to pulmonary vascular remodelling and endothelial dysfunction. However, the role of hypoxia as the sole driver of pulmonary hypertension in CLD is being reconsidered, particularly in chronic obstructive pulmonary disease (COPD), with evidence suggesting a potential role for cigarette smoke products in initiating pulmonary vascular impairment. On the other hand, interstitial lung disease (ILD) encompasses a group of heterogeneous lung disorders characterized by inflammation and fibrosis of the interstitium, leading to impaired gas exchange and progressive respiratory decline, which could also play a role as a cause of pulmonary hypertension. SUMMARY: Understanding the intricate interplay between the pulmonary vascular compartment and the parenchymal and airway compartments in respiratory disease is crucial for developing effective diagnostic and therapeutic strategies for patients with PVD and CLD, with implications for both clinical practice and research.

Combination of BeGraft and Solaris Stent Grafts for the Covered Endovascular Reconstruction of Aortic Bifurcation-BS-CERAB Technique.

Background: This study examines the impact of the use of the combination of BeGraft and Solaris stent grafts on the outcomes during the covered endovascular reconstruction of aortic bifurcation (BS-CERAB) technique and extension to the iliac arteries. Methods: Consecutive patients with aortoiliac occlusive disease who underwent endovascular treatment using BS-CERAB between January 2020 and December 2023 were included. Patient demographics, symptoms, lesion characteristics, and procedural and follow-up details were collected and analyzed. Perioperative complications and reinterventions were also identified. Results: A total of 42 patients met the inclusion criteria (32 men, 76.2%, median age 72 years, range 59-85). Indications for treatment were intermittent claudication (42.9%) and critical limb ischemia (57.1%). Procedure success was achieved in all cases. The median patient follow-up time was 14 months (1-36). One patient died at a 10-month follow-up due to lung cancer. The mean pre-operative ABI increased from 0.37 ± 0.19 before intervention to 0.71 ± 1.23 post-operatively at 12 months (p = 0.037). The estimated primary patency rates at 3, 6, and 12 months were 90.5%, 85.7%, and 81.0% and primary assisted patency rates were 90.5%, 90.5%, and 85.7%, respectively. Secondary patency was 95.2% at 3 and 6 months and 90.5% at a 12-month follow-up. Active cancer (p = 0.023, OR 2.12 95%CI 1.14-3.25) was a risk factor for restenosis. Conclusions: This mid-term experience shows that the CERAB technique using the combination of BeGraft and Solaris stents grafts, for the endovascular treatment of severe aortoiliac atherosclerotic disease, may allow an effective reconstruction of the aortic bifurcation and iliac arteries related to high-patency and lower-reintervention rates.

Experiences in the use of multiple doses of convalescent plasma in critically ill patients with COVID-19: An early phase 1 descriptive study.

Background: At the beginning of the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, transfusion of coronavirus disease 2019 (COVID-19) convalescent plasma (CCP) emerged as a potential therapeutic strategy to help patients severely afflicted by COVID-19. The efficacy of CCP has been controversial as it depends on many variables pertaining to the plasma donor and the patient with COVID-19, for example, time of convalescence or symptoms onset. This feasibility and descriptive study aimed to assess the safety of multiple doses of CCP in mechanically ventilated, intubated patients with respiratory failure due to COVID-19. Methods: A cohort of 30 patients all experiencing severe respiratory failure and undergoing invasive mechanical ventilation in an intensive care unit, received up to five doses of 300-600 mL of CCP on alternate days (0, 2, 4, 6, and 8) until extubation, futility, or death. Results: Nineteen patients received five doses, seven received four, and four received two or three doses. At 28-day follow-up mark, 57% of patients recovered and were sent home, and the long-term mortality rate was 27%. Ten severe adverse events reported in the study were unrelated to CCP transfusion. Independent of the number of transfused doses, most patients had detectable levels of total and neutralizing antibodies in plasma. Conclusion: This study suggests that transfusion of multiple doses of CCP is safe. This strategy may represent a viable option for future studies, given the potential benefit of CCP transfusions during the early stages of infection in unvaccinated populations and in settings where monoclonal antibodies or antivirals are contraindicated or unavailable.

Pulmonary vascular reactivity in growth restricted fetuses using computational modelling and machine learning analysis of fetal Doppler waveforms.

The aim of this study was to investigate the pulmonary vasculature in baseline conditions and after maternal hyperoxygenation in growth restricted fetuses (FGR). A prospective cohort study of singleton pregnancies including 97 FGR and 111 normally grown fetuses was carried out. Ultrasound Doppler of the pulmonary vessels was obtained at 24-37 weeks of gestation and data were acquired before and after oxygen administration. After, Machine Learning (ML) and a computational model were used on the Doppler waveforms to classify individuals and estimate pulmonary vascular resistance (PVR). Our results showed lower mean velocity time integral (VTI) in the main pulmonary and intrapulmonary arteries in baseline conditions in FGR individuals. Delta changes of the main pulmonary artery VTI and intrapulmonary artery pulsatility index before and after hyperoxygenation were significantly greater in FGR when compared with controls. Also, ML identified two clusters: A (including 66% controls and 34% FGR) with similar Doppler traces over time and B (including 33% controls and 67% FGR) with changes after hyperoxygenation. The computational model estimated the ratio of PVR before and after maternal hyperoxygenation which was closer to 1 in cluster A (cluster A 0.98 ± 0.33 vs cluster B 0.78 ± 0.28, p = 0.0156). Doppler ultrasound allows the detection of significant changes in pulmonary vasculature in most FGR at baseline, and distinct responses to hyperoxygenation. Future studies are warranted to assess its potential applicability in the clinical management of FGR.

Imaging of chronic thromboembolic pulmonary hypertension before, during and after balloon pulmonary angioplasty.

Balloon pulmonary angioplasty (BPA) has recently been elevated as a class I recommendation for the treatment of inoperable or residual chronic thromboembolic pulmonary hypertension (CTEPH). Proper patient selection, procedural safety, and post-procedural evaluation are crucial in the management of these patients, with imaging work-up playing a pivotal role. Understanding the diagnostic and therapeutic imaging algorithms of CTEPH, the imaging features of patients amenable to BPA, all imaging findings observed during and immediately after the procedure and the changes observed during the follow-up is crucial for all interventional radiologists involved in the care of patients with CTEPH. This article illustrates the imaging work-up of patients with CTEPH amenable to BPA, the imaging findings observed before, during and after BPA, and provides a detailed description of all imaging modalities available for CTEPH evaluation.

Innovative Predictive Approach towards a Personalized Oxygen Dosing System.

Despite the large impact chronic obstructive pulmonary disease (COPD) that has on the population, the implementation of new technologies for diagnosis and treatment remains limited. Current practices in ambulatory oxygen therapy used in COPD rely on fixed doses overlooking the diverse activities which patients engage in. To address this challenge, we propose a software architecture aimed at delivering patient-personalized edge-based artificial intelligence (AI)-assisted models that are built upon data collected from patients' previous experiences along with an evaluation function. The main objectives reside in proactively administering precise oxygen dosages in real time to the patient (the edge), leveraging individual patient data, previous experiences, and actual activity levels, thereby representing a substantial advancement over conventional oxygen dosing. Through a pilot test using vital sign data from a cohort of five patients, the limitations of a one-size-fits-all approach are demonstrated, thus highlighting the need for personalized treatment strategies. This study underscores the importance of adopting advanced technological approaches for ambulatory oxygen therapy.

Six-minute walking distance and desaturation-distance ratio in allogeneic stem cell transplantation.

BACKGROUND: Most patients with haematological malignancies who undergo allogeneic haematopoietic stem cell transplant (HSCT) receive chemotherapy before the transplant to control the disease. Certain chemotherapy drugs can cause lung toxicity. Conversely, in patients with chronic respiratory conditions, the 6-min walking test (6MWT) and the desaturation-distance ratio (DDR) have demonstrated prognostic significance. Our objective was to determine whether the 6MWD and DDR, assessed prior to HSCT, have a prognostic impact on survival at 24 months post-HSCT. METHODS: A prospective experimental study was conducted in consecutive patients referred for allogeneic HSCT at Hospital Clinic, Barcelona, Spain. A complete functional respiratory study, including the 6MWT and DDR, was conducted prior to admission. The area under the curve (AUC) and cut-off points were calculated. Data on patients' characteristics, HSCT details, main events, with a focus on lung complications, and survival at 24 months were analysed. RESULTS: One hundred and seventy-five patients (39% women) with mean age of 48 ± 13 years old were included. Before HSCT, forced vital capacity and forced expiratory volume in the first second were 96% ± 13% predicted and 92% ± 14% predicted, respectively; corrected diffusing capacity for carbon monoxide 79% ± 15% predicted; 6MWD was 568 ± 83 m and DDR of .27 (.20-.41). The cut-off points for 6MWD and DDR were 566 m, [.58 95% CI (.51-.64)], p = .024 and .306, [.63 95% CI (.55-.70)], p = .0005, respectively. The survival rate at 24 months was 55%. CONCLUSION: Our results showed that individuals who exhibit a 6MWD shorter than 566 ms or a decline in DDR beyond .306 experienced reduced survival rates at 24 months after HSCT.

Tolerancia al esfuerzo en la hipertensión pulmonar / Exercise tolerance in pulmonary hypertension

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[Translated article] Exercise Tolerance in Pulmonary Hypertension / Tolerancia al esfuerzo en la hipertensión pulmonar

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A New and More Sensitive Method to Integrate the Desaturation Distance Ratio During a 6-Minute Walking Test in Chronic Respiratory Diseases: Physiological Correlates / Un método nuevo y más sensible para integrar la relación entre distancia y desaturación durante la prueba de la marcha de seis minutos en las enfermedades respiratorias crónicas: correlaciones fisiológicas

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Prediction Equations for Maximal Aerobic Capacity on Cycle Ergometer for the Spanish Adult Population / Ecuaciones de referencia de la capacidad aeróbica máxima ciclo-ergoespirometría para la población española adulta

Background: Frequently used reference values for clinical exercise testing have been derived from non-random samples and some with poorly defined maximal criteria.Our objective was to obtain population based reference values for peak oxygen uptake (V?O2) and work rate (WR) for cardiopulmonary exercise testing in a representative sample of Caucasian Spanish men and women. Methods: 182 men and women, 20–85 years old, were included and exercised on cycle-ergometer to exhaustion. (V?O2) and WR were measured. The equations obtained from this sample were validated in an independent cohort of 69 individuals, randomly sampled form the same population. Then a final equation merging the two cohorts (=251) was produced. Results: Height, sex and age resulted predictive of both V?O2 peak and WR. Weight and physical activity added very little to the accuracy to the equations. The formulas V?O2peak=0.017·height?(cm)-0.023·age?(years)+0.864·sex?(female=0/male=1)±179?l?min-1, and peak WR=1.345 · height (cm) - 2.074 · age (years)+76.54 · sex (female=0/male=1)±21.2W were the best compromise between accuracy and parsimony. Conclusions: This study provides new and accurate V?O2 peak and WR rate reference values for individuals of European Spanish descent. (AU)

Antecedentes: Los valores de referencia utilizados con frecuencia para las pruebas de esfuerzo clínicas derivan de muestras no aleatorias y los criterios máximos para algunos de ellos están mal definidos. Nuestro objetivo fue obtener valores de referencia basados en la población general para el consumo máximo de oxígeno (VO2) y la carga de trabajo (CT) para las pruebas de ejercicio cardiopulmonar a partir de una muestra representativa de varones y mujeres caucásicos españoles. Métodos: Se incluyeron 182 varones y mujeres, de entre 20 y 85 años, que realizaron ejercicio en el cicloergómetro hasta el agotamiento. Se midieron el VO2 y la CT. Las ecuaciones obtenidas de esta muestra se validaron en una cohorte independiente de 69 individuos, seleccionados aleatoriamente de la misma población. A continuación, se creó una ecuación final que fusionó las dos cohortes (n=251). Resultados: La altura, el sexo y la edad resultaron predictivos tanto del V?O2 máximo como de la CT. El peso y la actividad física contribuyeron muy poco a la precisión de las ecuaciones. Las fórmulas V?O2 máximo=0,017×altura (cm)-0,023×edad (años)+0,864×sexo (mujer=0/varón=1)±179 L×min-1; y CT máxima=1,345×altura (cm)-2,074×edad (años)+76,54×sexo (mujer=0/varón=1)±21,2W fueron el mejor equilibrio entre precisión y parsimonia. Conclusiones: Este estudio proporciona valores de referencia del V?O2 máximo y la CT nuevos y precisos para personas de ascendencia española europea. (AU)

Hipertensión pulmonar tromboembólica crónica en España: una década de cambio / Chronic thromboembolic pulmonary hypertension in Spain: a decade of change

Introducción y objetivos El tratamiento de la hipertensión pulmonar tromboembólica crónica (HTPTEC) ha evolucionado en la última década. Sin embargo, apenas se dispone de información sobre el impacto de estos logros en la población general a escala nacional. Este estudio se diseñó para describir las características de los pacientes con HTPTEC en España en la última década. Métodos Se recogieron prospectivamente datos epidemiológicos, clínicos y pronósticos de los pacientes con HTPTEC consecutivos incluidos en el registro español REHAP desde el 1 de enero de 2007 al 31 de diciembre de 2018. Se evaluaron las diferencias entre diferentes periodos de tiempo, estableciendo 2013 como fecha de referencia para el análisis. Se calculó la puntuación de propensión para la intervención mediante un modelo multivariable de regresión logística. Resultados Se incluyó a 1.019 pacientes; se remitió a 659 (64,4%) a un centro nacional de referencia en HTPTEC. Del total, se seleccionó a 350 (34,3%) para cirugía y a 97 (9,6%) para tratamiento percutáneo. Entre los pacientes diagnosticados entre 2007 y 2012 hubo más frecuencia de muerte que entre los diagnosticados de 2013 en adelante (HR=1,83; IC95%, 1,07-3,15; p=0,027). En el grupo de pacientes ajustado por el modelo de puntuación de propensión, las resistencias vasculares pulmonares basales y la distancia recorrida en el test de 6 min de marcha también fueron determinantes del pronóstico (respectivamente, HR=1,24; IC95%, 1,15-1,33; p=0,011, y HR=0,93; IC95%, 0,90-0,97; p=0,001). Las tasas de supervivencia de los pacientes que se sometieron a un procedimiento intervencionista (trombendarterectomía pulmonar o angioplastia con balón de arterias pulmonares) resultaron llamativamente altas. Conclusiones Durante la última década, el diagnóstico y el pronóstico de la HTPTEC han mejorado de manera considerable. La gravedad de la enfermedad al diagnóstico determinó el perfil de riesgo. ... (AU)

Introduction and objectives Chronic thromboembolic pulmonary hypertension (CTEPH) treatment has evolved in the last decade. However, there is scarce information on the long-term impact of this progress in a real-life population at a national level. This study was designed to analyze the characteristics of CTEPH patients in Spain over the last decade. Methods We prospectively collected epidemiological, clinical, and prognostic data from CTEPH patients consecutively included in the Spanish REHAP registry from January 1, 2007, to December 31, 2018. We evaluated differences over time, establishing 2013 as the reference date for analysis. Propensity scores for interventional treatment were calculated using a multivariable logistic regression model. Results A total of 1019 patients were included; 659 (64.4%) were evaluated at a national CTEPH center. Overall, 350 patients (34.3%) were selected for surgery and 97 (9.6%) for percutaneous treatment. Patients diagnosed between 2007 and 2012 died more frequently than those diagnosed from 2013 onward (HR, 1.83; 95%CI, 1.07-3.15; P=.027). Within the subgroup of patients adjusted by propensity score, baseline pulmonary vascular resistance and the 6-minute walk test distance also determined the outcome (HR, 1.24; 95%CI, 1.15-1.33; P=.011; and HR, 0.93; 95%CI, 0.90-0.97; P=.001, respectively). High survival rates were found in patients who underwent an invasive procedure (pulmonary endarterectomy or balloon pulmonary angioplasty). Conclusions CTEPH diagnosis and prognosis have consistently improved in the last decade. Baseline disease severity determines the risk profile. Patients who undergo pulmonary endarterectomy or balloon pulmonary angioplasty have better outcomes. (AU)

Effects of Pulmonary Hypertension on Exercise Capacity in Patients With Chronic Obstructive Pulmonary Disease / Efectos de la hipertensión pulmonar en la capacidad de ejercicio en pacientes con enfermedad pulmonar obstructiva crónica

INTRODUCTION: The impact of pulmonary hypertension (PH) on exercise tolerance in chronic obstructive pulmonary disease (COPD) has not been fully elucidated. It is necessary to characterize pulmonary hemodynamics in patients with moderate to severe COPD in order to improve their management. The aim of the study was to determine whether in COPD the presence of PH is associated with reduced exercise tolerance in a cohort of stable COPD patients. METHODS: Cross-sectional analysis of 174 COPD patients clinically stable: 109 without PH and 65 with PH (COPD-PH). We assessed socio-demographic data, lung function, quality of life, dyspnea, cardiopulmonary exercise testing (CPET), constant workload endurance time (CWET), and six-minute walk test (6MWT). We elaborated a logistic regression model to explore the impact of PH on exercise capacity in COPD patients. RESULTS: COPD-PH patients showed lower exercise capacity both at maximal (CPET) (43 (20) versus 68 (27) Watts and 50 (19)% versus 71(18)% predicted peak oxygen consumption (VO2peak), COPD-PH and COPD, respectively), and at submaximal tests (6MWT) (382 (94) versus 486 (95) m). In addition, the COPD-PH group had lower endurance time than the non-PH COPD group (265 (113) s and 295 (164) s, respectively). CONCLUSIONS: The presence of PH is an independent factor that impairs exercise capacity in COPD

INTRODUCCIÓN: El impacto de la hipertensión pulmonar (HTP) en la tolerancia al ejercicio en la enfermedad pulmonar obstructiva crónica (EPOC) no se ha dilucidado en su totalidad. Es necesario caracterizar la hemodinámica pulmonar de los pacientes con EPOC moderada a grave para poder mejorar su manejo. El objetivo de este estudio fue determinar si la presencia de HTP en la EPOC se asociaba con una disminución en la tolerancia al ejercicio en una cohorte de pacientes con EPOC estable. MÉTODOS: Estudio transversal de 174 pacientes con EPOC clínicamente estables: 109 de ellos no mostraban HTP y 65 de ellos sí (EPOC-HTP). Valoramos la información sociodemográfica, la función pulmonar, la calidad de vida, la disnea, realizamos una prueba de ejercicio cardiopulmonar (PECP), medimos el tiempo de tolerancia de ejercicio constante y realizamos de marcha de seis minutos (6MWT, por sus siglas en inglés). Elaboramos un modelo de regresión logística para explorar el impacto de la HTP en la capacidad de ejercicio de los pacientes con EPOC. RESULTADOS: Los pacientes con EPOC-HTP mostraron una menor capacidad de ejercicio, tanto en las pruebas máximas (PECP) (43 (20)W frente a 68 (27)W y 50(19)% frente a 71 (18)% de consumo de oxígeno máximo predicho (VO2max), para pacientes con EPOC-HTP y pacientes con EPOC, respectivamente) como en las pruebas submáximas (6MWT) (382 (94)m frente a 486 (95)m). Además, el grupo de EPOC-HTP presentó un menor tiempo de resistencia que el grupo de EPOC sin HTP (265 (113) s y 295 (164) s, respectivamente). CONCLUSIONES: La presencia de HTP es un factor independiente que afecta a la capacidad de ejercicio en la EPOC

Guía de diagnóstico y tratamiento de la hipertensión pulmonar: resumen de recomendaciones / Guidelines on the Diagnosis and Treatment of Pulmonary Hypertension: Summary of Recommendations

La hipertensión pulmonar es un trastorno hemodinámico definido por el aumento anómalo de la presión arterial pulmonar, que puede presentarse en numerosas enfermedades y situaciones clínicas. Las causas de hipertensión pulmonar se clasifican en 5 grandes grupos: arterial, debida a cardiopatía izquierda, debida a enfermedad pulmonar y/o hipoxemia, tromboembólica crónica y de mecanismo no establecido y/o multifactorial. El presente documento expone de forma resumida las recomendaciones de la Guía de Diagnóstico y Tratamiento de la Hipertensión Pulmonar de la Sociedad Española de Neumología y Cirugía Torácica. En dicha guía se presentan las pautas actuales de diagnóstico y tratamiento de los distintos grupos de hipertensión pulmonar

Pulmonary hypertension is a hemodynamic disorder defined by abnormally high pulmonary artery pressure that can occur in numerous diseases and clinical situations. The causes of pulmonary hypertension are classified into 5 major groups: arterial, due to left heart disease, due to lung disease and/or hypoxemia, chronic thromboembolic, with unclear and/or multifactorial mechanisms. This is a brief summary of the Guidelines on the Diagnostic and Treatment of Pulmonary Hypertension of the Spanish Society of Pulmonology and Thoracic Surgery. These guidelines describe the current recommendations for the diagnosis and treatment of the different pulmonary hypertension groups

Tratamiento médico y quirúrgico de la hipertensión pulmonar tromboembólica crónica: experiencia en un único centro / Medical and Surgical Management for Chronic Thromboembolic Pulmonary Hypertension: A Single Center Experience

Introducción: La endarterectomía pulmonar (EP) es el tratamiento de elección para la hipertensión pulmonar tromboembólica crónica (HPTEC). El objetivo del estudio fue analizar nuestra experiencia en el tratamiento médico (TM) y quirúrgico de la HPTEC. Métodos: Se evaluaron 80 pacientes diagnosticados de HPTEC en el periodo enero 2000-julio 2012. En 32 casos se realizó EP, el resto recibió TM. Se analizaron: clase funcional (CF), distancia recorrida en seis minutos (PM6M) y hemodinámica pulmonar. Se analizó la mortalidad según el tratamiento y el periodo. Resultados: Los pacientes del grupo EP eran más jóvenes, mayoritariamente hombres y recorrieron mayor distancia en la PM6M. No hubo diferencias hemodinámicas ni de CF al diagnóstico. Al a˜no del tratamiento, el 100% del grupo EP y el 41% del grupo TM estaban en CF I-II. Al seguimiento, el grupo EP presentó mayor incremento en la PM6M y mayor reducción de la PAPm y la RVP que en el grupo TM (p < 0,05). La supervivencia global del grupo TM a 1 y 5 a˜nos fue del 83% y del 69%, respectivamente. La supervivencia condicionada de los pacientes vivos 100 días post-EP a 1 y 5 a˜nos fue del 95 y del 88%, respectivamente. La mortalidad quirúrgica en los pacientes operados en el período 2000-2006 fue del 31,3%, y en el período 2007-2012, del 6,3%. Conclusiones: La EP proporciona buenos resultados clínicos, hemodinámicos y de supervivencia en los pacientes que superan satisfactoriamente el postoperatorio inmediato. Tras un periodo de aprendizaje, la mortalidad perioperatoria actual en nuestro centro es superponible a los estándares internacionales

Introduction: Pulmonary endarterectomy (PE) is the treatment of choice for chronic thromboembolic pulmonary hypertension (CTEPH). The aim of this study was to analyze our experience in the medical and surgical management of CTEPH. Methods: We included 80 patients diagnosed with CTEPH between January 2000 and July 2012. Thirty two patients underwent PE and 48 received medical treatment (MT). We analyzed functional class (FC), sixminute walking distance (6MWD) and pulmonary hemodynamics. Mortality in both groups and periods were analyzed. Results: Patients who underwent PE were younger, mostly men, and had longer 6MWD. No differences were observed in pulmonary hemodynamics or FC at diagnosis. One year after treatment, all PE patients versus 41% in MT group were at FC I-II. At follow-up, the PE group showed greater increase in 6MWD, and greater reduction in mean pulmonary arterial pressure and pulmonary vascular resistance than the MT group (P < .05). Overall survival in the MT group at 1 and 5 years was 83% and 69%, respectively. Conditional survival in patients alive 100 days post-PE at 1 and 5 years was 95% and 88%, respectively. Surgical mortality in operated patients in the first period (2000-2006) was 31,3%, and 6,3% in the second (2007-2012). Conclusions: PE provides good clinical results, and improves pulmonary hemodynamics in patients who successfully overcome the immediate postoperative period. After a learning period, the current operatory mortality in our center is similar to international standards

Hipertensión arterial pulmonar en un paciente con telangiectasia hemorrágica hereditaria / Pulmonary Arterial Hypertension in a Patient With Hereditary Hemorrhagic Telangiectasia

La telangiectasia hemorrágica hereditaria (THH) es una enfermedad autosómica dominante caracterizada por la tríada de epistaxis, telangiectasias y malformaciones vasculares. Las complicaciones vasculares pulmonares asociadas a esta enfermedad incluyen malformaciones arteriovenosas (MAV) pulmonares y, de forma menos frecuente, hipertensión pulmonar (HP). El presente caso clínico hace referencia a un paciente con múltiples MAV pulmonares e HP en el contexto de posible THH. Se procedió a embolización de una MAV y se inició tratamiento específico de hipertensión arterial pulmonar con un antagonista de receptores de endotelina. A continuación se describe su mejoría funcional y hemodinámica tras 3 años de seguimiento(AU)

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by the triad of epistaxis, telangiectasia and vascular malformations. Pulmonary vascular complications associated with this disease include pulmonary arteriovenous malformations (AVM) and, less frequently, pulmonary hypertension (PH). We report the case of a patient who presented multiple pulmonary AVM and PH probably due to HHT. Embolization was carried out on one of the AVM and the patient received specific pulmonary arterial hypertension treatment with an endothelin receptor antagonist. We also described the patient's functional and hemodynamic improvement after almost 3 years of follow-up(AU)

Prueba de esfuerzo cardiopulmonar en el manejo del paciente con hipertensión pulmonar / The cardiopulmonary exercise test in the management of patients with pulmonary hypertension

La intolerancia al ejercicio es el principal síntoma de los pacientes con hipertensión pulmonar (HP). La prueba de referencia para la evaluación de la capacidad de ejercicio es la prueba de esfuerzo cardiopulmonar incremental (PECP) en cicloergómetro. La tolerancia al ejercicio en los pacientes con HP viene determinada principalmente por la capacidad de incrementar el gasto cardiaco a fin de atender las demandas metabólicas, que depende de la función del ventrículo derecho. Por ello, la disfunción ventricular derecha es el principal factor limitante de la tolerancia al esfuerzo en esta enfermedad. Los pacientes con HP también pueden presentar hipoxemia durante el ejercicio y es frecuente observar hiperventilación, tanto en reposo como durante el esfuerzo, que puede ser atribuible a una mayor quimiosensibilidad. En la presente revisión se analizan los mecanismos fisiológicos que determinan la tolerancia al ejercicio, la respuesta al esfuerzo en los pacientes con HP y las variables de mayor interés para su estudio, las similitudes y diferencias entre la PECP y otras pruebas más sencillas, como la prueba de marcha de 6 minutos, y el valor pronóstico de las pruebas de esfuerzo en esta enfermedad. La valoración de la tolerancia al esfuerzo constituye un elemento esencial en la valoración clínica de los pacientes con HP, por lo que un conocimiento detallado de la información que pueden proporcionar las pruebas de esfuerzo y de sus limitaciones tiene indudable interés para el manejo clínico de esta compleja enfermedad (AU)

The main symptom of patients with pulmonary hypertension (PH) is exercise intolerance. The gold standard for evaluation of exercise capacity is the incremental cardio-pulmonary exercise test (ICPET) on a bicycle ergometer. Exercise tolerance in patients with PH is mainly determined by the capacity to increase cardiac output to meet metabolic demands, which depends on right ventricular function. Therefore, right ventricular dysfunction is the main factor limiting exercise tolerance in PH. Patients with PH also show hypoxemia during exercise and hyperventilation is also common, both at rest and during exercise, which can be attributed to greater chemosensitivity. The present review analyzes the physiological mechanisms determining exercise tolerance, exercise response in patients with PH, the variables of greatest interest in the study of this disorder, the similarities and differences between ICPET and other, simpler tests such as the 6-minute walk test, and the prognostic value of exercise testing in these patients. Evaluation of exercise tolerance is an essential element in the clinical assessment of patients with PH. Consequently, detailed knowledge of the information provided by exercise testing and its limitation is of undoubted interest in the clinical management of this complex disease (AU)

Consumo máximo de oxígeno durante la prueba de marcha de 6 minutos en la enfermedad pulmonar intersticial difusa y en la hipertensión pulmonar / Peak oxygen uptake during the six-minute walk test in diffuse interstitial lung disease and pulmonary hypertension

Introducción: La prueba de marcha de 6 minutos (PM6M) es ampliamente utilizada en la evaluación de la enfermedad pulmonar intersticial difusa (EPID) y en la hipertensión pulmonar (HP). Sin embargo, sus determinantes fisiológicos no han sido bien caracterizados.ObjetivoEvaluar los cambios fisiológicos que ocurren durante la PM6M en la EPID y en la HP y compararlos con la prueba de esfuerzo cardiopulmonar (PECP).Material y métodosSe estudiaron 13 pacientes con EPID y 14 con HP mediante PM6M y PECP en cicloergómetro. Durante la PM6M se registraron las variables respiratorias mediante telemetría.ResultadosEl consumo de oxígeno (VO2), la ventilación y la frecuencia cardiaca mostraron una meseta desde el minuto 3 de la PM6M en ambas patologías. El valor de VO2 no difirió del valor pico en la PECP (14±2 y 15±2ml/kg/min, respectivamente, en la EPID; 16±6 y 16±6ml/kg/min, en la HP). En ambas patologías, disminuyó la saturación arterial de oxígeno, aunque más marcadamente en la EPID (−12±5%, p<0,01). En la HP, el equivalente ventilatorio de CO2 (VE/VCO2) durante la PM6M estaba fuertemente relacionado con la clase funcional (CF) (85±14 en CF III-IV, 44±6 en CF I-II; p<0,001).ConclusionesEn la EPID y en la HP la PM6M se comporta como una prueba de esfuerzo máxima, con valores de VO2 similares a la PECP, poniendo de manifiesto limitación de la capacidad de transporte de oxígeno. La monitorización mediante telemetría durante la PM6M puede ser útil para la evaluación clínica de los pacientes con EPID o con HP(AU)

Introduction: The six-minute walk test (6MWT) is widely used in evaluating diffuse interstitial lung disease (ILD) and pulmonary hypertension (PH). However, their physiological determining factors have not been well defined.ObjectiveTo evaluate the physiological changes that occur in ILD and PH during the 6MWT, and compare them with the cardiopulmonary exercise test (CPET).Material and methodsThirteen patients with ILD and 14 with PH were studied using the 6MWT and CPET on an ergometer cycle. The respiratory variables were recorded by means of telemetry during the 6MWT.ResultsOxygen consumption (VO2), respiratory and heart rate reached a plateau from minute 3 of the 6MWT in both diseases. The VO2 did not differ from the peak value in the CPET (14±2 and 15±2ml/kg/min, respectively, in ILD; 16±6 and 16±6ml/kg/min, in PH). The arterial oxygen saturation decreased in both diseases, although it was more marked in ILD (−12±5%, p<0,01). The ventilatory equivalent for CO2 (VE/VCO2) in PH during the 6MWT was strongly associated with functional class (FC) (85±14 in FC III-IV, 44±6 in FC I-II; p<0,001).ConclusionsThe 6MWT in ILD and PH behaves like a maximal effort test, with similar VO2 to the CPET, demonstrating a limit in oxygen transport capacity. Monitoring using telemetry during the 6MWT may be useful for the clinical evaluation of patients with ILD or PH(AU)