Simplified risk stratification based on cardiopulmonary exercise test: A Spanish two-center experience.

A simplified 4-strata risk stratification approach based on three variables is widespread in pulmonary arterial hypertension (PAH) at follow-up. This study aimed to assess the impact of replacing the 6-min walk test (6MWT) with the peak 02 uptake evaluated by the cardiopulmonary exercise test (CPET) on risk stratification by this scale. We included 180 prevalent patients with PAH from two reference hospitals in Spain, followed up between 2006 and 2022. Patients were included if all the variables of interest were available within a 3-month period on the Spanish Registry of Pulmonary Arterial Hypertension (REHAP): functional class (FC); NT-proBNP; 6MWT; and CPET. The original 4-strata model (NT-proBNP, 6MWT, FC) identified most patients at low or intermediate-low risk (36.7% and 51.1%, respectively). Notably, the modified scale (NT-proBNP, CPET, FC) improved the identification of patients at intermediate-high risk up to 18.9%, and at high risk up to 1.1% in comparison with the previous 12.2% and 0.0% in the original scale. This new model increased the number of patients correctly classified into higher-risk strata (positive NRI of 0.06), as well as classified more patients without events in lower-risk strata (negative NRI of 0.04). The proposed score showed a slightly superior prognostic capacity compared with the original model (Harrel's C-index 0.717 vs. 0.709). Using O2 uptake instead of distance walked in the 6MWT improves the identification of high-risk patients using the 4-strata scale. This change could have relevant prognostic implications and lead to changes in the specific treatment of PAH.

Impacto del cateterismo derecho de ejercicio en el diagnóstico diferencial de la enfermedad tromboembólica pulmonar crónica / Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease

Introduction and objectives Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing. Methods We included symptomatic patients with persistent pulmonary thrombosis after PE. We excluded patients with left heart disease or significant PH (mean pulmonary arterial pressure [mPAP] >25mmHg, pulmonary vascular resistance >3 WU, and pulmonary capillary wedge pressure [PCWP] >15mmHg). Cardiopulmonary exercise testing and exercise right heart catheterization were performed. Exercise-induced precapillary PH was defined as mPAP/CO slope >3 and PCWP/CO slope <2mmHg/l/min. The hemodynamic response and the values obtained in cardiopulmonary exercise testing were compared between patients with and without exercise-induced precapillary PH. Results We studied 36 patients; 4 were excluded due to incomplete hemodynamic data. Out of the 32 patients analyzed; 3 developed a pathological increase in PCWP. Among the remaining 29 patients (mean age, 49.4±13.7 years, 34.5% women), 13 showed exercise-induced PH. Resting mPAP was higher in those who developed exercise-induced PH (23.3±5.4 vs 19.0±3.8mmHg; P=.012), although CO was similar in the 2 groups. Patients with exercise-induced PH exhibited data of ventilatory inefficiency with reduced values of end-tidal CO2 pressure at the anaerobic threshold (32.8±3.0 vs 36.2±3.3mmHg; P=.021) and a higher Ve/VCO2 slope (34.2±4.8 vs 30.7±5.0; P=.049). Conclusions Exercise limitation and ventilatory inefficiency could be attributable to exercise-induced precapillary PH in a subgroup of patients with persistent pulmonary thrombosis and dyspnea (AU)

Introdución y objetivos La enfermedad tromboembólica crónica se define como la trombosis crónica de la vasculatura pulmonar y disnea, sin hipertensión pulmonar (HP) en reposo tras una embolia de pulmón. El estudio evaluó la hemodinámica al esfuerzo en estos pacientes y su relación con la ergoespirometría. Métodos Se incluyó a pacientes sintomáticos con trombosis pulmonar crónica tras una embolia de pulmón. Se excluyó a los pacientes con cardiopatía izquierda o HP significativa en reposo (presión arterial pulmonar media [PAPm] >25mmHg, resistencia vascular pulmonar >3 UW y presión capilar pulmonar [PCP] >15mmHg). Se realizó una ergoespirometría y un cateterismo derecho de ejercicio. La HP precapilar al ejercicio se definió como las pendientes PAPm/gasto cardiaco >3 y PCP/gasto cardiaco <2mmHg/l/min. Se comparó la respuesta hemodinámica y ergoespirométrica entre pacientes con y sin HP precapilar al ejercicio. Resultados Se estudió a 36 pacientes, excluyéndose 4 por calidad subóptima en el registro hemodinámico. Tres pacientes presentaron una elevación patológica de la PCP. De los 29 restantes (edad, 49,4±13,7 años; el 34,5% mujeres), en 13 se halló HP precapilar al ejercicio. La PAPm basal fue mayor en aquellos con HP al ejercicio (23,3±5,4 frente a 19,0±3,8mmHg; p=0,012), con similar gasto cardiaco. Aquellos con HP al ejercicio exhibieron valores reducidos de la presión de CO2 exahalada en el umbral anaeróbico (32,8±3,0 frente a 36,2±3,3mmHg; p=0,021) y mayor pendiente Ve/VCO2 (34,2±4,8 frente a 30,7±5,0; p=0,049). Conclusiones La HP precapilar al ejercicio se asocia con limitación funcional e ineficiencia ventilatoria en un subgrupo de pacientes con trombosis arterial pulmonar crónica y disnea persistente (AU)

Value of exercise right heart catheterization in the differential diagnosis of chronic thromboembolic pulmonary disease.

INTRODUCTION AND OBJECTIVES: Chronic thromboembolic disease refers to the presence of chronic thrombotic pulmonary vascular thrombosis without pulmonary hypertension (PH) at rest but with exercise limitation after pulmonary embolism (PE). Our aim was to evaluate the hemodynamic response to exercise in these patients and its correlation with the values reached in cardiopulmonary exercise testing. METHODS: We included symptomatic patients with persistent pulmonary thrombosis after PE. We excluded patients with left heart disease or significant PH (mean pulmonary arterial pressure [mPAP] >25mmHg, pulmonary vascular resistance >3 WU, and pulmonary capillary wedge pressure [PCWP] >15mmHg). Cardiopulmonary exercise testing and exercise right heart catheterization were performed. Exercise-induced precapillary PH was defined as mPAP/CO slope >3 and PCWP/CO slope <2mmHg/l/min. The hemodynamic response and the values obtained in cardiopulmonary exercise testing were compared between patients with and without exercise-induced precapillary PH. RESULTS: We studied 36 patients; 4 were excluded due to incomplete hemodynamic data. Out of the 32 patients analyzed; 3 developed a pathological increase in PCWP. Among the remaining 29 patients (mean age, 49.4±13.7 years, 34.5% women), 13 showed exercise-induced PH. Resting mPAP was higher in those who developed exercise-induced PH (23.3±5.4 vs 19.0±3.8mmHg; P=.012), although CO was similar in the 2 groups. Patients with exercise-induced PH exhibited data of ventilatory inefficiency with reduced values of end-tidal CO2 pressure at the anaerobic threshold (32.8±3.0 vs 36.2±3.3mmHg; P=.021) and a higher Ve/VCO2 slope (34.2±4.8 vs 30.7±5.0; P=.049). CONCLUSIONS: Exercise limitation and ventilatory inefficiency could be attributable to exercise-induced precapillary PH in a subgroup of patients with persistent pulmonary thrombosis and dyspnea.

Physical activity levels are low in patients with pulmonary hypertension.

BACKGROUND: Pulmonary hypertension (PH) leads to exercise capacity impairment, but limited data are available on the objective evaluation of physical activity (PA) levels in these patients. METHODS: We assessed PA levels using triaxial accelerometry in Spanish PH patients (n=75, 48±14 years, 65% female) and gender/age matched controls (n=107), and examined potential associations of meeting minimum international recommendations [moderate-vigorous PA (MVPA) ≥150 min/week] with survival predictors. RESULTS: With the exception of vigorous PA (with very low values in both groups), all accelerometry data showed significant differences between patients and controls, with lower PA levels and proportion of individuals meeting minimum PA guidelines, but higher inactivity time, in the former. Notably, the odds ratio (OR) of having a "low-risk" value of 6-minute walking distance (≥464 m) or ventilatory equivalent for carbon dioxide (≤39) was higher in patients following MVPA guidelines than in their less active peers [OR =4.3, 95% confidence interval (CI), 1.6-11.6, P=0.005, and OR =4.5, 95% CI, 0.9-21.1, P=0.054]. CONCLUSIONS: Daily PA is reduced in patients with PH, often to a level that may decrease their odds of survival. Efforts should be made to promote the implementation of healthy PA habits in this patient population.

La evolución de la hipertensión arterial pulmonar a lo largo de 30 años: experiencia de un centro de referencia / Trends in pulmonary hypertension over a period of 30 years: experience from a single referral centre

Introducción y objetivos: La hipertensión arterial pulmonar (HAP) se caracteriza por aumento de resistencias vasculares pulmonares, disfunción progresiva del ventrículo derecho y muerte. A pesar de los avances, sigue asociada a alta morbimortalidad. El objetivo del estudio es describir el tratamiento de esta enfermedad y determinar factores pronósticos de pacientes con HAP tratados en un centro de referencia nacional a lo largo de 30 años. Métodos: Se estudió a 379 pacientes consecutivos diagnosticados de HAP (enero de 1984-diciembre de 2014). Se los distribuyó en 3 intervalos de tiempo: previo a 2004, 2004-2009 y 2010-2014, y se analizaron los factores pronósticos de deterioro clínico. Resultados: La mediana de edad de los pacientes es 44 años (el 68,6% eran mujeres) y estaban en clase funcional III-IV el 72%. Se observó un incremento en etiologías más complejas: enfermedad venooclusiva e hipertensión portopulmonar en el último periodo. La terapia combinada de inicio aumentó (el 5% previo a 2004 frente al 27% posterior a 2010; p < 0,05). El análisis multivariable mostró como factores independientes de deterioro clínico edad, sexo, etiología y variables combinadas (p < 0,05). La supervivencia libre de muerte o trasplante al primero, el tercero y el quinto año fueron del 92,2, el 80,6 y el 68,5% respectivamente. La mediana de supervivencia fue 9 años (intervalo de confianza del 95%, 7,532-11,959). Conclusiones: La HAP es una enfermedad heterogénea y compleja. La mediana de supervivencia libre de muerte o trasplante en nuestra serie es 9 años. La estructura de una unidad multidisciplinaria de HAP debe adaptarse con rapidez a los cambios que se producen en el tiempo incorporando nuevas técnicas diagnósticas y terapéuticas (AU)

Introduction and objectives: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. Methods: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. Results: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) Conclusions: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques (AU)

Trends in Pulmonary Hypertension Over a Period of 30 Years: Experience From a Single Referral Centre.

INTRODUCTION AND OBJECTIVES: Pulmonary arterial hypertension (PAH) is characterized by increased pulmonary vascular resistance, right ventricular dysfunction and death. Despite scientific advances, is still associated with high morbidity and mortality. The aim is to describe the clinical approach and determine the prognostic factors of patients with PAH treated in a national reference center over 30 years. METHODS: Three hundred and seventy nine consecutive patients with PAH (January 1984 to December 2014) were studied. Were divided into 3 periods of time: before 2004, 2004-2009 and 2010-2014. Prognostic factors (multivariate analysis) were analyzed for clinical deterioration. RESULTS: Median age was 44 years (68.6% women), functional class III-IV: 72%. An increase was observed in more complex etiologies in the last period of time: Pulmonary venooclusive disease and portopulmonary hypertension. Upfront combination therapy significantly increased (5% before 2004 vs 27% after 2010; P < .05). Multivariate analysis showed prognostic significance in age, sex, etiology and combined clinical variables as they are independent predictors of clinical deterioration (P < .05). Survival free from death or transplantation for the 1st, 3rd and 5th year was 92.2%, 80.6% and 68.5% respectively. The median survival was 9 years (95% confidence interval, 7.532-11.959) CONCLUSIONS: The PAH is a heterogeneous and complex disease, the median survival free from death or transplantation in our series is 9 years after diagnosis. The structure of a multidisciplinary unit PAH must adapt quickly to changes that occur over time incorporating new diagnostic and therapeutic techniques.

Effects of an 8-month exercise intervention on physical capacity, NT-proBNP, physical activity levels and quality of life data in patients with pulmonary arterial hypertension by NYHA class.

This article provides descriptive detailed (pre and post) values of physical capacity variables, NT-proBNP, physical activity levels and quality of life in patients with pulmonary arterial hypertension (PH) (both, intervention and control group) by New York Heart Association (NYHA) class before and after an 8-month exercise intervention. The data are supplemental to our original Randomized Controlled Trial (RCT) entitled "Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial" (L. González-Saiz, C. Fiuza-Luces, F. Sanchis-Gomar, A. Santos-Lozano, C.A. Quezada-Loaiza, A. Flox-Camacho, D. Munguía-Izquierdo, I. Ara, A. Santalla, M. Morán, P. Sanz-Ayan, P. Escribano-Subías, A. Lucia A, 2017) [1].

Benefits of skeletal-muscle exercise training in pulmonary arterial hypertension: The WHOLEi+12 trial.

BACKGROUND: Pulmonary arterial hypertension is often associated with skeletal-muscle weakness. The purpose of this randomized controlled trial was to determine the effects of an 8-week intervention combining muscle resistance, aerobic and inspiratory pressure-load exercises on upper/lower-body muscle power and other functional variables in patients with this disease. METHODS: Participants were allocated to a control (standard care) or intervention (exercise) group (n=20 each, 45±12 and 46±11years, 60% women and 10% patients with chronic thromboembolic pulmonary hypertension per group). The intervention included five, three and six supervised (inhospital) sessions/week of aerobic, resistance and inspiratory muscle training, respectively. The primary endpoint was peak muscle power during bench/leg press; secondary outcomes included N-terminal pro-brain natriuretic peptide levels, 6-min walking distance, five-repetition sit-to-stand test, maximal inspiratory pressure, cardiopulmonary exercise testing variables (e.g., peak oxygen uptake), health-related quality of life, physical activity levels, and safety. RESULTS: Adherence to training sessions averaged 94±0.5% (aerobic), 98±0.3% (resistance) and 91±1% (inspiratory training). Analysis of variance showed a significant interaction (group×time) effect for leg/bench press (P<0.001/P=0.002), with both tests showing an improvement in the exercise group (P<0.001) but not in controls (P>0.1). We found a significant interaction effect (P<0.001) for five-repetition sit-to-stand test, maximal inspiratory pressure and peak oxygen uptake (P<0.001), indicating a training-induced improvement. No major adverse event was noted due to exercise. CONCLUSIONS: An 8-week exercise intervention including aerobic, resistance and specific inspiratory muscle training is safe for patients with pulmonary arterial hypertension and yields significant improvements in muscle power and other functional variables.

Rationale and Design of a Randomized Controlled Trial Evaluating Whole Muscle Exercise Training Effects in Outpatients with Pulmonary Arterial Hypertension (WHOLEi+12).

BACKGROUND AND AIMS: Physical exercise is an important component in the management of pulmonary artery hypertension (PAH). The aim of this randomized controlled trial (RCT) is to determine the effects of an 8-week intervention combining muscle resistance, aerobic and inspiratory pressure load exercises in PAH outpatients. METHODS: The RCT will be conducted from September 2015 to September 2016 following the recommendations of the Consolidated Standards of Reported Trials (CONSORT), with a total sample size of n ≥ 48 (≥24 participants/group). We will determine the effects of the intervention on: (i) skeletal-muscle power and mass (primary end points); and (ii) NT-proBNP, cardiopulmonary exercise testing variables (VO2peak, ventilatory equivalent for CO2 at the anaerobic threshold (VE/VCO2 at the AT), end-tidal pressure of CO2 at the anaerobic threshold (PETCO2 at the AT), 6-min walking distance (6MWD), maximal inspiratory pressure (PImax), health-related quality of life (HRQoL), objectively-assessed spontaneous levels of physical activity, and safety (secondary end points). CONCLUSIONS AND PERSPECTIVES: This trial will provide insight into biological mechanisms of the disease and indicate the potential benefits of exercise in PAH outpatients, particularly on muscle power.

Factores determinantes de la capacidad de ejercicio en pacientes con hipertensión arterial pulmonar severa / Factors Affecting the Response to Exercise in Patients with Severe Pulmonary Arterial Hypertension

IntroducciónLa ergoespirometría cuantifica objetivamente la capacidad de ejercicio. Hasta ahora, solo se ha descrito el comportamiento al ejercicio mediante ergoespirometría en la hipertensión arterial pulmonar en pacientes recién diagnosticados.El objetivo fue describir el comportamiento al ejercicio de pacientes con hipertensión arterial pulmonar severa bajo tratamiento y definir que parámetros lo determinan.Pacientes y métodoEstudio transversal realizado en 80 pacientes, 57 mujeres, 44 (13) años, con hipertensión arterial pulmonar severa (48 idiopática, 14 aceite colza, 13 colagenosis, 5 virus del sida), presión pulmonar media al diagnóstico 61 (15)mmHg y 49 (33) meses bajo tratamiento, en los que se determinó NTproBNP y realizó ecocardiograma y ergoespirometría.ResultadosNuestros pacientes, bajo tratamiento específico, presentaron el comportamiento típico de los pacientes con hipertensión arterial pulmonar en la ergoespirometría, aunque con menor limitación de capacidad aeróbica y de eficiencia ventilatoria. Ser varón (p=0,004), presentar elevados equivalente ventilatorio de dióxido de carbono en el umbral anaeróbico (p<0,001) y NTproBNP (p=0,006) se asociaron en al análisis multivariado con peor consumo de oxígeno en el máximo esfuerzo mientras que con peores cifras del valor porcentual respecto al predicho lo hicieron: menos tiempo de tratamiento (p=0,01), la dilatación del ventrículo derecho (p<0,001) y un elevado equivalente ventilatorio de dióxido de carbono en el umbral anaeróbico (p<0,001).ConclusionesEn la hipertensión arterial pulmonar, se pueden considerar predictores de peor capacidad funcional la disfunción ventricular derecha (dilatación del VD y elevación de NTproBNP), la ineficiencia ventilatoria, el sexo masculino y el reciente diagnóstico, que implica menor tiempo bajo tratamiento(AU)

IntroductionErgospirometry objectively quantifies exercise capacity. Up until now, the response to exercise evaluated by ergospirometry in patients with pulmonary arterial hypertension has only been described in recently diagnosed.patients.Our aim is to describe the response to exercise in patients with severe pulmonary arterial hypertension under specific treatment and define which parameters determine their exercise capacity.Patients and methodA cross-sectional study was performed on 80 patients, 57 women, aged 45 (14), with severe pulmonary arterial hypertension (48 idiopathic, 14 related to toxic rapeseed oil, 13 to connective tissue disease, 5 to human immunodeficiency virus), mean pulmonary pressure at diagnosis 61(15)mmHg and after 49(33) months under treatment since diagnosis. Biomarkers were measured and echocardiography and ergospirometry were performed the same day.ResultsOur patients, under specific treatment, showed the typical behaviour of patients with pulmonary arterial hypertension with less limitation of both aerobic capacity and ventilatory efficiency. Being male (p=0.004), high ventilatory equivalent for carbon dioxide at anaerobic threshold (p<0.001) or biomarkers (p=0.006) were the strongest predictors of impaired peak oxygen uptake in multivariate analysis, whereas for an impaired percentage achieved of predicted value were right ventricle diastolic diameter (p<0.001), months of treatment (p=0.01) and high ventilatory equivalent for CO2 (p<0.001).ConclusionsIn pulmonary arterial hypertension, right ventricle dysfunction (expressed by its dilation or high NTproBNP) and impaired ventilatory inefficiency as well as being male or a short time under treatment can be considered as determining factors of impaired exercise capacity(AU)

Factors affecting the response to exercise in patients with severe pulmonary arterial hypertension.

INTRODUCTION: Ergospirometry objectively quantifies exercise capacity. Up until now, the response to exercise evaluated by ergospirometry in patients with pulmonary arterial hypertension has only been described in recently diagnosed.patients. Our aim is to describe the response to exercise in patients with severe pulmonary arterial hypertension under specific treatment and define which parameters determine their exercise capacity. PATIENTS AND METHOD: A cross-sectional study was performed on 80 patients, 57 women, aged 45 (14), with severe pulmonary arterial hypertension (48 idiopathic, 14 related to toxic rapeseed oil, 13 to connective tissue disease, 5 to human immunodeficiency virus), mean pulmonary pressure at diagnosis 61(15)mmHg and after 49(33) months under treatment since diagnosis. Biomarkers were measured and echocardiography and ergospirometry were performed the same day. RESULTS: Our patients, under specific treatment, showed the typical behaviour of patients with pulmonary arterial hypertension with less limitation of both aerobic capacity and ventilatory efficiency. Being male (p=0.004), high ventilatory equivalent for carbon dioxide at anaerobic threshold (p<0.001) or biomarkers (p=0.006) were the strongest predictors of impaired peak oxygen uptake in multivariate analysis, whereas for an impaired percentage achieved of predicted value were right ventricle diastolic diameter (p<0.001), months of treatment (p=0.01) and high ventilatory equivalent for CO(2) (p<0.001). CONCLUSIONS: In pulmonary arterial hypertension, right ventricle dysfunction (expressed by its dilation or high NTproBNP) and impaired ventilatory inefficiency as well as being male or a short time under treatment can be considered as determining factors of impaired exercise capacity.

[Transition from prostacyclin to bosentan in five patients with severe pulmonary hypertension: the switch is possible]. / Transición de prostaciclina a bosentán en 5 pacientes con hipertensión pulmonar severa: el cambio es posible.

Prostacyclin improves symptoms, exercise tolerance, and survival in patients with pulmonary arterial hypertension. However, the difficulty of administration (whether intravenous, subcutaneous, or by inhalation) often causes side effects that can reduce the patient's quality of life and which may sometimes be serious. Bosentan, an orally active endothelin receptor antagonist, improves functional class and exercise tolerance in these patients. We describe the successful transition from prostacyclin to bosentan in five patients with severe pulmonary arterial hypertension who suffered serious side effects with prostacyclin treatment.

Transición de prostaciclina a bosentán en 5 pacientes con hipertensión pulmonar severa: el cambio es posible / Transition from prostacyclin to bosentan in five patients with severe pulmonary hypertension: the switch is possible

La prostaciclina mejora los síntomas, la capacidad de ejercicio y la supervivencia en los pacientes con hipertensión arterial pulmonar. Sin embargo, sus complejas vías de administración (intravenosa, inhalada, subcutánea) ocasionan frecuentes efectos adversos que disminuyen la calidad de vida y pueden ser graves. Bosentán, un antagonista oral de los receptores de la endotelina, mejora la clase funcional y la capacidad de ejercicio en estos pacientes. Describimos la transición de prostaciclina a bosentán en 5 pacientes con hipertensión arterial pulmonar severa e importantes complicaciones secundarias al tratamiento con prostaciclina

Prostacyclin improves symptoms, exercise tolerance, and survival in patients with pulmonary arterial hypertension. However, the difficulty of administration (whether intravenous, subcutaneous, or by inhalation) often causes side effects that can reduce the patient's quality of life and which may sometimes be serious. Bosentan, an orally active endothelin receptor antagonist, improves functional class and exercise tolerance in these patients. We describe the successful transition from prostacyclin to bosentan in five patients with severe pulmonary arterial hypertension who suffered serious side effects with prostacyclin treatment

[Primary cardiac lymphoma: diagnosis by transjugular biopsy]. / Linfoma cardíaco primario: diagnóstico mediante biopsia transyugular.

Primary cardiac lymphoma, defined as a non-Hodgkin's lymphoma involving only the heart and pericardium, is an extremely rare malignancy. It should be suspected in patients with a heart mass and heart failure, unexplained refractory pericardial effusion or rhythm disturbances. Transvenous intracardiac tumor biopsy under fluoroscopic or transesophageal echocardiographic guidance, is a minimally invasive technique which makes definite diagnosis possible. We describe a patient in whom primary cardiac lymphoma was diagnosed by this technique. He also underwent percutaneous balloon pericardiotomy because of severe refractory pericardial effusion. Seven months after diagnosis and treatment with standard chemotherapy, the patient remained free of disease.

Linfoma cardíaco primario: diagnóstico mediante biopsia transyugular / Primary cardiac lymphoma: diagnosis by transjugular biopsy

El linfoma cardíaco primario, definido como linfoma no hodgkiniano con afección exclusiva del corazón y pericardio, es un tumor maligno extremadamente raro. Debe sospecharse en pacientes con masas cardíacas y fallo cardíaco, derrame pericárdico refractario o trastornos del ritmo. La biopsia transvenosa, bajo control fluoroscópico o por ecocardiografía transesofágica, es un procedimiento mínimamente invasivo que permite el diagnóstico definitivo. Presentamos un caso de linforma cardíaco primario diagnosticado mediante esta técnica y al que se practicó una pericardiotomía percutánea con balón por derrame pericárdico severo recidivante. El paciente está libre de enfermedad tras tratamiento quimioterápico 7 meses después del diagnóstico (AU)