Portopulmonary hypertension: prognosis and management in the current treatment era - results from the REHAP registry.

BACKGROUND: Portopulmonary hypertension (PoPH) is a rare condition with poorer survival compared to idiopathic/familial pulmonary arterial hypertension (IPAH/FPAH). AIMS: To compare the characteristics, survival, prognostic factors and management of PoPH and IPAH/FPAH patients and to assess the impact of treatment on survival of PoPH patients. METHODS: Analysis of data of prevalent and incident PoPH patients enrolled in the Spanish registry of PAH (REHAP) from January 1998 to December 2017 and comparison with IPAH/FPAH patients. Variables analysed: patient and disease (PAH and liver) characteristics, first-line PAH-targeted therapy, causes of death, prognostic factors and survival (according to aetiology and treatment in PoPH patients). RESULTS: Compared to IPAH/FPAH patients (n = 678), patients with PoPH (n = 237) were predominantly men, older and had better functional class and higher prevalence of ascites. Haemodynamics were better. Biomarkers for heart failure were worse. Age- and sex-adjusted 5-year survival rate from diagnosis was 49.3% for PoPH patients and 68.7% for IPAH patients (P < 0.001). Treated PoPH had better survival than non-treated. PAH- and liver-related causes accounted for 30.2% and 24.7% of deaths in PoPH patients. PoPH patients were less likely to receive first-line PAH-targeted therapy and this was associated with greater mortality. Increasing age, worse exercise capacity and ascites were independent prognostic factors of poorer survival; first-line oral monotherapy was associated with improved survival. Eight (3.4%) PoPH patients underwent liver transplantation. CONCLUSIONS: PoPH patients are undertreated and show poorer survival than IPAH/FPAH patients. First-line treatment with PAH-targeted therapy was associated with better survival. Presence of ascites was a predictor of mortality.

Lipomatosis cardiaca masiva, hallazgo en la autopsia de un paciente con muerte súbita / Massive cardiac lipomatosis, an autopsy finding in a patient with sudden death

La sustitución grasa de las células miocárdicas es un proceso degenerativo que afecta en mayor medida al ventrículo derecho y se encuentra en el 50% de las personas ancianas. El problema se origina cuando esta degeneración se produce en grado masivo, planteando la necesidad de realizar el diagnóstico diferencial con otras enfermedades. Se presenta el caso de un paciente que falleció de forma súbita y en su autopsia se encontró esta dolencia como única explicación del desenlace (AU)

The fat replacement of myocardial cells is a degenerative process that usually affects the right ventricle and is found in 50% of the elderly. The problem arises when this degeneration occurs to a massive degree, a differential diagnosis with other pathologies being necessary. We present the case of a patient who died suddenly and a massive cardiac lipomatosis was found on autopsy, as the only explanation of the outcome (AU)

[Massive cardiac lipomatosis, an autopsy finding in a patient with sudden death]. / Lipomatosis cardiaca masiva, hallazgo en la autopsia de un paciente con muerte súbita.

The fat replacement of myocardial cells is a degenerative process that usually affects the right ventricle and is found in 50% of the elderly. The problem arises when this degeneration occurs to a massive degree, a differential diagnosis with other pathologies being necessary. We present the case of a patient who died suddenly and a massive cardiac lipomatosis was found on autopsy, as the only explanation of the outcome.

Hallazgos por tomografía de coherencia óptica en la hipertensión pulmonar tromboembólica / Optical coherence tomography in thromboembolic pulmonary hypertension

No disponible

[Recording the daily physical activity of COPD patients with an accelerometer: An analysis of agreement and repeatability]. / Registro de la actividad física cotidiana mediante un acelerómetro en pacientes con EPOC. Análisis de concordancia y reproducibilidad.

OBJECTIVE: The purpose of this study was to assess the agreement between different measurements of mean daily physical activity taken over a week in chronic obstructive pulmonary disease (COPD) patients with an accelerometer and to analyze the medium-term repeatability of these measurements. PATIENTS AND METHODS: The study enrolled 12 healthy control subjects and 23 patients with stable COPD (mean [SD] forced expiratory volume in 1 second [FEV1] of 45% [13%] of predicted and a ratio of FEV1 to forced vital capacity of 53% [13%]). Accelerometer output, measured in vector magnitude units, was recorded in a physical activity log for a 1-week period. The results were then analyzed to compare output for a conventional recording period (Friday to Sunday) to that for 2 other periods (Monday to Wednesday and Tuesday to Thursday). The measurements were repeated 3 to 5 weeks later. RESULTS: Activity counts were lower in the COPD patients than in the control subjects (184 [99] vs 314 [75]; P < .001). In the COPD patients, the results for the Friday to Sunday period correlated well with the results for both the Monday to Wednesday period (95% confidence interval, -29.21 to 28.81) and the Tuesday to Thursday period (95% confidence interval, -32.13 to 28.43). There were no significant differences in terms of medium-term repeatability of accelerometer readings between the COPD group and the control group (repeatability coefficient of 11.2% [4.6%] and 8.5% [4.7%], respectively). CONCLUSIONS: Both agreement between the different measurements of physical activity taken during a 1-week period and medium-term repeatability for COPD patients and control subjects were very good.

Registro de la actividad física cotidiana mediante un acelerómetro en pacientes con EPOC. Análisis de concordancia y reproducibilidad / Recording the daily physical activity of COPD patients with an accelerometer: an analysis of agreement and repeatability

Objetivo: Evaluar la concordancia intrasemanal de la medida con un acelerómetro de la actividad física cotidiana en pacientes con enfermedad pulmonar obstructiva crónica (EPOC). Analizar la reproducibilidad del registro a medio plazo. Pacientes y métodos: Se estudió a 23 pacientes con EPOC estable ­cifra media (± desviación estándar) de volumen espiratorio forzado en el primer segundo del 45 ± 13% y cociente entre este parámetro y la capacidad vital forzada del 53 ± 13%­ y 12 sujetos sanos. Se efectuó un registro con acelerómetro durante una semana y se analizó el vector de desplazamiento (VMU) en el período convencional (viernes a sábado) y en 2 períodos alternativos (lunes a miércoles y martes a jueves). El registro se repitió a las 3-5 semanas. Resultados: El VMU fue menor en los pacientes con EPOC que en los controles (184 ± 99 frente a 314 ± 75; p < 0,001). En el grupo con EPOC el registro de viernes a domingo mantenía una buena relación con el efectuado de lunes a miércoles (intervalo de concordancia del 95%, ­-29,21 a 28,81) y con el realizado de martes a jueves (intervalo de concordancia del 95%, ­-32,13 a 28,43). El coeficiente de reproducibilidad del VMU a medio plazo de los pacientes con EPOC (11,2 ± 4,6%) no resultó ser significativamente diferente del de los controles (8,5 ± 4,7%). Conclusiones: El registro de la actividad física cotidiana mediante un acelerómetro alcanza una elevada concordancia entre períodos de análisis comprendidos dentro de una misma semana y muestra una notable reproducibilidad a medio plazo, tanto en sujetos sanos como en pacientes con EPOC

Objective: The purpose of this study was to assess the agreement between different measurements of mean daily physical activity taken over a week in chronic obstructive pulmonary disease (COPD) patients with an accelerometer and to analyze the medium-term repeatability of these measurements. Patients and methods: The study enrolled 12 healthy control subjects and 23 patients with stable COPD (mean [SD] forced expiratory volume in 1 second [FEV1] of 45% [13%] of predicted and a ratio of FEV1 to forced vital capacity of 53% [13%]). Accelerometer output, measured in vector magnitude units, was recorded in a physical activity log for a 1-week period. The results were then analyzed to compare output for a conventional recording period (Friday to Sunday) to that for 2 other periods (Monday to Wednesday and Tuesday to Thursday). The measurements were repeated 3 to 5 weeks later. Results: Activity counts were lower in the COPD patients than in the control subjects (184 [99] vs 314 [75]; P<.001). In the COPD patients, the results for the Friday to Sunday period correlated well with the results for both the Monday to Wednesday period (95% confidence interval, ­-29.21 to 28.81) and the Tuesday to Thursday period (95% confidence interval, -­32.13 to 28.43). There were no significant differences in terms of medium-term repeatability of accelerometer readings between the COPD group and the control group (repeatability coefficient of 11.2% [4.6%] and 8.5% [4.7%], respectively). Conclusions: Both agreement between the different measurements of physical activity taken during a 1-week period and medium-term repeatability for COPD patients and control subjects were very good