Ventilación mecánica no invasiva en pacientes con síndrome de obesidad-hipoventilación. Evolución a largo plazo y factores pronósticos / Noninvasive Mechanical Ventilation in Patients with Obesity Hypoventilation Syndrome. Long-term Outcome and Prognostic Factors

Introducción: Desde el punto de vista respiratorio, la obesidad se asocia con 2 enfermedades muy relacionadas: el síndrome de obesidad-hipoventilación (SOH) y el síndrome de apnea-hipopnea del sueño (SAHS). Se ha demostrado que el tratamiento con ventilación mecánica no invasiva durante el sueño produce una mejoría clínica y funcional en estos pacientes. Analizamos a largo plazo la supervivencia con este tratamiento, y la diferencia en la evolución entre pacientes con SOH con y sin SAHS asociado. Metodología: Estudio longitudinal, observacional, de una cohorte de pacientes diagnosticados de SOH e incluidos en un programa de ventilación domiciliaria a lo largo de 12 años, distribuidos en 2 grupos: SOH puro y SOH asociado a SAHS. La ventilación se llevó a cabo con ventiladores de presión positiva continua binivel. Durante el tiempo de seguimiento se monitorizó y comparó su situación clínica (síntomas, exacerbaciones e ingresos), gasométrica y funcional, así como su supervivencia. Resultados: Ochenta y tres pacientes fueron válidos para el análisis, 60 mujeres (72,3%) y 23 hombres (27,7%), con una media de supervivencia de 8,47 años. Cincuenta pacientes (60,2%) fueron incluidos en el grupo sin SAHS (SOH) y 33 (39,8%) en el grupo con SAHS (SOH-SAHS). La PaCO2 del grupo SOH era significativamente mayor que la del grupo SOH-SAHS (p < 0,01), y también presentaban más hospitalizaciones (p < 0,05). Existió una mejoría significativa en ambos grupos en FEV1 y FVC, y en los valores de PaCO2 y PaO2, sin diferencias entre los grupos. Mientras que no se apreciaron diferencias en la supervivencia relacionadas con el subgrupo diagnóstico, valores bajos de FVC sí constituían un factor predictivo de mortalidad. Conclusiones: El uso de ventilación domiciliaria en pacientes con SOH con o sin SAHS es un tratamiento eficaz que corrige las alteraciones gasométricas y funcionales y permite alcanzar tiempos prolongados de supervivencia

Introduction: Obesity is associated with 2 closely related respiratory diseases: obesity hypoventilation syndrome (OHS) and obstructive sleep apnea-hypopnea syndrome (OSAHS). It has been shown that noninvasive ventilation during sleep produces clinical and functional improvement in these patients. The long-term survival rate with this treatment, and the difference in clinical progress in OHS patients with and without OSAHS are analyzed. Methodology: Longitudinal, observational study with a cohort of patients diagnosed with OHS, included in a home ventilation program over a period of 12 years, divided into 2 groups: pure OHS and OSAHS associated OHS. Bi-level positive airway pressure ventilation was administered. During the follow-up period, symptoms, exacerbations and hospitalizations, blood gas tests and pulmonary function tests, and survival rates were monitored and compared Results: Eighty-three patients were eligible for analysis, 60 women (72.3%) and 23 men (27.7%), with a mean survival time of 8.47 years. Fifty patients (60.2%) were included in the group without OSAHS (OHS) and 33 (39.8%) in the OSAHS-associated OHS group (OHS-OSAHS). PaCO2 in the OHS group was significantly higher than in the OHS-OSAHS group (P < .01). OHS patients also had a higher hospitalization rate (P < .05). There was a significant improvement in both groups in FEV1 and FVC, and no differences between groups in PaCO2 and PaO2 values. There were no differences in mortality between the 2 groups, but low FVC values were predictive of mortality. Conclusions: The use of mechanical ventilation in patients with OHS, with or without OSAHS, is an effective treatment for the correction of blood gases and functional alterations and can achieve prolonged survival rates

Noninvasive mechanical ventilation in patients with obesity hypoventilation syndrome. Long-term outcome and prognostic factors.

INTRODUCTION: Obesity is associated with 2 closely related respiratory diseases: obesity hypoventilation syndrome (OHS) and obstructive sleep apnea-hypopnea syndrome (OSAHS). It has been shown that noninvasive ventilation during sleep produces clinical and functional improvement in these patients. The long-term survival rate with this treatment, and the difference in clinical progress in OHS patients with and without OSAHS are analyzed. METHODOLOGY: Longitudinal, observational study with a cohort of patients diagnosed with OHS, included in a home ventilation program over a period of 12 years, divided into 2 groups: pure OHS and OSAHS-associated OHS. Bi-level positive airway pressure ventilation was administered. During the follow-up period, symptoms, exacerbations and hospitalizations, blood gas tests and pulmonary function tests, and survival rates were monitored and compared. RESULTS: Eighty-three patients were eligible for analysis, 60 women (72.3%) and 23 men (27.7%), with a mean survival time of 8.47 years. Fifty patients (60.2%) were included in the group without OSAHS (OHS) and 33 (39.8%) in the OSAHS-associated OHS group (OHS-OSAHS). PaCO2 in the OHS group was significantly higher than in the OHS-OSAHS group (P<.01). OHS patients also had a higher hospitalization rate (P<.05). There was a significant improvement in both groups in FEV1 and FVC, and no differences between groups in PaCO2 and PaO2 values. There were no differences in mortality between the 2 groups, but low FVC values were predictive of mortality. CONCLUSIONS: The use of mechanical ventilation in patients with OHS, with or without OSAHS, is an effective treatment for the correction of blood gases and functional alterations and can achieve prolonged survival rates.

Enfermedad pulmonar venooclusiva en una mujer jardinera / Pulmonary Veno-Occlusive Disease in a Female Gardener

La enfermedad pulmonar venooclusiva es una forma infrecuente de hipertensión arterial pulmonar y su pronóstico es sombrío. El diagnóstico suele ser tardío y el único tratamiento curativo en la actualidad es el trasplante pulmonar. Presentamos el caso de una mujer jardinera de 51 años cuyo diagnóstico se obtuvo por biopsia pulmonar abierta realizada previamente a su fallecimiento. Si bien existen casos reportados de enfermedad venooclusiva hepática secundaria a agentes tóxicos presentes en la naturaleza, como el alcaloide pirrolizidina, hasta el momento no se han relacionado con la enfermedad a nivel pulmonar

Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension with a poor prognosis. The diagnosis is usually delayed and treatment options other than lung transplantation are unfortunately limited. We report the case of 51-year-old female gardener diagnosed with PVOD by open lung biopsy before her death. Although there are many reported cases of hepatic veno-occlusive disease due to toxic agents present in nature, such as pyrrolizidine alkaloid exposure, to date this has not been linked to PVOD

Pulmonary veno-occlusive disease in a female gardener.

Pulmonary veno-occlusive disease (PVOD) is a subgroup of pulmonary arterial hypertension with a poor prognosis. The diagnosis is usually delayed and treatment options other than lung transplantation are unfortunately limited. We report the case of 51-year-old female gardener diagnosed with PVOD by open lung biopsy before her death. Although there are many reported cases of hepatic veno-occlusive disease due to toxic agents present in nature, such as pyrrolizidine alkaloid exposure, to date this has not been linked to PVOD.