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Vol. 45. Issue S1.
Pages 43-48 (February 2009)
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Vol. 45. Issue S1.
Pages 43-48 (February 2009)
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Hipertensión arterial pulmonar: un recorrido por el año 2008
Pulmonary Arterial Hypertension: a Voyage Around the Year 2008
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Adolfo Baloira
Servicio de Neumología, Complexo Hospitalario de Pontevedra, Pontevedra, España
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Resumen

La hipertensión arterial pulmonar (HAP) ha tenido un espectacular desarrollo, tanto en la terapéutica como en el conocimiento de su patogenia. Durante el año 2008 se han publicado algunos trabajos que ha contribuido a mejorar un poco más estos dos aspectos. En lo que se refiere a la patogenia, las mutaciones en BMPR2 siguen ganando puntos como actores fundamentales en el desarrollo de la enfermedad. Se ha podido comprobar que los pacientes que portan alguna de ellas tienen un curso clínico más rápido y grave. Existe una relación entre la vía de BMPR2 y la inflamación de árbol vascular pulmonar. En este año también ha aparecido en escena un nuevo fármaco antiendotelina: ambrisentán. Con una eficacia comparable con la de otros fármacos de su grupo, los efectos secundarios parecen ser bastante menores. Un trabajo importante ha sido la demostración de una mejoría en diversos parámetros de pacientes con HAP en clase funcional II con bosentán. También se han presentado resultados de algunas nuevas combinaciones, como sildenafilo y epoprostenol. Un tipo frecuente de HAP es el que aparece asociado a enfermedad tromboembólica. Tanto el tratamiento con sildenafilo como, en algunos casos seleccionados, la angioplastia percutánea obtienen respuestas favorables. Por último, en 2008 han visto la luz dos nuevos documentos de consenso, uno español y otro británico que, sobre la base de los conocimientos actuales, clarifican un poco más el manejo de esta grave enfermedad.

Palabras clave:
Hipertensión arterial pulmonar
BMPR2
Antiendotelina
Sildenafilo
Abstract

There have been spectacular developments in pulmonary arterial hypertension (PAH), both in its treatment and knowledge of its pathogenesis. Several studies have been published throughout 2008 that have contributed to improve these two aspects a little. As regards the pathogenesis, mutations in BMPR2 continue gaining points as fundamental factors in the development of the disease. It has been shown that patients who carry any of them have a more rapid and severe clinical course. There is a relationship between the BMPR2 pathway and inflammation of the pulmonary vascular tree. A new anti-endothelin drug, ambrisentan, has also appeared on the scene this year. With an efficacy comparable to other drugs of its group, the secondary effects appear to be a lot less. An important work has been the demonstration of an improvement in several parameters in functional class II in patients with PAH with bosentan. Results using new combinations, such as sildenafil and epoprostenol, have also been presented. A common type of PAH is that which seems to be associated with thromboembolic disease. Treatment with sildenafil and in some selected cases, percutaneous angioplasty, has obtained favourable responses. Finally, in 2008, two new consensus documents have emerged, one Spanish and the other British, which in the light of current knowledge, give a clearer insight into the management of this serious disease.

Keywords:
Pulmonary arterial hypertension
BMPR2
Anti-endothelin
Sildenafil
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Copyright © 2009. Sociedad Española de Neumología y Cirugía Torácica
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