Recientemente se han producido importantes avances en el conocimiento de la biopatología de la hipertensión arterial pulmonar (HAP) que han cambiado la perspectiva de la enfermedad. La disfunción del endotelio vascular induce un predominio del tono vasoconstrictor, de la hipercoagulabilidad y, fundamentalmente, incrementa la proliferación celular, produciendo un remodelado vascular con obliteración progresiva de la luz y un incremento de las resistencias vasculares pulmonares. Los nuevos tratamientos médicos actuarán sobre el remodelado vascular y la disfunción endotelial. Asimismo, el número creciente de enfermedades asociadas a la HAP y la aparición de nuevas técnicas diagnósticas obligan a sistematizar el procedimiento diagnóstico y definir una clasificación.
En esta revisión, se actualizan las principales novedadesen la patobiología pulmonar, las modificaciones en la clasificación clínica, los cambios en las definiciones diagnósticas, las nuevas estrategias terapéuticas y las perspectives futuras. Además, se exponen las recomendaciones de las principales sociedades científicas para el manejo del paciente con HAP.
Palabras clave
Hipertensión pulmonar
Prostaciclina
Sidenafilo
Bosentán
Trasplante pulmonar
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