Información del artículo
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
Abreviaturas:
AMPc
AD
CIA
CC
CML
ET-1
GMPc
HP
HAP
NO
PPs
PAD
SE
TGF-β
VD
VEGF
VI
VIH
Recent advances in the understanding of biopathology in pulmonary arterial hypertension have changed the perspective of the disease. Endothelial dysfunction induces a vasocontrictive response, a hypercoagulability state and, more importantly, an increase of cellular proliferation with subsequent vascular remodeling. Pulmonary vascular resistance is therefore increased due to progressive vessel obliteration. New therapeutic approaches are focused on vascular remodeling inhibition and endothelial dysfunction.
Pulmonary arterial hypertension is associated with an increasing number of different diseases, which makes necessary to establish a systematic diagnostic approach, and to define a classification. The main topics in this review will be the recent advances in pathobiology, the new clinical classification, the changes in diagnostic definitions and the last therapeutic approaches, pointing out the future directions. Finally, recent recommendations for diagnosis and treatment published by the main scientific societies are provided.
Key words:
Pulmonary hypertension
Prostacyclin
Sidenafil
Bosentan
Lung transplant
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