RESUMEN

El conocimiento bio-patológico actual de la hipertensión arterial pulmonar idiopática (HAPI), se sustenta en los hallazgos patológicos de la enfermedad descritos hace más de 30 años, consistentes en importantes alteraciones morfológicas y funcionales de células endoteliales, células del músculo liso vascular pulmonar (CMLVP) y de la propia adventicia. El concepto fisiopatológico de la HAPI centrado en vasoconstricción ha cedido su lugar al de un endotelio vascular entendido como un complejo formador de una innumerable cantidad de sustancias con propiedades vasoactivas, antitrombóticas y antiproliferativas, cuyo desequilibrio favorece la aparición de vasoconstricción, trombosis y proliferación celular anormal. Es en base a estos conceptos que en la actualidad se considera a la HAPI como una enfermedad con carácter angioproliferativo.

PALABRAS CLAVE

Patobiología, hipertensión arterial pulmonar idiopática, células endoteliales, células de músculo liso vascular, proliferación vascular.

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