Article information
Resumen
La hipertensión pulmonar tromboembólica crónica se caracteriza por la organización de material trombótico dentro de las arterias pulmonares. La falta de correlación entre la proporción de arterias pulmonares obliteradas y las cifras de hipertensión pulmonar sugiere que una teoría exclusivamente mecánica podría ser demasiado simplista. El embolismo pulmonar agudo podría ser el evento inicial, pero la progresión de la enfermedad resultaría del remodelado vascular progresivo de los pequeños vasos. Es posible que la trombosis arterial pulmonar no resuelta sea un factor decisivo para que células endoteliales vasculares inicien su transición mesenquimal. Se analizan los aspectos genéticos, mecanismos inflamatorios y condicionantes clínicos que influyen en el mosaico fisiopatológico de esta compleja entidad.
Palabras clave:
Hipertensión pulmonar postrombótica
Embolia pulmonar crónica
Fisiopatología
Enfermedad tromboembólica venosa
Etiopathogenesis and physiopathology
Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is characterized by the organization of thrombotic material within the pulmonary arteries. The lack of correlation between the proportion of obliterated pulmonary arteries and pulmonary hypertension values suggest that an exclusively mechanical theory could be too simplistic. Acute pulmonary embolism could be the initial event, but disease progression probably results from progressive vascular remodelling of the small vessels. Unresolved pulmonary arterial thrombosis may be a decisive factor in initiating endothelial-to-mesenchymal transition. The present article analyzes the genetic features, inflammatory mechanisms and clinical factors influencing the physiopathological mosaic of this complex entity.
Keywords:
Postthrombotic pulmonary hypertension
Chronic pulmonary embolism
Physiopathology
Venous thromboembolic disease
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