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Vol. 45. Issue S6.
Hipertensión pulmonar tromboembólica crónica
Pages 11-14 (June 2009)
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Vol. 45. Issue S6.
Hipertensión pulmonar tromboembólica crónica
Pages 11-14 (June 2009)
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Factores de riesgo de la hipertensión pulmonar tromboembólica crónica
Risk factors in chronic thromboembolic pulmonary hypertension
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David Jiméneza,b,
Corresponding author
djc_69_98@yahoo.com

Autor para correspondencia.
, Javier Gaudóa, Antonio Sueiroa,b
a Servicio de Neumología. Hospital Ramón y Cajal, Madrid, España
b Departamento de Medicina. Universidad de Alcalá de Henares, Madrid. España
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Aunque la preocupación de la comunidad científica por la hipertensión pulmonar tromboembólica crónica (HPTEC) es cada vez mayor, la patogenia de esta enfermedad no ha sido completamente aclarada, y no se conocen de manera precisa los factores predisponentes para su aparición. Algunos pacientes pueden estar condicionados genéticamente para desarrollar la HPTEC, pero sólo se han descrito algunas mutaciones del fibrinógeno y un aumento de la frecuencia de polimorfismos de los antígenos leucocitarios humanos. En los pacientes con HPTEC, los defectos hereditarios de la coagulación no son más prevalentes que en los controles, con la excepción del factor VIII y de los anticuerpos antifosfolípido. Algunos estudios han analizado diferencias en la expresión del inhibidor del activador tisular del plasminógeno tipo 1 en el trombo de los pacientes con HPTEC (comparado con los trombos del tromboembolismo pulmonar [TEP] agudo), y sugieren que la trombosis in situ puede contribuir a la persistencia del coágulo y a la progresión de la enfermedad. Algunas características del TEP agudo se han asociado a su progresión a HPTEC; entre ellas, el TEP idiopático, los defectos de perfusión grandes, el TEP masivo, el TEP recurrente, o la hipertensión pulmonar persistente a las 5 semanas del episodio trombótico. Se han descrito diferentes factores clínicos que aumentan el riesgo de HPTEC, como la esplenectomía, las derivaciones ventriculares o las enfermedades inflamatorias crónicas. La terapia tiroidea sustitutiva y el cáncer han emergido como nuevos predictores de HPTEC. La identificación de nuevos factores predisponentes proporcionará claves acerca de los mecanismos patogénicos de la enfermedad y facilitará un diagnóstico precoz y un tratamiento más efectivo.

Palabras clave:
Hipertensión pulmonar tromboembólica crónica
Tromboembolismo de pulmón
Factores predisponentes
Abstract

Although preoccupation with chronic thromboembolic pulmonary hypertension (CTEPH) among the scientific community is constantly increasing, the pathogenesis of this disease has not been completely elucidated and factors predisposing to its development are not precisely known. Some patients may be genetically conditioned to develop CTEPH, but only a few fibrinogen mutations and an increase in the frequency of polymorphisms of human leukocyte antigens have been described.

Hereditary coagulation defects are no more prevalent in patients with CTEPH than in controls, except for factor VIII and antiphospholipid antibodies. Some studies have analyzed differences in the expression of type 1 tissue plasminogen activator inhibitor in the thrombus of patients with CTEPH (compared with thrombi of acute pulmonary thromboembolism [PTE]) and suggest that thrombosis in situ can contribute to the persistence of the coagulum and disease progression. Some characteristics of acute PTE have been associated with its progression to CTEPH, such as idiopathic PTE, large perfusion defects, massive PTE, recurrent PTE and pulmonary hypertension persisting at 5 weeks after the thrombotic event. Several clinical factors that increase the risk of CTEPH have been described, such as splenectomy, ventricular shunts, and chronic inflammatory diseases. Thyroid replacement therapy and cancer have also emerged as new predictors of CTEPH. Identification of new predisposing factors will provide clues to the pathogenic mechanisms of the disease and will facilitate early diagnosis and more effective treatment.

Keywords:
Chronic thromboembolic pulmonary hypertension
Pulmonary thromboembolism
Predisposing factors
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