Tratamiento médico en pacientes con hipertensión pulmonar tromboembólica crónica / Medical treatment in patients with chronic thromboembolic pulmonary hypertension

La tromboendarterectomía pulmonar es el tratamiento de elección en los pacientes con hipertensión pulmonar tromboembólica crónica (HPTEC). Sin embargo, hay campos en los que el tratamiento médico específico de la hipertensión pulmonar (HP) puede ser una alternativa o desempeñar un papel complementario a la cirugía. Así, en los pacientes desestimados para cirugía por obstrucción trombótica distal, los que presentan HP residual o persistente tras la cirugía y los pacientes con HP muy severa y perfil hemodinámico de alto riesgo, el tratamiento médico puede contribuir a mejorar su evolución y el resultado final de la tromboendarterectomía. Los pacientes con obstrucción distal en el árbol pulmonar y los pacientes con HP residual tras la cirugía presentan deterioro clínico y hemodinámico por la progresión de la vasculopatía pulmonar en las arteriolas de pequeño calibre. El tratamiento convencional con diuréticos, antiacoagulantes y oxigenoterapia ha demostrado escasa eficacia. En la última década, se han incorporado multitud de fármacos al tratamiento de la HP: análogos de la prostaciclina, antagonistas de los receptores de la endotelina e inhibidores de la fosfodiesterasa-5, con acción fundamental en el remodelado vascular de las arteriolas de pequeño calibre. Aunque la evidencia de su eficacia en la HP y la similitud histológica de la vasculopatía de pequeño vaso en la HPTEC con la de otras formas de HP, aportan el fundamento racional para el uso de estos fármacos en los pacientes con HPTEC, la evidencia sustentada en ensayos clínicos es todavía limitada (AU)

Pulmonary thromboendarterectomy is the treatment of choice in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, specific medical treatment of pulmonary hypertension (PH) can be an alternative or play a complementary role to surgery. Thus, in patients unsuitable for surgery due to distal thrombotic obstruction, residual or persistent PH after surgery or very severe PH and a high-risk hemodynamic profile, medical treatment may improve their clinical course and the outcome of thromboendarterectomy. Patients with distal obstruction in the pulmonary tree and those with residual PH after surgery show clinical and hemodynamic deterioration due to progression of the pulmonary vascular disease in the small caliber arterioles. Conventional treatment with diuretics, anticoagulants and oxygen therapy has been demonstrated to have little effectiveness. In the last decade, numerous drugs have been developed for the treatment of PH: prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors acting principally in vascular remodelling of small-caliber arterioles. Although evidence of the effectiveness of these drugs in PH and the histological similarity of small-vessel vasculopathy in CTEPH to that of other forms of PH provide the main rationale for the use of these drugs in patients with CTEPH, the evidence from clinical trials is still limited (AU)

[Medical treatment in patients with chronic thromboembolic pulmonary hypertension]. / Tratamiento médico en pacientes con hipertensión pulmonar tromboembólica crónica.

Pulmonary thromboendarterectomy is the treatment of choice in patients with chronic thromboembolic pulmonary hypertension (CTEPH). However, specific medical treatment of pulmonary hypertension (PH) can be an alternative or play a complementary role to surgery. Thus, in patients unsuitable for surgery due to distal thrombotic obstruction, residual or persistent PH after surgery or very severe PH and a high-risk hemodynamic profile, medical treatment may improve their clinical course and the outcome of thromboendarterectomy. Patients with distal obstruction in the pulmonary tree and those with residual PH after surgery show clinical and hemodynamic deterioration due to progression of the pulmonary vascular disease in the smallcaliber arterioles. Conventional treatment with diuretics, anticoagulants and oxygen therapy has been demonstrated to have little effectiveness. In the last decade, numerous drugs have been developed for the treatment of PH: prostacyclin analogs, endothelin receptor antagonists, and phosphodiesterase-5 inhibitors acting principally in vascular remodelling of small-caliber arterioles. Although evidence of the effectiveness of these drugs in PH and the histological similarity of small-vessel vasculopathy in CTEPH to that of other forms of PH provide the main rationale for the use of these drugs in patients with CTEPH, the evidence from clinical trials is still limited.