RESUMEN
Pulmonary disease in liver cirrhosis and portal hypertension (PH) constitutes a challenging clinical scenario and may have important implications with regard to prognosis, liver transplantation (LT) candidacy, and post-LT outcome. Pre-LT evaluation should include adequate screening for pulmonary diseases that may occur concomitantly with liver disease as well as for those that may arise as a complication of end-stage liver disease and PH, given that either may jeopardize safe LT and successful outcome. It is key to discriminate those patients who would benefit from LT, especially pulmonary disorders that have been reported to resolve post-LT and are considered "pulmonary indications" for transplant, from those who are at increased mortality risk and in whom LT is contraindicated. In conclusion, in this article, we review the impact of several pulmonary disorders, including cystic fibrosis, alpha 1-antitrypsin deficiency, hereditary hemorrhagic telangiectasia, sarcoidosis, coronavirus disease 2019, asthma, chronic obstructive pulmonary disease, pulmonary nodules, interstitial lung disease, hepatic hydrothorax, hepatopulmonary syndrome, and portopulmonary hypertension, on post-LT survival, as well as the reciprocal impact of LT on the evolution of lung function.
Asunto(s)
Hipertensión Portal/complicaciones , Cirrosis Hepática/complicaciones , Trasplante de Hígado/mortalidad , Enfermedades Pulmonares/complicaciones , Adulto , Asma/diagnóstico , Asma/epidemiología , Asma/mortalidad , COVID-19/diagnóstico , COVID-19/epidemiología , COVID-19/mortalidad , COVID-19/virología , Niño , Fibrosis Quística , Enfermedad Hepática en Estado Terminal/complicaciones , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/epidemiología , Síndrome Hepatopulmonar/mortalidad , Humanos , Hipertensión Pulmonar/tratamiento farmacológico , Hipertensión Pulmonar/etiología , Trasplante de Hígado/métodos , Enfermedades Pulmonares/epidemiología , Enfermedades Pulmonares/patología , Enfermedades Pulmonares/fisiopatología , Tamizaje Masivo , Selección de Paciente/ética , Pronóstico , Enfermedad Pulmonar Obstructiva Crónica/diagnóstico , Enfermedad Pulmonar Obstructiva Crónica/epidemiología , Enfermedad Pulmonar Obstructiva Crónica/mortalidad , Pruebas de Función Respiratoria/métodos , SARS-CoV-2/genética , SARS-CoV-2/aislamiento & purificación , Sarcoidosis/diagnóstico , Sarcoidosis/epidemiología , Sarcoidosis/mortalidad , Tasa de Supervivencia/tendencias , Telangiectasia Hemorrágica Hereditaria/diagnóstico , Telangiectasia Hemorrágica Hereditaria/epidemiología , Telangiectasia Hemorrágica Hereditaria/mortalidad , Deficiencia de alfa 1-Antitripsina/diagnóstico , Deficiencia de alfa 1-Antitripsina/epidemiología , Deficiencia de alfa 1-Antitripsina/mortalidadRESUMEN
Hepatopulmonary syndrome (HPS) is a pulmonary vascular complication of liver disease, which adversely affects prognosis. The disease is characterised by intrapulmonary vascular dilatations and shunts, resulting in impaired gas exchange. A complex interaction between the liver, the gut and the lungs, predominately impacting pulmonary endothelial cells, immune cells and respiratory epithelial cells, is responsible for the development of typical pulmonary alterations seen in HPS. Liver transplantation is the only therapeutic option and generally reverses HPS. Since the implementation of the model for end-stage liver disease (MELD) standard exception policy, outcomes in patients with HPS have been significantly better than they were in the pre-MELD era. This review summarises current knowledge and highlights what's new regarding the diagnosis and management of HPS, and our understanding of pathogenesis based on experimental models and translational studies.