Format

Send to

Choose Destination
Presse Med. 2014 Sep;43(9):970-80. doi: 10.1016/j.lpm.2014.07.010. Epub 2014 Aug 20.

[Pulmonary hypertension in liver diseases].

[Article in French]

Author information

1
University Paris-Sud, faculté de médecine, 94270 Le Kremlin-Bicêtre, France; Service de pneumologie, département hospitalo-universitaire (DHU) thorax innovation (TORINO), centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, AP-HP, 94270 Le Kremlin-Bicêtre, France; Centre chirurgical Marie-Lannelongue, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), UMR_S 999, Inserm, 92350 Le Plessis-Robinson, France. Electronic address: laurent.savale@bct.aphp.fr.
2
University Paris-Sud, faculté de médecine, 94270 Le Kremlin-Bicêtre, France; Service de pneumologie, département hospitalo-universitaire (DHU) thorax innovation (TORINO), centre de référence de l'hypertension pulmonaire sévère, hôpital de Bicêtre, AP-HP, 94270 Le Kremlin-Bicêtre, France; Centre chirurgical Marie-Lannelongue, laboratoire d'excellence (LabEx) en recherche sur le médicament et l'innovation thérapeutique (LERMIT), UMR_S 999, Inserm, 92350 Le Plessis-Robinson, France.

Abstract

Portopulmonary hypertension (PoPH) is defined by the combination of portal hypertension and precapillary pulmonary hypertension (mPAP ≥ 25 mmHg, PCWP < 15 mmHg and PVR > 3 Wood units). PoPH is characterised by pathobiological mechanisms that are similar to other forms of pulmonary arterial hypertension. Prevalence of PoPH is estimated at 0.5-5% among patients with portal hypertension with or without cirrhosis. Treatment strategies most commonly employed for PoPH patients are based on recommendations for idiopathic PAH management. Indeed, the choice of specific PAH treatment must take account the severity of the underlying liver disease. Prognosis of PoPH patients is dependent on both the severity of PAH and of the underlying liver disease. PoPH may be a contraindication for orthotopic liver transplantation (OLT) if mean pulmonary arterial pressure is > 35 mmHg associated with severe right ventricular dysfunction or high level of pulmonary vascular resistance (> 3-4 Wood units). Bridge therapy with specific PAH therapies should be considered in those patients in an attempt to improve pulmonary hemodynamic and thereby allow OLT with acceptable risk. Recent data suggest that stabilize, improve or cure PoPH seems to be possible by combining specific PAH therapies and liver transplantation in selected patients. Clinical and experimental evidences suggest that IFN therapy may be a possible risk factor for PAH.

PMID:
25148949
DOI:
10.1016/j.lpm.2014.07.010
[Indexed for MEDLINE]

Supplemental Content

Full text links

Icon for Elsevier Science
Loading ...
Support Center