How should we treat portopulmonary hypertension?

Abstract

The development of pulmonary arterial hypertension in the setting of advanced liver disease (portopulmonary hypertension (POPH)) has certainly captured the attention of all liver transplant centres, but why? For patients with moderate-to-severe POPH (mean pulmonary artery pressure (mPAP) >35 mmHg and pulmonary vascular resistance (PVR) >400 dyne·s·cm-5), the risk of intra-operative death and post-transplant hospitalisation mortality in otherwise acceptable liver transplant candidates is high 1. The American Association for the Study of Liver Disease Practice Guidelines have espoused Doppler echocardiography screening for all liver transplant candidates 2. However, once POPH is confirmed by right-heart catheterisation, what therapeutic approach should be considered? More generally, what should the approach be to treating POPH, irrespective of liver transplant candidacy? POPH is not simply a liver transplant issue, but that area of clinical medicine has raised awareness of this entity over the past few years. Recent evidence from France underscores the importance of POPH: it was the third-most common type of pulmonary hypertension seen in a consortium of 17 French hospitals 3. The French evidence mirrors that of the Mayo Clinic (Rochester, MN, USA) over the past 10 yrs. POPH is the third-most common disorder documented in the Mayo Pulmonary Hypertension Clinic since 1996. In the current issue of the European Respiratory …