• portal hypertension
• pulmonary arterial h
• pulmonary hypertensi
• right ventricle
• sildenafil

BACKGROUND
Portopulmonary hypertension (PoPH) is a clinical condition associated with end‐stage liver disease, described by the coexistence of pulmonary arterial hypertension (PAH) and portal hypertension. In PoPH patients there is a right ventricle (RV) remodeling to compensate for the increased resistance in the lung circulation. There are no studies on the effects of the PAH-targeted pharmacological treatment on the RV dimension and function. The present study summarizes our experience in patients with PoPH treated with sildenafil in a period of 6 years (from 2013 to 2019).
METHODS
We enrolled 64 consecutive patients identified as PoPH, all treated with sildenafil (57.6% in monotherapy; in the other cases in association with macitentan; in 19.0% with an initial combination therapy). Hemodynamic invasive cardiopulmonary study was performed at baseline and after 6 months of sildenafil treatment.
RESULTS
In our population we showed a significative improvement in RV performance, with a significant increase in RV stroke volume (+ 33%), RV ejection fraction (+ 31%) and RV stroke work index (+17.5%). We registered the reduction of the RV cavity dimension over time in all patients treated with sildenafil (RV EDD decreased by 15% after 6 months of follow-up). Regarding diastolic function, we highlighted a very significant reduction in RV end-diastolic pressure (-50% respect to baseline). Sildenafil was effective both when used as monotherapy, and in combination with macitentan.
CONCLUSIONS
Sildenafil had a positive impact on RV systolic and diastolic function in patients with PoPH, and was able to conditionate the reverse remodeling of the RV.

BACKGROUND Portopulmonary hypertension (PoPH) is a clinical condition associated with end‐stage liver disease, described by the coexistence of pulmonary arterial hypertension (PAH) and portal hypertension. In PoPH patients there is a right ventricle (RV) remodeling to compensate for the increased resistance in the lung circulation. There are no studies on the effects of the PAH-targeted pharmacological treatment on the RV dimension and function. The present study summarizes our experience in patients with PoPH treated with sildenafil in a period of 6 years (from 2013 to 2019). METHODS We enrolled 64 consecutive patients identified as PoPH, all treated with sildenafil (57.6% in monotherapy; in the other cases in association with macitentan; in 19.0% with an initial combination therapy). Hemodynamic invasive cardiopulmonary study was performed at baseline and after 6 months of sildenafil treatment. RESULTS In our population we showed a significative improvement in RV performance, with a significant increase in RV stroke volume (+ 33%), RV ejection fraction (+ 31%) and RV stroke work index (+17.5%). We registered the reduction of the RV cavity dimension over time in all patients treated with sildenafil (RV EDD decreased by 15% after 6 months of follow-up). Regarding diastolic function, we highlighted a very significant reduction in RV end-diastolic pressure (-50% respect to baseline). Sildenafil was effective both when used as monotherapy, and in combination with macitentan. CONCLUSIONS Sildenafil had a positive impact on RV systolic and diastolic function in patients with PoPH, and was able to conditionate the reverse remodeling of the RV.