Transcript
Announcer:
Welcome to Clinician’s Roundtable on ReachMD. Today, Dr. Arun Jose will be discussing the role of liver transplantation in portopulmonary hypertension. Dr. Jose is an Associate Professor of Medicine at the University of Cincinnati College of Medicine. Let’s hear from him now.
Dr. Jose:
Much like other types of pulmonary arterial hypertension, it's treated with targeted PAH therapy. The challenge in portopulmonary hypertension is that the underlying liver disease or the presence of portopulmonary hypertension is often an exclusion criterion for clinical trials, so there aren't that many studies of patients with portopulmonary hypertension. Much of the evidence we have for treatment is extrapolated from other types of pulmonary arterial hypertension. Consequently, many of these people have not as profound of a treatment response, and they often have weaker responses to treatment and worse outcomes over time compared to other types of PAH.
Liver transplantation is important, because most people with portopulmonary hypertension have improvement following liver transplant. We think that in about half of them, they have resolution of their pulmonary arterial hypertension after liver transplantation. That's one of the few scenarios where you can have a cessation, resolution, or cure of almost of your pulmonary arterial hypertension. There are very few other scenarios where that can happen.
Because it's one of the few cases where you can actually have a resolution of your pulmonary arterial hypertension after a liver transplant, every portopulmonary hypertension patient should be evaluated for potential liver transplantation. And clinicians should work very hard to try and get them to liver transplant, just because the effects can be so profound on both their liver disease—because you get a new liver— and also the extent of their pulmonary vascular disease.
It's more challenging than that, though, because not everyone has a positive response to liver transplant. There's a small subset of patients who seem to develop worsening pulmonary arterial hypertension after liver transplant, and so managing people in the immediate perioperative and postoperative, post-transplant phase can be very challenging.
But I would say that the major issue that can confront clinicians is that you need to aggressively evaluate every portopulmonary hypertension patient for liver transplant. Not everyone is going to be a transplant candidate, but everyone should be evaluated to see if they can be a transplant candidate, because it could potentially be curative for multiple diseases.
Announcer:
You just heard Dr. Arun Jose explaining the importance of liver transplantation in portopulmonary hypertension. To access this and other episodes in our series, visit Clinician’s Roundtable on ReachMD.com, where you can Be Part of the Knowledge. Thanks for listening!