TY - JOUR
T1 - Clinical Outcomes After Liver Transplantation in Patients With Portopulmonary Hypertension
AU - Cartin-Ceba, Rodrigo
AU - Burger, Charles
AU - Swanson, Karen
AU - Vargas, Hugo
AU - Aqel, Bashar
AU - Keaveny, Andrew P.
AU - Heimbach, Julie
AU - Taner, Timucin
AU - Nyberg, Scott
AU - Rosen, Charles
AU - Cajigas, Hector
AU - Dubrock, Hilary
AU - Krowka, Michael J.
N1 - Funding Information:
This work was supported by the Brown Gift for Liver-Lung Research, Department of Transplantation, Mayo Clinic, Rochester, MN.
Publisher Copyright:
© 2021 Lippincott Williams and Wilkins. All rights reserved.
PY - 2021/10/1
Y1 - 2021/10/1
N2 - Background. Portopulmonary hypertension (POPH) is the presence of pulmonary arterial (PA) hypertension in patients with portal hypertension and is associated with significant morbidity and mortality. In a cohort of POPH patients, we describe the clinical outcomes of POPH patients who underwent liver transplantation (LT). Methods. Retrospectively collected data from a prospectively assembled cohort of all consecutive POPH adults evaluated in 3 transplant centers from 1996 to 2019. Results. From a cohort of 228 POPH patients, 50 patients underwent LT. Significant hemodynamic improvement after PA-targeted therapy was observed, with 58% receiving only monotherapy pretransplant. After LT, 21 (42%) patients were able to discontinue and remained off PA-targeted therapy. The 1-, 3-, and 5-y unadjusted survival rates after LT were 72%, 63%, and 60%, respectively. An elevated pulmonary vascular resistance (PVR) before LT was associated with worse survival rate (HR, 1.91; 95% CI, 1.07-3.74, P = 0.04). No survival difference was observed in those granted MELD exception or transplants performed before or after the year 2010. Conclusions. Significant number of POPH patients discontinued PA-targeted therapy after LT. Higher PVR before LT was associated with worse survival, as was monotherapy use. Despite effective PA-targeted therapies, POPH survival outcomes after LT in our cohort were modest and may reflect the need for more aggressive therapy.
AB - Background. Portopulmonary hypertension (POPH) is the presence of pulmonary arterial (PA) hypertension in patients with portal hypertension and is associated with significant morbidity and mortality. In a cohort of POPH patients, we describe the clinical outcomes of POPH patients who underwent liver transplantation (LT). Methods. Retrospectively collected data from a prospectively assembled cohort of all consecutive POPH adults evaluated in 3 transplant centers from 1996 to 2019. Results. From a cohort of 228 POPH patients, 50 patients underwent LT. Significant hemodynamic improvement after PA-targeted therapy was observed, with 58% receiving only monotherapy pretransplant. After LT, 21 (42%) patients were able to discontinue and remained off PA-targeted therapy. The 1-, 3-, and 5-y unadjusted survival rates after LT were 72%, 63%, and 60%, respectively. An elevated pulmonary vascular resistance (PVR) before LT was associated with worse survival rate (HR, 1.91; 95% CI, 1.07-3.74, P = 0.04). No survival difference was observed in those granted MELD exception or transplants performed before or after the year 2010. Conclusions. Significant number of POPH patients discontinued PA-targeted therapy after LT. Higher PVR before LT was associated with worse survival, as was monotherapy use. Despite effective PA-targeted therapies, POPH survival outcomes after LT in our cohort were modest and may reflect the need for more aggressive therapy.
UR - http://www.scopus.com/inward/record.url?scp=85097177074&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85097177074&partnerID=8YFLogxK
U2 - 10.1097/TP.0000000000003490
DO - 10.1097/TP.0000000000003490
M3 - Article
C2 - 33065725
AN - SCOPUS:85097177074
SN - 0041-1337
VL - 105
SP - 2283
EP - 2290
JO - Transplantation
JF - Transplantation
IS - 10
ER -