Clinical Outcomes After Liver Transplantation in Patients With Portopulmonary Hypertension

Cartin‐Ceba, Rodrigo; Burger, Charles D.; Swanson, Karen L.; Vargas, Hugo E.; Aqel, Bashar; Keaveny, Andrew P.; Heimbach, Julie K.; Taner, Timuçin; Nyberg, Scott L.; Rosen, Charles B.; Cajigas, Hector; DuBrock, Hilary M.; Krowka, Michael J.

doi:10.1097/tp.0000000000003490

Cited by 34 publications

(31 citation statements)

References 29 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“… 2 Long-term posttransplant survival rates are also overall similar to our recently reported survival rates for POPH patients at Mayo Clinic (n = 50) (1-y survival 72%, 3-y survival 63%, 5-y survival 60%). 15 Additionally, in contrast to older studies, acute perioperative cardiopulmonary mortality was low, and no patients died of POPH or right heart failure in the last decade. Our previously reported experience in patients without known POPH and an mPAP ≥ 35 mm Hg at the time of transplant has also been favorable with 96% (24/25) survival at 1-year posttransplant.…”

Section: Discussionmentioning

confidence: 93%

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

DuBrock

Runo

Sadd

et al. 2020

Transplantation Direct

Self Cite

View full text Add to dashboard Cite

Background. Portopulmonary hypertension (POPH), pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension, affects 5%–6% of patients with liver disease and is associated with significant morbidity and mortality. A mean pulmonary arterial pressure (mPAP) threshold of 35 mm Hg is used to stratify perioperative risk and liver transplant eligibility in treated POPH patients but does not take into account the specific factors that contribute to the pressure elevation. Methods. In this case series, we describe the characteristics and posttransplant outcomes of patients with treated POPH and an mPAP ≥35 mm Hg and pulmonary vascular resistance (PVR) <250 dynes-s-cm −5 at or just before liver transplantation (LT). We also describe the effect of PAH therapy on pulmonary hemodynamics in patients with POPH. Results. Sixteen patients were included. All patients were on PAH therapy at the time of LT. PAH therapy resulted in a decrease of mPAP (median 18.4%; interquartile range [IQR] 8.9%–27.0%) with a reduction in PVR (median 50.5%; IQR, 45.4%–70.7%), and an increase in both cardiac output (CO) (median 28.1%; IQR 5.7%–63.8%) and PAWP (median 50.0%; IQR 16.7%–108.3%) before LT. One year posttransplant survival was 69% (11/16); however, only 1 death was attributed to POPH. At 1-year posttransplant, 63.6% (7/11) of patients were weaned off all PAH therapy with clinical and echocardiographic resolution of POPH. Conclusions. In treated POPH patients with an mPAP ≥35 mm Hg and PVR < 250 dynes-s-cm −5 before LT, 1-year posttransplant survival was 69% and the majority of patients were able to discontinue PAH therapy.

show abstract

Section: Discussionmentioning

confidence: 93%

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

DuBrock

Runo

Sadd

et al. 2020

Transplantation Direct

Self Cite

View full text Add to dashboard Cite

show abstract

“…An elevated PVR before LT was associated with worse survival rate (HR, 1.91), again emphasizing the importance of RV afterload on outcome. 51 By comparison, retrospective review of a large (637 patients) French cohort demonstrated 1-, 3-, and 5-year survival rates after LT of 84%, 69%, and 51%, respectively. 52 Most patients (90%) received PAH-specific therapy with improved NYHA functional class, 6MWD, and PVR.…”

Section: Special Populations and Topicsmentioning

confidence: 99%

“…Clinical outcomes for patients with POPH were examined in pooled data from all 3 Mayo Clinic LT centers. 51 Of 228 patients with POPH, 50 underwent LT. Significant hemodynamic improvement was observed after PAH-targeted therapy, and after LT, 21 (42%) patients were able to discontinue and to remain off PAH-targeted therapy.…”

Section: Special Populations and Topicsmentioning

confidence: 99%

“…Survival in the subgroup receiving LT was significantly better, with 1-, 3-, and 5-year survival rates of 92%, 83%, and 81%, respectively, more favorable than the Mayo Clinic series. 51,52 A single-center experience of 24 POPH patients with excellent outcomes was published by the University of Wisconsin group. 53 All patients were optimized on medical therapy with 1-, 3-, and 5-year survival rates of 96%, 91%, and 91% in the cohort that underwent transplant.…”

Section: Special Populations and Topicsmentioning

confidence: 99%

See 1 more Smart Citation

Topic-Based, Recent Literature Review on Pulmonary Hypertension

Burger

DuBrock

Cartin‐Ceba

et al. 2021

Mayo Clinic Proceedings

Self Cite

View full text Add to dashboard Cite

Pulmonary hypertension is a complex condition but a relatively common manifestation of severe cardiopulmonary disease. By contrast, pulmonary arterial hypertension is uncommon and is more prevalent in young women. To better categorize patients and to guide clinical decision-making, 5 diagnostic groups and associated subgroups characterize the spectrum of disease. A multidisciplinary approach to evaluation and treatment is recommended by published guidelines and often entails referral to a designated pulmonary hypertension center. Several key publications during the last couple of years merit review. The PubMed database was searched for English-language studies and guidelines relating to pulmonary hypertension. The following terms were searched, alone and in combination: pulmonary hypertension, pulmonary arterial hypertension, portopulmonary hypertension, and chronic thromboembolic pulmonary hypertension. The focus was on those publications with new information on evaluation and management of pulmonary hypertension between January 1, 2019, and January 31, 2021. Of the subgroups, 2 were of particular interest for this review: portopulmonary hypertension and chronic thromboembolic pulmonary hypertension. Last, available data on the impact of the coronavirus disease 2019 pandemic and newer treatment agents in early trials were selectively reviewed. The review is therefore intended to serve as a practical, focused review of important topics germane to those clinicians caring for patients with pulmonary hypertension.pulmonary hypertension, pulmonary arterial hypertension, right ventricular failure

show abstract

“…Patients with POPH are suspected of having higher rates of mortality and graft failure following LT, but the precise impact of POPH and pretransplant hemodynamics on these important posttransplant outcomes is not well described. (19)(20)(21)(22) The purpose of this study is to better define the impact of POPH and pretransplant hemodynamics on posttransplant mortality and graft failure and identify hemodynamic thresholds that promote optimal outcomes in patients with POPH undergoing LT.…”

mentioning

confidence: 99%

Pulmonary Vascular Resistance Predicts Mortality and Graft Failure in Transplantation Patients With Portopulmonary Hypertension

et al. 2021

View full text Add to dashboard Cite

Earn MOC for this article: www.wileyhealthlearning.com/aasld.aspx Portopulmonary hypertension (POPH) is a pulmonary vascular disease associated with significant morbidity and mortality in those with liver disease, conferring a higher mortality in patients awaiting liver transplantation (LT). Although not a transplant indication, patients with POPH can experience significant clinical improvement following LT, and those maintaining a mean pulmonary artery pressure (MPAP) <35mm Hg and a pulmonary vascular resistance (PVR) <5 Woods units (WU) are granted additional listing points to expedite LT. The effect of POPH on posttransplant outcomes such as mortality and graft failure, however, is not well defined. We performed a retrospective cohort study of the US Organ Procurement and Transplantation Network database of all adult patients who underwent LT between January 1, 2006, and December 1, 2020. Using adjusted accelerated failure time models, we examined the relationship between a diagnosis of POPH and outcomes following LT and the relationship between pre-LT hemodynamics and post-LT survival (alive with a functioning graft) in patients with POPH. Compared with those undergoing transplants without exception points, patients with POPH had comparable post-LT survival rates but were significantly more likely to have graft failure. Both pre-LT MPAP and PVR predicted post-LT survival in POPH, with a pre-LT PVR of ≥1.6 WU, more than doubling the hazard for mortality (death or a nonfunctioning graft; coefficient, 2.01; standard error, 0.85; hazard ratio, 2.21; P = 0.02). POPH may confer a significantly higher risk of post-LT graft failure compared with patients with cirrhosis without POPH, and a pre-LT PVR of ≥1.6 WU may predict post-LT survival. Further investigation into the relationship between pre-LT hemodynamics, right ventricular function, and post-LT outcomes of mortality and graft failure in POPH is needed.

show abstract

Clinical Outcomes After Liver Transplantation in Patients With Portopulmonary Hypertension

Cited by 34 publications

References 29 publications

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

Topic-Based, Recent Literature Review on Pulmonary Hypertension

Pulmonary Vascular Resistance Predicts Mortality and Graft Failure in Transplantation Patients With Portopulmonary Hypertension

Contact Info

Product

Resources

About