Portopulmonary Hypertension: A Survey of Practice Patterns and Provider Attitudes

DuBrock, Hilary M.; Salgia, Reena; Sussman, Norman; Bartolome, Sonja; Kadry, Zakiyah; Mulligan, David C.; Jenkins, Sarah; Lackore, Kandace A.; Channick, Richard N.; Kawut, Steven M.; Krowka, Michael J.

doi:10.1097/txd.0000000000000900

Cited by 20 publications

(26 citation statements)

References 16 publications

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“…The 2017 survey of physicians at US liver transplant centers described in the Introduction found considerable heterogeneity in clinical practice and medications used to treat PoPH. 11 The authors concluded that this reflects how little is known about the optimal management of PoPH and called for more data from clinical trials and real-world experience to improve and standardize care. We believe that this need exists for all patients with PH and portal hypertension, not just those with PoPH.…”

Section: Discussionmentioning

confidence: 99%

“…A recent survey of 74 physicians at 35 liver transplant centers reported variability in provider attitudes and practice patterns across the US regarding PoPH management, highlighting the need to standardize care in patients with this dual comorbidity. 11 Understanding the current management and main cost drivers of portal hypertension in the setting of PH may identify strategies to improve quality of care. The objective of this study is to compare HCRU and costs between patients with portal hypertension and concomitant PH and patients with portal hypertension without PH.…”

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confidence: 99%

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Burden of pulmonary hypertension in patients with portal hypertension in the United States: a retrospective database study

et al. 2020

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Patients with portal hypertension may develop pulmonary hypertension (PH). The economic implications of these comorbidities have not been systematically assessed. We compared healthcare resource utilization and costs in the United States between patients with co-existing portal hypertension and PH (PH cohort) and a matched cohort of portal hypertension patients without PH (control cohort). In this retrospective analysis, adult PH and control patients were identified from the Optum® Clinformatics® Data Mart database between July 1, 2014, and June 30, 2018. All patients had ≥2 claims with diagnosis codes for portal hypertension; PH patients had ≥2 claims with diagnosis codes for PH; controls could not have PH diagnoses or any claims for pulmonary arterial hypertension-specific medications. Controls were matched to PH patients by age, sex, Charlson comorbidity index score, and liver diseases. We assessed 12-month healthcare resource utilization and costs. Each cohort included 146 patients. During follow-up, PH cohort patients were more likely than controls to experience a hospitalization (51% vs. 32%, P = 0.0014) and an emergency room visit (55% vs. 41%, P = 0.026). The average annual total cost was higher in PH patients than for matched controls ($119,912 vs. $81,839, P < 0.0001). After covariate adjustment, costs for PH cohort patients were 1.47 times higher than those for controls (P = 0.0197). These findings suggest that patients with portal hypertension and co-existing PH are at a greater risk for hospitalization and incur higher mean annual total costs than portal hypertension patients without PH.

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Section: Discussionmentioning

confidence: 99%

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Burden of pulmonary hypertension in patients with portal hypertension in the United States: a retrospective database study

et al. 2020

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“…It is possible that awareness of the POPH MELD exception has grown and that transplant teams have become more comfortable with the criteria and overall application process. Furthermore, over the past 15 y, there has been increased appreciation for the complex relationship between POPH and LT among hepatologists and PH specialists‚ 13 as well as increased understanding of the role and potential benefit of LT in the management of POPH. 14 , 15 Additionally, the general increase in exception frequency over time may be related to the shifting epidemiology in etiology of cirrhosis seen in this cohort, specifically the decline in HCV, which is associated with a decreased risk of POPH, and increase in other causes‚ including NASH.…”

Section: Discussionmentioning

confidence: 99%

Temporal Trends in Portopulmonary Hypertension Model for End-stage Liver Disease Exceptions and Outcomes

Valle

Krowka

Heimbach

et al. 2022

Transplantation Direct

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Background. Model for end-stage liver disease (MELD) exception criteria for portopulmonary hypertension (POPH) were created to prioritize patients for liver transplant before POPH progression. Little is known about trends in POPH exception frequency, disease severity, pulmonary hypertension treatment patterns, or outcomes since the POPH MELD exception began. Methods. Using data from the Organ Procurement and Transplantation Network database, we describe the frequency of POPH MELD exceptions between 2006 and 2019, compare baseline patient characteristics, and characterize trends in liver disease and POPH severity‚ as well as POPH treatment and outcomes‚ over time. To facilitate comparison, we divided this 14-y period into 3 “eras” (2006–2010, 2011–2015, and 2016–2019). Results. Between 2006 and 2019, 504 unique POPH MELD exceptions were granted. Both liver disease severity and patient age have increased over time ( P = 0.04 and P = 0.006, respectively). Posttreatment hemodynamic values (mean pulmonary arterial pressure and pulmonary vascular resistance) have significantly improved ( P < 0.001 and P = 0.008, respectively). Treatment with endothelin receptor antagonists has become more prevalent, whereas use of parenteral therapy and monotherapy regimens has decreased ( P < 0.001). Neither waitlist nor liver transplant mortality outcomes have significantly changed over the eras analyzed. Conclusions. In conclusion, 504 patients have received POPH MELD exceptions between 2006 and 2019. Since 2010, nearly all patients granted POPH MELD exceptions have met hemodynamic criteria for POPH. Over time, there has been a trend toward older age and higher MELD scores with significant changes in pulmonary arterial hypertension treatment patterns and an improvement in posttreatment hemodynamics without major change in outcomes.

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“…37,40,41,56 Because of inconsistent postoperative results, provider beliefs and attitudes on liver transplant in patients with POPH is far from unanimous, even among the experts in the field. A recent article by DuBrock et al 57 highlighted provider attitudes regarding POPH and liver transplantation at centers that performed > 50 transplants/year. The responses varied widely even among this select group (Figure 2).…”

Section: Prostacyclin Pathwaymentioning

confidence: 99%

Portopulmonary Hypertension: A Review

Ruggiero

Bartolome

2022

Advances in Pulmonary Hypertension

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Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.

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Portopulmonary Hypertension: A Survey of Practice Patterns and Provider Attitudes

Abstract: Supplemental Digital Content is available in the text.

Cited by 20 publications

References 16 publications

Burden of pulmonary hypertension in patients with portal hypertension in the United States: a retrospective database study

Burden of pulmonary hypertension in patients with portal hypertension in the United States: a retrospective database study

Temporal Trends in Portopulmonary Hypertension Model for End-stage Liver Disease Exceptions and Outcomes

Portopulmonary Hypertension: A Review

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