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Portopulmonary Hypertension: An Updated Review.

ABSTRACT: Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

SUBMITTER: Jasso-Baltazar EA

PROVIDER: S-EPMC10365198 | biostudies-literature | 2023 Aug

REPOSITORIES: biostudies-literature

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Portopulmonary Hypertension: An Updated Review.

Jasso-Baltazar Erick A EA Peña-Arellano Gonzalo A GA Aguirre-Valadez Jonathan J Ruiz Isaac I Papacristofilou-Riebeling Bruno B Jimenez Jose Victor JV García-Carrera Cristian J CJ Rivera-López Fabián E FE Rodriguez-Andoney Jesús J Lima-Lopez Francisco C FC Hernández-Oropeza José Luis JL Díaz Juan A Torres JAT Kauffman-Ortega Eric E Ruiz-Manriquez Jesus J Hernández-Reyes Pablo P Zamudio-Bautista Jorge J Rodriguez-Osorio Carlos A CA Pulido Tomás T Muñoz-Martínez Sergio S García-Juárez Ignacio I

Transplantation direct 20230721 8

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension ca ...[more]

PMID: 37492078

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Project description:BackgroundPortopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, can lead to right-sided heart failure and death. Being female is a known risk factor for POPH, but little is known about the effect of sex on clinical manifestations, hemodynamics, treatment response, and survival.Research questionWe sought to characterize sex differences in clinical characteristics, pulmonary hemodynamics, treatment response, and survival in patients with POPH.Study design and methodsWe performed a retrospective cohort study of adult candidates for liver transplant (LT) who had POPH within the Organ Procurement and Transplantation Network database. Females and males were compared. Multivariate regression was performed to assess the association between sex and pulmonary vascular resistance (PVR) and survival. Patients were also stratified by age (50 years) to determine how age modifies the relationship between sex and hemodynamics and survival.ResultsWe included 190 adults (103 male, 87 female). Compared with men, women had a lower model for end-stage liver disease (MELD) score (12.1± 4.2 vs 13.8 ± 4.9; P = .01) and were more likely to have autoimmune liver disease. Women had a higher baseline PVR (610.6 ± 366.6 vs 461.0 ± 185.3 dynes-s-cm-5; P < .001) and posttreatment PVR (244.6 ± 119.5 vs 202.0 ± 87.7 dynes-s-cm-5; P = .008) and a greater treatment response (ΔPVR) (-359.3 ± 381.9 vs -260.2 ± 177.3 dynes-s-cm-5; P = .03). In multivariate analysis, female sex (or gender) remained associated with a higher baseline PVR (P = .008). Women and men had overall similar survival (P > .05). When patients were stratified by age, being female was independently associated with worse waiting list survival after adjusting for MELD and PVR in younger patients (HR, 6.61; 95% CI, 1.25-35.08; P = .03) but not in older patients.InterpretationCompared with male candidates, female candidates for LT who had POPH had a higher PVR and lower MELD score and were more likely to have autoimmune liver disease. Women and men had similar overall survival, but female sex (or gender) was associated with worse survival in younger patients.

Project description:Portopulmonary hypertension (POPH) is a type of pulmonary arterial hypertension occurring exclusively in those with portal hypertensive liver disease. Liver transplantation (LT) can significantly improve outcomes. Current guidelines counsel against immediate adjustments to targeted therapy after LT and suggest routine echocardiography as sufficiently informative to guide therapeutic adjustments. Current practice patterns for adjusting targeted therapy after LT in POPH, and how they compare with guidelines, are not well established. To answer this question, we performed an institutional review board-approved, cross-sectional mixed-methods survey-based study of US POPH providers. Anonymized requests to complete the survey were sent through professional networks between January 20, 2022, and April 20, 2022. Responses were compared between cardiologists and pulmonologists using Fisher's exact tests, at a significance of 0.05. A total of 85 POPH physicians were included in the final analysis (66% pulmonologists and 34% cardiologists). Following LT, the majority of respondents routinely used a combination of standard cardiopulmonary assessment modalities to guide adjustment of targeted therapy following LT. Most respondents (69%) started by adjusting parenteral prostacyclins with small titrations and frequent reassessments within 3 months of LT, but some (19.7%) adjusted targeted therapy immediately after LT. Our results showed that the majority of respondents favored serial integrated cardiopulmonary testing (including routine right heart catheterization) to guide the adjustment of targeted therapy in POPH after LT, and almost one-fifth of respondents weaned therapy immediately after LT. Our study demonstrates heterogeneity in POPH practice patterns after LT, highlights differences between current practice patterns and the most recent guidelines, emphasizes the need for additional research, and supports a team-based approach to standardize care for these high-risk patients and optimize post-LT outcomes.

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Portopulmonary Hypertension: An Updated Review.

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Portopulmonary Hypertension: An Updated Review.

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