Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT‐1/‐2 studies

Portopulmonary hypertension (PoPH) is a form of pulmonary arterial hypertension (PAH) that can develop as complication of portal hypertension. Treatment of PoPH includes PAH-specific therapies and in certain cases, such therapies are necessary to facilitate a successful liver transplantation. A significant number of barriers may limit the adequate treatment of patients with PoPH and explain the poorer survival of these patients when compared to other types of PAH. Until recently, only one randomized controlled trial has included PoPH patients and the majority of treatment data is derived from relatively small observational studies. In the present manuscript we review some of the barriers in the treatment of patients with PoPH and implications for liver transplantation. This article is protected by copyright. All rights reserved.

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“…In addition, one patient died of sepsis related to bronchopneumonia, an event not ascribed to the study drug. (40)…”

Section: Soluble Guanylate Cyclase Stimulatormentioning

confidence: 99%

“…Riociguat appeared to improve functional and hemodynamic determinations in patients with PoPH; however, some patients experienced side effects (headaches (n=3) and peripheral edema (n=3)). In addition, one patient died of sepsis related to bronchopneumonia, an event not ascribed to the study drug (40).…”

Section: Introductionmentioning

confidence: 99%

Treatment Barriers in Portopulmonary Hypertension

2018

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“…PATENT-2 showed sustained improvements in functional class and 6MWD (71). A recently published subgroup analysis of the 13 patients with PoPH who were enrolled in PATENT-1 and 2 showed similar improvements in WHO functional class, 6MWD and PVR (72). Importantly, riociguat was well-tolerated by the PoPH patients.…”

Section: Treatment Of Pophmentioning

confidence: 76%

Portopulmonary Hypertension: From Bench to Bedside

et al. 2020

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Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) associated with portal hypertension and is a subset of Group 1 pulmonary hypertension (PH). PoPH is a cause of significant morbidity and mortality in patients with portal hypertension with or without liver disease. Significant strides in elucidating the pathogenesis, effective screening algorithms, accurate diagnoses, and treatment options have been made in past 20 years. Survival of PoPH has remained poor compared to IPAH and other forms of PAH. Recently, the first randomized controlled trial was done in this patient population and showed promising results with PAH specific therapy. Despite positive effects on hemodynamics and functional outcomes, it is unclear whether PAH specific therapy has a beneficial effect on long term survival or transplant outcomes. In this review, we will discuss the epidemiology, pathophysiology, clinical and hemodynamic characteristics of PoPH. Additionally, this review will highlight the lacunae in our current management strategy, challenges faced and will provide direction to potentially useful futuristic management strategies.

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“…For the first time, 13 patients with PoPH were included in the PATENT-1 trial with riociguat, a soluble guanylate-cyclase stimulator. Subgroup analysis was performed for patients with PοPH and showed that riociguat was well-tolerated and significantly improved World Health Organization functional class and the 6-min walking test after 12 weeks of treatment, while functional improvement was sustained for 2 years [103].…”

Section: Treatment and Prognosismentioning

confidence: 99%

Pulmonary manifestations of chronic liver disease: a comprehensive review

Σουλαϊδόπουλος

Goulis

2020

aog

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Hepatopulmonary syndrome (HPS) and porto-pulmonary hypertension (PoPH) represent relatively common pulmonary vascular complications of advanced liver disease. Despite distinct differences in their pathogenetic background, both clinical states are characterized by impaired arterial oxygenation and limited functional status, and are associated with increased pretransplantation mortality. Accumulation of ascitic fluid in the pleural cavity, known as hepatic hydrothorax (HH), is another frequent manifestation of decompensated cirrhosis, which may cause severe respiratory dysfunction, depending on the volume of the effusion, the rapidity of its development and its resistance to therapeutic measures. Orthotopic liver transplantation constitutes the only effective treatment able to resolve the pulmonary complications of liver disease. A prioritization policy for liver transplantation has evolved over the past years regarding advanced stages of HPS, yielding favorable outcomes regarding post-transplantation survival and HPS resolution. In contrast, severe PoPH is associated with poor post-transplantation survival. Hence, liver transplantation is recommended only for patients with PoPH and an acceptable reduction in pulmonary pressure values, after receiving PoPH-targeted vasodilating therapy. This review focuses on basic pathogenetic and diagnostic principles and discusses the current therapeutic approaches regarding HPS, PoPH, and HH.

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Riociguat treatment for portopulmonary hypertension: a subgroup analysis from the PATENT‐1/‐2 studies

Cited by 36 publications

References 20 publications

Treatment Barriers in Portopulmonary Hypertension

Treatment Barriers in Portopulmonary Hypertension

Portopulmonary Hypertension: From Bench to Bedside

Pulmonary manifestations of chronic liver disease: a comprehensive review

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