Treatment Barriers in Portopulmonary Hypertension

Abstract

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.

Portopulmonary Hypertension: From Bench to Bedside

“…Volume overload is managed with careful diuresis, as patients with liver disease are prone to kidney injury (64). A hyperdynamic state can be intrinsic to liver disease, but may also be due to other treatable medical comorbidities (such as anemia, arteriovenous shunts, and hyperthyroidism) (65)(66)(67). This common clinical scenario speaks to the challenge of diagnosing and treating PoPH, which is best done at expert centers which are most familiar with these complicated patients.…”

“…There is only one randomized controlled trial for PAH specific therapy in patients with PoPH. Advanced liver disease symptoms have significant overlap with the side effects of PAH specific therapies (e.g., nausea, fatigue, and fluid overload), which can make the initiation and maintenance of treatment challenging (67).…”

“…Many of the drugs used to treat PoPH are metabolized in the liver, and drug metabolism is altered by liver disease and other medications that patients may be taking (67). Additionally, patients may have hepatic encephalopathy or lack the social support needed to safely administer parenteral therapy or take medications multiple times per day (67).…”

“…Many of the drugs used to treat PoPH are metabolized in the liver, and drug metabolism is altered by liver disease and other medications that patients may be taking (67). Additionally, patients may have hepatic encephalopathy or lack the social support needed to safely administer parenteral therapy or take medications multiple times per day (67). More generally, many of the treatment barriers that apply to all patients with PAH apply to patients with PoPH: late referrals, lack of an expert center in close proximity to where the patient lives, and insurance issues.…”

Pulmonary manifestations of chronic liver disease: a comprehensive review

“…In the absence of specific guidelines, the treatment of PoPH needs to be individualized. Special characteristics and altered hemodynamics due to the underlying liver disease compared to PAH should be taken into consideration in the choice of the most appropriate medication [113]. Available data suggest that oral pulmonary vasoactive drugs are safe in cirrhotic patients and their positive effect on pulmonary hemodynamics is independent from the severity of the underlying liver disease, assessed by the Child-Pugh and MELD scores [114].…”