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… Show more
“…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.…”
Section: Right Heart Catheterization Hemodynamics Interpretation In Pophmentioning
confidence: 99%
“…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).…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
confidence: 99%
“…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).…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
confidence: 99%
“…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.…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
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.
“…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.…”
Section: Right Heart Catheterization Hemodynamics Interpretation In Pophmentioning
confidence: 99%
“…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).…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
confidence: 99%
“…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).…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
confidence: 99%
“…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.…”
Section: Challenges In the Diagnosis And Management Of Pophmentioning
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.
“…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].…”
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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