The Myths and Realities of Portopulmonary Hypertension

DuBrock, Hilary M.; Krowka, Michael J.

doi:10.1002/hep.31415

Cited by 16 publications

(7 citation statements)

References 31 publications

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“…Prior studies have described 1-year posttransplant survival rates of 63%–92% in POPH. 8 - 14 The 1-year posttransplant survival of 69% in patients with POPH and an elevated mPAP in our cohort is roughly similar to these prior estimates and higher than the 50% survival reported in older studies for patients with predominantly untreated POPH and an elevated mPAP ≥ 35 mm Hg. 2 Long-term posttransplant survival rates are also overall similar to our recently reported survival rates for POPH patients at Mayo Clinic (n = 50) (1-y survival 72%, 3-y survival 63%, 5-y survival 60%).…”

Section: Discussionsupporting

confidence: 87%

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

DuBrock

Runo

Sadd

et al. 2020

Transplantation Direct

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Background. Portopulmonary hypertension (POPH), pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension, affects 5%–6% of patients with liver disease and is associated with significant morbidity and mortality. A mean pulmonary arterial pressure (mPAP) threshold of 35 mm Hg is used to stratify perioperative risk and liver transplant eligibility in treated POPH patients but does not take into account the specific factors that contribute to the pressure elevation. Methods. In this case series, we describe the characteristics and posttransplant outcomes of patients with treated POPH and an mPAP ≥35 mm Hg and pulmonary vascular resistance (PVR) <250 dynes-s-cm −5 at or just before liver transplantation (LT). We also describe the effect of PAH therapy on pulmonary hemodynamics in patients with POPH. Results. Sixteen patients were included. All patients were on PAH therapy at the time of LT. PAH therapy resulted in a decrease of mPAP (median 18.4%; interquartile range [IQR] 8.9%–27.0%) with a reduction in PVR (median 50.5%; IQR, 45.4%–70.7%), and an increase in both cardiac output (CO) (median 28.1%; IQR 5.7%–63.8%) and PAWP (median 50.0%; IQR 16.7%–108.3%) before LT. One year posttransplant survival was 69% (11/16); however, only 1 death was attributed to POPH. At 1-year posttransplant, 63.6% (7/11) of patients were weaned off all PAH therapy with clinical and echocardiographic resolution of POPH. Conclusions. In treated POPH patients with an mPAP ≥35 mm Hg and PVR < 250 dynes-s-cm −5 before LT, 1-year posttransplant survival was 69% and the majority of patients were able to discontinue PAH therapy.

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Section: Discussionsupporting

confidence: 87%

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

DuBrock

Runo

Sadd

et al. 2020

Transplantation Direct

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“…Unpalliated portal hypertension from cirrhosis also increases the hepatic resistance to portal blood flow, driving the formation of portosystemic collaterals which enables abnormal shunting of portal blood directly to the systemic circulation, effectively bypassing the liver. The effects of this abnormal blood flow are wide ranging and can lead to various extrahepatic physical manifestations including hepatic encephalopathy (HE), 16 hepatopulmonary syndrome, 30 portopulmonary hypertension, 31 and cirrhotic cardiomyopathy. 32 A more common, and potentially devastating complication of cirrhosis and portal hypertension is the presence of esophageal varices which are well established to negatively impact clinical outcomes 33 and increase the risk for decompensation and mortality.…”

Section: Dynamic Evolution and Clinical Manifestations Of End-stage Liver Diseasementioning

confidence: 99%

The Inside-Out of End-Stage Liver Disease: Hepatocytes are the Keystone

et al. 2021

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Chronic liver injury results in cirrhosis and end-stage liver disease (ESLD) which represents a leading cause of death worldwide, affecting people in their most productive years of life. Medical therapy can extend life, but the only definitive treatment is liver transplantation (LT). However, LT remains limited by access to quality donor organs and suboptimal long-term outcomes. The degeneration from healthy-functioning livers to cirrhosis and ESLD involves a dynamic process of hepatocyte damage, diminished hepatic function, and adaptation. However, the mechanisms responsible for deterioration of hepatocyte function and ultimately hepatic failure in man are poorly understood. We review the current understanding of cirrhosis and ESLD as a dynamic process and outline the current mechanisms associated with the development of hepatic failure from the clinical manifestations to energy adaptations, regeneration, and regulation of nuclear transcription factors. A new generation of therapeutics could target stabilization of hepatocyte differentiation and function to avoid the need for transplantation in patients with cirrhosis and ESLD.

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“…Outcomes remain unpredictable. (1) Despite attaining these goals with the current buffet of pulmonary artery (PA)-targeted therapies, the risks of LT remain significant. Hepatectomy may be complicated by severe bleeding with the transfusion of blood products stressing an already impaired RV.…”

Section: See Article On Page 760mentioning

confidence: 99%

ECMO and POPH: More Help for the Right Heart

Findlay¹,

Krowka²

2020

Liver Transpl.

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The decision to offer liver transplantation (LT) in the presence of POPH is dependent upon improving pulmonary hemodynamics from baseline, as determined by right heart catheterization, and trying to optimize function of the right ventricle (RV) prior to surgery. Outcomes remain unpredictable 1. Despite attaining these goals with the current buffet of pulmonary artery (PA) targeted therapies, the risks of LT remain significant. Hepatectomy may be complicated by severe bleeding with the transfusion of blood products stressing an already impaired right ventricle. Reperfusion of the

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The Myths and Realities of Portopulmonary Hypertension

Cited by 16 publications

References 31 publications

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg

The Inside-Out of End-Stage Liver Disease: Hepatocytes are the Keystone

ECMO and POPH: More Help for the Right Heart

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