Portopulmonary Hypertension: The Interplay Between the Liver and Pulmonary Arteries.

Heart Lung Circ

Heart Transplant Unit, St Vincent's Hospital, Sydney, NSW, Australia; School of Medicine, University of New South Wales, Sydney, NSW, Australia.

Published: September 2022

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http://dx.doi.org/10.1016/j.hlc.2022.07.010DOI Listing

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Portopulmonary hypertension (POPH), a form of pulmonary artery hypertension, occurs because of portal hypertension. Pulmonary hemodynamics and right heart function are key entities that are considered when diagnosing and treating POPH. Medical management, combined with liver transplantation, offers a unique opportunity of POPH "cure," however transplant risk can be significant as it relates to the severity of POPH.

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Article Synopsis
  • * She had a splenectomy to address her thrombocytopenia caused by an enlarged spleen (hypersplenism), but nine months later, she experienced exertional dyspnea and was diagnosed with portopulmonary hypertension (PoPH).
  • * Treatment with the medication macitentan improved her symptoms and right heart pressure readings, marking a rare case of PoPH arising after splenectomy.
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: The prevalence of portopulmonary hypertension (PoPH) is relatively low; however, its presence significantly worsens patients' prognosis. When diagnosed, PoPH can be effectively treated, and specific therapies can lead to a substantial reduction in pulmonary circulation pressure, facilitating the safe performance of liver transplantation. Echocardiography is recommended as a first-line method for the non-invasive diagnosis of pulmonary hypertension and serves as a valuable screening tool for patients being evaluated for liver transplantation (LT).

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Cardiovascular complications are common in patients with severe liver disease and are an important cause of peri-operative and post-transplant morbidity and mortality. Cirrhotic cardiomyopathy (CCM), often found in advanced liver disease, is characterized by diastolic dysfunction, systolic dysfunction, and electrophysiological abnormalities. While CCM may not cause symptoms at rest, it can become evident during stressful activities, such as surgery.

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Objective: To determine the prevalence of portopulmonary hypertension in patients referred for liver transplant evaluation.

Methods: Medical records were reviewed for 986 consecutive patients referred for liver transplant evaluation who were screened for pulmonary hypertension with echocardiography from February 1, 2021, to January 31, 2022, across 3 liver transplant centers.

Results: Of 934 patients eligible for analysis, mean (SD) age was 57 (11) years, 558 (59.

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