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http://dx.doi.org/10.1164/ajrccm.180.4.378DOI Listing

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Background: Liver transplantation (LTx) is vital in patients with end-stage liver disease, with metabolic dysfunction-associated steatotic liver disease being the most common indication. Primary sclerosing cholangitis (PSC) is an important indication. Portopulmonary hypertension, associated with portal hypertension, poses a significant perioperative risk, making pretransplant screening essential.

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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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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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