Severe pulmonary hypertension and amelioration of hepatopulmonary syndrome after liver transplantation

Authors

  • Melanie D. Kaspar,

    1. Departments of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, TX
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  • Michael A.E. Ramsay, MD,

    Corresponding author
    1. Departments of Anesthesiology and Pain Management, University of Texas Southwestern Medical Center, Dallas, TX
    • Department of Anesthesiology and Pain Management, Baylor University Medical Center, 3500 Gaston Ave, Dallas, TX 75246
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  • Charles B. Shuey Jr,

    1. Department of Pulmonary Medicine, Baylor University Medical Center, Dallas, University of Texas Southwestern Medical Center, Dallas, TX
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  • Marlon F. Levy,

    1. Department of Transplantation, Baylor University Medical Center, Dallas, University of Texas Southwestern Medical Center, Dallas, TX
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  • Goran G.B. Klintmalm

    1. Department of Transplantation, Baylor University Medical Center, Dallas, University of Texas Southwestern Medical Center, Dallas, TX
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Abstract

A patient with end-stage liver disease as a result of α1-antitripsin deficiency presented for orthotopic liver transplantation. The liver cirrhosis was complicated by portal hypertension and hepatopulmonary syndrome resulting in varicosities and severe hypoxia (room air oxygen saturation 69%). After transplantation, the hepatopulmonary syndrome improved but, over the next 14 months, the patient developed severe pulmonary hypertension. Six years posttransplantation, his room air oxygen saturation was 95% with pulmonary artery pressures of 109 mm Hg systolic and 26 mm Hg diastolic (mean 55 mm Hg) and a pulmonary vascular resistance 688 dynes·sec·cm.5

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