Publications (3)22.39 Total impact
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ABSTRACT: The hepatopulmonary syndrome (HPS) occurs in as many as 15–20% of patients with cirrhosis; mortality is significantly increased compared to cirrhotic patients without HPS. The only proven effective therapy for HPS is orthotopic liver transplantation (OLT), which should be considered when severe hypoxemia is present. The natural history of HPS without liver transplantation is dismal. While post-OLT mortality is increased in patients with HPS relative to that reported in non-HPS patients, overall outcomes are favorable in properly selected patients. The higher mortality associated with HPS has led to the policy of increasing priority for OLT in selected HPS patients through a Model for End-Stage Liver Disease (MELD) score exception. There is currently no established protocol to screen for HPS in OLT candidates. However, a resting PaO2 < 65–60 mmHg identifies patients who qualify, or who may sufficiently deteriorate over a short time frame to qualify, for MELD exception criteria. In patients with HPS awaiting OLT, no specific therapies are available to improve intrapulmonary vasodilatation. The perioperative management of HPS patients presents particular clinical challenges. Key WordsHepatopulmonary syndrome–Hypoxia–Intrapulmonary shunt–Pulmonary disease
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ABSTRACT: Sleep alters respiratory mechanics and gas exchange, which can adversely affect arterial oxygenation. Whether sleep affects oxygenation in hepatopulmonary syndrome is unknown. The aim of this study was to assess oxygen desaturation during sleep in hepatopulmonary syndrome. Twenty adults with cirrhosis including 10 controls and 10 patients with hepatopulmonary syndrome underwent home pulse-oximetry during sleep. Subjects at high risk for obstructive sleep apnea were excluded through the Berlin questionnaire. Subjects who spent more than 10% of total sleep time with arterial oxygen saturation < 90% were classified as sleep-time oxygen desaturators. Sleep-time desaturation was correlated with clinical variables. The results showed that 7 of 10 hepatopulmonary syndrome subjects and none of the 10 controls had sleep-time oxygen desaturation. The median percentage of total sleep time with arterial oxygen saturation < 90% was significantly higher in hepatopulmonary syndrome subjects than in controls (medians 25% versus 0%, P = 0.005). Hepatopulmonary syndrome subjects had significantly lower wake-time arterial oxygen saturation level (median, 97% versus 95%; P = 0.003) and mean sleep-time arterial oxygen saturation level (median, 96% versus 91%; P = 0.0008) than did the controls. Sleep-time desaturation directly correlated with alveolar-arterial oxygen gradient (P = 0.0007) and inversely correlated with wake-time arterial oxygen tension (P = 0.0007) and oxygen saturation (P < 0.0001). Conclusion: Oxygen desaturation occurred during sleep in 70% of hepatopulmonary syndrome subjects, the degree of which correlated with the severity of hepatopulmonary syndrome. Marked hypoxemia during sleep may occur in hepatopulmonary syndrome patients who, according to wake-time oxygen values, have only mild to moderate hypoxemia.
Article: Hepatopulmonary Syndrome[Show abstract] [Hide abstract]
ABSTRACT: Purpose of review: To discuss the advances in the understanding of the pathophysiology of experimental and human hepatopulmonary syndrome (HPS) and in the management of HPS, particularly regarding liver transplantation. Recent findings: Advances have been made in defining the pathophysiology of HPS in experimental models as well as in human disease, including the role of endothelin-1, pulmonary monocytes, and angiogenesis. Additionally, the implications of the presence of HPS as it relates to prioritizing patients for liver transplantation and posttransplant outcomes will also be reviewed. Summary: Mechanisms of disease continue to be defined in HPS, providing potential targets for pharmacologic intervention. Outcomes after liver transplantation are also becoming clearer, including the management of HPS with severe hypoxemia.
University of Alabama at Birmingham
Birmingham, Alabama, United States
- Department of Medicine