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Related Experiment Video

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Invasive Hemodynamic Characterization of the Portal-hypertensive Syndrome in Cirrhotic Rats
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Impaired cardiovascular function in primary biliary cirrhosis.

David E J Jones1, Kieren Hollingsworth, Gulnar Fattakhova

  • 1Institute of Cellular Medicine, Newcastle Univ., UK.

American Journal of Physiology. Gastrointestinal and Liver Physiology
|February 6, 2010
PubMed
Summary

Primary biliary cirrhosis (PBC) patients show impaired cardiac function and reduced cardiac energy reserves, suggesting myocardial dysfunction rather than autonomic dysfunction. Fatigue severity correlates with decreased cardiac output during orthostasis in PBC.

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Area of Science:

  • Cardiology
  • Hepatology
  • Autonomic Neuroscience

Background:

  • Autonomic dysfunction is prevalent in primary biliary cirrhosis (PBC) and linked to fatigue.
  • The underlying mechanisms of autonomic dysfunction in PBC remain unclear.
  • This study investigates cardiac structure and function in PBC patients.

Purpose of the Study:

  • To explore cardiac structure and function in primary biliary cirrhosis (PBC) patients.
  • To investigate the relationship between cardiac function, bioenergetics, and fatigue in PBC.
  • To differentiate between primary cardiac dysfunction and autonomic dysfunction in PBC.

Main Methods:

  • Impedance cardiography (ICG) assessed beat-to-beat cardiac response to orthostasis (head-up tilt) in PBC patients (high/low fatigue), controls, and primary sclerosing cholangitis patients.
  • Magnetic resonance spectroscopy and cine imaging examined cardiac structure and bioenergetics (phosphocreatine-to-ATP ratio) in a subset of PBC patients and controls.
  • Fatigue Impact Scale assessed fatigue severity.

Main Results:

  • Left ventricular ejection time (LVET) response to orthostasis was impaired in PBC patients, independent of fatigue.
  • PBC patients had significantly lower cardiac muscle phosphocreatine-to-ATP (PCr/ATP) ratios, indicating impaired bioenergetic integrity.
  • 40% of PBC patients had PCr/ATP <1.6, a marker of increased cardiomyopathy risk.
  • The correlation between PCr/ATP and LVET was lost in PBC patients, suggesting myocardial dysfunction.
  • Fatigue severity in PBC correlated with a fall in cardiac output during orthostasis.

Conclusions:

  • Primary biliary cirrhosis is associated with altered myocardial function and impaired cardiac bioenergetics.
  • Autonomic "dysfunction" in PBC may be a compensatory mechanism for underlying myocardial issues.
  • These findings highlight potential cardiac involvement in the pathophysiology of PBC and its symptoms.