The Hepatopulmonary Syndrome refers to a respiratory condition associated with chronic liver disease. The authors are from the University of Barcelona in Spain.
Criteria for the hepatopulmonary syndrome:
(1) chronic liver disease
(2) abnormal pulmonary gas exchange with severe arterial hypoxemia.
(3) evidence of intrapulmonary vascular dilatations
(4) chest radiograph is normal or shows nodular basal shadowing
(5) need to exclude other possible explanations for these findings (absence of intrinsic cardiopulmonary disease)
One way to demonstrate intrapulmonary vascular dilatations is to monitor air microbubbles injected into the right heart with 2-dimensional contrast-enhanced echocardiography.
(1) Usually the microbubbles are trapped in the pulmonary capillary bed and so no microbubbles can be detected in the left atrium.
(2) With intrapulmonary vascular dilatations microbubbles can be detected in the left atrium within 3-6 heart beats.
(3) To be valid there must not be intracardiac communications (like atrial septal defect).
Other findings:
(1) abnormal diffusing capacity DLco
(2) areas with low ventilation-perfusion (V/Q) ratios.
(3) dyspnea with or without platypnea (shortness of breath when erect) and orthodeoxia
(4) high cardiac output with low systemic arterial pressure and a normal or low pulmonary artery pressure
(5) poor hypoxic pulmonary vascular response
The prognosis is poor but may reverse after liver transplantation.
Making the diagnosis is important, since underlying cardiopulmonary disease is usually a contraindication for liver transplantation.
