Clinical manifestations of hepatopulmonary syndrome

Abstract

Objective: Hepatopulmonary syndrome (HPS) occurs in one third of patients with decompensated cirrhosis and is defined by perturbed gas exchanges, arterial hypoxemia, and vasodilatation in the lungs, in the absence of primary cardiopulmonary disease. Respiratory symptoms are severe when Pa O2 levels are <60 mm Hg: centrally-induced cyanosis, dyspnea, hippocratic fingers, platypnea, and orthodeoxia. Portopulmonary hypertension (PPH) occurs in 2% of cirrhotic patients and it is suspected in hypoxemic patients presenting no pulmonary vasodilatation. It is clinically manifested by stress dyspnea, orthopnea, thoracic pain, fatigue, and dizziness. Systolic blood pressure in pulmonary artery is over 25 mm Hg and pulmonary capillary blood pressure is blocked below 15 mm Hg. Methods: A five-year (2008–2012) retrospective evaluation of pulmonary pathology occurring with hepatic cirrhosis showed 50 patients presenting dyspnea, a symptom suggesting pulmonary involvement. Echocardiography monitored the following parameters: pulmonary artery acceleration time, measured in the parsternal window; pulmonary artery systolic blood pressure was measured by means of tricuspid regurgitation method and right-atrium blood pressure was evaluated according to inferior vena cava collapse with inspiration. Results: The 50 patients in the study group presented dyspnea as a symptom suggestive of pulmonary involvement. Platypnea and orthodeoxia were present in 12% of the cases. In 88% of the cases, dyspnea did not display particular characteristics; it became stronger under stress conditions and in dorsal decubitus position, according to the pathology associated with hepatic cirrhosis. Patients’ average age was 52. There was no constant correlation between hepatic dysfunction biochemical markers and degree of dyspnea. Severe hypoxemia (SaO2 < 80%) was present in 12% of the cases. Pulmonary artery systolic blood pressure was higher in patients with portal hypertension, with measurements exceeding 35 mm Hg in 60% of the cases. Right-atrium blood pressure, evaluated according to IVC collapse with inspiration, was high (11 ± 4 mm Hg) in 62% of the cases and normal (5 mm Hg) in 36%. We considered that pulmonary hypertension associated with portal hypertension in the study group was caused by hyperdynamic status and it was not portopulmonary hypertension proper. Conclusions: Hepatopulmonary syndrome should be used among the diagnostic variants for a patient with hepatic involvement and/or portal hypertension, in whom significant hypoxemia is observed, which has no restrictive basis and is not justified by any co-occurring cardiopulmonary condition. Platypnea and orthodeoxia in a hepatic condition developing with portal hypertension increase the possibility of HPS suspicion. The only definitive and efficient treatment in hepatopulmonary syndrome is liver transplantation.