Severe Hypoxemia in a Young Patient: Unusual Presentation of Cryptogenic Cirrhosis with Hepatopulmonary Syndrome
Abstract
Cirrhosis can cause changes in micro-vasculature of any bodily system; when it involves pulmonary arteries, impaired gas exchange takes place due to increased production of vasodilatory substances like nitric oxide, vessel remodeling and angiogenesis, resulting in hypoxemia. We are reporting a case of a 35-year-old woman who presented to emergency department with bluish discoloration of her fingertips and exertional dyspnea, with no known prior liver disease. Examination revealed SpO levels of 60% via pulse oximetry at room air, palmar erythema, digital clubbing and 2 splenomegaly. Further evaluation revealed an A-a gradient of 66mmHg and contrast echocardiography confirmed intra pulmonary shunt, but no etiology of chronic liver disease was identified. Imaging confirmed cirrhosis, which was labelled cryptogenic, along with evidence for portal hypertension. She was diagnosed with Hepatopulmonary Syndrome (HPS) and was referred to liver transplant medicine based on MELD exception criteria. This case signifies the importance of considering HPS in any patient with unexplained hypoxemia and suspected chronic liver disease. Importance of MELD exception criteria is also highlighted for this patient as liver transplant remains the only treatment for HPS at the moment.Keywords:
Hepatopulmonary Syndrome, Cirrhosis, Hypoxemia, Contrast Echocardiography, MELD Exception, Liver Transplantation, Cryptogenic cirrhosisPublished
2025/08/23
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