Abstract Hepatopulmonary syndrome (HPS) is an underrecognized pulmonary vascular complication of chronic liver disease, defined by hepatic dysfunction, intrapulmonary vascular dilatation, and arterial hypoxemia. The condition leads to impaired oxygenation in the absence of intrinsic cardiopulmonary pathology. Recognition is vital, as HPS significantly increases morbidity and mortality among cirrhotic patients, and liver transplantation remains the only curative treatment. A 68-year-old man with untreated hepatitis C presented with one day of mild left-sided chest discomfort and a five-year history of progressive dyspnea, worsening over six months. Dyspnea was aggravated when upright or bending forward but improved when supine, consistent with platypnea-orthodeoxia. He reported a 50-pound unintentional weight loss, fatigue, and chronic watery diarrhea. On arrival, oxygen saturation was 66 % on room air, improving to 91 % with a non-rebreather mask. Physical examination revealed cyanotic lips and cheeks and bilateral lower-extremity edema without digital clubbing or stigmata of chronic liver disease.CT angiography of the chest, abdomen, and pelvis excluded pulmonary embolism and aortic disease but showed cirrhotic morphology with splenomegaly, varices, and portal hypertension. Liver enzymes were mildly elevated (AST ALT) with normal bilirubin and mild thrombocytopenia. Transthoracic echocardiography with agitated-saline contrast demonstrated delayed microbubble appearance in the left atrium, consistent with an intrapulmonary shunt. Right and left heart catheterization showed normal pressures (RA 0 mm Hg, PA 19/3 mm Hg mean 9 mm Hg, PCWP 5 mm Hg, LVEDP 3 mm Hg), preserved cardiac output/index (8.83 L/min, 4.11 L/min/m²), and no step-up in oxygen saturations, excluding an intracardiac shunt.An arterial blood gas obtained after 20 minutes on room air revealed a PaO2 of 47 mm Hg and an alveolar-arterial gradient of 71.5 mm Hg (expected ≈ 21 mm Hg), confirming severe hypoxemia out of proportion to pulmonary findings. In the context of chronic liver disease and intrapulmonary shunting, a diagnosis of hepatopulmonary syndrome was established. The patient was transferred for liver-transplant evaluation.HPS occurs in 10-30 % of patients with cirrhosis and manifests with positional hypoxemia from pulmonary vasodilatation and diffusion-perfusion impairment. Diagnosis relies on contrast echocardiography and arterial blood gas analysis, with right heart catheterization excluding intracardiac causes. Supportive oxygen therapy is essential, but liver transplantation is the only definitive treatment and often leads to resolution of hypoxemia within one year. This case highlights the importance of recognizing HPS in cirrhotic patients with unexplained hypoxemia to ensure timely diagnosis and transplant referral. This abstract is funded by: None
Alachraf et al. (Fri,) studied this question.