Abstract Hepatopulmonary syndrome (HPS) is a triad of liver disease, hypoxemia, and pulmonary vasodilation. Most patients with clinically significant HPS will have signs and symptoms of chronic liver disease. Definitive treatment is liver transplantation. A 65-year-old female presented for dyspnea. She reported using two liters of oxygen, a history of heart failure, prior hepatitis B, and chronic cough. On admission she was hypoxic with significant lower extremity swelling, started on non-invasive ventilation. Initially congestive heart failure was suspected, and the patient was diuresed. AST and ALT were elevated at 151 U/L and 70 U/L, respectively. A hepatitis panel identified active hepatitis C, with a viral load of 637,025 IU/mL. CT of the abdomen noted fatty infiltration of the liver. Despite over a 20 liter net output from diuresis and resolution of her lower extremity edema, her respiratory status did not improve. She required 45 lpm of 70% FiO2 at rest and had significant oxygen desaturation with activity. Transthoracic echocardiogram bubble study was concerning for a large shunt, which prompted a transesophageal echocardiogram that confirmed suspicion for an extracardiac shunt. A right heart catheterization with shunt run was not indicative of intracardiac shunt, also favoring an extracardiac shunting process. HPS was confirmed by lung perfusion scintigraphy. Her HPS was considered severe with a right to left shunt fraction of 26.6% and a PaO2 of 45 mmHg off supplemental oxygen. A transplant evaluation determined she was not a candidate due to severity of HPS and her level of hypoxemia. Patient discharged with hospice services. HPS is typically seen in the setting of overt chronic liver disease. However, in this patient her relatively modest elevation in liver enzymes and only mildly abnormal liver morphology on imaging, without signs of cirrhosis is an unusual presentation for HPS. Literature in these types of patients is limited and primarily based on a few case reports. Her chronic hepatitis C may have contributed but did not produce the cirrhotic features that traditionally prompt thoughts of HPS. This patient’s presentation adds to the theory that HPS may be more prevalent in non-cirrhotic patients than previously thought. The dyspnea workup in this patient highlights the various diagnostics tests that can lead to diagnosis of HPS. This case contributes to the need for research into HPS detection in the absence of glaring cirrhosis and expanded treatment options. This abstract is funded by: None
Feen et al. (Fri,) studied this question.