S5546 Maintaining a Comprehensive Differential Diagnosis in Acute Liver Failure: A Critical Approach to Early Identification and Management

Introduction: Acute liver failure (ALF) is defined as acute liver injury (<26 weeks) with coagulopathy (international normalized ratio [INR] >1.5) and altered mentation. ALF requires urgent transfer to a transplant center for expediated evaluation to determine the etiology, initiation of treatment and consideration of transplantation. We present a case of a patient transferred for suspected ALF of unclear etiology. Case Description/Methods: A 26-year-old man with history of benign heart murmur presented with cough, vomiting, and diarrhea that began the day prior. On presentation, vital signs revealed a markedly widened pulse pressure. On physical exam, he was somnolent but arousable and interactive with trace lower extremity edema and ascites. Exam negative for asterixis. Cardiac exam revealed systolic murmur. Laboratory analysis revealed rapidly rising aspartate aminotransferase of 5,940 U/L up from 821 U/L the day prior, alanine aminotransferase of 3890 U/L up from 837, INR of 8.1 up from 3.8, arterial ammonia of 346 umol/L, and pH of 7.30. Computed tomography (CT) revealed hepatomegaly and small volume ascites. Liver doppler revealed patent vasculature. Extensive serologic workup including acetaminophen, salicylate, ethanol, phosphatidylethanol, hepatitis panel, and autoimmune serologies were unremarkable. Given the indeterminate etiology of ALF and progressive elevation of INR, liver transplantation was considered; however urgent transthoracic echocardiogram (TTE) was requested, especially in the setting of widened pulse pressure. TTE revealed normal ejection fraction, no valvular disease, but evidence of left-to-right shunt. Transesophageal echocardiogram revealed a 1.8 cm fistula between the sinus of Valsalva and right ventricular outflow tract. With this information, the etiology of ALF was suspected to be cardiogenic shock, secondary to a congenital supracristal ventricular septal defect leading to ruptured sinus of Valsalva aneurysm. The patient underwent successful surgical closure. Liver enzymes, coagulopathy, and mental status improved. He was discharged to inpatient rehab and at 3-month follow-up had full return to independent function. Discussion: Congestive hepatopathy due to cardiogenic shock can mimic ALF, and it can be challenging to differentiate between them without performing a detailed physical examination plus serologic and imaging workup. This case highlights the importance of maintaining a broad differential when working up patients with ALF, including cardiac conditions in younger patients, to ensure that timely and appropriate care be implemented.