Abstract 13247: Premature Closure in Need of Percutaneous Closure: An Elusive Case of Recurrent Liver Failure
A 54-year-old man with alcoholic cirrhosis and a transjugular intrahepatic portosystemic shunt (TIPS) was hospitalized for hepatic encephalopathy. During evaluation, a bicuspid aortic valve without stenosis or regurgitation and thoracic aortic aneurysm of 5.3cm were identified. Surgical repair of the aorta was deferred given his end-stage liver disease (ESLD). He underwent orthotopic liver transplantation (OLT) but suffered primary graft non-function and failure. He was re-listed emergently and underwent repeat OLT. 4 weeks later he developed volume overload and recurrent liver failure. The liver failure was initially attributed to acute rejection. Liver biopsy revealed centrilobular necrosis and sinusoidal dilation. The patient was re-listed and received a third deceased cadaveric liver transplantation. The lack of a unifying diagnosis at this time required re-evaluation of prior testing. On repeat examination, the jugular venous pulse was elevated and notable for a v-wave; however, there was no significant tricuspid regurgitation on physical exam or echocardiography. Invasive hemodynamic testing confirmed tall right atrial v-waves, a high cardiac output, and elevated pulmonary arterial saturation of 88%. The aggregate data suggested the possibility of an intracardiac shunt as the culprit of high-output heart failure and liver failure. 3D transesophageal echocardiography was performed and revealed an aorto-atrial fistula with left-to-right shunting, possibly due to iatrogenic injury during his TIPS procedure. As the patient was deemed at prohibitively high risk for open repair, percutaneous closure was performed with a 10-mm Amplatzer device with immediate reduction in right-sided filling pressures and normalization of v-waves. This case illustrates (1) the problem of “premature closure”, acceptance of a diagnosis (and failure to consider alternatives) before sufficient verification has occurred; and (2) the critical importance of re-examining the primary cardiac data to eliminate bias in the clinical reasoning process. Percutaneous closure led to successful resolution of the aorto-atrial shunt with complete recovery and reversal of decompensated liver failure in this patient who continues to do well 2 years later.
Author Disclosures: S.S. Khan: None. K. Maganti: None. D.C. Lee: None. J.D. Flaherty: None. S. Intwala: None. B.B. Shetuni: None. S.J. Shah: None.
- © 2014 by American Heart Association, Inc.