Postshunt hemochromatosis leading to cardiogenic shock in a patient presenting for orthotopic liver transplant: a case report.

INTRODUCTION: In this case report, we have presented a patient whose liver transplant course was greatly affected by a previously undiagnosed disease process that ultimately led to an unexpected perioperative death. CASE REPORT: A 52-year-old woman with idiopathic hepatoportal sclerosis presented for liver transplantation 2 years postmesocaval shunt placement. Lab and pathology studies at the time of liver biopsy and shunt placement were negative for iron deposition. Preoperative workup was negative for cardiac disease. At the outset of her liver transplant, the patient developed refractory hypotension secondary to cardiogenic shock. Intraoperative transesophageal echocardiography and postoperative transthoracic echocardiography demonstrated dilated cardiomyopathy with severely depressed systolic function. Upon succumbing to ventricular fibrillation cardiac arrest, an autopsy revealed hemochromatosis of the heart, pancreas, kidneys, adrenals, and explanted liver.
CONCLUSION: Dilated cardiomyopathy, congestive heart failure, and other unexpected disease processes resulting from hemochromatosis can greatly influence the care of postshunt liver failure patients.