HLH in a patient with post-surgical Ebstein’s anomaly: Case report

Abstract

This case was interesting as it presents a rare complication in a rare congenital anomaly. We present it to raise awareness about serious fatal complications that could occur in a CHD postoperative patient which may not be related to their surgery. The heart surgery was successful but the patient passed away from complications which arose postoperatively. A 34-year-old male with Ebstein’s anomaly and severe tricuspid regurgitation status post ASD closure, atrialized ventricular plication and tricuspid annuloplasty at 6 years old presented with symptoms of congestive heart failure. He underwent tricuspid valve replacement and insertion of epicardial dual chamber pacemaker. Patient developed recurrent high fevers on the first night following surgery that were unresponsive to antibiotics and antipyretics. Evaluation revealed hyperferritinemia, thrombocytopenia, anemia and splenomegaly consistent with Hemophagocytic Lymphohistiocytosis (HLH). EVB titers were found to be elevated as a possible inciting infection. He was treated with Dexamethasone, Etoposide and Cyclopsorine, however he developed Heparin Induced Thrombocytopenia (HIT) followed by Disseminated Intravascular Coagulopathy (DIC) and died from a presumed pulmonary embolism. Conclusion: Surgical intervention outcomes in complex congenital heart disease patients can be complicated by infectious and hematological complications. If more cases of cardiac bypass related HLH are identified, the presence or absence of cold agglutinins as a factor should be studied. Timely recognition will prevent unnecessary mortality and morbidity.