Abstract: PUB026
Atypical Hemolytic Uremic Syndrome Secondary to Transcatheter Aortic Valve Replacement (TAVR)
Session Information
Category: Acute Kidney Injury
- 101 AKI: Epidemiology, Risk Factors, and Prevention
Authors
- Ojeniyi, Solabomi Oyeronke, MedStar Union Memorial Hospital, Baltimore, Maryland, United States
- Gonzalez Hernandez, Dina R., MedStar Union Memorial Hospital, Baltimore, Maryland, United States
Introduction
Atypical hemolytic uremic syndrome (aHUS) is a rare subtype of TMA mediated by complement dysregulation with an incidence of 0.23-0.43 cases per million population. It can manifest in a diverse range of conditions and presentations, but acute kidney injury is a common prominent feature because of the apparent propensity of the glomerular circulation to endothelial damage and occlusion. We present a case of aHUS developed after an elective TAVR.
Case Description
An 86-year-old man with PMH of aortic stenosis who presented for an elective TAVR which was successfully done.
However, post-operative day 1, he developed new onset hematuria [dark brown to tea colored urine] and elevated BUN/Creatinine [Baseline serum creatinine of 0.9 to 1 mg/dL. He continued to have hematuria and worsening renal function.
Laboratory studies were remarkable for thrombocytopenia, low haptoglobin, and low fibrinogen. Normocytic anemia. Schistocytes on peripheral smear. Elevated D-dimer, CK, LDH, total bilirubin, direct bilirubin, AST. ADAMS 13 activity was greater than 5%. UA showed trace blood and 4-5 RBC. Complements, vasculitis panel, hepatitis panel, coagulation panel, cryoglobulinemia, and direct antiglobulin test were all negative. The patient remained asymptomatic throughout the hospital stay. He was managed symptomatically with antihypertensives for blood pressure control, intravenous hydration with normal saline, and platelet transfusion for thrombocytopenia. With the improvement of renal function, thrombocytopenia, and resolution hematuria. The patient was discharged home to follow-up with Nephrology and hematology.
Discussion
Atypical hemolytic uremic syndrome is most commonly due to a loss of function of a regulatory protein either by genetic mutation or autoantibody. The most common triggers include infections, autoimmune conditions, drugs, malignancies, or pregnancy. Treatment can be supportive, plasma exchange and anticomplement therapy. Although aHUS are associated with significant mortality and morbidity, including end-stage renal disease [ESRD], prompt diagnosis and initiation of supportive and specific management can transform outcome.