APCU25CR18 Fragmentation syndrome in native valve mitral regurgitation

Introduction This is a case study of common but rarely described in literature. Valve hemolysis is a common complication that can be observed post valve replacement surgery and severe stenotic native valve.Case Presentation Patient is a 86-year-old gentleman with underlying Diffuse Large B-cell Lymphoma and non-valvular Atrial Fibrillation. He was diagnosed with DLBCL primary cervical lymph node disease stage IIB and completed cycles of R-CHOP and PET scan post treatment cycle shows complete remission. Subsequently he defaulted follow up.He presented after 5 years later with 2 months symptoms of jaundice, shortness of breath, bilateral leg and scrotal swelling and orthopnea. He denies angina, loss of weight, or any new lymph node swellings. Clinical examinations show scleral jaundice, irregular heart rate, congested lungs with pansystolic murmur at the apex with radiation to axilla.Results Lab investigations show macrocytic anemia with hemoglobin of 5.8 MCV 101 fL, platelet 240 (109/L), white cells 5.1 (109/L), indirect bilirubin 44, direct bilirubin 14, LDH 333, Coombs test is negative, ferritin 625, serum B12 and folate is adequate. Full blood picture shows many fragmented red blood cells with iron deficiency features.Other investigations include ECG which shows atrial fibrillation with right bundle branch block. ECHO shows, EF 79%, dilated RA/LA, thickened AMVL, PMVL with moderate to severe mitral regurgitation.Discussion He is treated for decompensated HFpEF, non-valvular atrial fibrillation and non-immune hemolytic anemia likely secondary to fragmentation syndrome due to severe mitral regurgitation. He is treated with IV frusemide, blood transfusion, subcutaneous Darbepoetin, T apixaban and optimizing pillars of HF medications. Subsequent follow up in clinic shows improving hemolysis with further optimizing his heart failure medications.Conclusion This instance demonstrates how cardiac decompensation can exacerbate valve hemolysis, which in turn leads to a vicious cycle of both cardiac decompensation and valve hemolysis. It’s critical to optimize the therapy of heart failure while concurrently addressing other anemia-causing factors. Definitive surgical mitral valve replacement may treat this condition; however, this patient is deemed high risk for major cardiac surgery.