Hemolytic Anemia: Sneaky Cause, Leaky Valve

Intravascular hemolysis is a known complication of prosthetic heart valves. Severe hemolysis is rare (<1%) with the use of newer generation prosthetic valves. This usually occurs due to paravalvular leaks (PVLs). We present a case of hyperbilirubinemia and hemolytic anemia occurring as a result of a PVL of a prosthetic mechanical mitral valve. The patient was a 49-year-old female with a past medical history of rheumatic heart disease status following two mitral valve replacements each with a mechanical prosthesis; she presented with a complaint of worsening fatigue, epigastric pain, nausea, and vomiting. On examination, she had scleral icterus. Heart auscultation revealed a crisp mechanical S1 click and a soft 2/6 systolic murmur in the left lower sternal border. Her abdomen was soft with mild epigastric and right upper quadrant tenderness, and no Murphy's sign. Her labs revealed a white blood cell count of 7.0 x 10³/microliter, hemoglobin 10.5 g/dL, hematocrit 29.7%, total bilirubin 6.9 mg/dL, direct bilirubin 0.8 mg/dL, alkaline phosphatase (ALP) 62 U/L, aspartate aminotransferase (AST) 79 U/L, and alanine aminotransferase (ALT) 56 U/L. An ultrasound of the abdomen revealed cholelithiasis without pericholecystic fluid collection and no ultrasonographic Murphy's sign. Magnetic resonance cholangiopancreatography ruled out acute cholecystitis or intra- or extra-hepatic biliary ductal dilatation. A transesophageal echocardiogram showed a well-seated mitral valve prosthesis with a significant PVL and likely moderate mitral regurgitation. The patient was evaluated for possible hemolysis. Lactate dehydrogenase was 1155 U/L, haptoglobin was <30 mg/dL, and reticulocyte count was 5.2%. She underwent a mitral valve re-replacement with a mechanical prosthesis. An echocardiogram after the surgery showed the mechanical prosthesis mitral valve with no residual PVL.