Hemolytic Anemia: Sneaky Cause, Leaky Valve

Abstract

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.

Keywords: anemia and hyperbilirubinemia; hemolytic anemia; mechanical mitral valve complications; paravalvular leaks.

Figure 1. MRCP (sagittal and axial planes) revealing cholelithiasis (blue arrows) with mild gallbladder wall thickening (green arrow) indicative of chronic cholecystitis

MRCP, magnetic resonance cholangiopancreatography

Figure 2. MRCP showing mild hepatomegaly with hepatic steatosis (orange arrows)

MRCP, magnetic resonance cholangiopancreatography

Figure 3. TEE image showing paravalvular leak (pink arrow)

TEE, transesophageal echocardiogram