What is the best course of action for a patient with severe anemia, likely due to hemolytic anemia from a perivalvular leak of a mechanical mitral valve, with a planned CT (Computed Tomography) surgery appointment?

Proceed with Planned Surgical Mitral Valve Replacement

This patient with severe hemolytic anemia (Hgb 4.4) from a perivalvular leak of a mechanical mitral valve requires surgical intervention—specifically mitral valve replacement (MVR) redo—as this represents intractable hemolysis requiring repeated transfusions on her third admission. 1

Definitive Management: Surgery is Indicated

Surgery is a Class I recommendation (highest level) for patients with intractable hemolysis from prosthetic paravalvular leak unless surgical risk is prohibitive. 1, 2 This patient meets criteria for "intractable" hemolysis given:

• Severe anemia requiring third hospitalization for transfusion 1
• Persistent elevation in LDH and low haptoglobin with appropriate reticulocyte response 1
• Failed medical management (iron, folic acid supplementation) 1

The planned CT surgery appointment should proceed as scheduled. 2

Why Percutaneous Closure is Not Appropriate Here

Percutaneous closure was already appropriately ruled out by cardiology (Dr. Woo). 1 The ACC/AHA guidelines reserve percutaneous repair (Class IIa recommendation) only for patients who meet ALL three criteria: 1

1. High or prohibitive surgical risk
2. Intractable hemolysis OR NYHA Class III-IV symptoms
3. Suitable anatomy for catheter-based therapy

Critically, percutaneous closure could worsen hemolysis in this mechanical valve setting, as noted by the cardiology consultation. 2 Even technically successful percutaneous procedures can cause new-onset severe hemolytic anemia requiring surgical rescue. 3

Surgical Approach: Complete Valve Replacement

Complete replacement of the mechanical mitral valve is the most frequently performed and recommended procedure for symptomatic perivalvular leaks causing hemolysis. 2 This is preferred over attempted repair because:

• The defect is causing clinically significant hemolysis requiring repeated transfusions 2
• Mechanical valves with paravalvular leaks causing hemolysis typically require replacement rather than repair 1, 2
• Direct repair has higher failure rates in this setting 2

Critical Preoperative Considerations

Pulmonary hypertension assessment via right heart catheterization is essential before this reoperation (Class I recommendation). 2 This provides:

• Precise hemodynamic data that echocardiography estimates cannot fully replace 2
• Critical risk stratification, as elevated pulmonary artery pressures significantly increase operative mortality in mitral reoperations 2

Perioperative Risk Acknowledgment

Reoperative mitral valve surgery carries substantially higher risk than primary operations, with operative mortality ranging from 4.7% to 17.5%. 2, 4 However, this must be weighed against:

• Ongoing severe hemolysis causing multi-organ dysfunction (acute kidney injury, hepatomegaly noted in similar cases) 4
• Progressive deterioration with medical management alone 4
• High mortality without definitive intervention 4

Bridging Strategy Until Surgery

While awaiting surgery, continue: 1

• Folic acid and iron supplementation to support erythropoiesis 1
• Periodic transfusion as needed to maintain adequate hemoglobin 1
• Beta-blockers may reduce hemolysis severity 1, 4
• Erythropoietin can be considered in severe cases 1, 4

Exclude Endocarditis Before Surgery

New paravalvular leak late after valve implantation raises concern for infective endocarditis, which must be excluded. 1 Endocarditis would require:

• Antibiotic treatment before surgical therapy 1
• Different surgical approach and timing 1

Ensure blood cultures and clinical assessment exclude active infection before proceeding. 1

Post-Surgical Expectations

Expect: 4

• Long surgical times (aortic cross-clamp ~124 minutes, cardiopulmonary bypass ~182 minutes) 4
• Significant blood product requirements (typically 6-8 units RBCs) 4
• Potential complications including cardiac dysfunction (22%), arrhythmia (43%), and infection (22-26%) 4

However, successful reoperation typically resolves the hemolytic process completely. 5, 6