What is the management plan for a patient with aplastic anemia and complex cardiac issues including severe mitral and tricuspid regurgitation, mild to moderate mitral stenosis, and elevated pulmonary artery systolic pressure?

Management of Aplastic Anemia with Complex Valvular Heart Disease

Surgical intervention with combined mitral and tricuspid valve repair/replacement is the recommended treatment for this patient with aplastic anemia who has severe mitral and tricuspid regurgitation, mild-to-moderate mitral stenosis, and pulmonary hypertension.

Cardiac Assessment and Management Priorities

Valvular Disease Evaluation

• The patient has multiple significant valvular abnormalities requiring intervention:
  • Severe mitral regurgitation with mild-to-moderate mitral stenosis (valve area 2.8 cm²) 1
  • Severe tricuspid regurgitation 1
  • Moderate-to-severe aortic regurgitation (+3) 1
  • Pulmonary hypertension (PASP 50 mmHg) 1

Surgical Approach

• Combined valve surgery is indicated due to:

  • Presence of severe mitral regurgitation with thickened/calcified leaflets (Wilkins score 8) 1
  • Severe tricuspid regurgitation with right heart chamber dilation 1
  • Elevated pulmonary artery pressure (50 mmHg) 1

• Percutaneous mitral commissurotomy (PMC) is not recommended because:

  • The patient has severe mitral regurgitation, which is a contraindication for PMC 1
  • The valve has significant calcification (Wilkins score 8) 1
  • The mitral stenosis is degenerative rather than rheumatic, with no commissural fusion 1

Surgical Considerations

• Mitral valve replacement is likely necessary due to:

  • Degenerative mitral stenosis with calcification 1
  • Severe regurgitation with structural abnormalities 1

• Tricuspid valve repair should be performed concurrently:

  • Ring annuloplasty is preferred for tricuspid repair when possible 1, 2
  • Valve replacement may be necessary if leaflets are significantly tethered 1

Aplastic Anemia Management in the Perioperative Period

Preoperative Preparation

• Complete hematologic evaluation with bone marrow assessment to determine disease severity 3, 4
• Optimize hemoglobin levels with blood transfusions prior to surgery 4, 5
• Consider platelet transfusions to maintain adequate platelet count (>50,000/μL) for surgery 5, 6

Perioperative Management

• Close monitoring of complete blood counts with ready availability of blood products 4, 7
• Minimize blood loss during surgery with meticulous hemostasis 5
• Consider specialized hematology consultation for perioperative management 6

Postoperative Care

• Intensive monitoring for bleeding complications 4, 7
• Continued transfusion support as needed 5, 6
• Early mobilization to prevent complications of prolonged bed rest 7

Long-term Management Considerations

Cardiac Follow-up

• Regular echocardiographic monitoring of valve function and pulmonary pressures 1
• Anticoagulation management if mechanical valves are used 1
• Ongoing heart failure management with appropriate medications 1

Aplastic Anemia Treatment

• Definitive treatment options to consider after cardiac recovery:
  • Hematopoietic stem cell transplantation for eligible patients 4, 6
  • Immunosuppressive therapy with ATG and cyclosporine for non-transplant candidates 6, 7
  • Consider thrombopoietin receptor agonists for persistent thrombocytopenia 6

Special Considerations and Pitfalls

• Cardiac surgery in aplastic anemia carries high risk due to:

  • Increased bleeding risk from thrombocytopenia 4, 7
  • Impaired wound healing and infection risk from neutropenia 5, 7
  • Potential for transfusion-related complications 6

• Timing of interventions is critical:

  • Cardiac surgery should be performed before severe right ventricular dysfunction develops 1, 2
  • Definitive treatment of aplastic anemia may need to be delayed until after cardiac recovery 4, 6

• Avoid medications that may worsen aplastic anemia, including certain antibiotics and antiarrhythmics 5, 7