How to manage a 34-year-old postpartum woman with pulmonary hypertension secondary to Eisenmenger syndrome due to atrial septal defect (ASD), currently with hypotension, requiring oxygen therapy via nasal prongs, and recent impaired renal function?

Management of Postpartum Patient with Eisenmenger Syndrome Secondary to ASD

The postpartum patient with Eisenmenger syndrome due to ASD requires immediate pulmonary vasodilator therapy with an endothelin receptor antagonist (ERA) such as bosentan as first-line treatment, along with careful hemodynamic monitoring and optimization of fluid status to manage hypotension while continuing oxygen therapy. 1

Current Clinical Status Assessment

This 34-year-old woman (G2P0L0A2) is in a critical period following medical termination at 23 weeks due to WHO class IV pulmonary hypertension secondary to Eisenmenger syndrome from an atrial septal defect. Her current status shows:

• POD 4 (Post-operative day 4)
• Oxygen requirement: 3 L/min via nasal prongs
• Systolic BP: 86 mmHg (hypotensive)
• Serum creatinine: improved from 1.0 mg/dL to 0.7 mg/dL
• Urine output: Initially compromised but now improved
• Serum lactate: 1.5 mmol/L (within normal range)

Immediate Management Priorities

1. Respiratory Support

• Continue supplemental oxygen to maintain arterial oxygen saturation >90% 2
• Monitor oxygen saturation continuously
• Consider increasing oxygen flow if saturation drops below 90%

2. Hemodynamic Management

• Address hypotension (SBP 86 mmHg) with careful volume management:
  • Administer judicious IV fluids if no signs of fluid overload
  • Target euvolemia with close monitoring of urine output and vital signs
  • Avoid excessive fluid administration which can worsen right heart failure 2
• Monitor for signs of right ventricular failure (increased JVP, peripheral edema)
• Consider low-dose inotropic support (dobutamine) if hypotension persists despite optimal volume status 2

3. Pulmonary Hypertension Therapy

• Initiate endothelin receptor antagonist (ERA) therapy (bosentan) as first-line treatment for Eisenmenger syndrome 1
• Consider adding phosphodiesterase-5 inhibitor (sildenafil) if monotherapy is insufficient 1, 3
• Avoid calcium channel blockers as they are contraindicated in Eisenmenger syndrome and can cause catastrophic systemic vasodilation 1

4. Anticoagulation Considerations

• Consider anticoagulation due to high risk of pulmonary artery thrombosis in Eisenmenger syndrome
• Balance against risk of bleeding, especially in the postpartum period 1
• If anticoagulation is initiated, use prophylactic doses initially with close monitoring

Monitoring and Additional Care

Laboratory Monitoring

• Daily complete blood count to assess for secondary erythrocytosis
• Monitor renal function (BUN, creatinine) daily until stable
• Liver function tests (elevated in right heart failure)
• Iron studies (iron deficiency is common and should be treated) 1
• BNP/NT-proBNP to assess heart failure status

Cardiovascular Assessment

• Echocardiography to assess right ventricular function, estimate pulmonary artery pressure, and evaluate shunt direction
• ECG monitoring for arrhythmias which are common in Eisenmenger syndrome

Fluid Balance

• Maintain strict input/output records
• Daily weights to assess fluid status
• Avoid excessive fluid shifts which can destabilize patients with Eisenmenger syndrome 1

Special Considerations and Pitfalls

1. Postpartum Period Risks: The first 4 weeks postpartum represent the highest risk period for mortality in women with pulmonary hypertension 2

2. Avoid Common Pitfalls:

   • Excessive diuresis causing hypovolemia and worsening hypotension
   • Inadequate oxygen therapy
   • Failure to recognize deteriorating right heart function
   • Inappropriate use of vasopressors without addressing underlying cardiac issues

3. Multidisciplinary Approach: Management should involve adult congenital heart disease specialists, pulmonary hypertension experts, and critical care physicians 1

4. ECMO Consideration: For severe decompensation refractory to medical therapy, extracorporeal membrane oxygenation (ECMO) may be considered as a bridge to recovery 2

Long-term Management Plan

1. Pulmonary Vasodilator Therapy:

   • Continue ERA therapy (bosentan) long-term
   • Consider combination therapy with PDE-5 inhibitor if monotherapy is insufficient 1

2. Follow-up:

   • Regular assessment at a specialized center with expertise in adult congenital heart disease and pulmonary hypertension
   • Follow-up echocardiography, 6-minute walk test, and laboratory studies every 3-6 months

3. Contraception:

   • Absolute contraindication to future pregnancies due to extremely high mortality risk 1
   • Arrange for permanent contraception before discharge

4. Patient Education:

   • Avoid situations that can destabilize Eisenmenger physiology (dehydration, high altitude, extreme exertion)
   • Recognize early signs of deterioration requiring medical attention

This patient requires specialized care at a center with expertise in adult congenital heart disease and pulmonary arterial hypertension to optimize outcomes and reduce mortality risk.