What is the appropriate management for a patient with an atrial septal defect that has progressed to Eisenmenger syndrome?

Management of Atrial Septal Defect with Eisenmenger Syndrome

Defect closure is absolutely contraindicated in ASD with Eisenmenger syndrome, and management focuses on pulmonary arterial hypertension-targeted medical therapy with specialized care at a grown-up congenital heart disease (GUCH) center. 1

Absolute Contraindication to Closure

Surgery or device closure must be avoided when Eisenmenger physiology is present. 1 The defining features that prohibit intervention include:

• Net right-to-left shunt (shunt reversal with cyanosis) 1
• Pulmonary artery systolic pressure greater than two-thirds systemic pressure 1
• Pulmonary vascular resistance greater than two-thirds systemic resistance 1
• Severe pulmonary arterial hypertension at systemic levels 1

Early surgical attempts at closure in Eisenmenger patients were associated with unacceptably high mortality and the practice was abandoned. 1 This remains a Class III (Harm) recommendation with Level C evidence. 1

Medical Management Strategy

Patients with ASD and Eisenmenger syndrome require lifelong management at specialized GUCH centers with pulmonary hypertension expertise. 1 The management algorithm includes:

Pulmonary Arterial Hypertension Therapy

• Initiate PAH-targeted medical therapy (endothelin receptor antagonists, phosphodiesterase-5 inhibitors, prostacyclin analogs) under the guidance of pulmonary hypertension specialists 2, 3
• Monitor response to vasodilator therapy, as some patients may show improvement in pulmonary vascular resistance and potential shunt reversal 2
• Regular assessment of functional capacity and oxygen saturation 1

Supportive Care Measures

• Limit physical activity to low-intensity recreational activities only 1
• Avoid dehydration, which increases blood viscosity and thrombotic risk 1
• Manage secondary erythrocytosis with phlebotomy only when symptomatic hyperviscosity occurs (typically hematocrit >65%) 1
• Provide supplemental oxygen for symptomatic relief, though it does not alter disease progression 1

Pregnancy Counseling

Pregnancy is absolutely contraindicated in Eisenmenger syndrome due to maternal mortality rates of 30-50%. 1 Effective contraception counseling is mandatory, with barrier methods plus progesterone-only options preferred over estrogen-containing contraceptives. 1

Follow-Up Protocol

Annual evaluation at a specialized GUCH center (Level 2 care) is required. 1 Each visit should include:

• Clinical assessment of functional status and cyanosis 1
• Echocardiography to assess right ventricular function, pulmonary pressures, and shunt direction 1
• Six-minute walk test or cardiopulmonary exercise testing 3
• Complete blood count to monitor erythrocytosis 1
• Assessment for arrhythmias (atrial fibrillation/flutter are common complications) 1, 3

Prognosis and Advanced Therapies

The 25-year survival for unoperated ASD with Eisenmenger syndrome is only 42%, significantly worse than small defects (96% survival). 1 When medical therapy fails and refractory right heart failure develops despite optimal PAH treatment, bilateral lung transplantation with ASD repair is the preferred option over combined heart-lung transplantation. 4

Transplant outcomes data show:

• BLT with ASD repair: 88.3% survival at 1 year, 71.1% at 3 years 4
• Combined heart-lung transplant: 63.2% survival at 1 year, 49.8% at 3 years 4

The superior outcomes with BLT plus repair reflect that ASD-related Eisenmenger syndrome causes isolated and reversible right ventricular failure, unlike VSD-related Eisenmenger where ventricular interdependence makes HLT preferable. 4

Critical Pitfalls to Avoid

Never attempt defect closure based on improvement with PAH therapy alone—even if pulmonary pressures decrease, closure in established Eisenmenger physiology carries prohibitive mortality risk. 1

Do not delay referral to specialized centers—these patients require multidisciplinary expertise in both congenital heart disease and pulmonary hypertension that is unavailable in general cardiology practices. 1

Avoid routine phlebotomy—remove blood only for symptomatic hyperviscosity (headache, visual changes, paresthesias) with hematocrit >65%, as overzealous phlebotomy worsens iron deficiency and increases stroke risk. 1