What is the recommended approach for dilatation in a patient with Eisenmenger syndrome?

Dilatation in Eisenmenger Syndrome: Atrial Septostomy

In patients with Eisenmenger syndrome who have failed medical therapy, atrial septostomy using blade septostomy followed by a series of balloon dilatations is the recommended approach for right ventricular decompression, but only when performed electively in moderate to severe disease—not as rescue therapy. 1

Mechanism and Rationale

Atrial septostomy creates an iatrogenic atrial septal defect to offload the failing right ventricle in the setting of severe pulmonary vascular resistance. 1 This approach is supported by evidence that Eisenmenger patients with patent foramen ovale demonstrate better long-term survival. 1

Technical Approach

• Perform blade septostomy as the initial technique, followed by serial balloon dilatations to achieve the desired defect size 1
• The procedure must be performed by experienced interventional cardiologists in specialized centers 1
• This technique increases cardiac index, decreases right atrial pressure, and improves symptoms and exercise tolerance 1

Patient Selection Criteria

Critical timing consideration: Early selection in moderate to severe disease is essential—the procedure must be elective, not rescue therapy. 1

Avoid septostomy in patients with:

• Markedly elevated pulmonary vascular resistance with maximal arterial oxygen saturations ≤80% at rest 1
• Severe right heart failure with low cardiac output and high right atrial pressure 1
• These high-risk features predict mortality and contraindicate the procedure 1

Expected Outcomes and Risks

Benefits: Improved cardiac index, reduced right atrial pressure, enhanced exercise tolerance, and symptom relief 1

Substantial risks include:

• Cardiac tamponade 1
• Arrhythmias 1
• Refractory hypoxemia 1

Clinical Context

While some centers use atrial septostomy purely as bridge to transplantation or palliative salvage, evidence suggests earlier performance is both safer and more effective. 1 The procedure should be considered within a comprehensive management strategy that includes optimization of pulmonary arterial hypertension-specific therapies. 1

Important caveat: This intervention addresses end-stage disease in patients who have exhausted medical options. The ultimate goal remains early detection and surgical correction of intracardiac shunts before Eisenmenger syndrome develops, as this condition is preventable. 2