Which Statement About Eisenmenger Syndrome is FALSE?
Statement C is FALSE: Treatment does NOT involve closure of the ventricular septal defect when Eisenmenger syndrome has developed 1, 2.
Why Closure is Contraindicated in Established Eisenmenger Syndrome
Once Eisenmenger syndrome develops with established pulmonary hypertension and shunt reversal (right-to-left or bidirectional flow), closure of the defect carries an unacceptably high risk of mortality and should not be performed 1. The 2018 AHA/ACC guidelines explicitly state that "closure of nonrestrictive VSD in adults with Eisenmenger syndrome who do not demonstrate left-to-right shunting and a decline in pulmonary vascular resistance with PAH therapies carries a high risk of mortality and should not be performed" 1. Early surgical attempts at closure in these patients were abandoned due to prohibitively high mortality rates 1.
The pathophysiology explains why: In Eisenmenger syndrome, the pulmonary vascular resistance has risen to systemic or suprasystemic levels, causing shunt reversal. The defect now serves as a "pop-off valve" for the failing right ventricle. Closing it would eliminate this decompression mechanism, leading to acute right heart failure and death 3, 4.
Why the Other Statements Are TRUE
A) Cyanotic Congenital Heart Disease - TRUE
Eisenmenger syndrome is definitively a cyanotic condition. Central cyanosis is the primary clinical manifestation, resulting from the reversed (right-to-left) or bidirectional shunting that develops when pulmonary vascular resistance exceeds systemic resistance 2, 5. This chronic hypoxemia leads to secondary erythrocytosis and multiorgan complications 5, 4.
B) Associated with Pulmonary Hypertension - TRUE
Pulmonary arterial hypertension is the fundamental pathophysiologic feature of Eisenmenger syndrome. It represents the most severe phenotype of PAH associated with congenital heart disease 2, 5. The syndrome develops when large unrepaired shunts cause progressive pulmonary vascular disease, with pulmonary vascular resistance rising to systemic levels 4, 6.
D) ASD Can Be Complicated by Eisenmenger Syndrome - TRUE
While ventricular septal defects are more commonly associated with Eisenmenger syndrome, atrial septal defects can also lead to this complication 2, 7. The 2015 ESC/ERS guidelines explicitly include both intra- and extra-cardiac defects in their classification 2. A recent case report documented Eisenmenger syndrome secondary to an atrial septal defect in a 52-year-old woman 7.
E) Pregnancy Contraindicated - TRUE
Pregnancy carries extremely high maternal mortality risk in women with Eisenmenger syndrome due to the hemodynamic changes that occur during pregnancy and delivery, which the fragile cardiopulmonary system cannot tolerate 4, 6. This is a well-established absolute contraindication in the management of these patients.
Critical Management Principle
The only scenario where defect closure might be considered is in patients with large defects and PAH who still demonstrate net left-to-right shunting either at baseline or with PAH therapies, using fenestrated devices or patches to allow right heart decompression 1. However, this is not Eisenmenger syndrome by definition—Eisenmenger requires established shunt reversal 2.
Current treatment focuses on pulmonary arterial hypertension-targeted therapies (endothelin receptor antagonists, phosphodiesterase-5 inhibitors) to improve functional capacity and quality of life, not defect closure 5, 8, 6.