8th March 2024

Eisenmenger Syndrome

Eisenmenger syndrome occurs as a result of a chronic process in which an uncorrected left-to-right shunt leads to increased pulmonary blood flow, causing pathological remodelling of the pulmonary vasculature and finally, pulmonary hypertension. It consists of the triad: 

1. Congenital structural defect causing initial left-to-right shunt
2. Subsequent pulmonary hypertension causing shunt reversal from right-to-left
3. Cyanosis 

Clinical manifestations include clubbing, raised JVP, central cyanosis, right parasternal heave and loud P2. Complications of Eisenmenger syndrome include: 

• RV dilatation and dysfunction 
• Cyanotic heart disease- erythrocytosis; bleeding
• Intrapulmonary haemorrhage / haemoptysis 
• Pulmonary artery aneurysm 
• Pulmonary thrombosis 
• Stroke/TIA – Paradoxical embolism 
• Brain abscess 
• Arrhythmias 
• Sudden cardiac death
• Infective endocarditis