Eisenmenger syndrome Part 1

Eisenmenger Syndrom
Eisenmenger Syndrome
Eisenmenger syndrome is a complication of long-standing left-to-right congenital cardiac shunts that leads to severe pulmonary arterial hypertension (PAH) and reversal of the shunt to right-to-left, causing cyanosis.
In simple words:
Congenital defect → L→R shunt → pulmonary hypertension → shunt reversal (R→L) → cyanosis
2. Congenital heart diseases that cause Eisenmenger
Most common defects:
VSD (most common)
PDA
ASD
AV canal defect
Truncus arteriosus
Aortopulmonary window
Mnemonic: “VPAAT”
VSD
PDA
ASD
AV canal
Truncus
3. Pathophysiology (Very Important for Exams)
Stepwise progression:
1. Initial defect
Large left-to-right shunt
Example: VSD
2. Increased pulmonary blood flow
Pulmonary arteries exposed to high pressure and flow
3. Pulmonary vascular remodeling
Medial hypertrophy
Intimal proliferation
Pulmonary vascular resistance (PVR) increases
4. Pulmonary hypertension develops
5. Shunt reversal
When PVR -- systemic vascular resistance
Blood flows right → left
Deoxygenated blood enters systemic circulation → cyanosis
4. Age of Development
Depends on the defect:
Defect
Typical Age of Eisenmenger Development
VSD / PDA
Childhood
ASD
Adolescence or adulthood
FOLLOW FOR PART 2
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Видео Eisenmenger syndrome Part 1 канала pediatrics pearls