Eisenmenger’s syndrome natural history

Eisenmenger’s syndrome passes through different stages, including the causative left-to-right shunt, development of pulmonary hypertension, polycythemia, and shunt reversal. The complications of Eisenmenger’s syndrome include intracranial hemorrhage, stroke, congestive heart failure, angina pectoris, hyperviscosity syndrome, infection (cerebral abscess), renal failure, and sudden death. How well the infant or child does depends onwhether another medical condition is present and the age at which high blood pressure develops in the lungs. Patients with Eisenmenger’s syndrome can live 20 to 50 years.

Patients with Eisenmenger’s syndrome pass through the following stages^[1]:

• An underlying heart defect that initially allows a left-to-right shunt between the left and right sides of the heart.
• The development of pulmonary hypertension.
• Polycythemia, an increase in the number of red blood cells.
• Finally, a reversal of the left-to-right shunt so that there is a right-to-left shunt.
• At the final stage, the patient is liable to the complications mentioned below.

Therefore, they may present with a history of symptoms for each of these stages.

The complications of Eisenmenger’s syndrome include^[2][3]:

• Intracranial hemorrhage
• Stroke
• Congestive heart failure
• Angina Pectoris
• Hyperviscosity syndrome
• Infection (cerebral abscess)
• Renal failure
• High risk pregnancy and fetal loss.
• Sudden death

• How well the infant or child does depends on^[4]:
  • whether another medical condition is present
  • the age at which high blood pressure develops in the lungs.
• Patients with Eisenmenger’s syndrome can live 20 to 50 years.
• The predictors of mortality in Eisenmenger’s syndrome include^[5]:
  • Age
  • Pretricuspid shunt
  • Oxygen saturation at rest
  • Presence of sinus rhythm
  • Presence of pericardial effusion

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