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Eisenmenger Syndrome

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Overview

Eisenmenger syndrome describes the reversal of a left-to-right cardiac shunt caused by a congenital heart disease. (CHD) This occurs due to increased blood entering the pulmonary circulation from the left-to-right shunt leading to pulmonary hypertension. The right ventricle hypertrophies (enlarges) to compensate for this pulmonary hypertension.

Eventually, the right ventricle generates a higher pressure than the left ventricle, and blood starts to flow from right to left, forming a right-to-left shunt. This leads to decreased deoxygenated blood getting to the lungs and it instead enters the systemic circulation leading to cyanosis. Chronic hypoxaemia can lead to polycythaemia, which introduces further complications.

Causes

Causes of Eisenmenger syndrome include conditions that lead to left-to-right shunts:

  • Ventricular septal defects
  • Atrial septal defects
  • Patent ductus arteriosus

Epidemiology

  • Due to improvements in antenatal screening, Eisenmenger syndrome is relatively rare in people with good healthcare access

Presentation

Worsening shortness of breath in a person with a known CHD may suggest Eisenmenger syndrome. Features include:

  • Features of chronic hypoxaemia:
    • Cyanosis
    • Dyspnoea
    • Clubbing
  • Features associated with pulmonary hypertension:
    • Peripheral oedema
    • Right ventricular heave
    • Loud second heart sound
  • Features due to polycythaemia:
  • Features of an underlying septal defect:
    • Ventricular septal defects: pansystolic murmur
    • Atrial septal defects: ejection systolic murmur with fixed second heart sound splitting
    • Patent ductus arteriosus: continuous systolic and diastolic ‘machinery-like’ murmur
  • The original murmur may disappear:
    • Reversal of the shunt may lead to reduced turbulence of blood flow, making the murmur quieter/disappear

Investigations

Overview

Some investigations include:

  • ECG:
    • May show features of right ventricular hypertrophy
  • Chest x-ray:
    • May show features associated with an underlying CHD
  • Echocardiography:
    • Can diagnose Eisenmenger syndrome

Management

Overview

The only curative option is a heart-lung transplant. Other aspects of management involve treating pulmonary hypertension, arrhythmia, polycythaemia, and thrombosis.

Complications

Some complications include:

Prognosis

  • The prognosis of Eisenmenger syndrome is relatively poor. Mortality is high in 20-40 years.

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