Eisenmenger syndrome (ASD, VSD, PDA With PAH with reversal of shunt – Right-to-Ieft shunt) with Treatment

Eisenmenger syndrome (ASD, VSD, PDA With PAH with reversal of shunt – Right-toIeft shunt)

  • Eisenmenger described a syndrome in which pulmonary vascular disease developed in patients with a nonrestrictive ventricular septal defect,
  • There is cyanosis, JVP is normal, there is no paraster­nal heave, palpable pulmonary trunk in left second intercostal space. There are signs of pulmonary hy­pertension – pulmonary ejection systolic murmur, pulmonary ejection sound, loud and single S2, Gra­ham Steell murmur.
  • The triad of systemic-to-pulmonary communication, pulmonary vascular disease and cyanosis is called Eisenmenger syndrome,

Eisenmenger syndrome 1

Eisenmenger syndrome (ASD, VSD, PDA With PAH with reversal of shunt – Right-to-Ieft shunt)

diagnosis

  • diagnosis of Eisenmenger syndrome requires the presence of congenital heart disease,
  • Physical examination of the patient with Eisenmenger syndrome demonstrates central cyanosis and digital clubbing,
  • risk of developing the Eisenmenger syndrome appears to be determined by the size of the initial left-to-right shunt

ECG

  • Shows normal or right atrialp tricular hyportrophy.

X-ray

  • Shows oligemic lung fields, dilated pulmonary trunk and central branches and normal cardiac shadow.
  • chest x-ray usually shows dilatation of the central pulmonary arteries, peripheral pulmonary artery “pruning”

Complications

  • Infective endocarditis
  • Coagulation defects
  • Cerebral infarction or abscess
  • Paradoxic embolism
  • Erythrocytosis

Eisenmenger syndrome Treatment

  • Corrective operation should be done at suitable age.
  • If there is marked hypoplasia of pulmonary ar­teries, a palliative operation like a systemic ar­terial to pulmonary arterial shunt is done fol­lowed by complete correction years later.
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