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

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.