Eisenmenger's syndrome

Question 1

Pathophysiology?

Answer 1

Reversal of a left-to-right shunt in a congenital heart defect due to pulmonary hypertension. This occurs when an uncorrected left-to-right leads to remodeling of the pulmonary microvasculature, eventually causing obstruction to pulmonary blood and pulmonary hypertension.

Question 2

Associated with?

Answer 2

• ventricular septal defect
• atrial septal defect
• patent ductus arteriosus

Question 3

Features?

Answer 3

• original murmur may disappear
• cyanosis
• clubbing
• right ventricular failure
• haemoptysis, embolism

Question 4

Management?

Answer 4

Heart-lung transplantation is required