Pediatrics · Congenital Heart Diseases (Acyanotic, Cyanotic)

A cyanotic neonate is found on chest radiograph to have a narrow mediastinum (egg-on-a-string appearance) with increased pulmonary vascular markings. Echocardiography confirms d-transposition of the great arteries (d-TGA). The immediate intervention to improve oxygenation until definitive arterial switch operation is:

  • A Prostaglandin E1 infusion to keep PDA open
  • B Emergency Mustard procedure to redirect atrial flows
  • C Balloon atrial septostomy (Rashkind procedure) to create/enlarge ASD and mix oxygenated blood
  • D Indomethacin to close the PDA
Correct answer: C. Balloon atrial septostomy (Rashkind procedure) to create/enlarge ASD and mix oxygenated blood

Explanation

In d-TGA with intact ventricular septum, oxygenated pulmonary venous blood recirculates in the left heart and deoxygenated systemic blood recirculates in the right — two parallel circuits. Adequate mixing at atrial level is essential for survival. Balloon atrial septostomy (Rashkind) creates an ASD to allow mixing and is the immediate palliative measure. PGE1 is used to maintain patency of PDA, which also allows some mixing, but septostomy is more reliable for TGA. Mustard/Senning procedures are atrial repairs, now superseded by the neonatal arterial switch operation as definitive repair.

Reference: Ghai Essential Pediatrics, 10th ed.

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