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Tetralogy of Fallot hypercyanotic spell — MCCQE Part 1 MCQ

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HardHeart MurmurTetralogy of Fallot hypercyanotic spellMCCQE Part 1

A 10-month-old with known cyanotic congenital heart disease becomes deeply cyanotic and irritable after crying. Squatting position improves symptoms. Which of the following is the most appropriate immediate management?

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Correct answer: A — Knee-chest positioning, oxygen, calming and urgent paediatric/cardiology support

A hypercyanotic spell in tetralogy physiology is managed with knee-chest positioning, oxygen, calming, fluids and medications such as morphine or beta-blocker in consultation. Vigorous crying worsens right-to-left shunting. This is an emergency even if symptoms partially improve.

Reference: Canadian paediatric cardiology practice; paediatric emergency pathways