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LVOT Obstruction Physiology in HCM — EECC MCQ

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HardCardiomyopathyLVOT Obstruction Physiology in HCMEECC

A 42-year-old after a Mustard operation has severe systemic tricuspid regurgitation, systemic RV ejection fraction 42% and worsening exercise capacity. Pulmonary-venous and systemic-venous baffles are unobstructed. What should be considered before RV dysfunction becomes advanced?

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Correct answer: C — Systemic tricuspid-valve surgery at an adult-congenital centre

After atrial switch, the morphologic tricuspid valve supports the systemic RV. Severe regurgitation accelerates systemic-RV failure, and surgery should be considered while ventricular function remains moderately preserved because late outcomes are poor once dysfunction is advanced. The other procedures do not address the systemic atrioventricular valve or delay beyond the useful window.

Reference: 2020 ESC Guidelines for adult congenital heart disease. https://academic.oup.com/eurheartj/article/42/6/563/5898606