UMEM Educational Pearls

Category: Pediatrics

Title: Hypoplastic Left Heart Syndrome

Posted: 2/25/2011 by Rose Chasm, MD (Updated: 4/20/2024)
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disorder in which the entire left side of the heart is underdeveloped
the right side of the heart is dilated and hypertrophied, and supports both the systemic and pulmonary circulations via PDA
accounts for nearly 1/4 of all cardiac deaths in the first year of life
infants present within the first days or weeks of life acutely ill with signs of CHF
PE often shows cyanosis and poor pulses but hyperdynamic cardiac impulses
CXR shows cardiac enlargement and prominent pulmonary vasculature
EKG shows RA and RV hypertrophy
echo is diagnostic
acute treatment is PGE1 to maintain the PDA.

Pediatrics Board Review Core Curriculum

MedStudy 1st edition, Book 3