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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.
References
Pediatrics Board Review Core Curriculum
MedStudy 1st edition, Book 3