•  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