UMEM Educational Pearls

Category: Pediatrics

Title: Hypoplastic Left Heart Syndrome

Posted: 2/25/2011 by Rose Chasm, MD (Updated: 7/14/2024)
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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.

References

Pediatrics Board Review Core Curriculum

MedStudy 1st edition, Book 3