hypoplastic left heart syndrome
underdevelopment of the left side of the heart characterized by:
most common cause of CHF in neonate
- 25% of cardiac deaths in 1st week of life
- prognosis if untreated: 100% fatal by 6 weeks
hemodynamics:
- pulmonary venous return is diverted from LA to RA through
ASD
- RV supplies pulmonary artery, ductus arteriosus,
descending aorta (antegrade flow), aortic arch, ascending
aorta, coronary circulation (retrograde flow)
- leads to RV work overload and CHF
Rx:
- Norwood procedure (palliative) - three stage surgery
that results in the RA feeding the pulmonary circulation
and the RV feeding the systemic circulation.
- transplant
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© 2002, C.E. Kahn, Jr.
2002-04-18