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Hypoplastic left heart syndrome (Clinical presentation (Cyanosis, Shock…
Hypoplastic left
heart syndrome
Definition
Congenital structural heart
disorder with underdeveloped
left side of the heart
Epidemiology
Rare
Pathophysiology
Defect
Small/atretic mitral and aortic valves
Very small LV and ascending aorta
Coarctation of aorta
Mechanism
LV dysfunction and no flow through L side
Duct-dependent circulation, at 1-2d at duct closure
they become extremely cyanosed and shocked
Clinical
presentation
Cyanosis
Shock
Collapse
Diagnosis
Examination
Cardio
Weak/absent ALL peripheral pulses
Cyanosis, collapse
Investigations
Bedside
Obs (shock, hypoxia)
Bloods
Cap blood gas, FBC, U+E, LFT
Imaging
ECHO: diagnostic
History
DH
Meds, allergies
FH
Cardiac disorders
Cosanguinuity
PMH
Known conditions
POH
Antenatal: scans, bloods, growth
Perinatal: gestation, delivery, weight
Postnatal: growth, complications
PC/HPC
Sudden collapse/cyanosis at 1-2d
SH
Social support
Management
Initial ABCDE
Definitive
Medical
Prostaglandins
Indication: ASAP cyanosed neonate
E.g. aloprostil
MOA: preserves ductus arteriosus
in a duct-dependent system
Surgical
Norwood proceedure
Indication: asap neonatal period plus FU ops
MOA: surgical repair of L heart
Conservative
Information, advice, support
Cardio/paeds referral ASAP
Screening
Antenatal screening programme
Foetal anomaly scan (counselling and planning)
