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Complex Congenital Heart Disease (Anaesthetic Plan (Airway, Breathing,…
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Fontan Circulation
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Surgical stages
- Artificial shunt between R SCA and R PA (aged~6mth)
- Shunt from SVC to R PA (Aged~2)
- Extra-cardiac conduit to allow flow from IVC to L PA (aged ~4)
Mortality most from stage 1 - mainly due to PVR (highest, dont do as neonate
Adult fontan:
- Ventricular function limited
- Dependent on ventricular pre-load and can't increase it --> poor exercise tolerance
- Therefore dont respond to b-blockers, inotropes, pressors
Complications
Arrhythmia 45%, poorly tolerated, need anticoagulation
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Must have:
- sinus rhythm
- adequate sized pulm a
- good LV Fn
Indications:
- pulm atresia
- tricuspid atresia
- double inlet LV
- hypo plastic left heart
- double outlet RV
- complete AV septal defects
Tetralogy of Fallot
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Treatment
If shunt is very large, needs blalock-Taussig from R SCA to R PA to provide pulmonary flow and reduce cyanosis but still has high PVR
TOF repair at 6-18mths when PVR reducing naturally
May have RVH
Pulmonary regurgitation/PVR may be an issue
RVOT dependent on preload and continuous flow to maintain OT open
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