Long term survival after 10 yrs: 94% (IAA and VSD) 72% (IAA and TGA). Long term antihypertensive treatment required in 30%. Neurological injury due to deep hypothermic circulatory arrest.Spinal cord injury (spinalis anterior injury): 0.4-1.5%.PHT if high PBF was preceding (VSD or ASD).Postcoarctectomy syndrome: hypertension, abdominal pain, ileus (2-3 days post repair).Acute hypertension (increase of noradrenaline release due to sympathetic stimulation during repair): SNP or Esmolol infusion.Fluid restriction: 1ml/kg/hr, careful trophic feeds.Haemodynamics: age adjusted, in neonates: SBP >60 mmHg but 40 mmHg prevent hypertension (SNP or Esmolol infusion).Keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding high PBF elective cases can be extubated earlier.Preferred single stage repair with end-to-end or end-to-side anastomosis, patch augmentation, subclavian-flap aortoplasty or extended resection with primary anastomosis and also VSD closure. Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates. Calcium infusion if there is Di-George Syndrome (see inotropes)ĮCG, CXR, CUS, FBE, xlotting, UECs, FISH, PRBC(4), FFP (2), platelets (2), cryoprecipitate (2).Careful fluid resuscitation – this is an obstructive lesion, not hypovolaemic!.Dopamine (5-10 mcg/kg/min), dobutamine (5-10 mcg/kg/min) or adrenaline (0.02-0.1 mcg/kg/min) may be required to stabilise for a low CO.Balanced circulation with PDA open and/or VSD present (aim SpO2 75-85%).Hypoventilation to higher the PVR and to lower the SVR. Intubate and sedate to lower the oxygen consumption. Commence Prostaglandin E1 (20ng/kg/min) to maintain systemic perfusion. Hypertension of upper limbs is usually not present before Day 5, but usually after PDA closure with signs of CCF of various degree.ĬXR: cardiomegaly and pulmonary congestion.Ĭardiac catheterisation (diagnostic and interventional) This in turn causes increased PBF and severe CCF with systemic hypotension. This leads to CCF (in extreme: myocardial ischemia) and shunt reversal along the PFO (and VSD if present). With PDA closure there is an acute increase in LV afterload, a decreased CO, increased LVEDP. Genetic association to Di-George-Syndrome. Most of them are associated with a VSD or other defects. Type C (2%): proximal to left carotid artery. Type B (78%): between left subclavian and left carotid artery. Type A (20%): IAA distal to left subclavian artery. Definition: obstructive anomaly of the aortic arch.
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