Definition: obstructive anomaly of the aortic arch.
Classification by Celoria et al:
Type A (20%): IAA distal to left subclavian artery.
Type B (78%): between left subclavian and left carotid artery.
Type C (2%): proximal to left carotid artery.
Most of them are associated with a VSD or other defects.
Prevalence 1% of all CHD. Incidence 4:10.000 of all life births. Genetic association to Di-George-Syndrome.
With PDA closure there is an acute increase in LV afterload, a decreased CO, increased LVEDP. This leads to CCF (in extreme: myocardial ischemia) and shunt reversal along the PFO (and VSD if present). This in turn causes increased PBF and severe CCF with systemic hypotension.
Hypertension of upper limbs is usually not present before Day 5, but usually after PDA closure with signs of CCF of various degree.
ECG: signs of RVH.
CXR: cardiomegaly and pulmonary congestion.
Cardiac catheterisation (diagnostic and interventional)
- Commence Prostaglandin E1 (20ng/kg/min) to maintain systemic perfusion. Intubate and sedate to lower the oxygen consumption. Hypoventilation to higher the PVR and to lower the SVR.
- Balanced circulation with PDA open and/or VSD present (aim SpO2 75-85%)
- 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
- Careful fluid resuscitation – this is an obstructive lesion, not hypovolaemic!
- Calcium infusion if there is Di-George Syndrome (see inotropes)
ECG, CXR, CUS, FBE, xlotting, UECs, FISH, PRBC(4), FFP (2), platelets (2), cryoprecipitate (2).
Methylprednisolone 10mg/kg 12hrs and 6hrs pre surgery in neonates.
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.
- Keep intubated, ventilated, sedated and paralysed for 24 hours for patients with preceding high PBF; elective cases can be extubated earlier
- Inotropes: milrinone plus dopamine
- Haemodynamics: age adjusted, in neonates: SBP >60 mmHg but <80 mmHg; MAP >40 mmHg; prevent hypertension (SNP or Esmolol infusion)
- Respiratory: normoxaemia, normocapnea
- Fluid restriction: 1ml/kg/hr, careful trophic feeds
- Haemostasis: Hb 100-140
- Maintain normothermia
- Acute hypertension (increase of noradrenaline release due to sympathetic stimulation during repair): SNP or Esmolol infusion
- Postcoarctectomy syndrome: hypertension, abdominal pain, ileus (2-3 days post repair)
- PHT if high PBF was preceding (VSD or ASD)
- Thoracic duct injury with chylothorax
- Laryngeal nerve injury
- Spinal cord injury (spinalis anterior injury): 0.4-1.5%
- Neurological injury due to deep hypothermic circulatory arrest
Perioperative mortality: 5-7%
Long term antihypertensive treatment required in 30%.
Long term survival after 10 yrs: 94% (IAA and VSD); 72% (IAA and TGA). 5 yrs: 47% (IAA and other defects)
 Critical Heart Disease in Infants and Children; 2nd ed, Nichols et al: Interrupted Aortic Arch
 Eur J Cardiothorac Surg. 2009 Apr;35(4):565-8: Kobayashi et al: Outcomes following surgical repair of aortic arch obstructions with associated cardiac anomalies