Anomalous left coronary artery (ALCAPA)

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Definition: anomalous origin of left coronary artery (LCA) from pulmonary artery; also known as Bland-White-Garland syndrome; reaches 100% mortality if untreated. Prevalence: 0.2-0.5 % in all congenital heart defects.

Incidence: 1:300,000

ARCAPA (anomalous right coronary artery from pulmonary artery) possible


Physiology:

High PVR in the infant period supports an appropriate LCA perfusion pressure; while PVR falls → coronary steal from LCA → myocardial ischaemia, papillary muscle infarction, MR (left sided coronary blood flow depending on collaterals and dilated right coronary artery).


Diagnosis:

Average onset at 6 months to 1 year with poor weight gain, exercise intolerance, CHF, ECG abnormalities (Q wave, negative T, inversed T, LVH, MI), Echo (WMA, MI, MR), angiogram.


Preoperative management:

Treatment of congestive heart failure.


Preoperative preparation:

ECG, CXR, CUS, FBE, clotting, UECs, PRBC (4), FFP (2), platelets (2), cryoprecipitate (2).

Methylprednisolone 10mg/kg 12 hrs and 6 hrs pre surgery in neonates.


Surgery:

Excision of ALCAPA and aortic reimplantation (direct or via tunnel – Takeuchi operation).


Postoperative management:

  • Keep intubated, ventilated, sedated and paralysed for 24-48 hrs
  • Inotropes: milrinone plus dopamine or adrenaline (plus noradrenaline, aim MAP >40 mmHg in neonates), aim for an appropriate coronary perfusion pressure
  • Haemodynamics: age adjusted, SBP >60 mmHg, DBP >30 mmHg, MAP >40 mmHg in neonates (increasing over time), CVP 8-12 mmHg, LAP 8-12 mmHg
  • Respiratory: normoxaemia, normocapnea
  • Fluid restriction: 1ml/kg/hr
  • Haemostasis

Specific problems:

  • Low CO (most common): keep paralysed, don’t wean inotropes <24 hrs) → early mechanical assist!
  • Anatomical coronary artery problems (check ECG, troponin).  Early investigations (echo, Cathlab)
  • Coronary artery spasm (start GTN 5-10 mcg/kg/min)
  • Arrhythmia: SVT (adenosine, digoxin), JET (slow amiodarone), bradycardia (pacing), AV block (pacing)
  • Mitral valve regurgitation → functional MR, treat underlying cause, correct arrhythmia, decrease SVR
  • Low urine output: start PD

Outcome:

Mean ICU stay: 8 days.

Mortality 30 days: up to 20%

Normal cardiac function within 1 yr post surgery


References:

[1] Am Heart J 8:787-801; 1993: Bland et al: Congenital anomalies of the coronary arteries: report of an unusual case associated with cadiac hypertrophy

[2] Interact Cardiovasc Thorac Surg. 2010 Jan;10(1):70-5: Ojala et al: Excellent functional result in children after correction of anomalous origin of left coronary artery from the pulmonary artery–a population-based complete follow-up study

[3] Interact Cardiovasc Thorac Surg. 2013 Mar 7: Kazmierczak et al: Repair of anomalous origin of the left coronary artery from the pulmonary artery in infants


 All Marc’s PICU cardiology FOAM can be found on PICU Doctor and can be downloaded as a handy app for free on iPhone or AndroidA list of contributors can be seen here.

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