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Anomalous left coronary artery (ALCAPA)


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


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).


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.


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


Mean ICU stay: 8 days.

Mortality 30 days: up to 20%

Normal cardiac function within 1 yr post surgery


[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

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