test 5 anomalous left coronary atery to pulmonary artery (ALCAPA)

Question 1

anomalous left coronary atery to pulmonary artery (ALCAPA)

Answer 1

• Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA)
• Rare malformation in which the left coronary artery originates from the pulmonary artery
• Anomaly leads to severe coronary hypoperfusion and left ventricular dysfunction when PVR falls in the postnatal period.

Question 2

Anomalous left coronary artery

Answer 2

• Can also connect to aorta but connection is with in the right aortic sinus
• LCA can pass between the aorta and the pulmonary valve
• LCA can pass anterior to the pulmonary artery

Question 3

anomalous left coronary atery to pulmonary artery (ALCAPA) pathophys

Answer 3

• In fetal/early neonatal life, the left coronary artery (LCA) from the pulmonary artery (PA) is well tolerated
▫ PAP = systemic pressure (leading to antegrade flow in both the anomalous LCA and the normal RCA)
• When PAP < Systemic pressure, flow in the LCA decreases and then reverses, which leads to myocardial ischemia and
infarction
• As long as PVR is high, coronary perfusion is maintained
▫ Perfusion with desaturated blood is better than no flow through the LCA
▫ When pulmonary artery pressure falls, perfusion of the left coronary artery becomes inadequate.

Question 4

If the LCA is dominant and if intercoronary collaterals are inadequate

Answer 4

• severe left ventricular dysfunction with ischemic mitral regurgitation develops; the prognosis is poor.

Question 5

If the RCA is dominant and if collaterals develop efficiently

Answer 5

• normal LCA perfusion may be maintained while left-to-right shunt from the right coronary artery to the left coronary artery and the pulmonary artery progressively increases
▫ But can steal flow from the cardiac muscle

Question 6

Surgical Technique

Answer 6

• Surgical correction performed on making a diagnosis of ALCAPA syndrome is considered to be the standard treatment.
• The aim of surgery is to restore a two-coronary artery circulation system
• 2 Techniques are utilized

Question 7

Surgical technique: Coronary Reimplantation

Answer 7

• remove the button of the coronary artery from the pulmonary artery and attach it to the aorta

Question 8

Surgical technique: Takeuchi procedure (creating an A-P window)

Answer 8

• A transpulmonary baffle (tunnel) between the coronary ostium in the PA and the Ao is created.
• Baffles (tunnels) blood across Aorta -> PA

Question 9

anomalous left coronary atery to pulmonary artery (ALCAPA) cannulation

Answer 9

• Aortic Arterial cannulation
• Bicaval cannulation: (open procedure)
• Hypothermia: Normothermic cardiopulmonary bypass
▫ Althoughmoderate hypothermia may be necessary to allow low flow bypass if needed
• LV Vent: a left ventricular vent is inserted through the superior right pulmonary vein (RSPV) and both pulmonary arteries are snared to avoid runoff of coronary perfusion into the pulmonary circulation.

Question 10

anomalous left coronary atery to pulmonary artery (ALCAPA) cardioplegia

Answer 10

• Cardioplegia: Aortic root + ostial:
• The first cardioplegic administration is performed in the aortic root (and thus right coronary artery) and it is completed by direct administration into the anomalous LCA (ostial)
• Both RCA and LCA ostial are given for maintenance doses

Question 11

Coming off bypass when they have anomalous left coronary atery to pulmonary artery (ALCAPA)

Answer 11

• It is very useful to create a small atrial septal defect as a way to unload the failing left ventricle during the early postoperative period.
• After aortic unclamping, a LA line is inserted and weaning from cardiopulmonary bypass is prepared.
• However, cardiopulmonary bypass must be prolonged for a while until left atrial pressure (which is initially high) reaches an acceptably low level, allowing weaning with a moderate inotropic support.
• If this is not the case, a left heart VAD or ECMO must be done to allow cardiac assistance for a few days until left ventricular function recovers enough to allow weaning in good hemodynamic conditions.