Discussion.
ALCAPA is a very rare condition in which the left main coronary artery originates on the pulmonary artery. The decreased flow into the left coronary arteries due to coronary artery steal from the low pulmonary artery resistance leads to ischemia and infarction of the myocardial tissue an usually is lethal if is not corrected as an infant (3).
The infant type of ALCAPA is considered to be very fatal if untreated with a 1-year mortality of approximately 90% (5). Infants commonly present with failure to thrive, breathing difficulties, irritability, and distress while crying or eating. The adult type of ALCAPA is related to sudden cardiac death and symptoms of ischemia, mitral valve regurgitation, and left ventricular dysfunction (3,4).
The treatment of the ALCAPA infant type is absolutely surgically with either two-coronary system coronary button transfer, Takeuchi procedure (trans- pulmonary baffling), coronary artery bypass grafting (CABG) combined with ligation of the anomalous left coronary artery (4,5). ALCAPA Adult type treatment is sometimes controversial, although the preferred approach is surgical including CABG combined with anomalous left coronary artery ligation and patch of the pulmonary artery (4,5). This approach should only be considered if the patient adult has or develops evidence of ischemia, severe mitral regurgitation or heart failure secondary to a significant shunt and volume overload (4).
Currently there are no guidelines or gold standard management for patients with adult form of ALCAPA that present with minimal to non-disease of the myocardium? How can we monitor the disease course and possibly risk stratify patients for possible complications? Is there a screening benefit for patients with high probability of ALCAPA? How much real benefit does the surgical intervention really offers to this patient’s population group? What type of conduit is appropriate if CABG planned vein or double mammary. This case raises questions regarding the management of this rare congenital coronary anomaly
Reference:
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2.- Arciniegas E, Farooki ZQ, Hakimi M, Green EW. Management of anomalous left coronary artery from the pulmonary artery. Circulation 1980;62(2 pt 2):I180–I189.
3- Fernandes ED, Kadivar H, Hallman GL, Reul GJ, Ott DA, Cooley DA. Congenital malformations of the coronary arteries: the Texas Heart Institute ex- perience. Ann Thorac Surg 1992;54:732–740.
4.- H. Wesselhoeft, J.S. Fawcett, A.L. Johnson. Anomalous origin of the left coronary artery from the pulmonary trunk: Its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases. Circulation, 38 (1968), pp. 403-425.
5.- Ishihata T, Takeda H, Katohno E, et al. An adult case of Bland-White-Garland syndrome with col- laterals from the bronchial artery. Heart Vessels 1994;9:218–222.
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