a 49-year - old woman presented with breathlessness on exertion with gradual onset over the previous one month ; there was no other significant past medical history .
cardiac and respiratory examinations were normal , and the 12-lead electrocardiogram ( ecg ) was inconspicuous .
two - dimensional echocardiography showed an anomalous left main coronary artery ( lmca ) arising from the main pulmonary artery ( mpa ) , moderate left ventricular dysfunction , an ejection fraction of 40% , and mild mitral regurgitation .
the coronary angiogram revealed anomalous origin of the left coronary artery from the pulmonary artery ( alcapa ) .
multislice computed tomography ( ct ) coronary angiography revealed an alcapa from the posterior wall of the mpa , which bifurcated into the left anterior descending ( lad ) artery and the left circumflex artery . the right coronary artery ( rca ) was tortuous , dilated , and a rising from the aorta ( fig .
the patient was otherwise healthy and had an uneventful life history ( from childhood to adulthood ) , without any evidence of arrhythmic or syncopal attacks .
the patient underwent coronary artery bypass grafting ( cabg ) with closure of the lmca opening from inside the mpa .
after sternotomy and opening of the pericardium , we observed the rca arising from the aorta , which was hugely dilated , tortuous , and the lmca was arising from the lateral aspect of the mpa ( fig .
it was not possible to mobilize the lmca for reimplantation to the aorta , therefore we planned a cabg with closure of the left main orifice of the lmca .
the left internal mammary artery ( lima ) was harvested and cardiopulmonary bypass ( cpb ) was established with right atrial and aortic cannulation .
after aortic cross clamping , cardioplegia was given by two methods : root cardioplegia was given in the aorta for the rca and followed by left main ostial cardioplegia after opening the mpa ( fig .
the lima was grafted to the lad and the reversed saphenous vein was grafted to the second obtuse marginal artery .
the left main ostium was closed with 6 - 0 prolene sutures from inside the mpa .
aortic cross clamp was released , cabg completed , the patient was weaned from cpb , and the chest was closed .
the patient was transferred to the intensive care unit with minimal inotropes , stable hemodynamics , and an isoelectric ecg .
on postoperative day 2 , the patient had atrial fibrillation with a fast ventricular rate , which required amiodarone infusion , oral beta - blockers , and oral digoxin for rate control .
after 72 hours ( i.e. , postoperative day 5 ) , the patient converted to normal sinus rhythm and was maintained with oral amiodarone and beta - blockers . on postoperative day 8 , a ct coronary angiogram was performed , which showed blood flowing through the bypass grafts and no communication between the mpa and lmca ( fig .
first described by brooks   in 1886 , alcapa has an incidence of 1 in 300,000 live births ( 0 .
the anomaly , if left untreated , has a mortality rate approaching 90% in the first year of life . due to extensive intercoronary collaterals with the large dominant rca ,
alcapa occurs from either an abnormal septation of the conotruncus or from the persistence of the pulmonary buds combined with involution of the aortic buds that form the coronary arteries .
it usually occurs as an isolated entity , but may be associated with atrial septal defect , ventricular septal defect , and coarctation of the aorta .
alcapa is well tolerated in utero and in infancy because the pulmonary arterial pressure equals the systemic pressure , which leads to antegrade flow in the anomalous left coronary artery ( lca ) and the normal rca .
development of the extensive collateral circulation between the rca and lca during this time period determines the extent of myocardial injury and the outcome .
patients with alcapa who survive into adulthood have an enormous enlargement of the rca and extensive intercoronary collaterals .
variable clinical presentation of alcapa is seen in this adult cohort , as patients may be asymptomatic or present with mitral insufficiency , ischemic cardiomyopathy , and cardiac failure or with malignant ventricular dysrhythmias .
coronary angiography is the traditional standard method used for diagnosis ; however , cardiac ct and cardiac magnetic resonance imaging have been increasingly employed for the diagnosis of alcapa .
echocardiography serves as an important tool to aid in diagnosis , because it visualizes the abnormal origin of the lca , dilated rca , retrograde filling of the anomalous coronary , abnormal diastolic flow in the mpa , and abnormal septal or epicardial color flow signals from the collateral vessels .
examples of surgical correction methods include direct reimplantation of the left coronary into the aorta , baffle repair , interposition of the free subclavian artery , and use of the internal thoracic artery as a bypass graft .
establishment of a dual coronary system is a widely used corrective surgery to repair alcapa in adult patients .
all variants of cabg incorporate ligation of the alcapa at the pulmonary origin in order to avoid competitive flow .
administration of cardioplegia for myocardial protection is a crucial element common to all surgical techniques . in this case
, we gave cardioplegia in the aortic root and direct ostial cardioplegia for the left coronary system .
management of alcapa in adults is controversial and may consist of ligation of the anomalous left coronary artery , cabg , or direct reimplantation .
a high index of suspicion and appropriate diagnostic modalities should allow rapid diagnosis of alcapa in any patient seen with dilated congestive cardiac failure .
recently , operations that reestablish two coronary arterial circulations have dramatically improved surgical results for an otherwise fatal disease .
close and long - term follow - up of this patient is necessary to better understand the corrected natural history after surgical treatment of alcapa .
we report a case of a 49-year - old woman with alcapa who presented with dyspnea on exertion .
management involved cabg to the lad and obtuse marginal arteries , closure of the lmca ostium , and reestablishment of the dual coronary artery system .
 anomalous origin of the left coronary artery from the pulmonary artery ( alcapa ) is an extremely rare , potentially fatal , congenital anomaly with a high mortality rate in the first year of life . 
 it occurs rarely in adulthood and may appear with malignant ventricular a rrhythmia or sudden death . 
 we report a case of a 49-year - old woman with alcapa who presented with dyspnea on exertion . 
 management was coronary artery bypass grafting to the left anterior descending artery and obtuse marginal arteries , closure of the left main coronary artery ostium , and reestablishment of the dual coronary artery system . 
Case report
Discussion