ABC | Volume 114, Nº4, Suplement, April 2020

Case Report Image Diagnosis: An Anomalous Origin of Left Coronary Artery from the Pulmonary Artery Haisong Bu 1 and Tianli Zhao 2 Second Xiangya Hospital, 1 Changsha, Hunan – China Second Xiangya Hospital - Central South University - Department of Cardiovascular Surgery, 2 Changsha – China Mailing Address: Tianli Zhao • Second Xiangya Hospital, Central South University - Department of Cardiovascular Surgery - Department of Cardiovascular Surgery, Middle Renmin Road 139, Changsha 410011 – China E-mail: zhaotianli@csu.edu.cn Manuscript received September 09, 2018, revised manuscript December 16, 2018, accepted January 23, 2019 Keywords Heart Defects,Congenital/surgery; Insufficiency Mitral Valve/ surgery; Myocardial Ischemia; Diagnostic Imaging; Magnetic Resonance Imaging/methods; Pulmonary Artery/abnormalities; Echocardiography/methods. DOI: https://doi.org/10.36660/abc.20180207 Introduction Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly with a mortality of 90% by 1 year of age without surgical intervention. 1 Nowadays the procedure of choice for correction of ALCAPA depends on the establishment of a dual coronary artery system by direct reimplantation of the anomalous left coronary artery (LCA) into the ascending aorta. However, anatomic variations of the origin of the anomalous LCA often make this aim difficult to achieve, especially in patients undergoing reoperation. Chronic ischemic mitral regurgitation (MR) develops as a consequence of coronary artery disease in the absence of primary leaflet abnormalities or chordal pathology: 2 ischemic cardiopathy causes remodeling of left ventricular geometry, displacement of papillary muscles, leaflet tethering and annular dilatation, leading to functional mitral insufficiency. The outcome of these patients represents a challenging problem for both cardiologists and cardiac surgeons. In fact, the role of mitral valve surgery (MVS) associated with coronary artery revascularization is still debated. Case report 1 11-year-old boy from a remote village in southern China, s/p mitral valvuloplasty without significant symptomatic improvement. Transthoracic echocardiography (TTE) showed an enlarged left atrium of 61 mm, moderate MR (Figure 1A), and an ejection fraction of 60%. The right coronary artery (RCA) diameter was increased to 7 mm at the proximal end (Figure 1B), and the LCA was not from a left coronary sinus (Figure 1C) and inserted into the pulmonary artery through a 5-mm fistula (Figure 1D). Three-dimensional coronary artery computed tomography angiography (CTA) showed an ALCAPA (Figure 2B and D) with a giant and twisted RCA (Figure 2A and C). Ascending aorta angiography showed dilated and twisted RCA (Figure 3A and B) and ALCAPA. The direction of coronary blood flow was RCA-communicating branch-LCA-pulmonary artery (Figure 3C and D) and hence there was a coronary steal. Case report 2 A 9-year-old Chinese boy status post mitral valve replacement for about seven years presented with repeated fever and exertional dyspnea and referred to our department. TTE showed an enlarged left atrium of 58mm, moderate mitral parabasilar leak, and an ejection fraction of 62%. The RCA diameter was increased to 5 mm at the proximal end, and the LCA was not from left crown sinus. Three-dimensional coronary artery CTA showed an ALCARPA (Figure 4B and C) with a giant RCA (Figure 4A, B and C). Ascending aorta angiography showed dilated and twisted RCA (Figure 4D). There was a phenomenon of stealing blood. The direction of coronary blood flow was from RCA-communicating branch‑LCA-pulmonary artery (Figure 4E and F). Result and conclusion ALCAPA is a rare congenital anomaly with a mortality of 90% by 1 year of age without surgical intervention. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. 3 Adult survivors, however, are either asymptomatic or present with dyspnea, angina, MR, myocardial ischemia, or ventricular arrhythmia, pulmonary hypertension, and sudden death. This contrasts with the clinical presentation of myocardial ischemia and infarction (palpitations, angina and fatigue) in children, and failure to thrive, irritability, excessive sweating, and listlessness in babies. 4,5 During the infancy phase, there is a decrease in pulmonary pressures and decline in oxygen levels leading to decreased coronary perfusion and ischemia, especially during feeding or crying when myocardial oxygen demand is increased. Chronic mycardial ischemia leads to impaired function not only of the myocardium, but often also of the mitral valve apparatus with cardiac failure and mitral valve regurgitation following. If this phase is tolerated, then compensatory changes occur over time and the myocardium remodels during in children. Due to the development of intercoronary collaterals from the increasingly large RCA providing collateral supply to the LCA leads to a reversal of flow from the anomalous left coronary into the pulmonary artery. 3 Finally, there are excessive collateral vessels that lead to the shunting of blood from the RCA via collaterals to the LCA and into pulmonary artery during in adults. 3,5 This is seen as an example of a phenomenon of stealing blood. 4