Anomalous Left Coronary Artery from the Pulmonary Artery: How to Diagnose and Treat

Abstract

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary anomaly that can present in childhood or adulthood with a multitude of symptoms depending on the age of presentation. It should be suspected in infants presenting with heart failure in the setting of left ventricular systolic dysfunction and associated mitral regurgitation from papillary muscle ischemia. Adults with ALCAPA may present with cardiac ischemic symptoms. Prompt diagnosis with echocardiography and cross-sectional chest imaging is important to guide surgical intervention and improve the patients' survival and prognosis. The goal of surgery is to establish a dual-coronary system with mid-term results revealing progressive recovery of left ventricular function and improvement in mitral regurgitation. Patients with ALCAPA should maintain life-long follow-up with a cardiologist with congenital heart disease expertise for surveillance of post-operative complications.

Keywords: adult congenital heart disease; anomalous coronary artery; anomalous left coronary artery from the pulmonary artery (ALCAPA).

Figure 4

Electrocardiogram of anomalous left coronary artery from the pulmonary artery reveals Q waves and ST depressions and T wave changes in the anterolateral leads (leads I, AVL, V5, and V6). Open access figure from Al-Fayyadh, M.; Alwadai, A.; Al Huzaimi, A.; Al Halees, Z. Near missed reversible cardiomyopathy: The value of the electrocardiogram. Int. J. Pediatr. Adolesc. Med. 2015, 2, 29–33. [16].

Figure 5

Computed tomography of the heart revealing anomalous left coronary artery from the pulmonary artery (blue) as well as the dilated and tortuous right coronary artery.

Figure 1

(a) Selective angiography of the right coronary artery (white arrow) revealing a dilated and tortuous vessel with multiple collaterals to the left coronary artery. (b) The injected intrave-nous contrast spills from the left coronary artery into the main pulmonary artery (MPA; asterisks), confirming anomalous left coronary artery from the pulmonary artery. Used with permission from Crean, A.; Ahmed, F.; Motwani, M. The Role of Radionuclide Imaging in Congenital Heart Disease. Curr. Cardiovasc. Imaging Rep. 2017, 10, 38. https://doi.org/10.1007/s12410-017-9434-0; Executive Summary. J. Am. Coll. Cardiol. 2019, 73, 1494–1563. [12].

Figure 2

Echocardiography of anomalous left coronary artery from the pulmonary artery (ALCAPA) obtained from the parasternal short axis view at the base of the heart reveals anomalous origin of the left coronary artery (orange arrow) from the posterior aspect of the pulmonary artery (PA) far from the aorta (Ao). It divides into the left anterior descending artery (LAD) and left circumflex artery (Cx). Used with permission from Eidem, B. W.; Cetta, F.; O’Leary, P. W. Echocardiography in Pediatric and Adult Congenital Heart Disease, 3rd ed.; Wolters Kluwer: Alphen am Rhein, The Netherlands, 2021; pp. 514–530. [13].

Figure 3

Echocardiography of anomalous left coronary artery from the pulmonary artery show-ing retrograde flow in the left anterior descending (LAD) coronary artery. Flow in the LAD is blue as it moves away from the transducer toward the pulmonary artery (PA), which is abnormal be-cause it should flow away from the aortic root (red Doppler signal) rather than toward it. A turbu-lent flow signal (arrows) is also seen in the pulmonary artery as the anomalous left coronary ar-tery empties into the low-pressure pulmonary artery. Used with permission from Eidem, Benjamin W., Jonathan Johnson, Leo Lopez and Frank Cetta. Echocardiography in Pediatric and Adult Congenital Heart Disease. Available from: Wolters Kluwer, (3rd Edition), 2021: 514-530. [13].

Figure 6

Schematic diagram outlining the coronary button transfer technique. (a) The main pulmonary artery (MPA) is transected with harvesting of the coronary button. (b,c) The MPA is reconstructed with a pericardial patch at the site of coronary button excision, and an aortotomy is created in the posterolateral aortic wall. (d) The left coronary artery is reimplanted end-to-side to the posterolateral aorta. (e) Re-anastomosis of the MPA. Used with permission from Mishra, A. Surgical management of anomalous origin of coronary artery from pulmonary artery. Indian J. Thorac. Cardiovasc. Surg. 2021, 37, 131–143. [19].

Figure 7

(A) The main pulmonary artery is transected above the origin of the left coronary artery (LCA). (B) The pulmonary artery flap and aortic trap door are designed as shown. (C) The LCA is anastomosed to the aortic trap door anteriorly and directly to the aorta posteriorly. (D) The pulmonary artery is repaired with autologous pericardium and the main pulmonary artery is reconstructed. LCA: left coronary artery; RC and RCA: right coronary artery. Used with permission from Kim YS, Lee M, Cho YH, Yang JH, Jun TG. An alternative surgical technique for repair of anomalous origin of the left coronary artery from the pulmonary arteryKorean J Thorac Cardiovasc Surg. 2014 Jun; 47(3):220-4. [21].

Figure 8

Three-dimensional-reconstructed image from coronary computed tomography angiogram demonstrating intrapulmonary baffle following Takeuchi repair method. Used with permission from Cramer, J.W.; Cinquegrani, M.; Cohen, S.B. Takeuchi repair of anomalous left coronary artery from the pulmonary artery. J Cardiovasc. Comput. Tomogr. 2015, 9, 457–458. [22].