Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) diagnosed in children and adolescents

Jinmei Z, Yunfei L, Yue W, Yongjun Q.J Cardiothorac Surg. 2020 May 12;15(1):90. doi: 10.1186/s13019-020-01116-z.PMID: 32398101 Free PMC article.

 

Abstract

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare but potentially fatal congenital coronary anomaly associated with early infant mortality and sudden adult death. By the development or lack of coronary collateral, it can be classified as infantile or adult type. However, even with the compensatory mechanism in adult patients, there is an estimated 80 to 90% incidence of sudden death at the mean age of 35 years.

Methods: We enrolled 9 patients with ALCAPA within the age group 5 to 16 years.

Results: Only one patient developed symptoms (apsychia), whereas other patients were asymptomatic, and there was no evident left ventricular dysfunction found in any of the cases.

Conclusion: With the development of imaging techniques, asymptomatic adult-type ALCAPA patients could be identified and diagnosed in childhood or adolescence. As a potential cause of sudden death, ALCAPA should be surgically repaired soon after the diagnosis.

 

Fig. 1 a 2D on parasternal long axis view. White arrows show the dilated left ventricle (LV). b Color 2D on parasternal long axis view. White arrows show Mitral regurgitation (MR). c Color 2D on parasternal short axis view. White arrow shows the retrograde flow from the left coronary artery (LCA) into the pulmonary artery. d Color 2D on parasternal short axis view. White arrow shows the increased flow in the intraventricular collateral vessels from the RCA to the LCA

 

Fig. 2 CT coronary angiogram – 3D reconstruction shows different levels of collateral vessels from the right coronary artery (RCA) to the left coronary artery (LCA) in 5-year-old boy (a), 7-year-old girl (b), 16-year-old boy(c)

Fig. 3 Surgical steps of the re-implantation technique are shown in order. a The pulmonary artery is transected just proximal to the bifurcation; one-third circumference of the posterior pulmonary artery is excised including the ALCAPA orifice and the entire sinus, creating an ample-sized autologous flap. b By suturing the edges of this flap longitudinally with the autologous pericardial patch, a long rolled conduit is obtained. c The rolled conduit-extended left main coronary artery is re-implanted laterally to the aortic wall

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