Misdiagnosis of anomalous origin of the left coronary artery from the pulmonary artery by echocardiography: Single‐center experience from China

Abstract

Objectives To identify the risk factors causing misdiagnosis by echocardiography and missed diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). Materials and Methods Echocardiographic results of 16 patients with ALCAPA confirmed by surgery in Union Hospital, Wuhan, were analyzed retrospectively. The influencing factors leading to echocardiographic misdiagnosis were analyzed from the aspects of confusing image characteristics, special pathological anatomy of the left coronary artery (LCA), and operators' working years. Results Echocardiography diagnosed 11 cases with an accuracy rate of 68.8%. Five cases were misdiagnosed, three cases as endocardial fibroelastosis, one case as mitral prolapse with severe insufficiency, and one case as coronary‐pulmonary artery fistula. Display rate of the specific echocardiographic features for confirmed group and misdiagnosed group was statistically significantly different（P = .014）. But the working years of the operator for confirmed group and misdiagnosed group were not statistically significantly different（P = .267）. Some special pathological anatomy and pathophysiological features could be also the cause of misdiagnosis. Conclusions Echocardiography is the first diagnostic choice of the ALCAPA in China. It is essential for the operator to have the knowledge, diagnostic awareness, and proficiency in manipulation in the accurate interpretation of echocardiography results.

Case Report: Neonatal Complex Congenital Heart Disease With Anomalous Origin of the Left Coronary Artery From the Right Pulmonary Artery: Analysis of Missed Diagnosis and Improvement Procedures

“…The direct signs include that the left coronary artery is not connected with the left coronary sinus of the aorta, but with the pulmonary artery, and the blood flow of the left coronary artery retrogradely enters the pulmonary artery in diastole. Indirect signs include compensatory dilation of the right coronary artery, the formation of left and right coronary collateral circulation in the myocardium, left ventricular enlargement, mitral insufficiency, and endocardial elastic fiber hyperplasia (7,8). In the neonatal period, due to the existence of pulmonary hypertension, the blood supply of the ALCAPA mainly or completely comes from the pulmonary artery (8).…”

“…If severe left ventricular dysfunction occurs after the operation, it is necessary to be vigilant and look for evidence of the ALCAPA. An exploration of the coronary artery is mainly recommended for children with complex CHD in the neonatal period and children with CHD with unexplained cardiac insufficiency and abnormal mitral valve development, which may be indirect signs of the ALCAPA (1,7,8). The above improvement procedures are strongly recommended in neonatal complex CHD surgery, which can avoid the missing diagnosis of the ALCAPA.…”

Echocardiographic Diagnosis of Bland-White-Garland Syndrome in an Asymptomatic Adult

“…Although it most commonly presents in infancy, 10% of children with this congenital anomaly will survive to adulthood. Case reports on adults with ALCAPA show that their first presentation is on a spectrum of being asymptomatic to acute heart failure, myocardial ischemia, and sudden cardiac death ( 3 , 4 ). The degree of collateral formation between the RCA and LCA serves as a significant predictor for symptoms.…”

Imaging options for defining anomalous origin of the coronary artery from the pulmonary artery

“…Extensive left ventricular fibrosis was observed on 2D echocardiography in some ALCAPA patients, particularly in the subendocardial region ( 8 , 24 ). Attention should be paid to distinguishing this from endocardial fibroelastosis ( 22 , 25 , 26 ), a CHD caused by an increase in collagen and elastic fibers and characterized by thickening of the endocardium, enlargement of the cardiac chambers, and decreased myocardial motor function.…”