Objectives: To analyze the reasons of missed diagnosis or misdiagnosis on anomalous origin of left coronary artery from pulmonary artery (ALCAPA) by echocardiography. Methods: This is a retrospective study. Patients with ALCAPA who underwent surgical treatment in Union Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2008 to December 2021 were included. According to the results of preoperative echocardiography and surgical diagnosis, the patients were divided into confirmed group or missed diagnosis/misdiagnosis group. The results of preoperative echocardiography were collected, and the specific echocardiographic signs were analyzed. According to the experience of the doctors, the echocardiographic signs were divided into four types, namely clear displayed, vague/doubtful displayed, no display and no notice, and the display rate of each sign was calculated (display rate=number of clearly displayed cases/total number of cases×100%). By referring the surgical data, we analyzed and recorded the pathological anatomy and pathophysiological characteristics of the patients, and the rate of missed diagnosis/misdiagnosis of echocardiography in patients with different characteristics was compared. Results: A total of 21 patients were enrolled, including 11 males, aged 1.8 (0.8, 12.3) years (range 1 month to 47 years). Except for one patient with anomalous origin of left anterior descending artery, the others were all originated from the main left coronary artery (LCA). There were 13 cases of ALCAPA in infant and children, and 8 cases of adult ALCAPA. There were 15 cases in the confirmed group (diagnostic accuracy was 71.4% (15/21)), and 6 cases in the missed diagnosis/misdiagnosis group (three cases were misdiagnosed as primary endocardial fibroelastosis, two cases were misdiagnosed as coronary-pulmonary artery fistula; and one case was missed diagnosis). The working years of the physicians in the confirmed group were longer than those in the missed diagnosis/misdiagnosed group ((12.8±5.6) years vs. (8.3±4.7) years, P=0.045). In infants with ALCAPA, the detection rate of LCA-pulmonary shunt (8/10 vs. 0, P=0.035) and coronary collateral circulation (7/10 vs. 0, P=0.042) in confirmed group was higher than that in missed diagnosis/misdiagnosed group. In adult ALCAPA patients, the detection rate of LCA-pulmonary artery shunt was higher in confirmed group than that in missed diagnosis/misdiagnosed group (4/5 vs. 0, P=0.021). The missed diagnosis/misdiagnosis rate of adult type was higher than that of infant type (3/8 vs. 3/13, P=0.410). The rate of missed diagnosis/misdiagnosis was higher in patients with abnormal origin of branches than that of abnormal origin of main trunk (1/1 vs. 5/21, P=0.028). The rate of missed diagnosis/misdiagnosis in patients with LCA running between the main and pulmonary arteries was higher than that distant from the main pulmonary artery septum (4/7 vs. 2/14, P=0.064). The rate of missed diagnosis/misdiagnosis in patients with severe pulmonary hypertension was higher than that in patients without severe pulmonary hypertension (2/3 vs. 4/18, P=0.184). The reasons with an echocardiography missed diagnosis/misdiagnosis rate of≥50% included that (1) the proximal segment of LCA ran between the main and pulmonary arteries; (2) abnormal opening of LCA at the right posterior part of the pulmonary artery; (3) abnormal origin of LCA branches; (4) complicated with severe pulmonary hypertension. Conclusions: Echocardiography physicians' knowledge of ALCAPA and diagnostic vigilance are critical to the accuracy of diagnosis. Attention should be paid to the pediatric cases with no obvious precipitating factors of left ventricular enlargement, regardless of whether the left ventricular function is normal or not, the origin of coronary artery should be routinely explored.
Male ; Adult ; Infant ; Child ; Humans ; Bland White Garland Syndrome/diagnostic imaging* ; Pulmonary Artery/diagnostic imaging* ; Retrospective Studies ; Missed Diagnosis ; Hypertension, Pulmonary ; Echocardiography ; Coronary Vessel Anomalies/diagnostic imaging*

Aorta ; Constriction, Pathologic ; Coronary Angiography ; Coronary Vessel Anomalies ; Decapitation ; Humans ; Pulmonary Artery

Coronary Aneurysm ; Coronary Angiography ; Coronary Vessel Anomalies ; Humans ; Vascular Diseases/congenital*

Adult ; Anomalous Left Coronary Artery ; Coronary Angiography ; Coronary Vessel Anomalies/diagnostic imaging* ; Heart Arrest/etiology* ; Humans ; Pulmonary Artery/diagnostic imaging*

coronary artery from the left coronary sinus (R-ACAOS) with an interarterial course, assess the relationship of CT-FFR with the anatomical features of interarterial R-ACAOS on coronary computed tomographic angiography (CCTA), and determine its clinical relevance.MATERIALS AND METHODS: Ninety-four patients with interarterial R-ACAOS undergoing CCTA were retrospectively included. Anatomic features (proximal vessel morphology [oval or slit-like], take-off angle, take-off level [below or above the pulmonary valve], take-off type, intramural course, % proximal narrowing area, length of narrowing, minimum luminal area [MLA] at systole and diastole, and vessel compression index) on CCTA associated with CT-FFR ≤ 0.80 were analyzed. Receiver operating characteristic analysis was performed to describe the diagnostic performance of CT-FFR ≤ 0.80 in detecting interarterial R-ACAOS.RESULTS: Significant differences were found in proximal vessel morphology, take-off level, intramural course, % proximal narrowing area, and MLA at diastole (all p < 0.05) between the normal and abnormal CT-FFR groups. Take-off level, intramural course, and slit-like ostium (all p < 0.05) predicted hemodynamic abnormality (CT-FFR ≤ 0.80) with accuracies of 0.69, 0.71, and 0.81, respectively. Patients with CT-FFR ≤ 0.80 had a higher prevalence of typical angina (29.4% vs. 7.8%, p = 0.025) and atypical angina (29.4% vs. 6.5%, p = 0.016).CONCLUSION: Take-off level, intramural course, and slit-like ostium were the main predictors of abnormal CT-FFR values. Importantly, patients with abnormal CT-FFR values showed a higher prevalence of typical angina and atypical angina, indicating that CT-FFR is a potential tool to gauge the clinical relevance in patients with interarterial R-ACAOS.]]>
Angiography ; Coronary Sinus ; Coronary Vessel Anomalies ; Coronary Vessels ; Diastole ; Hemodynamics ; Humans ; Phenobarbital ; Prevalence ; Retrospective Studies ; ROC Curve ; Systole

Objective: To analysis the clinical characteristics and to summarize therapy experience of pediatric patients with cardiac syncope caused by anomalous origin of the left coronary artery from the right sinus (ALCA-R). Methods: We retrospectively analyzed the clinical data including clinical manifestations, myocardial injury biomarkers, radiological features, treatments and prognoses of pediatric patients with ALCA-R who were admitted to Beijing Children's Hospital from November 2015 to June 2018. Results: Four female patients were included in this analysis, age of onset was 7 to 14 years. All the patients presented with exercise-induced syncope and acute myocardial infarction. During the course, three patients presented with acute left heart failure, and one patient had history of sudden cardiac arrest. Laboratory data showed significant elevation of both the creatine kinase and troponin levels in four patients. All electrocardiogram (ECG) showed left main coronary artery occlusion, echocardiography suggested the possible anomalous origin of the left coronary artery in one child. Coronary CT angiography (CTA) revealed there was no coronary ostium in the left coronary sinus, and the left coronary artery had an anomalous origin from the right sinus. The left main coronary artery passed between the ascending artery and the root of the main pulmonary artery, which was compressed by these two large vessels. Two patients underwent cardiac magnetic resonance examination, which detected late gadolinium enhancement in ALCA-R with an interarterial course. Unroofing of the left coronary ostium (cut-back procedure) was performed in two patients, and the other two patients who were not operated were recommended to restrict their physical activities. During a regular follow-up period of 12-43 months, all the children survived without recurrent cardiovascular event. Conclusion: If an adolescent presents with exercise-induced syncope, acute myocardial infarction and even sudden death, and ECG shows left main coronary artery occlusion characteristics, we should consider the possibility of developmental abnormality of coronary artery, particularly the ALCA-R. Once diagnosed as ALCA-R, patients should be recommended to avoid strenuous activities，early recognition and surgical treatment are imperative for these patients.
Adolescent ; Child ; Contrast Media ; Coronary Angiography ; Coronary Vessel Anomalies ; Female ; Gadolinium ; Humans ; Retrospective Studies ; Syncope

Coronary artery fistula is an abnormal communication between the coronary artery and the cardiac chambers. In particular, an abnormal connection between the conus branch of the right coronary artery and the proximal left anterior descending coronary artery is defined as Vieussens’ arterial ring. Coronary artery fistulas are usually asymptomatic, but some can cause complications such as infective endocarditis. Here, we report a case of Vieussens’ arterial ring causing infective endocarditis with severe mitral regurgitation.
Conus Snail ; Coronary Vessel Anomalies ; Coronary Vessels ; Endocarditis ; Fistula ; Mitral Valve Insufficiency ; Pulmonary Artery

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare coronary artery anomaly and double right coronary artery (RCA) is a very rare coronary anomaly. Because patients with ALCAPA usually die within 1 year of being born due to myocardial infarction (MI) and heart failure, ALPACA is very rarely seen in adults. Here, we report an extremely rare asymptomatic case of MI, presumably caused by ALCAPA and double RCA, and provide a review of the literature. This is the first reported case of coronary artery anomaly that had both ALCAPA and double RCA.
Adult ; Bland White Garland Syndrome ; Camelids, New World ; Coronary Vessel Anomalies ; Coronary Vessels ; Heart Failure ; Humans ; Myocardial Infarction ; Pulmonary Artery

Arterio-Arterial Fistula ; epidemiology ; China ; epidemiology ; Coronary Angiography ; Coronary Artery Disease ; epidemiology ; Coronary Vessel Anomalies ; Humans ; Prevalence

Coronary arteriovenous fistula (CAVF) is a rare condition defined as an anomalous termination of the coronary arteries. The etiology of CAVF is either congenital or acquired, and iatrogenic CAVF is most commonly caused by cardiovascular surgery or percutaneous intervention. Most of the prenatally diagnosed CAVFs were related to complex heart disease, and only few cases of an isolated CAVF have been reported to date. We successfully diagnosed an isolated CAVF by fetal echocardiography at 25.3 weeks of gestation. Accurate prenatal diagnosis resulted in the prompt decision for postnatal surgical correction, and the neonate thrived well without any complications.
Arteriovenous Fistula ; Coronary Vessel Anomalies ; Coronary Vessels ; Echocardiography ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Infant, Newborn ; Pregnancy ; Prenatal Diagnosis