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Objective: Although studies have compared fetal echo results with autopsy findings, investigations that compared multiple categories of congenital heart disease (CHD) are lacking. This study, therefore, aimed to compare fetal echocardiographic diagnoses with cardiac autopsy findings and evaluate the diagnostic accuracy of fetal echocardiography (FE).Methods: One hundred seventy-one specimens from fetuses diagnosed with CHD were collected after termination of pregnancy, and fetal autopsies were performed. FE and autopsy diagnoses were compared and the degree of their correspondence was categorized as "complete agreement" (FE results were in accordance with autopsy findings), "minor discrepancies" (autopsies verified the main FE diagnoses but added and/or revised some minor information), or "discordance" (autopsy findings were different from the primary diagnoses of FE).Results: The "complete agreement" group accounted for 87.1% (149/171) of the total specimens. In 11.7% (20/171) of cases, autopsies disclosed new deformities and/or revised some echo results (minor discrepancies group). Minor abnormalities were frequently embodied in small septal defects and vascular malformations. A rare malformation of common pulmonary vein atresia was confirmed by autopsy in two fetuses, but both were misdiagnosed by FE (discordance group).Conclusions: Fetal echocardiographic diagnoses were mostly consistent with autopsy findings. The diagnostic discrepancies mainly consisted of rare cases and minor abnormalities missed or misdiagnosed by FE. Autopsies may help confirm, modify, or add information to prenatal echo results. They may also help sonographers have a better understanding of the anatomic structures of CHD, especially for rare lesions, which could further improve the diagnostic accuracy and integrity of FE. [ABSTRACT FROM AUTHOR]

Background: Idiopathic cardiac osseous metaplasia in the right atrium of a 9-year-old boy, accompanied by right atrial Chiari network and right pulmonary artery embolism. This case is rare and can easily be misdiagnosed., Case Description: We encountered a case of a 9-year-old boy with a 3.5 cm diameter neoplasm in the right atrium. Preoperative imaging diagnosis could not determine the nature of the tumor, and the initial clinical suspicion of cardiac myxoma. After admission, a cardiotomy to remove foreign bodies and a pulmonary artery thrombectomy were performed., Conclusions: Idiopathic cardiac osseous metaplasia is relatively rare, and it is even rarer to be accompanied by a Chiari network in the right atrium. Due to the location and characteristics of the lesion in this case, it is easy to be misdiagnosed as atrial myxoma in clinical practice. Whether it is idiopathic osseous metaplasia or myxoma, it needs to be performed surgical treatment and pathological examination can easily rule out the diagnosis of myxoma. However, as idiopathic cardiac metaplasia is difficult to encounter in clinical work and there are few reports in the literature, clinicians and pathologists need to consult more relevant literature. Learn to understand and master the disease through multi-party consultation., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-23-168/coif). The authors report that the study was supported by the Beijing Hospitals Authority InnovationStudio of Young Staff Funding Support (No. 202114). The authors have no other conflicts of interest to declare., (2024 AME Case Reports. All rights reserved.)

Objective: To improve the diagnostic accuracy of fetal pulmonary venous abnormalities through the analysis of the fetal pulmonary vein anatomy., Methods: 234 cases of congenital cardiac abnormalities were detected by echocardiography during pregnancy in An Zhen Hospital, Capital Medical University from May 2010 to August 2015. Autopsy was then performed. The type of fetal pulmonary venous malformation, cardiac abnormalities, systemic venous malformations, and other internal organs deformities were documented., Results: There were ninteen cases of pulmonary venous malformations among the 234 cases of fetal congenital heart disease. These included two cases of congenital pulmonary venous hypoplasia (CPVH) or atresia, four cases of partial anomalous pulmonary venous drainage (PAPVD), seven cases of total anomalous pulmonary venous drainage (TAPVD), five cases of atresia of common pulmonary vein (CPV), one case of congenital pulmonary venous hypoplasia with total anomalous pulmonary venous drainage. There were eleven cases with single ventricle, eight cases with right aortic arch, seven cases with single atrium and six cases with pulmonary valve stenosis. Eleven cases had pulmonary hypoplasia and nine cases had abnormal spleen., Conclusions: There are many variations in pulmonary venous abnormalities associated with severe and complex cardiac abnormalities and internal organs malformation. Care should be exercised during autopsy examination to look for all branches of the pulmonary vein.

Objective: To summarize the echocardiograhic features of atrial appendage aneurysm (AAA) in fetuses, children and adults and evaluate the value of echocardiography in the diagnosis of AAA., Methods: The AAA echocardiographic features of 6 fetuses, 1 child and 4 adults were retrospectively analyzed. And the diagnoses were confirmed by autopsy, postnatal follow-ups, computed tomography (CT) and surgery., Results: Left atrial appendage aneurysm was more common than right atrial appendage aneurysm. AAA had the same echocardiographic characteristics in fetuses, children and adults. And it was usually combined with other complex cardiac malformations in fetuses while single cardiac abnormality was predominant in children and adults., Conclusion: Fetuses, children and adults with AAA may be found by echocardiography in time so that echocardigraphy has an important role in diagnosing AAA.

Objective: To investigate clinicopathological features, immunophenotype and differential diagnosis of intravenous leiomyomatosis with intracardiac extension., Methods: Clinical manifestations, morphologic features, and immunohistochemical staining were retrospectively analyzed in 19 cases of intravenous leiomyomatosis with intracardiac extension., Results: The patients' age ranged from 33 to 59 years (mean 44 years). Clinical presentation included chest tightness, palpitation, dyspnea, edema of low extremity, abdominal distention or hypermenorrhea. However, a few patients were asymptomatic. Grossly, intravenous leiomyomatosis in most cases demonstrated coiled or nodular growth within the myometrium with worm-like involvement of the uterine vein in broad ligament or other pelvic veins with continued extension into the vena cava and the heart. The intravenous tumor surface was generally smooth and rubbery, with a greyish-white color. Microscopically, the tumors were composed of spindle cells with rare mitotic figures and the presence of abundant thick-walled vessels. Marked fibrosis, hyalinization, myxoid and edematous changes were common. The tumor cells were positive for SMA, ER, PR and desmin but negative for HMB45 and S-100. CD10 and CD34 were positive in 4 patients and negative in the remaining cases., Conclusion: Intravenous leiomyomatosis with intracardiac extension is a rare disease among women of child-bearing age, with specific morphologic manifestations and immunohistochemical profiles.