Prognostic value of left ventricular global longitudinal strain for major adverse cardiovascular events in patients with aortic valve disease：a meta-analysis.

INTRODUCTION: Valvular heart disease is one of the most common heart diseases. It is characterized by abnormal function or structure of the heart valves. There may be no clinical symptoms in the early stages. Clinical symptoms of arrhythmia, heart failure or thromboembolic events may occur in the late stages of the disease, such as palpitation after activities, breathing difficulties, fatigue and so on. Aortic valve disease is a major part of valvular heart disease. The main treatment for aortic valve disease is valve replacement or repair surgery, but it is extremely risky. Therefore, a rigorous prognostic assessment is extremely important for patients with aortic valve disease. The global longitudinal strain is an index that describes the deformation capacity of myocardium. There is evidence that it provides a test for systolic dysfunction other than LVEF (left ventricular ejection fraction) and provides additional prognostic information. METHOD: Search literature published between 2010 and 2023 on relevant platforms and contain the following keywords: "Aortic valve disease", "Aortic stenosis", "Aortic regurgitation" and "longitudinal strain" or "strain". The data is then extracted and collated for analysis. RESULTS: A total of 15 articles were included. The total population involved in this study was 3678 individuals. The absolute value of LVGLS was higher in the no-MACE group than in the MACE group in patients with aortic stenosis (Z=8.10, P<0.00001), and impaired LVGLS was a risk factor for MACE in patients with aortic stenosis (HR=1.14, P<0.00001, 95% CI: 1.08-1.20). There was also a correlation between impaired LVGLS and aortic valve surgery in patients with aortic valve disease (HR=1.16, P<0.0001, 95% CI: 1.08-1.25) or patients with aortic valve regurgitation (HR=1.21, P=0.0004, 95% CI: 1.09-1.34). We also found that impaired LVGLS was no significant association between LVGLS and mortality during the period of follow-up in patient with aortic valve stenosis (HR =1.08, 95%CI: 0.94-1.25, p=0.28), but it was associated with mortality in studies of prospective analyses (HR =1.34, 95%CI:1.02-1.75, p=0.04). CONCLUSIONS: Impaired LVGLS correlates with major adverse cardiovascular events in patients with aortic valve disease, and it has predictive value for the prognosis of patients with aortic valve disease.

Aortic sinus aneurysm invading ventricular septum and dissection caused by Behcet's disease: a case report and literature review.

Few case reports have mentioned the aortic sinus aneurysm invading ventricular septum and dissection caused by Behcet's disease. Here, we reported a 36-year-old male patient with an aortic sinus aneurysm invading the ventricular septum and dissection caused by Behcet's disease, who manifested as recurrent chest tightness and shortness of breath. Cardiac ultrasound showed the rupture of the right aortic sinus and the formation of ventricular septal dissection. Ascending aortic valve prosthesis replacement, mitral valvuloplasty with ring implantation and tricuspid valvuloplasty were performed. Postoperatively, he was treated with hormones, hydroxychloroquine sulfate, mycophenolate mofetil tablets, thalidomide and warfarin, and his symptoms were relieved. This is a rare case easily being misdiagnosed and missed, early diagnosis and in-time treatment are crucial to avoid surgical complications. The diagnostic and therapeutic approaches of this patient were reported and related literature was reviewed in this case report.

Evaluation of Right Ventricular Myocardial Mechanics by 2- and 3-Dimensional Speckle-Tracking Echocardiography in Patients With an Ischemic or Non-ischemic Etiology of End-Stage Heart Failure.

Background: The aims of our study were (1) to assess the right ventricular (RV) myocardial mechanics by two-dimensional (2D) and three-dimensional (3D) speckle-tracking echocardiography (STE) in patients with an ischemic or non-ischemic etiology of end-stage heart failure (HF) and (2) to explore which RV index evaluated by 2D- and 3D-STE was the most powerful indicator for identifying the ischemic and non-ischemic etiologies of end-stage HF. Methods: A total of 96 patients with left ventricular ejection fraction (LVEF) < 30% were enrolled in our study: 42 patients (mean age, 52 ± 10 years; 9.5% female) with ischemic cardiomyopathy and 54 patients (mean age, 46 ± 14 years; 16.7% female) with non-ischemic cardiomyopathy. A total of 45 healthy subjects (mean age, 46 ± 13 years; 24.4% female) served as controls. The longitudinal strain of the RV free wall (RVFWLS) was determined by both 2D- and 3D-STE. Results: Compared to controls, patients with an ischemic or non-ischemic etiology of end-stage HF had lower 2D-RVFWLS, 3D-RVFWLS and RV ejection fraction (RVEF) values (P < 0.05). Patients with non-ischemic cardiomyopathies (NICMs) had significantly lower 3D-RVFWLS and RVEF values than in those with ischemic cardiomyopathies (ICMs), whereas 2D-RVFWLS and conventional RV function parameters did not differ between the two subgroups. RVEF was highly related to 3D-RVFWLS (r = 0.72, P < 0.001), modestly related to 2D-RVFWLS (r = 0.51, P < 0.001), and weakly related to conventional RV function indices (r = -0.26 to 0.46, P < 0.05). Receiver operating characteristic curve analysis revealed that the optimal 3D-RVFWLS cut-off value to distinguish NICM from ICM patients was -14.78% (area under the curve: 0.73, P < 0.001), while 2D-RVFWLS and conventional RV echocardiographic parameters did not. Conclusion: Our study demonstrated the superiority of 3D-RVFWLS over 2D-RVFWLS and conventional RV function indices in identifying the ischemic and non-ischemic etiologies of end-stage HF. These findings support the idea that 3D-RVFWLS may be a promising non-invasive imaging marker for distinguishing NICM from ICM.

Comparison of fetal echocardiogram with fetal cardiac autopsy findings in fetuses with congenital heart disease.

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.

Multiple Primary Cardiac Malignant Peripheral Nerve Sheath Tumors in the Left Atrium: Case Report.

Malignant peripheral nerve sheath tumors are rare sarcomas of the heart. Herein, we report the case of a 24-year-old man who complained of dyspnea, cough, and upper left back pain. He was found to have multiple primary heart tumors obstructing the right superior pulmonary vein in the left atrium, which were diagnosed as malignant peripheral nerve sheath tumors. The patient underwent successful resection of the tumors and immunohistochemistry was utilized for diagnosis.

Expanding the phenotype of STRA6-related disorder to include left ventricular non-compaction.

BACKGROUND: Syndromic microphthalmia-9 (MCOPS9) is a rare autosomal recessive disorder caused by mutations in STRA6, an important regulator of vitamin A and retinoic acid metabolism. This disorder is characterized by bilateral clinical anophthalmia, pulmonary hypoplasia/aplasia, cardiac malformations, and diaphragmatic defects. The clinical characteristics of this disorder have not been fully determined because of the rarity of clinical reports. METHODS: A comprehensive genotyping examination including copy number variation sequencing (CNV-Seq) and whole-exome sequencing (WES) was applied to a fetus of Han Chinese with bilateral anophthalmia, bilateral pulmonary agenesis, interrupted aortic arch type A, and left ventricular non-compaction (LVNC). RESULTS: No aneuploidy or pathogenic CNV were identified by CNV-seq. WES analysis revealed a previously reported homozygous splice site (NM_022369.4:c.113+3_113+4del) in the STRA6 gene. This variant was confirmed by Sanger sequencing. The diagnosis of MCOPS9 was confirmed given the identification of the STRA6 mutation and the association of bilateral anophthalmia, pulmonary agenesis, and cardiac malformations. CONCLUSION: This case adds to the phenotypic spectrum of MCOPS9, supporting the association with LVNC, and the presence of interruption of aortic arch further demonstrates the variability of the cardiac malformations.

DW-Net: A cascaded convolutional neural network for apical four-chamber view segmentation in fetal echocardiography.

Fetal echocardiography (FE) is a widely used medical examination for early diagnosis of congenital heart disease (CHD). The apical four-chamber view (A4C) is an important view among early FE images. Accurate segmentation of crucial anatomical structures in the A4C view is a useful and important step for early diagnosis and timely treatment of CHDs. However, it is a challenging task due to several unfavorable factors: (a) artifacts and speckle noise produced by ultrasound imaging. (b) category confusion caused by the similarity of anatomical structures and variations of scanning angles. (c) missing boundaries. In this paper, we propose an end-to-end DW-Net for accurate segmentation of seven important anatomical structures in the A4C view. The network comprises two components: 1) a Dilated Convolutional Chain (DCC) for "gridding issue" reduction, multi-scale contextual information aggregation and accurate localization of cardiac chambers. 2) a W-Net for gaining more precise boundaries and yielding refined segmentation results. Extensive experiments of the proposed method on a dataset of 895 A4C views have demonstrated that DW-Net can achieve good segmentation results, including the Dice Similarity Coefficient (DSC) of 0.827, the Pixel Accuracy (PA) of 0.933, the AUC of 0.990 and it substantially outperformed some well-known segmentation methods. Our work was highly valued by experienced clinicians. The accurate and automatic segmentation of the A4C view using the proposed DW-Net can benefit further extractions of useful clinical indicators in early FE and improve the prenatal diagnostic accuracy and efficiency of CHDs.

Echocardiographic features of interventricular septal dissection in patients with Behçet's disease.

Involvement of the heart in Behçet's disease (BD) is rare. We retrospectively analyzed these three patients with interventricular septal (IVS) dissection in BD and discussed the echocardiographic manifestations of IVS dissections. In our patients, the echocardiographic characteristics of IVS dissection were echo-free space in the IVS basal segment or basal to middle segment, dilatation in the diastole and contraction in systole, and abnormal turbulent blood flow in the heart.

The prenatal diagnosis of aortopulmonary window by fetal echocardiography.

OBJECTIVE: The purpose of this study was to analyze the fetal echocardiographic features and the associated anomalies of prenatal aortopulmonary window (APW). METHODS: We retrospectively reviewed the fetal echocardiographic database (n = 24 000) in our hospital between May 2012 and December 2017. The general clinical information, fetal echocardiographic features, and the associated anomalies in patients with APW were analyzed. Four patients had undergone whole genome sequencing using fetal tissues. RESULTS: Six cases of APW confirmed by autopsy were identified in our fetal echocardiographic database. On the three-vessel view, a communication between the pulmonary artery trunk and ascending aorta was noted above the two semilunar valves in all cases. The most frequent type of APW among the cases was type II, and all cases were associated with other cardiac anomalies. No pathogenic or suspected pathogenic copy number variation or insertion-deletions were detected in this series. CONCLUSION: Prenatal diagnosis of APW is feasible, which is helpful during prenatal consultations, so that parents can make better decisions regarding postpartum treatment options and pregnancy outcomes.