Project description:A 40 year-old athletic woman presented with worsening dyspnea on exertion over the preceding several months. Chest radiograph showed borderline cardiomegaly and subsequent echocardiography demonstrated a 5.0-cm left atrial mass as well as left-to-right interatrial shunting through a patent foramen ovale. Cardiac magnetic resonance imaging was performed, which demonstrated signal characteristics consistent with an atrial myxoma. The patient then underwent urgent surgical treatment with good technical and clinical outcome. Histologic examination confirmed an atrial myxoma. Cardiac magnetic resonance imaging was valuable in characterizing the nature of the atrial mass and patent foramen ovale, helping guide the surgical approach.
Project description:Atrial septal defects (ASDs) are common congenital heart defects (CHD). The clinical course in patients without closure of the ASD is associated with significant morbidity and mortality in advanced age. A small percentage of patients may develop pulmonary arterial hypertension (PAH) due to left to right shunting that impacts morbidity and mortality. Advances in prenatal screening and fetal echocardiography have allowed timely interventions. Nonetheless, some patients still may be diagnosed with ASD in adulthood as an incidental finding or presenting with clinical symptoms such as shortness of breath from right heart failure. We report a case of an adult female presenting with shortness of breath due to ASD causing PAH with Eisenmenger physiology.
Project description:Background Current guidelines recommend at least 6 months of antithrombotic therapy and antibiotic prophylaxis after septal-occluding device deployment in transcatheter closure of atrial septal defect. It has been estimated that it takes ≈6 months for complete neo-endothelialization; however, neo-endothelialization has not previously been assessed in vivo in humans. Methods and Results The neointimal coverage of septal occluder devices was evaluated 6 months after implantation in 15 patients by angioscopy from the right atrium. Each occluder surface was divided into 9 areas; the levels of endothelialization in each area were semiquantitatively assessed by 4-point grades. Device neo-endothelialization was sufficient in two thirds of patients, but insufficient in one third. In the comparison between patients with sufficiently endothelialized devices of average grade score ≥2 (good endothelialization group, n=10) and those with poorly endothelialized devices of average grade score <2 (poor endothelialization group, n=5), those in the poor endothelialization group had larger devices deployed (27.0 mm [25.0-31.5 mm] versus 17.0 mm [15.6-22.5 mm], respectively) and progressive right heart dilatation. The endothelialization was poorer around the central areas. Moreover, the prevalence of thrombus formation on the devices was higher in the poorly endothelialized areas than in the sufficiently endothelialized areas (Grade 0, 94.1%; Grade 1, 63.2%; Grade 2, 0%; Grade 3, 1.6%). Conclusions Neo-endothelialization on the closure devices varied 6 months after implantation. Notably, poor endothelialization and thrombus attachment were observed around the central areas and on the larger devices.
Project description:With increasing atrial septal defect (ASD) repairs, more women of childbearing age will have ASD closure devices. Current ASD closure trials have excluded women planning pregnancy, making their management challenging. We present a pregnant woman, with a repaired ASD, who presented with device-related infective endocarditis. (Level of Difficulty: Beginner.).
Project description:Atrial septal defect (ASD) is a common congenital defect that leads to various hemodynamic complications if untreated. Transcatheter closure (TCC) of isolated secundum ASD is the preferred treatment. Herein we describe a unique malaligned ASD secondary to a membranous chord. With balloon sizing and intracardiac echocardiography (ICE), TCC was successfully pursued. (Level of Difficulty: Beginner.).
Project description:Atrial septal defects (ASDs) are the most common types of cardiac septal defects in congenital heart defects. In addition to traditional therapy, interventional closure has become the main treatment method. However, the molecular events and mechanisms underlying the repair progress by occlusion device remain unknown. In this study, we aimed to characterize differentially expressed genes (DEGs) in the blood of patients treated with occlusion devices (metal or poly-L-lactic acid devices) using RNA-sequencing, and further validated them by qRT-PCR analysis to finally determine the expression of key mediating genes after closure of ASD treatment. The result showed that total 1,045 genes and 1,523 genes were expressed differently with significance in metal and poly-L-lactic acid devices treatment, respectively. The 115 overlap genes from the different sub-analyses are illustrated. The similarities and differences in gene expression reflect that the body response process involved after interventional therapy for ASDs has both different parts that do not overlap and the same part that crosses. The same portion of body response regulatory genes are key regulatory genes expressed in the blood of patients with ASDs treated with closure devices. The gene ontology enrichment analysis showed that biological processes affected in metal device therapy are immune response with CXCR4 genes and poly-L-lactic acid device treatment, and the key pathways are nuclear-transcribed mRNA catabolic process and proteins targeting endoplasmic reticulum process with ribosomal proteins (such as RPS26). We confirmed that CXCR4, TOB1, and DDIT4 gene expression are significantly downregulated toward the pre-therapy level after the post-treatment in both therapy groups by qRT-PCR. Our study suggests that the potential role of CXCR4, DDIT4, and TOB1 may be key regulatory genes in the process of endothelialization in the repair progress of ASDs, providing molecular insights into this progress for future studies.
Project description:Background Delayed brain development, brain injury, and neurodevelopmental disabilities are commonly observed in infants operated for complex congenital heart defect. Our previous findings of poorer neurodevelopmental outcomes in individuals operated for simple congenital heart defects calls for further etiological clarification. Hence, we examined the microstructural tissue composition in cerebral cortex and subcortical structures in comparison to healthy controls and whether differences were associated with neurodevelopmental outcomes. Methods and Results Adults (n=62) who underwent surgical closure of an atrial septal defect (n=33) or a ventricular septal defect (n=29) in childhood and a group of healthy, matched controls (n=38) were enrolled. Brain diffusional kurtosis imaging and neuropsychological assessment were performed. Cortical and subcortical tissue microstructure were assessed using mean kurtosis tensor and mean diffusivity and compared between groups and tested for associations with neuropsychological outcomes. Alterations in microstructural tissue composition were found in the parietal, temporal, and occipital lobes in the congenital heart defects, with distinct mean kurtosis tensor cluster-specific changes in the right visual cortex (pericalcarine gyrus, P=0.002; occipital part of fusiform and lingual gyri, P=0.019). Altered microstructural tissue composition in the subcortical structures was uncovered in atrial septal defects but not in ventricular septal defects. Associations were found between altered cerebral microstructure and social recognition and executive function. Conclusions Children operated for simple congenital heart defects demonstrated altered microstructural tissue composition in the cerebral cortex and subcortical structures during adulthood when compared with healthy peers. Alterations in cerebral microstructural tissue composition were associated with poorer neuropsychological performance. Registration URL: https://www.clinicaltrials.gov; Unique identifier: NCT03871881.