Nanoscale Structural Comparison of Fibrillin-1 Microfibrils Isolated from Marfan and Non-Marfan Syndrome Human Aorta.

Fibrillin-1 microfibrils are essential elements of the extracellular matrix serving as a scaffold for the deposition of elastin and endowing connective tissues with tensile strength and elasticity. Mutations in the fibrillin-1 gene (FBN1) are linked to Marfan syndrome (MFS), a systemic connective tissue disorder that, besides other heterogeneous symptoms, usually manifests in life-threatening aortic complications. The aortic involvement may be explained by a dysregulation of microfibrillar function and, conceivably, alterations in the microfibrils' supramolecular structure. Here, we present a nanoscale structural characterization of fibrillin-1 microfibrils isolated from two human aortic samples with different FBN1 gene mutations by using atomic force microscopy, and their comparison with microfibrillar assemblies purified from four non-MFS human aortic samples. Fibrillin-1 microfibrils displayed a characteristic "beads-on-a-string" appearance. The microfibrillar assemblies were investigated for bead geometry (height, length, and width), interbead region height, and periodicity. MFS fibrillin-1 microfibrils had a slightly higher mean bead height, but the bead length and width, as well as the interbead height, were significantly smaller in the MFS group. The mean periodicity varied around 50-52 nm among samples. The data suggest an overall thinner and presumably more frail structure for the MFS fibrillin-1 microfibrils, which may play a role in the development of MFS-related aortic symptomatology.

What Patients Find on the Internet When Looking for Information About Percutaneous Coronary Intervention: Multilanguage Cross-sectional Assessment.

BACKGROUND: The internet provides general users with wide access to medical information. However, regulating and controlling the quality and reliability of the considerable volume of available data is challenging, thus generating concerns about the consequences of inaccurate health care-related documentation. Several tools have been proposed to increase the transparency and overall trustworthiness of medical information present on the web. OBJECTIVE: We aimed to analyze and compare the quality and reliability of information about percutaneous coronary intervention on English, German, Hungarian, Romanian, and Russian language websites. METHODS: Following a rigorous protocol, 125 websites were selected, 25 for each language sub-sample. The websites were assessed concerning their general characteristics, compliance with a set of eEurope 2002 credibility criteria, and quality of the informational content (namely completeness and accuracy), based on a topic-specific benchmark. Completeness and accuracy were graded independently by 2 evaluators. Scores were reported on a scale from 0 to 10. The 5 language subsamples were compared regarding credibility, completeness, and accuracy. Correlations between credibility scores on the one hand, and completeness and accuracy scores, on the other hand, were tested within each language subsample. RESULTS: The websites' compliance with credibility criteria was average at best with scores between 3.0 and 6.0. In terms of completeness and accuracy, the website subsets qualified as poor or average, with scores ranging from 2.4 to 4.6 and 3.6 to 5.3, respectively. English language websites scored significantly higher in all 3 aspects, followed by German and Hungarian language websites. Only German language websites showed a significant correlation between credibility and information quality. CONCLUSIONS: The quality of websites in English, German, Hungarian, Romanian, and Russian languages about percutaneous coronary intervention was rather inadequate and may raise concerns regarding their impact on informed decision-making. Using credibility criteria as indicators of information quality may not be warranted, as credibility scores were only exceptionally correlated with content quality. The study brings valuable descriptive data on the quality of web-based information regarding percutaneous coronary intervention in multiple languages and raises awareness about the need for responsible use of health-related web resources.

Blood-filled cyst of the tricuspid valve: Multiple cardiac disorders, one surgical case.

BACKGROUND: Intracardiac blood cysts are an exceedingly rare occurrence in adulthood. Detailed imaging modalities aid in the diagnosis of such incidental lesions and procedure planning. METHODS: We report the case of a 51-year-old male accusing dyspnea on exertion as a sole symptom which led to the discovery of multiple cardiac anomalies, namely, severe aortic valve insufficiency on a bicuspid aortic valve, ascending aortic aneurysm, a cystic mass on the tricuspid valve, patent foramen ovale, and an occluded right coronary artery. RESULTS: The disorders were managed in a single surgical intervention, the resected mass being confirmed as a blood cyst. CONCLUSIONS: Our case presents a unique association of cardiac disorders, including a highly uncommon intracardiac blood-filled cyst, and underlines the importance of multimodality imaging and interdisciplinary approach in the successful management of such complex cases.