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Objectives: Our goal was to conduct a hemodynamic analysis of a novel animal model of Fontan physiology. Poor late-term outcomes in Fontan patients are believed to arise from Fontan-induced hemodynamics, but the mechanisms remain poorly understood. Recent advances in surgical experimentation have resulted in the development of a chronic sheep model of Fontan physiology; however, detailed analysis of this model is lacking. Methods: We created a single-stage Fontan model in juvenile sheep with normal biventricular circulation. The superior vena cava was anastomosed to the main pulmonary artery, and the inferior vena cava was connected to the main pulmonary artery using an expanded polytetrafluoroethylene conduit. Longitudinal hemodynamics, including catheterization and magnetic resonance imaging were evaluated. Results: Four out of 12 animals survived, with the longest surviving animal living 3 years after single-stage Fontan. We showed a significant era effect regarding survival (1 out of 8 and subsequently 3 out of 4 animals surviving beyond 2 months) attributed in large part to the procedural learning curve. Key characteristics of Fontan hemodynamics, namely systemic venous hypertension and low normal cardiac output, were observed. However, recapitulation of passive human Fontan hemodynamics is affected by volume loading of the right ventricle given an anatomic difference in sheep azygous venous anatomy draining to the coronary sinus. Conclusions: A significant learning curve exists to ensure long-term survival and future surgical modifications, including banding of the main pulmonary artery and ligation of the azygous to coronary sinus connection are promising strategies to improve the fidelity of model hemodynamics.

In this survey study of institutions across the US, marked variability in evaluation, treatment, and follow-up of adolescents 12 through 18 years of age with mRNA coronavirus disease 2019 (COVID-19) vaccine-associated myopericarditis was noted. Only one adolescent with life-threatening complications was reported, with no deaths at any of the participating institutions.

Background Data on the use of implanted hemodynamic monitoring (IHM) in patients with Fontan circulation are limited. This study reports our experience using the CardioMEMS HF system in adults with Fontan circulation. Methods and Results This single‐center, retrospective study evaluated heart failure hospitalizations, procedural complications, and device‐related complications in patients with Fontan circulation referred for IHM placement (2015–2022). The association of pulmonary artery pressure (by most recent catheterization and median IHM pressure within 30 days of placement) with both death and follow‐up Model for End‐Stage Liver Disease Excluding International Normalized Ratio score were evaluated. Of 18 patients referred for IHM placement, 17 were successful (median age, 30 [range 21–48] years, 6 women). Procedural complications (access site hematomas, pulmonary artery staining) occurred in 3 patients, without device‐related procedural complications. In follow‐up (median, 35 [range, 6–83] months), 1 patient developed a pulmonary embolism (possibly device‐related). Heart failure hospitalizations/year were similar before and after IHM (median, 1 [interquartile range, 0–1.0] versus 0.6 [0–2.3]; P=0.268), though only 46% of heart failure hospitalizations had associated IHM transmissions. IHM pressures were associated with Model for End‐Stage Liver Disease Excluding International Normalized Ratio scores (R2=0.588, P

Introduction: Transthoracic (TTE) follow up guidelines after PFO device closure are vague. The primary goal of this study was to perform a literature search to characterize the timing of complications that occur after PFO device placement to determine the utility of long‐term routine TTE. Methods: A search was performed in Medline (PubMed) with English language and publication date (2000–2023) filters were applied. Studies were included if they reported on patients who underwent a PFO device closure. Studies were excluded if they only reported on ASD device closure patients, were meta‐analysis or review papers, or did not report any outcomes for the PFO device procedure. Results: A total of 305 articles met the criteria (observational cohort study, n = 196; case study, n = 99; and randomized control study, n = 10). Age at the time of PFO device closure was 47.5 ± 9.3 years. The incidence of complications was 6.9% (3358/48 348). Maximum range of follow‐up was 0–17 years. The plurality of complications was due to arrhythmias (46.3%, 1554/3358), followed by residual shunts (29.7%, 999/3358). The majority of complications occurred ≤6 months post‐device placement (57.8%, 1941/3358). Only 0.8% (27/3358) of complications took place >5 years. All patients with complications >5 years of device placement were presented with clinical symptoms related to their complications. Conclusion: The incidence of patients presenting with PFO‐reported complications beyond 5 years post‐procedure diminished. The utility of routine TTE beyond 5 years post‐PFO after device placement should be further assessed. [ABSTRACT FROM AUTHOR]

Abstract Purpose Ectodermal dysplasias are characterized by developmental abnormalities in ectodermal structures. Hypohidrotic ectodermal dysplasias (HED) are the most common subtype. They are most commonly inherited via X-linked recessive routes. We report on a novel ectodysplasin-A (EDA) mutation that is expected to be involved in pathogenesis of HED. Methods Hypohidrotic ectodermal dysplasia genes, including EDA, EDAR and EDARADD, were analyzed using next-generation sequencing (NGS). The detected mutation on the EDA gene was confirmed in the patient and his mother using Sanger sequencing. Results The patient presented with adontia, absence of gum development, hyperthermia and hypohidrosis. Our genetic analysis of the patient revealed a novel frameshift hemizygous mutation (c.898_924 + 8del35ins4CTTA) on the EDA gene. The patient’s mother showed a mild HED phenotype. Direct sequencing of the EDA gene in the region where her son had the mutation showed the same mutation in a heterozygous state. Conclusion We identified a novel frameshift mutation in the EDA gene in an Iranian patient affected by X-linked HED. The difference between our patient’s symptoms and those recorded for some previous subjects may be due to the differences in the mutations involved.

IntroductionA 2-year-old female with hypoplastic left heart syndrome (HLHS)-variant, a complex congenital heart defect (CHD) characterized by the underdevelopment of the left ventricle, presented with complications following single ventricle palliation. Diagnostic work-up revealed elevated Fontan pathway pressures, as well as significant dilation of the inferior Fontan pathway with inefficient swirling flow and hepatic venous reflux. Due to the frail condition of the patient, the clinical team considered an endovascular revision of the Fontan pathway. In this work, we performed a computational fluid dynamics (CFD) analysis informed by data on anatomy, flow, and pressure to investigate the hemodynamic effect of the endovascular Fontan revision.MethodsA patient-specific anatomical model of the Fontan pathway was constructed from magnetic resonance imaging (MRI) data using the cardiovascular modeling software CardiovasculaR Integrated Modeling and SimulatiON (CRIMSON). We first created and calibrated a pre-intervention 3D-0D multi-scale model of the patient’s circulation using fluid-structure interaction (FSI) analyses and custom lumped parameter models (LPMs), including the Fontan pathway, the single ventricle, arterial and venous systemic, and pulmonary circulations. Model parameters were iteratively tuned until simulation results matched clinical data on flow and pressure. Following calibration of the pre-intervention model, a custom bifurcated endograft was introduced into the anatomical model to virtually assess post-intervention hemodynamics.ResultsThe pre-intervention model successfully reproduced the clinical hemodynamic data on regional flow splits, pressures, and hepatic venous reflux. The proposed endovascular repair model revealed increases of mean and pulse pressure at the inferior vena cava (IVC) of 6 and 29%, respectively. Inflows at the superior vena cava (SVC) and IVC were each reduced by 5%, whereas outflows at the left pulmonary artery (LPA) and right pulmonary artery (RPA) increased by 4%. Hepatic venous reflux increased by 6%.ConclusionOur computational analysis indicated that the proposed endovascular revision would lead to unfavorable hemodynamic conditions. For these reasons, the clinical team decided to forgo the proposed endovascular repair and to reassess the management of this patient. This study confirms the relevance of CFD modeling as a beneficial tool in surgical planning for single ventricle CHD patients.

Children with hypoplastic left heart syndrome (HLHS) must undergo multiple surgical stages to reconstruct the anatomy to a sustainable single ventricle system. Stage I palliation, or the Norwood procedure, provides circulation to both pulmonary and systemic vasculature. The aorta is reconstructed and attached to the right ventricle and a fraction of systemic flow is redirected to the pulmonary arteries (PAs) through a systemic-to-PA shunt. Despite abundant hemodynamic data available 4–5 months after Norwood palliation, data is very scarce immediately following stage I. This data is critical in determining post-operative success. In this work, we combined population data and computational fluid dynamics (CFD) to characterize hemodynamics immediately following stage I (post-stage I) and prior to stage II palliation (pre-stage II). A patient-specific model was constructed as a baseline geometry, which was then scaled to reflect population-based morphological data at both time-points. Population-based hemodynamic data was then used to calibrate each model to reproduce blood flow representative of HLHS patients. The post-stage I simulation produced a PA pressure of 22 mmHg and high-frequency oscillations within the flow field indicating highly disturbed hemodynamics. Despite PA mean pressure dropping to 14 mmHg, the pre-stage II model also produced high-frequency flow components and PA wall shear stress increases. These suboptimal conditions may be necessary to ensure adequate PA flow throughout the pre-stage II period, as the shunt becomes relatively smaller compared to the patient’s somatic growth. In the future, CFD can be used to optimize shunt design and minimize these suboptimal conditions.

Rivers play an important role in water supply, waterway transport, and riverine species habitations. The underwater depth of a river channel is a fundamental geometric element and a key input to studies for the aforementioned and other applications. Traditional in-situ field surveys for flow depth measurements would incur high costs and encounter technical and/or logistical difficulties, particularly for river channels of large extents, very shallow channels, and remote, inaccessible channels. Recent advances in satellite remote sensing of inland water bodies like rivers, streams, and creeks have allowed mapping flow depth in an inexpensive, convenient, and efficient manner. The purpose of this paper is to demonstrate the retrieval of river flow depth from high-resolution (1.2 m) WorldView-3 satellite imagery. The depth retrieval methods are based on the ratio of top-of-atmosphere reflectance between two pairing wavelength bands of multispectral imagery. The originality of the methods lies in using analytical relationships without resorting to ground data of river flow depth for calibration, which improves from previous studies of remote sensing of river flow depth. The methods are successfully applied to mapping the underwater bathymetry of a 26 km reach of the Nicolet River in Quebec, Canada. This study shows the importance of geometric and radiometric corrections to the satellite images. The obtained flow depths using the ratio of reflectance of the red band (630–690 nm) to that of the green band (510–580 nm), among the eight bands in the visible spectrum, agree best with in-situ measurements. This study is perhaps the first use of the analytical approach for mapping river bathymetry. It is feasible to implement the approach to other river channels, with a good potential to reduce the costs and increase the efficiency of mapping river bathymetries.

This document provides a concise summary of a study on the use of implanted haemodynamic monitoring (IHM) in adults with subaortic right ventricles (sRV) who have congenital heart disease. The study explores the novel uses of IHM, including monitoring pulmonary artery pressures for heart transplant referral and management after a ventricular assist device. The study found that IHM had multiple uses and could potentially reduce the need for repeat cardiac catheterizations. The document also provides supplemental tables and references related to the study, which can be useful for researchers and healthcare professionals in the field. [Extracted from the article]

Background: Periodontal disease has a multifactorial etiology. A combination of microbial agents and environmental, habitual, systemic, and genetic risk factors is responsible for the development of periodontal disease. Host immune response causes the destruction of tooth-supporting structure and eventual tooth loss. This study aimed to assess the correlation of interleukin 6 (IL-6) -174-GC and IL-6-572-GC gene polymorphisms with periodontal disease in an Iranian population. Materials and Methods: This case–control analytical study was conducted on 129 subjects presenting to the laboratory of Taleghani Hospital. Subjects underwent clinical and periodontal examinations and divided into five groups of healthy, gingivitis and mild, moderate and severe periodontitis. Blood samples (2 ml) were obtained. Genomic DNA was extracted manually using the salting-out method. IL-6 sequence amplification was performed using polymerase chain reaction with three thermal protocols. Digested products were analyzed by electrophoresis through 2% agarose gel using Gel Red staining. Data were analyzed using Chi-square, Kruskal–Wallis, and Mann–Whitney tests, and P < 0.05 was considered significant. Results: The frequency of GG polymorphism at IL-6-174 and IL-6-572 genomic regions was 51.2% and 71.3%, respectively. The frequency of IL-6-572-GG polymorphism was significantly greater than that of IL-6-572-GC polymorphism (P < 0.001). Comparison of the mean and maximum pocket depth and clinical attachment level, as well as bleeding on probing percentage, revealed significant differences between the healthy controls and periodontitis patients (P < 0.001). The frequency percentages of GC and GG polymorphisms were almost equal in the healthy, gingivitis, and periodontitis groups. In other words, the frequency of the two polymorphisms was not significantly different between the health and disease states (P = 0.065 for IL-6-572 and P = 0.63 for IL-6-174). Conclusion: This study found no association between IL-6-174 and IL-6-572 gene polymorphisms and periodontitis in the studied population.