Contribution of rare chromosome 22q11.2 copy number variants to non-syndromic bicuspid aortic valve.

Background: Bicuspid aortic valve (BAV) is the most common congenital heart defect in adults, often leading to complications such as thoracic aortic aneurysms and aortic stenosis. While BAV is frequently associated with 22q11.2 deletion syndrome (22q11.

Impact of Hypertension and Physical Exercise on Hemolysis Risk in the Left Coronary Artery: A Computational Fluid Dynamics Analysis.

: Hypertension increases the risk of developing atherosclerosis and arterial stiffness, with secondarily enhanced wall stress pressure that damages the artery wall. The coexistence of atherosclerosis and hypertension leads to artery stenosis and microvascular angiopathies, during which the intravascular mechanical hemolysis of red blood cells (RBCs) occurs, leading to increased platelet activation, dysfunction of the endothelium and smooth muscle cells due to a decrease in nitric oxide, and the direct harmful effects of hemoglobin and iron released from the red blood cells. This study analyzed the impact of hypertension and physical exercise on the risk of hemolysis in the left coronary artery.

Rare genomic copy number variants implicate new candidate genes for bicuspid aortic valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms.

Cardiovascular Imaging, Climate Change, and Environmental Sustainability.

Environmental exposures including poor air quality and extreme temperatures are exacerbated by climate change and are associated with adverse cardiovascular outcomes. Concomitantly, the delivery of health care generates substantial atmospheric greenhouse gas (GHG) emissions contributing to the climate crisis. Therefore, cardiac imaging teams must be aware not only of the adverse cardiovascular health effects of climate change, but also the downstream environmental ramifications of cardiovascular imaging.

Fetal Cardiac MRI Using Doppler US Gating: Emerging Technology and Clinical Implications.

Fetal cardiac MRI using Doppler US gating is an emerging technique to support prenatal diagnosis of congenital heart disease and other cardiovascular abnormalities. Analogous to postnatal electrocardiographically gated cardiac MRI, this technique enables directly gated MRI of the fetal heart throughout the cardiac cycle, allowing for immediate data reconstruction and review of image quality. This review outlines the technical principles and challenges of cardiac MRI with Doppler US gating, such as loss of gating signal due to fetal movement.

Whole Exome Sequencing Uncovers the Genetic Complexity of Bicuspid Aortic Valve in Families with Early Onset Complications.

Bicuspid Aortic Valve (BAV) is the most common adult congenital heart lesion with an estimated population prevalence of 1%. We hypothesize that early onset complications of BAV (EBAV) are driven by specific impactful genetic variants. We analyzed whole exome sequences (WES) to identify rare coding variants that contribute to BAV disease in 215 EBAV families.

4D Flow Cardiac MR in Primary Mitral Regurgitation.

Background: Four-dimensional-flow cardiac MR (4DF-MR) offers advantages in primary mitral regurgitation. The relationship between 4DF-MR-derived mitral regurgitant volume (MR-Rvol) and the post-operative left ventricular (LV) reverse remodeling has not yet been established.

Purpose: To ascertain if the 4DF-MR-derived MR-Rvol correlates with the LV reverse remodeling in primary mitral regurgitation.

Rare Genomic Copy Number Variants Implicate New Candidate Genes for Bicuspid Aortic Valve.

Bicuspid aortic valve (BAV), the most common congenital heart defect, is a major cause of aortic valve disease requiring valve interventions and thoracic aortic aneurysms predisposing to acute aortic dissections. The spectrum of BAV ranges from early onset valve and aortic complications (EBAV) to sporadic late onset disease. Rare genomic copy number variants (CNVs) have previously been implicated in the development of BAV and thoracic aortic aneurysms.

4D Flow cardiovascular magnetic resonance consensus statement: 2023 update.

Hemodynamic assessment is an integral part of the diagnosis and management of cardiovascular disease. Four-dimensional cardiovascular magnetic resonance flow imaging (4D Flow CMR) allows comprehensive and accurate assessment of flow in a single acquisition. This consensus paper is an update from the 2015 '4D Flow CMR Consensus Statement'.

Rationale and clinical applications of 4D flow cardiovascular magnetic resonance in assessment of valvular heart disease: a comprehensive review.

Background: Accurate evaluation of valvular pathology is crucial in the timing of surgical intervention. Whilst transthoracic echocardiography is widely available and routinely used in the assessment of valvular heart disease, it is bound by several limitations. Although cardiovascular magnetic resonance (CMR) imaging can overcome many of the challenges encountered by echocardiography, it also has a number of limitations.

Acute vasodilator response testing in the adult Fontan circulation using non-invasive 4D Flow MRI: a proof-of-principle study.

Background: Pulmonary vasodilator therapy in Fontan patients can improve exercise tolerance. We aimed to assess the potential for testing of acute vasodilator response using four-dimensional (D) flow MRI during oxygen inhalation.

Materials And Methods: Six patients with well-functioning Fontan circulations were prospectively recruited and underwent cardiac MRI.

Le Cœur en Sabot: shape associations with adverse events in repaired tetralogy of Fallot.

Background: Maladaptive remodelling mechanisms occur in patients with repaired tetralogy of Fallot (rToF) resulting in a cycle of metabolic and structural changes. Biventricular shape analysis may indicate mechanisms associated with adverse events independent of pulmonary regurgitant volume index (PRVI). We aimed to determine novel remodelling patterns associated with adverse events in patients with rToF using shape and function analysis.

Comprehensive Neonatal Cardiac, Feed and Wrap, Non-contrast, Non-sedated, Free-breathing Compressed Sensing 4D Flow MRI Assessment.

Background: Cardiac MRI is an important imaging tool in congenital cardiac disease, but its use has been limited in the neonatal population as general anesthesia has been needed for breath-holding. Technological advances in four-dimensional (4D) flow MRI have now made nonsedated free-breathing acquisition protocols a viable clinical option, but the method requires prospective validation in neonates.

Purpose: To test the feasibility of compressed sensing (CS) 4D flow MRI in the neonatal population and to compare with standard previously validated two-dimensional (2D) phase-contrast (PC) flow MRI.

Three-dimensional quantification of circulation using finite-element methods in four-dimensional flow MR data of the thoracic aorta.

Purpose: Three-dimensional (3D) quantification of circulation using a Finite Elements methodology.

Methods: We validate our 3D method using an in-silico arch model, for different mesh resolutions, image resolution and noise levels, and we compared this with a currently used 2D method. Finally, we evaluated the application of our methodology in 4D Flow MRI data of ascending aorta of six healthy volunteers, and six bicuspid aortic valve (BAV) patients, three with right and three with left handed flow, at peak systole.

Four-Dimensional Flow Magnetic Resonance Imaging in the Assessment of Blood Flow in the Heart and Great Vessels: A Systematic Review.

Four-dimensional (4D) flow magnetic resonance imaging (MRI) allows multidirectional quantification of blood flow in the heart and great vessels. Comparability of the technique to the current reference standards of flow assessment-two-dimensional (2D) flow MRI and Doppler echocardiography-varies in the literature. Image acquisition parameters likely impact upon the accuracy and reproducibility of 4D flow MRI.

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3.

Summary: International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional, and research purposes.

This International evidence-based nomenclature and classification consensus on the congenital bicuspid aortic valve and its aortopathy recognizes 3 types of bicuspid aortic valve: 1. Fused type, with 3 phenotypes: right-left cusp fusion, right-non cusp fusion and left-non cusp fusion; 2. 2-sinus type with 2 phenotypes: Latero-lateral and antero-posterior; and 3.

International Consensus Statement on Nomenclature and Classification of the Congenital Bicuspid Aortic Valve and Its Aortopathy, for Clinical, Surgical, Interventional and Research Purposes.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3.

International consensus statement on nomenclature and classification of the congenital bicuspid aortic valve and its aortopathy, for clinical, surgical, interventional and research purposes.

This International Consensus Classification and Nomenclature for the congenital bicuspid aortic valve condition recognizes 3 types of bicuspid valves: 1. The fused type (right-left cusp fusion, right-non-coronary cusp fusion and left-non-coronary cusp fusion phenotypes); 2. The 2-sinus type (latero-lateral and antero-posterior phenotypes); and 3.