Aortic valvulopathy

BACKGROUND: Left ventricular outflow track (LVOT) obstruction (LVOTO) is a severe complication of transcatheter mitral valve replacement (TMVR) procedures, with an uncertain prognosis and only few strategies available to prevent its occurrence. TMVR is thus contraindicated in some patients because of a high risk of LVOTO onset. We demonstrate how LVOTO can be managed with a balloon inflation in the LVOT and a D-shaped deformation of the bioprosthetic valve. CASE SUMMARY: A 64-year-old female presented with acute pulmonary oedema 2 weeks following aortic valve replacement and aorto-coronary bypass surgeries...

OBJECTIVE: Surgery for ascending aneurysms in bicuspid aortic valve syndrome primarily includes Bentall root replacement, aortic valve replacement with supracoronary ascending aorta replacement (AVRSCAAR), and valve-sparing root reimplantation (VSRR). Comparative analysis of long-term clinical and functional outcomes of these procedures is detailed. METHODS: From 1997 to 2017, 635 patients with bicuspid aortic valve undergoing root complex-focused procedures electively were stratified by valvulopathy (ie, aortic stenosis vs aortic insufficiency) and substratified into ascending or root aneurysm phenotype...

In patients with bicuspid aortic valves, guidelines call for regular follow-up to monitor disease progression and guide intervention. We aimed to evaluate how closely these recommendations are followed at a tertiary care center. Among 48,504 patients who received echocardiograms (2013-2018) at a tertiary care center, 245 patients were identified to have bicuspid aortic valve. Bivariate analyses compared characteristics between patients who did and did not receive follow-up by a cardiovascular specialist. During a median follow-up of 3...

A 60-year-old patient, professor of physics, presented in 1999 with sudden-onset vitiligo associated with hyperprolactinemia and a prolactinoma. Fearful of potential surgical complications at the peak of his career, the patient declined surgery and opted for medical management with bromocriptine. The decreasing effectiveness of bromocriptine after 5 years required a switch to cabergoline. After a 15-year-course of cabergoline therapy with a cumulative dose of 572 mg, echocardiographic monitoring demonstrated aortic and mitral valve thickening and regurgitation...

Cellular communication network factor 2 (CCN2/CTGF) has been traditionally described as a downstream mediator of other profibrotic factors including transforming growth factor (TGF)-β and angiotensin II. However, recent evidence from our group demonstrated the direct role of CCN2 in maintaining aortic wall homeostasis and acute and lethal aortic aneurysm development induced by angiotensin II in the absence of CCN2 in mice. In order to translate these findings to humans, we evaluated the potential association between three polymorphisms in the CCN2 gene and the presence of a thoracic aortic aneurysm (TAA)...

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Aortic regurgitation (AR) is a common valvular pathology. Multimodality noninvasive cardiovascular imaging is routinely used to assess the mechanism of AR, degree, and its hemodynamic impact on the cardiovascular system. Collecting this information is crucial in establishing the prognosis and in guiding patient management and follow-up. While echocardiography remains the primary test to assess AR, a comprehensive assessment of this valvulopathy can be obtained by combining the information from different techniques...

The bicuspid aortic valve (BAV) is associated with significant aortic valve dysfunction. We aimed to study Mexican patients with BAV to assess phenotypic expressions of BAV, and associations of valvulopathy and aortopathy and their predictors. A cross-sectional, retrospective study was designed. The patients were divided according to (1) normally or minimally dysfunctional BAV, (2) predominant aortic regurgitation (AR), and (3) predominant aortic stenosis (AS). A total of 189 patients were included with a high prevalence of males (68%)...

Background: The mechanism underlying aortic dilatation is still unknown. Vascular dilatation is thought to be the result of progressive aortic media degeneration caused by defective vascular matrix hemostasis, including TGF-β1 dysregulation. The goal of this study is to draw attention to the potential utility of TGF-β1 as a diagnostic marker in non-syndromic patients with aortic dilatation. Methods: TGF-β1 levels in plasma were measured in 50 patients who had undergone surgery and had a tricuspid or bicuspid aortic valve as well as a normal or dilated ascending aorta...

Pregnancy is associated with profound hemodynamic changes that are particularly impactful in patients with underlying cardiovascular disease. Management of pregnant women with cardiovascular disease requires careful evaluation that considers the well-being of both the woman and the developing fetus. Clinical assessment begins before pregnancy and continues throughout gestation into the post-partum period and is supplemented by cardiac imaging. This review discusses the role of imaging, specifically echocardiography, cardiac MRI, and cardiac CT, in pregnant women with valvular diseases, hypertrophic cardiomyopathy, and aortic pathology...

Loss-of-function pathogenic variants in somatic and germline cells in SMAD4 may cause cancer and juvenile polyposis-Hereditary Hemorrhagic Telangiectasia (SMAD4-JP-HHT), respectively. In a similar manner, gain-of-function somatic and germline pathogenic variants in SMAD4 can cause various forms of cancer as well as Myhre syndrome. The different SMAD4 molecular mechanisms result in contrasting clinical phenotypes demonstrated by SMAD4-JP-HHT and Myhre syndrome. We report an additional patient with SMAD4-JP-HHT and aortopathy, and expand the phenotype to include severe valvulopathy, cutaneous, ophthalmologic, and musculoskeletal features consistent with an inherited disorder of connective tissue...

BACKGROUND: The gender difference of the bicuspid aortic valve (BAV) is not well understood. OBJECTIVES: We evaluated the impact of gender on the Sievers types, valvulopathy, aortopathy, and outcomes of aortic valve replacement (AVR) of BAV patients in a cohort of Chinese patients. METHODS: Among 992 BAV patients without aortic dissection nor congenital heart disease, 658 underwent AVR. The demography, Sievers types, valvulopathy, aortopathy, and outcomes of AVR were compared between genders...

BACKGROUND: Mucopolysaccharidoses (MPSs) are a group of lysosomal storage disorders caused by the deficit of lysosomal hydrolases involved in the degradation of glycosaminoglycans (GAGs). The course is chronic and progressive, with multisystemic involvement that often leads to cardiovascular disease. We describe the overall incidence and type of cardiac damage in a cohort of Italian MPS patients, and their progression over time, also with reference to treatment efficacy in patients under Enzyme Replacement Therapy (ERT)...

Prosthetic valve replacement is the standard treatment for aortic valvulopathy. Due to the structural valve deterioration of biological prosthesis and the anticoagulation requirements of mechanical valves, the repair of aortic valve disease is receiving more attention in recent years. The Ozaki technique consists in the reconstruction of a trileaflet valve using autologous pericardium. We report our first two cases of successful reconstruction of the aortic valve using this technique with 12-month echocardiographic and 24-month clinical follow up...

Introduction: and importance: Although radiotherapy is a well-known cancer treatment and an important part of the therapeutic strategy for achieving long-term remission or disease control, the radiation-induced heart disease rates are high and may occur years later. This article aims to raise clinician awareness of cardiac side effects that can occur years after radiation therapy. In order to develop effective prevention strategies and improve clinical outcomes. Case presentation: Here, we present a rare case of a young female, on remission from breast carcinoma, who received mediastinal radiotherapy 13 years earlier, admitted in our department for recurrent syncope of sudden on-set...

BACKGROUND: Anorectic drugs are overlooked as a cause of valvular heart disease (VHD). AIM: To describe the characteristics of a large population of patients with severe VHD who underwent cardiac surgery and had a history of benfluorex intake. METHODS: Retrospective observational and cross-sectional study of patients from a large French database (Office National d'Indemnisation des Accidents Médicaux). Clinical, echocardiographic, surgical and pathology findings were comprehensively collected from medical files...

Coxiella burnetti is the causative organism of the zoonotic infection Q fever, of which endocarditis is one of the most common manifestations of the chronic form. Polymicrobial endocarditis with Q fever is extremely rare and is yet to be described among an Australasian cohort. SUMMARY: We present the case of a 32-year-old gardener with culture-negative chronic Q fever prosthetic valve endocarditis concomitant with another bacterial pathogen, leading to aortic root abscess formation, requiring a Bentall procedure, extracorporeal membrane oxygenation, and prolonged antimicrobial therapy, with a fatal outcome...

Aortic valve neocuspidization or Ozaki procedure represents an advanced surgical strategy for the management of patients with aortic valvulopathy. It has been shown to have clinical and hemodynamic outcomes that compare favorably with aortic valve replacement as it restores physiological aortic valve function and left ventricular remodeling. There are, however, a new set of issues including structural valve deterioration, leaflet tear/perforation, and need for reoperation. A keen understanding of the technical nuances involved with the Ozaki procedure may help in reducing the incidence of such adverse outcomes...

Cardiac magnetic resonance (CMR) imaging is a well-set diagnostic technique for assessment of valvular heart diseases and is gaining ground in current clinical practice. It provides high-quality images without the administration of ionizing radiation and occasionally without the need of contrast agents. It offers the unique possibility of a comprehensive stand-alone assessment of the heart including biventricular function, left ventricle remodeling, myocardial fibrosis, and associated valvulopathies. CMR is the recognized reference for the quantification of ventricular volumes, mass, and function...

Calcific aortic valve disease (CAVD) is a common acquired valvulopathy, which carries a high burden of mortality. Chronic inflammation has been postulated as the predominant pathophysiological process underlying CAVD. So far, no effective medical therapies exist to halt the progression of CAVD. This review aims to outline the known pathways of inflammation and calcification in CAVD, focussing on the critical roles of mechanical stress and mechanosensing in the perpetuation of valvular inflammation. Following initiation of valvular inflammation, dysregulation of proinflammatory and osteoregulatory signalling pathways stimulates endothelial-mesenchymal transition of valvular endothelial cells (VECs) and differentiation of valvular interstitial cells (VICs) into active myofibroblastic and osteoblastic phenotypes, which in turn mediate valvular extracellular matrix remodelling and calcification...