PubMed Journals Articles About "Aortic Stenosis Patient With Hurler Syndrome After Bone" RSS

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 23,000+

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Transcatheter aortic valve implantation in a 54-year-old patient with aggressive HIV.

We report a case of a 54-year-old patient who was denied surgical replacement for severe aortic stenosis because of complicated acquired immunodeficiency syndrome and who successfully underwent transcatheter aortic valve implantation at our institution.

Progressive supra-aortic stenosis in a young adult with the findings of Singleton Merten Syndrome.

Singleton Merten Syndrome is an autosomal dominant disorder of unknown origin. Patients often present with muscular weakness, failure to thrive, abnormal dentition, glaucoma, psoriatic skin lesions, aortic calcification and musculoskeletal abnormalities. In this case, we present a young girl with a history of aortic root replacement, who had an unusual progressive supra-aortic stenosis managed with urgent surgery during the course of the syndrome. Cardiovascular involvement needs special attention, since it...

A patient with Marfan's syndrome presented with severe rheumatic mitral stenosis and successfully treated with percutaneous transmitral balloon commissurotomy - Report of first case.

Marfan's syndrome (MFS) is an inherited autosomal dominant disorder of connective tissue with mutation on the fibrillin-1 gene encoding for fibrillin. This frequently involves the cardiovascular system with prevalence is 1:5000-10,000. The clinical major criteria involve the skeletal and ocular apparatus and the cardiovascular and central nervous system. In Marfan's syndrome mitral valve prolapse and aortic dilation are the main cardiovascular manifestations. According to the literature database the prevale...

Fetal intervention for critical aortic stenosis: advances, research and postnatal follow-up.

Fetal aortic valvuloplasty is intended to alter the natural history of aortic stenosis evolving to hypoplastic left heart syndrome. The most recently reported data and advances on this procedure were reviewed.

Combined severe valvular aortic and valvular pulmonary stenosis and its management - A case report and literature review.

Isolated congenital valvular stenosis of either aortic or pulmonary valve is commonly seen yet the presence of both these lesions in the same patient is rare. This combination presents unusual diagnostic as well as management problems. Apart from a few case reports, there is little in the literature on the combined stenosis of both semilunar valves. We present here a case report of a three and half year old boy diagnosed as a combined congenital severe valvular aortic stenosis with valvular pulmonary stenos...

Endovascular Treatment for Primary Aortic Angiosarcoma to Relieve Thoracic Aortic Stenosis.

Aortic aneurysms and stenosis are widely treated with endovascular procedures in the current era. In this report, we present endovascular stent grafting for symptomatic treatment of severe thoracic aortic stenosis caused by an aortic sarcomatoid carcinoma in a 73-year-old female. The peri- and post-operative courses were complicated with tumor embolisms that were managed both endovascular and surgical measures.

The impact of the metabolic syndrome on the outcome after aortic valve replacement.

The aim of this study was to examine the influence of the metabolic syndrome on the left ventricular geometry as well as on the early and mid-time outcome in patients with aortic stenosis who underwent aortic valve replacement.

Percutaneous balloon aortic valvuloplasty in different age groups.

Aortic stenosis is a congenital or acquired reduction in the area of the aortic valve, resulting in obstruction of the blood flow from the left ventricle to the aorta. Aortic stenosis accounts for 2-5% of all congenital heart defects and is a potentially life-threatening disorder. In adults aortic stenosis represents 34% of all valvular heart diseases. Degenerative etiology is present in 80% of cases. Patients with mild aortic stenosis are usually asymptomatic. Symptoms of the disease occur along with the d...

Postcardiotomy mechanical circulatory support in two infants with williams' syndrome.

Supravalvar aortic stenosis (SVAS) in patients with Williams' syndrome is often accompanied by coronary, pulmonary, and even myocardial lesions and therefore associated with increased perioperative morbidity and mortality. Extracorporeal membrane oxygenation (ECMO) provides reliable short-term mechanical circulatory support to patients, especially young, in acute postoperative cardiac failure when conventional means are ineffective. The incorporation of centrifugal pumps in these systems has made their use ...

Treatment of aortic stenosis with a self-expanding transcatheter valve: the International Multi-centre ADVANCE Study.

Transcatheter aortic valve implantation has become an alternative to surgery in higher risk patients with symptomatic aortic stenosis. The aim of the ADVANCE study was to evaluate outcomes following implantation of a self-expanding transcatheter aortic valve system in a fully monitored, multi-centre 'real-world' patient population in highly experienced centres.

97 Value of Cardiopulmonary Exercise and Non-invasive Haemodynamic assessment during Exercise in Patients with Asymptomatic Severe Aortic Stenosis.

Timing of aortic valve replacement in patients with severe asymptomatic aortic stenosis (VD) is controversial. Exercise testing may uncover symptoms and early cardiac dysfunction. However there is little information on the value of cardiopulmonary exercise (CPX) testing combined with haemodynamic assessment during exercise in this patient group. We report preliminary results of CPX and haemodynamic data in this patient cohort.

Anaesthesia-related haemodynamic complications in Williams syndrome patients: A review of one institution's experience.

Williams syndrome is a genetic disorder associated with cardiac pathology, including supravalvular aortic stenosis and coronary artery stenosis. Sudden cardiac death has been reported in the perioperative period and attributed to cardiovascular pathology. In this retrospective audit, case note and anaesthetic records were reviewed for all confirmed Williams syndrome patients who had received an anaesthetic in our institution between July 1974 and November 2009. There were a total of 108 anaesthetics adminis...

18F-FDG Uptake and Calcifications on Positron Emission Tomography/Computed Tomography in octogenarians with severe aortic stenosis.

The degree of inflammation within the atherosclerotic plaque can be detect non--invasively by positron emission tomography (PET) with 18F--fluorodeoxyglucose (18F--FDG). The incidence of aortic plaques with 18F--FDG increased uptake in octogenarians with aortic stenosis is unknown. Aim of this study was to evaluate the frequency of inflamed aortic atherosclerotic plaques in octogenarians with or without severe aortic stenosis and their correlations with calcifications.

Acute coronary syndrome as a result of left main coronary artery stenosis after aortic valve replacement. A report of three cases.

Acute coronary syndrome (ACS) as a result of iatrogenic coronary ostial stenosis (ICOS) is a rare but potentially life-threatening complication of aortic valve replacement (AVR). We present three cases of patients with ACS shortly after AVR, in whom ICOS were revealed. They refused an operation and thus they were treated with percutaneous coronary intervention. The potential pathomechanisms of ICOS and treatment options are discussed.

What is the ultimate test that lowering lipoprotein(a) is beneficial for cardiovascular disease and aortic stenosis?

Lipoprotein(a) [Lp(a)] is a risk factor for cardiovascular disease (CVD) and calcific aortic valve stenosis. We review recent studies that highlight Lp(a) in CVD and calcific aortic valve stenosis and propose pathways to clinical registration of Lp(a)-lowering agents.

The aortic valve: structure, complications and implications for transcatheter aortic valve replacement.

The aortic valve operates in a complex haemodynamic environment, opening and closing over 100,000 times a day. When complications arise, such as aortic stenosis, prognosis can be very poor, leading to death within the first few years. Surgical valve replacement is currently the standard treatment for aortic stenosis. A thorough understanding of the anatomy and function of the native valve is imperative when developing a prosthetic replacement that can withstand the complex demands of the heart. This review ...

Similar morphology, but different function: acute improvement of myocardial longitudinal strain after percutaneous transcatheter aortic valve implantation therapy in a severe aortic stenosis patient.

Novel SMAD3 Mutation in a Patient with Hypoplastic Left Heart Syndrome with Significant Aortic Aneurysm.

Aneurysms-osteoarthritis syndrome (AOS) caused by haploinsufficiency of SMAD3 is a recently described cause of syndromic familial thoracic aortic aneurysm and dissection (TAAD). We identified a novel SMAD3 mutation in a patient with hypoplastic left heart syndrome (HLHS) who developed progressive aortic aneurysm requiring surgical replacement of the neoaortic root, ascending aorta, and proximal aortic arch. Family screening for the mutation revealed that his father, who has vascular and skeletal features of...

Early and mid-term results of isolated aortic valve replacement for aortic stenosis in octogenarians.

As transcatheter aortic valve implantation (TAVI) may become a potential treatment for high-risk patients with aortic stenosis (AS), evaluation of outcomes after open aortic valve replacement (AVR) in elderly patients is warranted. We documented early and late outcomes after isolated AVR in octogenarians compared with younger age groups.

Expression of uPA, tPA, and PAI-1 in Calcified Aortic Valves.

Purpose. Our physiopathological assumption is that u-PA, t-PA, and PAI-1 are released by calcified aortic valves and play a role in the calcification of these valves. Methods. Sixty-five calcified aortic valves were collected from patients suffering from aortic stenosis. Each valve was incubated for 24 hours in culture medium. The supernatants were used to measure u-PA, t-PA, and PAI-1 concentrations; the valve calcification was evaluated using biphotonic absorptiometry. Results. Aortic stenosis valves expr...

High-Risk Patients With Inoperative Aortic Stenosis: Use of Transapical, Transaortic, and Transcarotid Techniques.

Patient characteristics and procedural outcomes from nontransfemoral (non-TF) transcatheter aortic valve replacement (TAVR) in high-risk or inoperable patients with aortic stenosis have been incompletely reported. The purpose of this study was to compare outcomes with non-TF TAVR access techniques including transapical (TA), transaortic (TAo), and transcarotid (TC) TAVR with a balloon-expandable valve.

High-sensitivity troponin I concentrations are a marker of an advanced hypertrophic response and adverse outcomes in patients with aortic stenosis.

High-sensitivity cardiac troponin I (cTnI) assays hold promise in detecting the transition from hypertrophy to heart failure in aortic stenosis. We sought to investigate the mechanism for troponin release in patients with aortic stenosis and whether plasma cTnI concentrations are associated with long-term outcome.

Circulating Levels of Matrix Gla Protein and Progression of Aortic Stenosis: A Substudy of the Aortic Stenosis Progression Observation: Measuring Effects of Rosuvastatin (ASTRONOMER) Trial.

Matrix γ-carboxyglutamate protein is an inhibitor of cardiovascular calcification. The objective of this substudy of the Aortic Stenosis Progression Observation: Measuring Effects of Rosuvastatin (ASTRONOMER) trial was to examine the relationship between total (ie, carboxylated [active] form + uncarboxylated [inactive] form) circulating desphosphorylated matrix γ-carboxyglutamate protein (dpMGP) level and the progression rate of aortic stenosis (AS).

Coronary microvascular dysfunction and aortic stenosis: An update.

The coronary microcirculatory impairment is a key feature of the pathophysiology of aortic stenosis (AS), the most operated valvular disease over the world. Several studies showed this coronary microcirculatory impairment in AS, using different tools and protocols, in various patient population of AS. This article will review the impairment of the coronary microcirculation in AS underlining its multifactorial origin, its functional part related to the hemodynamic consequences of AS, its complex relationship...

Coronary artery disease and transcatheter aortic valve replacement: current treatment paradigms.

Aortic stenosis is the most common form of valvular heart disease in the elderly population and is often diagnosed in individuals who also have coronary artery disease. Surgical aortic valve replacement has been the standard of care for the treatment of aortic stenosis during the past decades, but the availability of transcatheter aortic valve replacement has now allowed different options for high or extreme surgical risk patients. The management of coronary artery disease in patients undergoing transcathet...

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