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

05:30 EDT 23rd October 2017 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 20,000+

Outcome of Combined Mitral and Aortic Valve Replacement in Adults With Mucopolysaccharidosis (the Hurler Syndrome).

We describe 2 adult sisters with type I mucopolysaccharidosis (MPS) who underwent combined mitral and aortic valve replacement for mitral and aortic valve stenosis. One died early postoperatively and the other survived but had a repeat double-valve replacement 1 month after the first. We analyzed previously reported patients with MPS and valve replacement to learn of their outcomes. The study of our 2 patients and those previously reported suggests that valve replacement in patients with MPS should be viewe...


Severe Valvular Aortic Stenosis and Fixed Subvalvular Aortic Stenosis: A Rare and Challenging Combination.

A 58-year-old man with a history of hypertension presented with accelerating angina. Transthoracic echocardiography revealed a thickened aortic valve with pressure gradients and an estimated aortic valve area suggestive of mild aortic stenosis. Left heart catheterization demonstrated non-significant coronary artery disease. Pressure tracings showed a high left ventricular pressure and a mean gradient across the aortic valve of 69 mmHg. Subsequent transesophageal echocardiography revealed a subvalvular aorti...

Outcome of isolated aortic valve replacement in patients with classic and paradoxical low-flow, low-gradient aortic stenosis.

To analyze operative outcomes and mid-term results after isolated aortic valve replacement (AVR) in low-flow, low-gradient aortic stenosis (LFLG AS) by comparing the 2 subcategories (classic low-flow, low-gradient aortic stenosis [CLFLG] and paradoxical low-flow, low-gradient aortic stenosis [PLFLG]).


The medically managed patient with severe symptomatic aortic stenosis in the TAVR era: Patient characteristics, reasons for medical management, and quality of shared decision making at heart valve treatment centers.

Little is known about patients with severe symptomatic aortic stenosis (AS) who receive medical management despite evaluation at a heart valve treatment center.

Lipoprotein(a) in patients with aortic stenosis: Insights from cardiovascular magnetic resonance.

Aortic stenosis is the most common age-related valvular pathology. Patients with aortic stenosis and myocardial fibrosis have worse outcome but the underlying mechanism is unclear. Lipoprotein(a) is associated with adverse cardiovascular risk and is elevated in patients with aortic stenosis. Although mechanistic pathways could link Lipoprotein(a) with myocardial fibrosis, whether the two are related has not been previously explored. In this study, we investigated whether elevated Lipoprotein(a) was associat...

High- Versus Low-Gradient Severe Aortic Stenosis: Demographics, Clinical Outcomes, and Effects of the Initial Aortic Valve Replacement Strategy on Long-Term Prognosis.

There is considerable debate on the management of patients with low-gradient severe aortic stenosis (LG-AS), defined as aortic valve area

Should TAVR Replace Surgery for Aortic Stenosis in Low- to Intermediate-Risk Patients?

Transcatheter aortic valve replacement (TAVR) was initially envisioned as a less invasive option for patients with severe symptomatic aortic stenosis who either were not candidates or were very high-risk candidates for surgical aortic valve replacement (SAVR). Based on data from the original Placement of Aortic Transcatheter Valve (PARTNER) trials as well as the CoreValve Pivotal trials, TAVR is now approved and accepted in the treatment of severe symptomatic aortic stenosis in extreme-risk and high-risk pa...

Watchful Waiting in Aortic Stenosis: the Problem of Acute Decompensation.

Acute decompensation with heart failure, angina or syncope may be the first indication of undiagnosed aortic stenosis, but should be uncommon when the disorder is known and managed by watchful waiting. There is a lack of information on the proportion of patients with acute decompensated aortic stenosis with and without a prior diagnosis and their outcomes.

Imaging adult patients with discrete subvalvar aortic stenosis.

Distinction between discrete subvalvar aortic stenosis and other causes of left ventricular outflow obstruction has important implications for predicting natural history and guiding the timing and type of intervention. Imaging, primarily transthoracic echocardiography (TTE), plays a pivotal role in the diagnosis and management of adults with subvalvar aortic stenosis.

P650Influence of fetunin-a level on progression of calcific aortic valve stenosis The COFRASA - GENERAC Study.

In recent years, pathophysiology of aortic stenosis (AS) has been considered as a possibly active inflammatory process, but its determinants remain unclear. Calcium tissue deposition observed in dilaysis patients have been linked to low level of Fetuin-A, a powerful inhibitor of ectopic calcification. It is thus suspected to play a role in development of aortic stenosis.

Prognostic implications of left ventricular asymmetry in patients with asymptomatic aortic valve stenosis.

Left ventricular (LV) regional hypertrophy in the form of LV asymmetry is a common finding in patients with aortic valve stenosis. The aim of this study was to test the hypothesis that LV asymmetry predicts future symptomatic status and indication for aortic valve replacement (AVR) in patients with asymptomatic aortic valve stenosis.

Full metal jacket: transfemoral aortic valve implantation for regurgitant valve after endovascular aortic repair†.

Transfemoral aortic valve implantation has become an almost routine interventional procedure for severe aortic stenosis in high-risk patients. Over time an increased number of experiences has led to unusual procedures. In this report, we present a successful valve-in-valve transfemoral aortic valve implantation in a patient with aortic regurgitation, who previously had debranching and thoracic endovascular aortic repair operations.

Recommendations on the echocardiographic assessment of aortic valve stenosis: a focused update from the European Association of Cardiovascular Imaging and the American Society of Echocardiography.

Echocardiography is the key tool for the diagnosis and evaluation of aortic stenosis. Because clinical decision-making is based on the echocardiographic assessment of its severity, it is essential that standards are adopted to maintain accuracy and consistency across echocardiographic laboratories. Detailed recommendations for the echocardiographic assessment of valve stenosis were published by the European Association of Echocardiography and the American Society of Echocardiography in 2009. In the meantime...

Comparative study of bicuspid vs. tricuspid aortic valve stenosis.

To examine differences and similarities in bicuspid aortic valve (BAV) and tricuspid aortic valve (TAV) stenosis occurring during the same age and whether any differences impact outcomes following aortic valve replacement (AVR).

Comparison of preoperative and intraoperative assessment of aortic stenosis severity by echocardiography.

General anaesthesia and surgically induced changes in cardiac loading conditions may alter flow across the aortic valve. This study examined how echocardiographic assessment of the severity of aortic stenosis (AS) changes during surgery.

Prevalence and Impact of Prosthesis-Patient Mismatch Following Surgical Aortic Valve Replacement for Pure Aortic Regurgitation.

Prosthesis-patient mismatch (PPM) is highly prevalent among patients undergoing aortic valve replacement (AVR) to treat aortic stenosis. Data regarding the prevalence and impact of PPM on left ventricular remodeling and outcomes in patients who have undergone surgical AVR to treat pure severe aortic regurgitation (AR) are, however, scarce.

Differential Expression of MicroRNAs in Calcific Aortic Stenosis.

Calcific aortic stenosis (CAS) is the most common heart valve disorder. To explore the underlying mechanisms, we investigated whether key microRNAs in calcified aortic valves are differentially expressed compared to those in the non-calcified valves.

Do you know this syndrome? Leopard syndrome.

Hypertrophic cardiomyopathy is known as Leopard syndrome, which is a mnemonic rule for multiple lentigines (L), electrocardiographic conduction abnormalities (E), ocular hypertelorism (O), pulmonary stenosis (P), abnormalities of genitalia (A), retardation of growth (R), and deafness (D). We report the case of a 12-year-old patient with some of the abovementioned characteristics: hypertelorism, macroglossia, lentigines, hypospadias, cryptorchidism, subaortic stenosis, growth retardation, and hearing impairm...

How should I treat a patient with a symptomatic and severe low-flow low-gradient aortic stenosis and an incidental abdominal aortic aneurysm?

Long term survival and cardiopulmonary outcome in children with Hurler syndrome after haematopoietic stem cell transplantation.

Premature death in untreated children with Hurler syndrome (HS) in the first decade of life is largely due to life-threatening cardiopulmonary complications. We examined the long-term survival and cardiopulmonary outcome in 54 children undergoing haematopoietic stem cell transplantation (HSCT) at the Royal Manchester Children's Hospital from 1985 to 2008. The median age at first HSCT was 15.1 months. Eighteen had graft failure and nine died after first HSCT. Of 18 patients with graft failure, 17 underwent s...

Severe Aortic Stenosis in Dialysis Patients.

Characteristics and prognosis of hemodialysis patients with severe aortic stenosis have not yet been well defined.

Impact of Vascular Hemodynamics on Aortic Stenosis Evaluation: New Insights Into the Pathophysiology of Normal Flow-Small Aortic Valve Area-Low Gradient Pattern.

About 50% of normal-flow/low-gradient patients (ie, low mean gradient [MG] or peak aortic jet velocity and small aortic valve area) have severe aortic valve calcification as measured by computed tomography. However, they are considered to have moderate aortic stenosis (AS) in current American College of Cardiology/American Heart Association guidelines. The objective was thus to evaluate the effect of hypertension and reduced arterial compliance (rAC) on MG and Vpeak measurements.

Comparative outcomes of transcatheter aortic valve replacement in African American and Caucasian patients with severe aortic stenosis.

Racial disparities in cardiovascular care have been extensively investigated. The introduction of transcatheter aortic valve replacement (TAVR) revolutionized the treatment of aortic stenosis (AS) in the last decade. Whether a racial disparity in the utilization and outcome of TAVR exists is unknown.

Staging classification of aortic stenosis based on the extent of cardiac damage.

In patients with aortic stenosis (AS), risk stratification for aortic valve replacement (AVR) relies mainly on valve-related factors, symptoms and co-morbidities. We sought to evaluate the prognostic impact of a newly-defined staging classification characterizing the extent of extravalvular (extra-aortic valve) cardiac damage among patients with severe AS undergoing AVR.

End-stage renal disease and severe aortic stenosis: Does valve replacement improve one-year outcomes?

Treatment for patients with end-stage renal disease (ESRD) and severe aortic stenosis (AS) includes balloon aortic valvuloplasty (BAV), surgical (SAVR), or transcatheter (TAVR) aortic valve replacement. We compared outcomes among these strategies.


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