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

01:49 EST 17th January 2017 | BioPortfolio

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

Statins for aortic valve stenosis.

Aortic valve stenosis is the most common type of valvular heart disease in the USA and Europe. Aortic valve stenosis is considered similar to atherosclerotic disease. Some studies have evaluated statins for aortic valve stenosis.


Percutaneous Implantation of the self-expanding valve Prosthesis a patient with homozygous familial hypercholesterolemia severe aortic stenosis and porcelain aorta.

Transcatheter aortic valve implantation (TAVI) has shown favorable outcomes in patients with severe symptomatic aortic valve stenosis who are at high surgical risk or inappropriate for open heart surgery. However, concerns exist over treating patients who have porcelain aorta and familial hypercholesterolemia, due to the potential complications of aortic root and aortic annulus. In this case report, we present a patient with familial hypercholesterolemia, symptomatic severe aortic stenosis, previous coronar...

A novel explanation of corneal clouding in a bone marrow transplant-treated patient with Hurler syndrome.

One common complication of mucopolysaccharidosis I-Hurler (MPS1-H) is corneal clouding, which occurs despite current treatments, including bone marrow transplantation. Human corneas were obtained from a 14 year old subject with MPS1-H and visual disability from progressive corneal clouding despite prior bone marrow transplant at age 2. This was compared to a cornea from 17 year old donated to our eye bank after his accidental death. The corneas were analyzed microscopically after staining with Alcian blue, ...


Pathological Investigation of Congenital Bicuspid Aortic Valve Stenosis, Compared with Atherosclerotic Tricuspid Aortic Valve Stenosis and Congenital Bicuspid Aortic Valve Regurgitation.

Congenital bicuspid aortic valve (CBAV) is the main cause of aortic stenosis (AS) in young adults. However, the histopathological features of AS in patients with CBAV have not been fully investigated.

The frailty syndrome and mortality among very old patients with symptomatic severe aortic stenosis under different treatments.

The role of frailty as a prognostic factor in non-selected patients with symptomatic severe aortic stenosis (SAS) is still uncertain. This study aims to examine the association between the frailty syndrome and mortality among very old patients with symptomatic SAS, and to assess whether the association varies with the type of SAS treatment.

Low-gradient aortic stenosis.

An important proportion of patients with aortic stenosis (AS) have a 'low-gradient' AS, i.e. a small aortic valve area (AVA

Transcatheter Aortic Valve Replacement After Intraoperative Discovery of Porcelain Aorta in a Patient With Aortic Stenosis.

Transcatheter Aortic Valve Implantation for Treatment of Aortic Valve Stenosis: A Health Technology Assessment.

Surgical aortic valve replacement (SAVR) is the gold standard for treating aortic valve stenosis. It is a major operation that requires sternotomy and the use of a heart-lung bypass machine, but in appropriately selected patients with symptomatic, severe aortic valve stenosis, the benefits of SAVR usually outweigh the harms. Transcatheter aortic valve implantation (TAVI) is a less invasive procedure that allows an artificial valve to be implanted over the poorly functioning valve.

An Early Canadian Experience With the Correx Automated Coring and Apical Connector Device for Aortic Valve Bypass.

Aortic valve replacement is the standard of care for severe, symptomatic aortic valve stenosis (AS); however, anatomy or pre-existing comorbidities may preclude conventional or alternative transcatheter approaches. Aortic valve bypass (AVB) may be performed as a salvage procedure for the relief of symptomatic aortic stenosis in patients who are not suitable candidates for aortic valve replacement.

Implantation of a SAPIEN 3 Valve in a Patient with Pure Aortic Regurgitation.

Transcatheter aortic valve implantation (TAVI) is an emerging treatment for high-risk patients with aortic stenosis. Aortic regurgitation is considered to be a relative contraindication for transcatheter procedures, as a non-calcified aortic annulus poses the risk of an insufficient anchoring of the transcatheter aortic valve prosthesis. Herein is described the case of a patient who suffered from recurrent aortic valve regurgitation after valve-sparing repair, and which was successfully treated by the trans...

Repair of Bicuspid Aortic Valve Syndrome with Anomalous Right Coronary Artery in Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is an inherited connective tissue disorder caused by the defective synthesis of type I collagen. The clinical phenotype is dominated by bone fragility, but cardiovascular tissue involvement has also been reported. Here, the case is described of a 37-year-old man with OI who presented with aortic insufficiency, bicuspid aortic valve, dilated aortic root, and anomalous right coronary artery. The patient was treated successfully with a mechanical valved conduit aortic root replacem...

Prognostic Significance of Sleep Apnea Syndrome on False Lumen Aortic Expansion in Post-Acute Aortic Syndrome.

Obstructive sleep apnea syndrome (OSAS) is a risk factor for resistant arterial hypertension and aortic dilatation. We assessed the value of systematic screening for OSAS in patients soon after the onset of acute aortic syndrome (AAS).

Balloon valvuloplasty for aortic stenosis using umbilical vein access in a newborn: First experience in Turkey.

Balloon valvuloplasty is an effective therapy for severe congenital aortic valve stenosis, with mild aortic insufficiency and minimal intermediate-term restenosis. No consensus currently exists regarding optimal vascular approach for balloon dilatation in newborns with critical or severe aortic valve stenosis. Critical aortic valve stenosis in newborns must be treated promptly and effectively. Transcatheter therapy may offer marked advantages, as surgical therapy has been associated with significant rates o...

Discordant Grading of Aortic Stenosis Severity: Echocardiographic Predictors of Survival Benefit Associated With Aortic Valve Replacement.

This study sought to assess the survival benefit associated with aortic valve replacement (AVR) according to different strata of echocardiographic parameters of aortic stenosis (AS) severity, and especially in patients with an aortic valve area (AVA) comprised between 0.8 cm(2) and 1 cm(2).

Lipoprotein(a) and oxidized phospholipids in calcific aortic valve stenosis.

As the incidence of calcific aortic valve stenosis increases with the aging of the population, improved understanding and novel therapies to reduce its progression and need for aortic valve replacement are urgently needed.

Valve-in-valve-in homograft: A case of a repeat transcatheter aortic valve replacement in a patient with an aortic homograft.

In recent years, the use of transcatheter aortic valve replacement (TAVR) has extended beyond the treatment of native aortic valve stenosis in patients with high surgical risk. TAVR is increasingly being performed for bioprosthetic aortic valve failure, i.e., the valve-in-valve (VIV) procedure. Establishing the success of a VIV procedure can be challenging in these cases. Furthermore, the limited availability of prostheses sizes further complicates the management of these patients. We present an unusual cas...

Detection of a primary tumor in the area of the renal artery with 18F-FDG PET/CT in a patient with metastatic undifferentiated sarcoma and a history of mid-aortic syndrome: A case report.

We present a case of a 57-year-old woman patient with a history of mid-aortic syndrome, treated with several vascular procedures, who was referred for investigation of metastatic disease of an undifferentiated sarcoma of unknown origin.

Aortic Valve Morphology Correlates With Left Ventricular Systolic Function and Outcome in Children With Congenital Aortic Stenosis Prior to Balloon Aortic Valvuloplasty.

We sought to determine the relationship between aortic valve morphology and left ventricular (LV) systolic function in children with aortic stenosis (AS) prior to balloon aortic valvuloplasty (BAV).

The role of TTE in assessment of the patient before and following TAVI for AS.

ranscatheter aortic valve implantation is now accepted as a standard mode of treatment for an increasingly large population of patients with severe aortic stenosis. With the availability of this technique, echocardiographers need to be familiar with the imaging characteristics that can help to identify which patients are best suited to conventional surgery or transcatheter aortic valve implantation, and what parameters need to be measured. This article highlights the major features that should be assessed d...

Contained ruptured abdominal aortic aneurysm presenting as cauda equina syndrome.

This is the case report of a contained ruptured aortic aneurysm presenting with acute cauda equina syndrome. The patient was a 79-year-old man. A literature search revealed various unusual presentations of abdominal aortic aneurysm (AAA), including femoral neuropathy, hip pain and others; however, there are no other reports of cauda equina-like syndrome. The present case is therefore another unusual presentation of ruptured abdominal aortic aneurysm and reiterates the utmost importance of careful history ta...

Integration of Flow-Gradient Patterns Into Clinical Decision Making for Patients With Suspected Severe Aortic Stenosis and Preserved LVEF: A Systematic Review of Evidence and Meta-Analysis.

This study was designed to evaluate the evidence base for the 2014 American Heart Association/American College of Cardiology (AHA/ACC) guidelines for severe aortic stenosis (SAS) and preserved left ventricular ejection fraction by comparing the natural history of the disease in subgroups of low-gradient (LG) aortic stenosis patients with high-gradient (HG) patients.

Systolic hypertension and progression of aortic valve calcification in patients with aortic stenosis: results from the PROGRESSA study.

Hypertension is highly prevalent in patients with aortic stenosis (AS) and is associated with worse outcomes. The current prospective study assessed the impact of systolic hypertension (SHPT) on the progression of aortic valve calcification (AVC) measured by multidetector computed tomography (MDCT) in patients with AS.

Posttranscatheter Aortic Valve Replacement Ventricular Septal Defect During Transfemoral Edwards SAPIEN Valve Implantation.

Transcatheter aortic valve replacement (TAVR) is gaining favor as a treatment for aortic stenosis in patients at high risk for the open surgical approach. The following is a report of a 95-year-old woman with severe aortic stenosis who presented for TAVR with an Edwards SAPIEN valve. Her medical history included pacemaker-dependent complete heart block and a recent episode of congestive heart failure secondary to a non-ST segment elevation myocardial infarction. The TAVR was performed successfully through a...

High-Risk Transcatheter Aortic Valve Replacement with Evolut-R CoreValve to Simultaneously Treat Severe Calcific Aortic Stenosis and a Large Aortic Root Pseudoaneurysm.

Choice of Treatment for Aortic Valve Stenosis in the Era of Transcatheter Aortic Valve Replacement in Eastern Denmark (2005 to 2015).

The aim of this study was to evaluate the choice of treatment for severe aortic valve stenosis in the era of transcatheter aortic valve replacement (TAVR) in eastern Denmark.


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