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

17:25 EDT 24th May 2017 | BioPortfolio

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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 21,000+

Misconceptions and Facts about Aortic Stenosis.

Aortic stenosis is the most common valvular heart disease leading to intervention and it is typically a disease of the elderly. Recent clinical advances have expanded the role of transcatheter aortic valve intervention in patients with severe aortic stenosis making aortic valve intervention feasible and effective even in patients at intermediate, high and prohibitive surgical risk. With the rapid advances in treatment proper diagnosis becomes crucial for a wide range of patients with aortic stenosis: from '...


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.

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 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.

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.

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.

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

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...

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.

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 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.

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...

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...

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...

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.

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.

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...

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.

Infective endocarditis by Abiotrophia defectiva diagnosed by mass spectrometry (MALDI-TOF MS) in Argentina.

We report the case of a 63-year-old woman with congestive heart failure due to a bicuspid aortic valve and severe aortic stenosis. The patient had a febrile syndrome with positive blood cultures for Abiotrophia defectiva, Transesophageal echocardiogram revealed the presence of paravalvular abscess, which was treated by a successful valve replacement. The patient received appropriate antibiotic therapy with intravenous vancomycin, leading to a successful response. The use of MALDI-TOF MS as a rapid and speci...

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).

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...

Sutureless aortic valves: who is the right patient?

Sutureless aortic valve replacement (AVR) has emerged as an alternative to traditional AVR for patients with aortic stenosis who present a higher surgical risk, such as the elderly, or those with small or highly calcified aortic roots. With transcatheter aortic valve implantation - the other major AVR alternative - being used in increasingly lower-risk patients, the place of sutureless valves in the AVR landscape needs to be defined. In this review, we discuss recent data and expert opinion as it pertains t...

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...

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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