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

03:07 EDT 25th June 2016 | BioPortfolio

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

Urgent resection of a giant left atrial appendage aneurysm and mitral valve replacement in a complex case of Hurler-Scheie syndrome.

Hurler-Scheie syndrome is a rare lysosomal storage disease affecting the cardiovascular system. Besides the cardiac manifestations, it presents with complications from abnormal proteoglycan deposition in soft tissues in many locations, resulting in joint contractures, paraplegia, impaired vision, airway narrowing and restrictive lung function, to name a few. There are very few reports of surgical management of valvular heart disease due to mucopolysaccharidosis (MPS). We describe the successful management o...


Regression of ventriculomegaly following medical management of a patient with Hurler syndrome.

Hurler syndrome is the most severe form of mucopolysaccharidosis (MPS) Type 1. Progressive neurocognitive decline in this condition can be accompanied by macrocephaly, ventriculomegaly, and/or periventricular signal changes on MRI, which often leads to a neurosurgical referral. In this case, the authors describe a 2-year-old boy with ventriculomegaly and periventricular T2 signal changes, both of which decreased following medical management of Hurler syndrome. The authors discuss the possible mechanisms for...

Osseous and chondromatous metaplasia in calcific aortic valve stenosis.

Aortic valve replacement for calcific aortic valve stenosis is one of the more common cardiac surgical procedures. However, the underlying pathophysiology of calcific aortic valve stenosis is poorly understood. We therefore investigated the histologic findings of aortic valves excised for calcific aortic valve stenosis and correlated these findings with their associated clinical features.


Paradoxical low-flow low-gradient aortic stenosis.

In approximately one third of patients presenting with suspected severe aortic stenosis, there is a discrepancy between a severely reduced aortic valve opening area (

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, ...

Severe aortic valve stenosis in the elderly: high prevalence of sleep-related breathing disorders.

Aortic valve stenosis is common in the elderly, with a prevalence of nearly 3% in patients aged 75 years or older. Despite the fact that sleep-related breathing disorders (SRBD) are thought to be associated with cardiac disease, little is known about their prevalence in this patient cohort. The purpose of this study was to evaluate the prevalence of SRBD in older patients with aortic valve stenosis admitted for transcatheter aortic valve implantation.

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

Type B aortic dissection triggered by heart transplantation in a patient with Marfan syndrome.

Heart transplantation in patients with Marfan syndrome is challenging and raises concerns with regards to the haemodynamic and immunosuppressive-induced effects on the inherently fragile aorta. Most aortic events following transplantation reported so far in the literature occurred in patients with pre-existent distal aortic dissection. We report a case of successful orthotopic heart transplantation in a patient with Marfan syndrome that was complicated by late-onset type B dissection in pre-existing mild an...

The association of severe aortic stenosis and narrow aortic root in a young patient - What is the etiology: Rheumatic valvulitis or Lambl's excrescences?

Patient-defined goals for the treatment of severe aortic stenosis: a qualitative analysis.

Patients with severe aortic stenosis (AS) at high risk for aortic valve replacement are a unique population with multiple treatment options, including medical therapy, surgical aortic valve replacement and transcatheter aortic valve replacement (TAVR). Traditionally, in elderly populations, goals of treatment may favour quality of life over survival. Professional guidelines recommend that clinicians engage patients in shared decision making, a process that may lead to decisions more aligned with patient-def...

Anesthetic management for craniotomy in a patient with massive cerebellar infarction and severe aortic stenosis: a case report.

Severe aortic stenosis combined with coronary heart disease remarkably increases the risk of perioperative morbidity and mortality during noncardiac surgery. Surgery and anesthesia often complicate the perioperative outcome if adequate monitoring and proper care are not taken. Therefore, understanding of the hemodynamic changes and anesthetic implications is an important for successful perioperative outcome. This report described the anesthetic management of a patient with a massive cerebellar infarction wh...

TCRαβ CD19 depletion in allogeneic haematopoietic stem cell transplantation performed for Hurler syndrome.

Aortic valve decalcification for severe aortic valve stenosis in the elderly: medium-term results.

To present the results of a novel technique of aortic valve decalcification (AVD) in a consecutive population of elderly patients with severe aortic valve stenosis (AVS) and small aortic annulus.

Expanding the phenotype of feingold syndrome-2.

Feingold syndrome-2 has been recently shown to be caused by germline heterozygous deletions of MIR17HG with 10 reported patients to date. Manifestations common to both Feingold syndrome-1 and Feingold syndrome-2 include microcephaly, short stature, and brachymesophalangy; but those with Feingold syndrome-2 lack gastrointestinal atresias. Here we describe a 14-year-old male patient who presented to our Cardiovascular Genetics Clinic with a history of a bicuspid aortic valve with aortic stenosis, short statur...

Aortic root, not valve, calcification correlates with coronary artery calcification in patients with severe aortic stenosis: A two-center study.

The underlying pathology in aortic stenosis (AS) and coronary artery stenosis (CAS) is similar including atherosclerosis and calcification. We hypothesize that coronary artery calcification (CAC) is likely to correlate with aortic root calcification (ARC) rather than with aortic valve calcification (AVC), due to tissue similarity between the two types of vessel rather than with the valve leaflet tissue.

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.

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).

Transcatheter aortic valve implantation using the Lotus valve system in severe aortic stenosis in an orthotopic heart transplant patient.

Aortic Valve Stenosis and Left Main Coronary Disease: Hybrid Approach.

We describe a technique of combined transcatheter aortic valve replacement (TAVR), off-pump single coronary artery bypass, and percutaneous coronary intervention (PCI) in a high-risk patient presenting with unstable angina and severe heart failure. This patient had documented moderate to severe aortic stenosis, left ventricular dysfunction, and a heavily calcified ascending aorta. A robotic-assisted left internal thoracic artery harvesting was aborted owing to inability to tolerate single-lung ventilation. ...

Transfemoral aortic valve implantation in severe aortic stenosis patients with prior mitral valve prosthesis.

Transcatheter aortic valve implantation for severe symptomatic aortic stenosis in patients with a previous mitral valve prosthesis is technically challenging, and pre-procedural comprehensive assessment of these patients before transcatheter aortic valve implantation is vital for an uncomplicated and successful procedure.

Prosthesis-patient mismatch in high-risk patients with severe aortic stenosis: A randomized trial of a self-expanding prosthesis.

We compared the incidence of prosthesis-patient mismatch (PPM) between transcatheter aortic valve replacement (TAVR) using a self-expanding bioprosthesis and surgical aortic valve replacement (SAVR) in the CoreValve US High Risk Pivotal Trial. We sought to determine the influence of PPM on clinical outcomes.

Transcatheter Aortic Valve Replacement Results in Improvement of Pulmonary Function in Patients With Severe Aortic Stenosis.

Chronic obstructive pulmonary disease (COPD) has been identified as a risk factor for morbidity and mortality after transcatheter aortic valve replacement (TAVR). We hypothesized that a portion of pulmonary dysfunction in patients with severe aortic stenosis may be of cardiac origin, and has potential to improve after TAVR.

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...

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.


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