PubMed Journals Articles About "Aortic Stenosis Patient With Hurler Syndrome After Bone" 
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Showing "Aortic stenosis patient with Hurler syndrome after bone" PubMed Articles 1–25 of 45,000+
Aortic stenosis in a patient with Hurler's syndrome after bone marrow transplantation.
We describe a case of severe aortic stenosis in a 16-year-old male with Hurler's syndrome who had prior bone marrow transplantation. The excised aortic valve leaflets showed characteristic pathologic findings of Hurler's syndrome. This is the first case report of aortic valve replacement in a patient with Hurler's syndrome treated with bone marrow transplantation that demonstrates progression of the aortic valve disease despite treatment.
Long-term outcome of large artificial patch aortic repair for diffuse stenosis in Williams syndrome.
There have been only a few reports concerning the long-term results of a surgical procedure using a large artificial patch for patients with Williams syndrome. Twelve years have passed since a patient with William's syndrome underwent a surgery with a patch angioplasty for the diffuse supravalvular aortic stenosis and deformities of the neck branch arteries. The patient had a well-balanced aortic growth without stenotic or aneurysmal changes, which was confirmed during the time of the second surgery when re...
Alagille Syndrome: An Unusual Presentation of an Uncommon Disease.
Alagille syndrome is a rare genetic disorder that results in intrahepatic cholestasis. Cardiac involvement mainly involves stenosis of branches of the pulmonary arteries. Aortic valve involvement is less common. To our knowledge, this is the first case of Alagille syndrome associated with severe aortic stenosis due to a bicuspid aortic valve.
A 39-year-old woman with familial homozygous hypercholesterolemia had supravalvular and valvular aortic stenosis. Modified Nick's procedure and aortic valve replacement was performed to relieve both the supravalvular and annular stenoses. At surgery, the ascending aorta was found to be narrowing at the level of the sinotubular junction, which was compatible with congenital supravalvular aortic stenosis. Histological examination of the aortic cusps showed sclerotic change due to hypercholesterolemia. These f...
Aim: To assess the effect of bone marrow transplantation (BMT), enzyme replacement therapy (ERT), and a fiberoptic endotracheal intubation technique in patients with mucopolysaccharidosis type I (MPS I, Hurler syndrome). Background: The mucopolysaccharidoses are inherited metabolic conditions with a well-documented association with difficult airway management. We present the largest series to date of patients with Hurler syndrome (MPS I) and look at the impact of new treatments, such as BMT and ERT, o...
This study examined the clinical course of patients with asymptomatic severe aortic stenosis (AS) according to the new proposed aortic valve stenosis grading classification.
Surgical treatment of aortic valve disease.
The development of intraoperative transoesophageal echocardiography together with improved understanding of the functional anatomy of the aortic valve have allowed the design of several new conservative procedures, such as aortic valve-sparing operations, to treat patients with aortic root aneurysms or aortic insufficiency. The long-term results of these procedures have been excellent, and >90% of patients are free from reoperation on the aortic valve 10-15 years after surgery. Incompetent bicuspid aortic v...
This report describes a technique for repairing an aortic stenosis in an elderly patient with a small aortic annulus and a narrow left ventricular outflow tract. Preoperative echocardiography in an 80-year-old woman showed severe aortic stenosis with a narrow outflow tract: the aortic valve area was 0.48 cm(2), the aortic annular diameter was 14 mm, and the left ventricular outflow tract diameter was 14 mm. The Konno procedure was used to enlarge both the small aortic annulus and the left ventricular outflo...
Impact of metabolic syndrome on progression of aortic stenosis: influence of age and statin therapy.
The aims of this study were to examine prospectively the relationship between metabolic syndrome (MetS) and aortic stenosis (AS) progression and to evaluate the effect of age and statin therapy on AS progression in patients with or without MetS.
Management of Concomitant Large Aortic Aneurysm and Severe Stenosis of Aortic Arc.
Primary large saccular aortic aneurysm with high grade stenosis of aortic arc is rare, and no standard therapy is available. We have encountered one case and successfully treated using a hybrid interventional approach. A 59-year-old woman with a 7-day history of headache, dizziness and chest pain, and a 5-year history of hypertension admitted and was diagnosed with transverse aortic aneurysm with sever aortic stenosis, the huge saccular aneurysm was located behind the transverse aortic arc. During surgery,...
We sought to evaluate whether the presence of pulmonary stenosis (PS), amongst other factors, influences the mortality and the rate of reoperations in the long-term follow-up of patients with supravalvular aortic stenosis (SVAS). We identified all patients with SVAS from our surgical database. The patients with multi-level aortic stenosis or concomitant cardiac procedures were excluded from this study. Follow-up (100 %) was conducted between 2008 and 2010. Twenty-six patients underwent surgery for SVAS bet...
Quality of life among patients with severe aortic stenosis.
BACKGROUND: The disease burden of patients with severe aortic stenosis is not often explored, while the incidence is increasing and many patients who have an indication for aortic valve replacement are not referred for surgery. We studied the quality of life of 191 patients with severe aortic stenosis, hypothesising that symptomatic patients have a far worse quality of life than the general population, which could enforce the indication for surgery. METHODS: The SF-36v2 Health Survey was completed by 191 co...
Hurler disease bone marrow stromal cells exhibit altered ability to support osteoclast formation.
Mucopolysaccharidosis type I (MPS IH; Hurler syndrome) is a rare genetic disorder that is caused by mutations in the α-L-iduronidase (IDUA) gene, resulting in the deficiency of IDUA enzyme activity and intra-cellular accumulation of glycosaminoglycans. A characteristic skeletal phenotype is one of the many clinical manifestations in Hurler disease. Since the mechanism(s) underlying these skeletal defects are not completely understood, and bone and cartilage are mesenchymal lineages, we focused on the chara...
Effect of Bisphosphonates on the Progression of Degenerative Aortic Stenosis.
Bisphosphonates appear to regulate mineralization in both bone and vasculature. Degenerative aortic stenosis (AS) is thought to be due to vascular calcification. We studied the effect of bisphosphonates on the progression of degenerative AS. A retrospective study was performed on patients >70 years, who had transthoracic echocardiograms (TTE) >1 year apart and an initial aortic valve area (AVA) of 0.6-2.0 cm(2). Patients were excluded if they had an ejection fraction
The study sought to compare echocardiographic with invasive hemodynamic data in patients with "paradoxic" aortic stenosis and in patients with conventionally defined severe aortic stenosis.
Assessment and management of aortic valve disease in patients with left ventricular dysfunction.
The onset of symptoms or left ventricular systolic dysfunction heralds a poor prognosis for patients with either aortic stenosis or aortic regurgitation. Echocardiography is the primary imaging modality for assessment of aortic valvular lesions. Cardiac catheterization is indicated to determine the severity of the aortic valve lesion when there is a discrepancy between the clinical findings and the results of echocardiography in patients with either symptoms or left ventricular dysfunction. For patients wit...
The aim of the study was to examine the predictive value of QRS duration and morphology during watchful waiting in asymptomatic patients with aortic stenosis (AS).
Aortic valve replacement for aortic stenosis caused by alkaptonuria.
We report a case of aortic stenosis associated with ochronosis in a 70-year-old man who underwent biologic aortic valve replacement. Intraoperative findings included ochronosis of a severely calcified pigmented aortic valve along with pigmentation of the intima of the aorta.
Atherosclerosis, Degenerative Aortic Stenosis and Statins.
Aortic stenosis is the most common valvular heart disease among adult subjects in western countries The current treatment for aortic stenosis is aortic valve replacement. The possibility of a medical treatment that can slow the progression of aortic stenosis is very fascinating and statins have been tested to reduce the progression of degenerative aortic stenosis (DAS). The rationale for statin treatment in DAS has a deep pathophysiological substrate, in fact inflammation and lipid infiltration constitute t...
Transcatheter aortic-valve replacement for inoperable severe aortic stenosis.
Transcatheter aortic-valve replacement (TAVR) is the recommended therapy for patients with severe aortic stenosis who are not suitable candidates for surgery. The outcomes beyond 1 year in such patients are not known.
The prognostic impact of ECG left ventricular strain and left ventricular hypertrophy (LVH) in asymptomatic aortic stenosis is not well described.
A prosthesis-patient mismatch (PPM) is present when the prosthetic valve is too small in relation to the patient's body size. The purpose of the present study was to investigate the frequency of PPM after the implantation of the Medtronic CoreValve System, and its relation to the clinical outcome. The indexed effective orifice area (EOA) was measured in 74 patients with symptomatic severe aortic stenosis, who had undergone successful transcatheter aortic valve implantation with the Medtronic CoreValve Syste...
The purpose of this study was to determine the prevalence of valvular aortic stenosis requiring surgery in patients with a pre-existing diagnosis of subaortic stenosis.
The purpose of this study was to clarify the impact of sex-related differences in transcatheter aortic valve implantation (TAVI) for high-risk patients with severe aortic stenosis.
Girls and women with Turner syndrome are at risk for aortic dissection and rupture. However, the size of the aorta and the clinical characteristics among those with Turner syndrome and dissection have received little attention.
