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Jurecka A.,The Childrens Memorial Health Institute | Jurecka A.,University of Gdansk | Golda A.,Silesian Center for Heart Diseases | Opoka-Winiarska V.,Medical University of Lublin | And 2 more authors.
Molecular Genetics and Metabolism

We present here the first literature description of a predominantly cardiac phenotype in a patient homozygous for missense mutation p.R152W in the N-acetylogalactosamine-4-sulfatase (arylsulfatase B, ARSB) gene. An adult Caucasian woman, who displayed very few symptoms up to her late thirties, was diagnosed with mucopolysaccharidosis type VI (MPS VI) after her hospitalization due to acute heart failure originating mainly from valve disease. In addition to her cardiac phenotype some musculoskeletal involvement without other MPS characteristic features were found. Despite the common pharmacologic treatment and implementation of enzyme replacement therapy with galsulfase the patient died at the age of 38. years because of decompensation of chronic heart failure. © 2011 Elsevier Inc. Source

Zembala M.O.,Silesian Center for Heart Diseases | Irimie V.,Cardiovascular Clinic Bad Neustadt | Urbanski P.P.,Cardiovascular Clinic Bad Neustadt
Interactive Cardiovascular and Thoracic Surgery

A rare case of aortic arch aneurysm combined with chronic aortic dissection is reported. Because the visceral arteries originated from different, equivalently perfused lumens and the descending aorta was circumferentially calcified (porcelain aorta) limiting the possibilities of anastomosing, careful planning of the surgical strategy was of utmost importance. The complex surgery consisted of ascending and total arch replacement using the 'frozen elephant trunk' technique with Thoraflex™ Hybrid Prosthesis (Vascutek, Terumo, Inchinnan, Scotland); however, before insertion of the stent graft, an angioscopic resection of the dissection membrane in the proximal part of the descending aorta was carried out to ensure a complete expansion of the distal edge of the stent within the entire common lumen of the aorta and unimpaired distal flow in both lumens below the stent graft. The surgery and the postoperative course were uneventful. © 2016 The Author 2016. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. Source

Radomska E.,Swietokrzyskie Cardiology Center | Sadowski M.,Swietokrzyskie Cardiology Center | Kurzawski J.,Swietokrzyskie Cardiology Center | Gierlotka M.,Swietokrzyskie Cardiology Center | Polonski L.,Silesian Center for Heart Diseases
Diabetes Care

OBJECTIVE-To evaluate the effect of type 2 diabetes on the clinical course and prognosis of women with ST-segment elevation myocardial infarction (STEMI) and diabetes. RESEARCH DESIGN AND METHODSdA total of 26,035 consecutive patients with STEMI who were hospitalized in 456 hospitals in Poland during 1 year were analyzed. The data were obtained from the Polish Registry of Acute Coronary Syndromes (PL-ACS). RESULTS-Type 2 diabetes occurred more frequently in women than in men (28 vs. 16.6%; P < 0.0001). The proportion of women was larger among patients with diabetes (47.1 vs. 31.3%; P < 0.0001), and compared with women without diabetes, diabetic women had worse clinical profiles. Women with diabetes were most frequently treated conservatively. Both women and men with diabetes had significantly more advanced atherosclerotic lesions than women without diabetes.Women with diabetes had the highest in-hospital, 6-month, and 1-year mortality rates. Multivariate analysis indicated that type 2 diabetes was a significant independent risk factor for in-hospital and 1-year mortality in women with STEMI. Primary percutaneous coronary intervention (pPCI) was a significant factor associated with the decreased 1-year mortality in women without diabetes. CONCLUSIONS-Type 2 diabetes was a significant independent risk factor for in-hospital and 1-year mortality in women with STEMI. Women with diabetes had the poorest early and 1-year prognoses after STEMI when compared with women without diabetes and men with diabetes. Although pPCI improves the long-term prognosis of women with diabetes, it is used less frequently than in women without diabetes or men with diabetes. © 2013 by the American Diabetes Association. Source

Angert D.,Cardiovascular Research Center | Berretta R.M.,Cardiovascular Research Center | Kubo H.,Cardiovascular Research Center | Zhang H.,Cardiovascular Research Center | And 6 more authors.
Circulation Research

Rationale: The ability of the adult heart to generate new myocytes after injury is not established. Objective: Our purpose was to determine whether the adult heart has the capacity to generate new myocytes after injury, and to gain insight into their source. Methods and Results: Cardiac injury was induced in the adult feline heart by infusing isoproterenol (ISO) for 10 days via minipumps, and then animals were allowed to recover for 7 or 28 days. Cardiac function was measured with echocardiography, and proliferative cells were identified by nuclear incorporation of 5-bromodeoxyuridine (BrdU; 7-day minipump infusion). BrdU was infused for 7 days before euthanasia at days 10, 17, and 38 or during injury and animals euthanized at day 38. ISO caused reduction in cardiac function with evidence of myocyte loss from necrosis. During this injury phase there was a significant increase in the number of proliferative cells in the atria and ventricle, but there was no increase in BrdU+ myocytes. cKit+ cardiac progenitor cells were BrdU labeled during injury. During the first 7 days of recovery there was a significant reduction in cellular proliferation (BrdU incorporation) but a significant increase in BrdU+ myocytes. There was modest improvement in cardiac structure and function during recovery. At day 38, overall cell proliferation was not different than control, but increased numbers of BrdU+ myocytes were found when BrdU was infused during injury. Conclusions: These studies suggest that ISO injury activates cardiac progenitor cells that can differentiate into new myocytes during cardiac repair. © 2011 American Heart Association, Inc. Source

Opolski G.,Medical University of Warsaw | Strojek K.,Silesian Center for Heart Diseases | Kurzelewski M.,Sanofi S.A. | Ostrowski M.,Sanofi S.A. | Rabczenko D.,National Institute of Public Health
Polskie Archiwum Medycyny Wewnetrznej

Introduction: Diabetes mellitus (DM) and coronary artery disease (CAD) are associated with increased cardiovascular risk. Objectives: The aim of the study was to compare management of high-risk patients with DM and patients with CAD in Poland. Patients and methods: Randomly selected primary care offices enrolled patients aged 55 years and older, with DM and no documented CAD (n = 210) or with CAD and no documented DM (n = 186). Results: Statins were given to 64% vs. 87% ( P <0.05), acetylsalicylic acid (ASA) to 53% vs. 84% ( P <0.05), and angiotensin-converting enzyme inhibitors to 70% vs. 69% ( P = 0.8) of the patients with DM and CAD, respectively. Screening tests to detect glucose abnormalities in patients with CAD or to detect CAD in patients with DM were not performed in 26% of patients with DM and 24% of those with CAD (P = 0.64). Mean systolic blood pressure was 136.8 ± 13.6 vs. 131.7 ± 15.8 mmHg ( P = 0.001), diastolic blood pressure was 80.4 ± 7.4 vs. 79.4 ±11.6 mmHg ( P = 0.316), and total cholesterol was 196 ± 42 vs. 183 ± 42 mg/dl ( P = 0.003) in patients with DM and CAD, respectively. The percentage of patients with blood pressure below 140/90 mmHg, total cholesterol below 175 mg/dl, and low-density lipoprotein (LDL) cholesterol below 100 mg/dl was 15% vs. 25% (P = 0.055), while the percentage of patients with blood pressure below 130/80 mmHg, total cholesterol below 175 mg/dl, and LDL cholesterol <70 mg/dl was 1% vs. 3% (P = 0.016) in the DM vs. CAD groups, respectively. Conclusions: Use of statins and ASA was more frequent in patients with CAD than in patients with DM. Control of risk factors in the study population was better in the CAD group but still unsatisfactory in most patients. Copyright by Medycyna Praktyczna, 2012. Source

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