Golda A.,Silesian Center for Heart Diseases |
Jurecka A.,Children Memorial Hospital |
Jurecka A.,University of Gdansk |
Tylki-Szymanska A.,Children Memorial Hospital
International Journal of Cardiology | Year: 2012
The aim of the article is to gather and summarize the published data about the incidence, course of illness, treatment possibilities and complications of cardiovascular disorders in patients with mucopolysaccharidosis type VI (MPS VI) also known as Maroteaux-Lamy syndrome. MPS VI is a lysosomal storage disorder caused by deficient activity of N-acetylogalactosamine-4-sulfatase leading to progressive intracellular accumulation of glycosaminoglycans. The relatively low birth prevalence ranging from 1 in 43,000 to1 in 1.5 million births mirrors the limited descriptions of the cardiovascular disorders in the medical literature. Patients with MPS VI can be specifically treated with enzyme replacement therapy. Extra-cardiac features include growth retardation, coarse facial features, stiff joints, skeletal malformations (dysostosis multiplex), respiratory problems, corneal clouding, and hepatosplenomegaly. The clinical presentation varies considerably, however the development of heart disease and cardiac dysfunction is a serious problem in the majority of patients. The most characteristic cardiac presentation is valvular disease, while other MPS VI patients also develop cardiomyopathy, fibroelastosis, pulmonary hypertension, cardiac conduction system disorders and other complications. There are also reports on acute heart failure. Early cardiovascular manifestation may escape detection since joint stiffness or skeletal malformations limit maximal exercise levels and respiratory system involvement may mask the underlining cardiac insufficiency. A correct and timely diagnosis offers the possibility of disease-specific treatment leading to sustained clinical benefits for cardiac and non-cardiac MPS VI manifestations. © 2011 Elsevier Ireland Ltd.
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 | Year: 2012
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.
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 | Year: 2011
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.
Sadowski M.,Swietokrzyskie Centrum Kardiologii |
Gasior M.,Silesian Center for Heart Diseases |
Gierlotka M.,Silesian Center for Heart Diseases |
Janion M.,Swietokrzyskie Centrum Kardiologii |
And 2 more authors.
EuroIntervention | Year: 2011
Aims: Clinical outcomes in the treatment of acute ST-segment elevation myocardial infarction (STEMI) differ between men and women. The aim of the study was to compare results of STEMI management in a large multicentre national registry. Methods and results: A total of 456 hospitals (including 58 interventional centres) participated in the registry during one year. The study group consisted of 8,989 (34.5%) females and 17,046 (65.5%) males. Women were older (69.7±11 vs. 62±12 years; p<0.0001) and had more risk factors. Percutaneous coronary intervention was performed significantly less in women (47.8% vs. 57.4%; p<0.0001). There was a longer time delay in women at each stage of treatment. The incidence of in-hospital complications was higher in women. In-hospital (11.9% vs. 6.9%; p<0.0001) and 12-months (22% vs. 14.1%; p<0.0001) mortality was significantly higher in women. In multivariate analysis pulmonary oedema, cardiogenic shock, cardiac arrest, age, diabetes and anterior infarction significantly increased both in-hospital and long-term mortality. The in-hospital mortality was higher in the female group. Conclusions: Despite poor clinical characteristics, less than satisfactory management and a worse prognosis of STEMI in women, being a women itself is not a risk factor for increased long-term mortality, however, other well known risk factors affecting the prognosis relate frequently to the female gender. © Europa Edition 2011. All rights reserved.
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 | Year: 2011
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.
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 | Year: 2016
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.
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 | Year: 2013
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.
Banaszak B.,Medical University of Silesia, Katowice |
Banaszak P.,Silesian Center for Heart Diseases
Pediatric Nephrology | Year: 2012
Background Recently, a number of reports have highlighted changes in the histopathology and response to corticosteroid treatment in childhood nephrotic syndrome; however, these involved ethnically mixed populations. For comparison, the purpose of our research was to search for changes in the characteristics of nephrotic syndrome in a homogeneous population of Caucasian children over two consecutive decades. Methods Chart analysis was performed to identify children with new-onset nephrotic syndrome. The children were admitted to the Division of Pediatric Nephrology, Zabrze, during two periods: 1986-1995 (76 patients) and 1996- 2005 (102 patients). Specifically, a comparison of clinical characteristics and morphology of nephrotic syndrome between the two groups was performed. Steroid resistance was defined as no remission within 8 weeks of corticosteroid treatment. Histopathology was available in 36.8% and 43.1% of patients respectively. Results There was a significant increase in primary steroid resistance in the latter decade: 15.8% vs 31.4% (P00.017). Changes in the histopathology did not reach the level of statistical significance: minimal change nephrotic syndrome 25% vs 9% (P00.095), mesangial proliferative glomerulonephritis 46.4% vs 61.3% (P00.21), focal segmental glomerulosclerosis 17.9%vs 20.4%(P00.78), membranoproliferative glomerulonephritis 7.1% vs 6.8% (P01.0), membranous glomerulonephritis 3.6% vs 0% (P00.39). Conclusions Our results show the increasing incidence of primary steroid resistance in childhood nephrotic syndrome. © The Author(s) 2012.
Knapik P.,Silesian Center for Heart Diseases |
Rychlik W.,Intensive Care Unit |
Duda D.,Intensive Care Unit |
Golyszny R.,Intensive Care Unit |
And 2 more authors.
Resuscitation | Year: 2012
Objectives: Therapeutic hypothermia improves survival and neurological outcome in patients successfully resuscitated after cardiac arrest. Accurate temperature control during cooling is essential to prevent cooling-related side effects. Methods: Prospective observational study of 12 patients assessed during therapeutic hypothermia (32-34°C) achieved by intravascular cooling following cardiac arrest. Simultaneous temperature measurements were taken using a Swan-Ganz catheter (blood temperature BLT), nasopharyngeal probe (nasopharyngeal temperature NPT) and the urinary bladder catheter (urinary bladder temperature UBT). A total of 1728 measurements (144 measurements per patient) were recorded over a 48-h period and analyzed. Blood temperature was considered as the reference measurement. Results: Temperature profiles obtained from BLT, NPT and UBT compared with the use of analysis of variance did not differ significantly. Pearson correlation revealed that the correlation between BLT and NPT as well as BLT and UBT was statistically significant (r=0.96, p<0.001 and r=0.95, p<0.001, respectively). Bland-Altman analysis proved that the agreement between all measurements was satisfactory and the differences were not clinically important. Conclusions: In 12 post-cardiac arrest patients undergoing intravascular cooling, both nasopharyngeal and urinary bladder temperature measurements were similar to blood temperatures measured using a pulmonary artery catheter. © 2011 Elsevier Ireland Ltd.
Knapik P.,Silesian Center for Heart Diseases |
Ciesla D.,Silesian Center for Heart Diseases |
Filipiak K.,Silesian Center for Heart Diseases |
Knapik M.,Silesian Center for Heart Diseases |
Zembala M.,Silesian Center for Heart Diseases
European Journal of Cardio-thoracic Surgery | Year: 2011
Objective: To establish what is the prevalence of elevated HbA1c among diabetic patients scheduled for coronary surgery and whether this may influence their postoperative outcome. Methods: We performed a retrospective review of our departmental cardiac surgical database over a 3-year period (2006-2008). Among the 2665 patients, who underwent coronary revascularization, 782 (29.3%) patients had diabetes mellitus, with 735 (94.0%) patients having their HbA1c assessed preoperatively up to 3 days before the start of the procedure. Patients with preoperative normal or elevated HbA1c (>7%) were compared regarding their hospital mortality, morbidity (defined as any postoperative complication such as stroke, renal failure, wound infection, perioperative myocardial infarction (MI), and others) as well as mean intensive care unit (ICU) and hospital stay. Patients for comparisons were matched to achieve similar preoperative status with a use of a Greedy matching procedure. Chi-square test was performed to compare variables of interest; p< 0.05 was considered significant. Results: Elevated HbA1c levels were present in 38.4% of diabetic patients - 57.1% of patients among insulin-dependent diabetics, 27.3% of patients on oral medication, and in 7.7% of patients whose diabetes was treated with diet only. In a direct comparison and after matching for preoperative variables, elevated HbA1c levels increased only the frequency of perioperative MI (p= 0.01). Other complications, length of ICU and hospital stay as well as early mortality were similar in both the groups. Conclusions: Elevated HbA1c levels are common among diabetic patients scheduled for coronary surgery, particularly in patients receiving insulin, and are associated with more frequent occurrence of perioperative MI. © 2010 European Association for Cardio-Thoracic Surgery.