Biomedical subjects

A Linhart

Publications and source records attributed to A Linhart.

At least 37 records · Page 2Linked to original sources

PubMed2003

[Comparison of the cost effectiveness of treatment of acute myocardial infarct with primary angioplasty and thrombolysis ].

INTRODUCTION: Primary angioplasty (PTCA) or intravenous thrombolysis are the recommended treatment of acute myocardial infarction. According to results of clinical investigations however primary PTCA provides a more favourable short-term as well as long-term prognosis. As this method is much more expensive we were interested in its cost-effectiveness as compared with cheaper intravenous thrombolysis. METHODS: We constructed an decision analysis model (programme DATA 3.5, TreeAge Software) to compare the strategy of primary PTCA and intravenous thrombolysis in acute myocardial infarction. Probabilities of clinical outcomes were obtained from a long-term randomized clinical trial (Zijlstra et al. NEJM, 1999). The relative risk of death in PTCA was 0.54, rehospitalization 0.52 and reinfarction 0.27. The costs of PTCA (100,000,- crowns), of streptokinase thrombolysis (4000,- crowns) and hospitalization (2820,- crowns) were estimated from costs of the catheterization laboratory and information obtained from health insurance companies. We assumed that the subsequent costs of treatment and quality of life after the first infarction were the same in both strategies. In patients with reinfarction we anticipated a reduced quality of life (coefficient of life quality 0.9). The average effect of treatment and costs of both strategies were evaluated in the course of five years. As an acceptable cost-effectiveness (ratio of difference in costs and effect) we considered costs up to 200,000,- crowns per one gained year of life. RESULTS: In the basic analysis we revealed that after 5 years the strategy of primary PTCA is more expensive (125,000,- crowns vs. 4500,- crowns) but has a greater effect, i.e. a longer life span (4.38 vs. 3.81) adjusted to quality of life). The cost-effectiveness (ratio of difference in costs and effect) expressing the costs of one gained year of life when using primary PTCA as compared with thrombolysis was despite the high cost of PTCA acceptable and amounted to 140,350,- crowns. Analysis of the sensitivity of the model confirmed the stability of favourable cost-effectiveness within a wide range of costs and therapeutic effect. CONCLUSION: Primary PTCA is in acute myocardial infarction a cost-effective strategy) providing effect for an acceptable cost) despite the markedly higher costs of the procedure.

Angioplasty, Balloon, Coronary↗

PubMed2003

[Comparison of the occurrence of positive and negative vascular remodeling in patients with stable vs unstable angina pectoris].

BACKGROUND: The vascular remodelling refers to the increase or decrease in EEM (external elastica membrane) area that occurs during development of atherosclerosis. The positive remodeling, which was thought only as a compensatory factor during atheroma development, was also found as a one of the main features of unstable plaque. The intravascular ultrasound is very good tool to measure different type of arterial remodelling. These findings correlate with histologic post-mortem specimen with excellent results. AIM OF THE STUDY: To correlate the remodelling index from patients suffered from stable angina pectoris (SAP) and from patients suffered from unstable angina (UA). METHOD: We performed IVUS in 51 patients. In the group of patients with unstable angina were included patients with worsening angina symptoms in last six weeks or with angina in rest. We used IVUS--endosonics In-Vision with 30 MHz probe Awanar with mechanical pull-back. We studied the occurrence of positive remodelling, negative remodeling (an index that describes remodeling is expressed as: lesion EEM CSA/reference EEM CSA. If the lesion EEM area is greater than the reference EEM area, positive remodelling has occurred, and the index will be > 1.0. If the lesion EEM area is smaller than the reference EEM area, negative remodelling has occurred, and the index will be < 1.0). RESULTS: We performed the intravascular ultrasound in 51 patients, 22 patients (43.1%) with unstable angina (UA) and in 29 patients (56.9%) stable angina (SAP). The positive remodelling was found in 14 patients (63.6%) in UA group vs. 5 patients (17.2%) in group with SAP, p < 0.05. The negative remodelling was found in 6 patients (27.3%) in UA group vs. 23 patients (79.3%) in SAP group, p < 0.05. CONCLUSION: Positive remodelling is more often found in patients with UA vs. in patients with SAP. Positive arterial remodelling is therefore one of the features of unstable atherosclerotic plaque.

Angina Pectoris↗

PubMed2003

[Contrast nephropathy and its prevention].

Contrast-induced nephropathy is one of the adverse events of diagnostic and therapeutic intravascular application of contrast agent. In general, the condition was defined as an increase in the serum creatinine concentration of more than 44 mmol/l or of more than 25% within 48 hours after the contrast agent administration. Other cause of creatinine increase should be excluded. Contrast-induced nephropathy has been reported to be the third leading cause of acute nephropathy in hospitalized patients, occurring at a rate of 1-6% in unselected population and of 30-50% in high-risk patients. One year mortality can be as high as 45% in high-risk patient population. The most important risk factors are chronic renal insufficiency, diabetes mellitus and high volume of contrast agent. Clinical presentation is mostly asymptomatic, but in some patients acute renal failure with necessity of hemodialysis can occur. Prevention is underlying tool in reducing of contrast-induced nephropathy incidence. It is based on the identification of risk patients, stop of medication which can increase risk of contrast-induced nephropathy and proper hydratation of patients before, during and after the contrast agent administration. In high-risk patients, non-ionic and low-osmolarity contrast agent should be used. Several clinical studies testing different drugs to prevent contrast-induced nephropathy were performed, but no convincing result has been found. Promising substancies are N-acetylcysteine and fenoldopam.

Contrast Media↗

PubMed2003

[Idiopathic aneurysm of the pulmonary artery].

Aneurysm of pulmonary artery (PAA) is relatively rare clinical finding and appears mostly in association with significant cardiovascular or pulmonary abnormalities and is frequently caused by pulmonary hypertension. Its diagnosis is not difficult. However, guidelines for the treatment were not yet established. Patients with PAA are at risk for sudden death by a rupture of aneurysm. Therefore, surgical correction (graft replacement) is the method of choice. Nevertheless, it seems that certain group of patients with asymptomatic idiopathic aneurysm of pulmonary artery without presence of any serious cardiovascular or pulmonary abnormality can profit from conservative approach. We refer a 62-years old man with idiopathic PAA, in whom we did not indicate surgical correction. However, the long-term follow-up is necessary.

Aneurysm↗

PubMed2003

[Long-term treatment with statins in patients with ischemic heart disease after coronary angioplasty].

UNLABELLED: Long-term statin therapy is the corner-stone in management of patients with coronary artery disease. PURPOSE OF STUDY: The aim of our study was to analyze the state of the statin therapy at patients undergoing percutaneous coronary intervention (PCI) and to determine predictors of long-term statin treatment. METHODS: We performed a retrospective study in 520 patients who underwent percutaneous coronary angioplasty in 2nd Dept. of Internal Medicine, 1st School of Medicine, Charles University, Prague during the year 2000. Data were collected from hospital records and from a mailed questionnaire. RESULTS: The response rate was 61.9% and the average response time was 11.6 +/- 3.5 months after PCI. Long-term statin therapy was prescribed in 52.5%. In patients with hypercholesterolemia 67.1% were treated in comparison with 32.3% treated patients without this diagnosis (p < 0.0001). Patients aged 70 years and older were treated significantly less frequently then younger individuals (30.6% vs. 61.3%, p < 0.0001). Patients with a history of prior revascularization procedure were treated significantly more often then patients undergoing the first procedure (64.8% vs. 49.8%, p < 0.05). Multivariate logistic regression analysis was applied to detect significant predictors of long-term statin therapy. Only hypercholesterolemia and statin prescription at discharge were identified as independent positive predictors, whereas age > or = 70 years and male gender had negative predictive value. CONCLUSION: By course of evidence-based medicine, patients who underwent PCI in our study are undertreated by statins. Statin treatment should be initiated in all patients treated by PCI with increased cardiovascular risk. Patients at defined risk for undertreatment are mainly older patients and men. The prescription of statin therapy at the time of hospital discharge appears to be a very effective tool to improve long-term statin therapy.

Aged↗

PubMed2003

Interventional treatment of multiple vascular involvement in young male with heterozygous familial hypercholesterolemia.

We report a case of 31 year old man with heterozygous familial hypercholesterolemia and excessive tobacco use leading to acute myocardial infarction as the first manifestation of premature atherosclerosis. The patvent was treated by primary PTCA of occluded first marginal artery and at this time an attempt of recanalisation of occluded LAD was unsuccessful. The patient was referred for mini coronary bypass graft of the LIMA to LAD. During evaluation of carotid arteries we found a significant stenosis of the left internal carotid artery and occlusion of the left subclavian artery which made the use of LIMA unsuitable. Therefore, another attempt of PTCA of the occluded LAD was performed, this time with success. Hence PTA of the occluded subclavian artery was performed with good result. The patient was treated with the standard therapy of CAD and combined lipid lowering agents with significant reduction of plasma cholesterol. However, 2 years after the first MI, he suddenly died after swimming at the age of 31. In this patient the risk of premature CAD was increased by the presence of another powerful risk factor--the excessive tobacco use. Acute physical exercise probably acted as a trigger of acute coronary events at the time of both MI. Interventional methods were very effective in the treatment of multiple atherosclerotic lesions in this patient and provided significant relief of symptoms. Treatment of heterozygous FH is briefly discussed in this article.

Adult↗

PubMed2003

[Long-term prognosis in patients with acute myocardial infarct and treatment with direct coronary angioplasty].

BACKGROUND: Primary coronary angioplasty (PTCA) has a beneficial effect on the immediate prognosis for patients with acute myocardial infarction. Number of information about effects of direct PTCA on the long-term prognosis are less numerous. The aim of the work was to establish the long-term prognosis for not-selected patients treated by direct PTCA. METHODS AND RESULTS: The studied group consisted of 279 patients with acute myocardial infarction treated by direct PTCA in years 1995 to 1999 for the period of 38 +/- 12 months. Part of them were out-door patients of our clinic. The necessary data of the other patients were obtained by a questionnaire and by a telephone contact. 45 (16%) patients were lost from the follow up. The mortality rate of the study group was compared with data in the central register of Czech Republic. Positive angiographic effect of the direct PTCA (residual stenosis < 50% + flow TIMI 3) was achieved in 90% of patients. 30-day mortality was 6.8%, after excluding patients with cardiogenic shock it decreased to 3.2%. 6 patients (2.2%) had non-fatal infarction within 30 days after the first attack. From 259 patients who survived the acute infarction phase 24 died during the next period of follow up, 18 (7%) patients had a relapse of non-fatal infarction. PTCA of the infarcted artery was done in 15% of patients, PTCA of another artery in 9% of patients. The aorthocoronary bypass was indicated in 6% of patients. Almost half of relapses occurred during the first year after the hospitalisation. The risk factors of the death during the follow up were the age > 70 years, ejection fraction < 35%, impairment of 3 or more coronary artery branches, i.m. in the history, duration of ischemia > 4 hours, and diabetes mellitus. The total mortality was 11.4% in the first year, 1.4% in the second and 3.3% in the third year of the follow up. CONCLUSIONS: The beneficial prognostic effect of the direct PTCA on patients with acute infarction carries through the whole period of follow up. Prognosis of the risk patients remains critical. Next revascularization of the infarcted artery was in our cohort of patients necessary in 21% of patients.

Angioplasty, Balloon, Coronary↗

PubMed2003

[Cardiogenic shock--a complex therapeutic approach].

Cardiogenic shock belongs to the most severe and immediately life-threatening complications of the acute myocardial infarction. Despite development of modern diagnostic and therapeutic methods the incidence and mortality of cardiogenic shock has not significantly declined in the past decades. Early reperfusion strategy with percutaneous revascularization has become a cornerstone of therapy. The complex approach to cardiogenic shock comprises pharmacological and mechanical hemodynamic support, ventilatory support utilizing new ventilator regimens, metabolic and renal support/replacement with continuous renal replacement therapies and psychological, eventually psychopharmacological support. All these measures enable prevention of the multiple organ failure syndrome development and positively influence high mortality of patients suffering from cardiogenic shock.

Humans↗

PubMed2002

Cardiac involvement in Wilson disease.

BACKGROUND: Wilson disease is an inherited autosomal recessive disorder of copper metabolism resulting in pathological accumulation of copper in the liver, brain and other tissues. One of the reported manifestations is cardiac involvement. METHODS: We studied 42 patients with Wilson disease (19 men and 23 women, mean age 34 +/- 10 y) and 42 age- and sex-matched healthy volunteers. All subjects underwent complete echocardiographic examination; 24 h ECG Holter monitoring was performed in 23 Wilson disease patients. RESULTS: In comparison to healthy subjects, patients with Wilson disease had increased thickness of the interventricular septum (9.5 +/- 1.4 vs 8.6+/-1.1 mm, p < 0.01) and left ventriclular (LV) posterior wall (9.1 +/- 1.3 vs 8.2 +/- 1.0 mm, p < 0.01). While the two groups did not differ in LV mass index, relative LV wall thickness was significantly increased in Wilson disease patients compared to control subjects (0.39 +/- 0.06 vs 0.34 +/- 0.04 p < 0.001). Concentric LV remodelling was present in 9 patients (21%) and LV hypertrophy in one patient. Systolic LV function showed a nonsignificant trend towards lower values in Wilson disease patients (EF 62 +/- 5% vs 64 +/- 50%, p = 0.06). Diastolic filling and the frequency of valvular abnormalities were comparable in both groups. The established echocardiographic abnormalities did not correlate with the type of Wilson disease manifestation, the presence of the His1069Gln mutation, laboratory parameters or the duration and type of therapy. Twenty-four-hour ECG Holter monitoring detected ECG abnormalities in 10 patients (42%), the most frequent findings being runs of supraventricular tachycardias and frequent supraventricular ectopic beats. CONCLUSIONS: Cardiac involvement in Wilson disease patients was mild, characterized by LV parietal thickening with an increased prevalence of concentric LV remodelling and a relatively high frequency of benign supraventricular tachycardias and extrasystolic beats.

Adult↗

PubMed2002

Cardiac involvement in Fabry disease.

UNLABELLED: Fabry disease is a rare X-linked defect of the lysosomal enzyme alpha-galactosidase A. The disease is characterized by progressive intracellular accumulation of neutral glycosphingolipids. The storage occurs within various tissues and cells, including cardiocytes, the cardiac conduction system, and valvular fibrocytes. Cardiac involvement may be the sole manifestation of the disease, particularly in individuals with residual enzyme activity. In general, hemizygous men are more seriously affected than heterozygous women. The main cardiac manifestations include myocardial hypertrophy, which, in some patients, mimics hypertrophic cardiomyopathy. Conduction system involvement leads to PR shortening or, in later stages, to AV blocks. Arrhythmias presenting with variable severity also appear to be common. Valvular involvement is frequently noted but generally mild and clinically non-significant. Newly available enzyme replacement therapy has produced promising results in preventing further functional deterioration of affected organs and possibly also in reversing impaired function. CONCLUSIONS: With the advent of effective enzyme replacement therapy, early diagnosis of Fabry disease may be crucial for patient prognosis.

Fabry Disease↗

PubMed2002

[Direct percutaneous transluminal coronary angioplasty in patients with acute myocardial infarct treated at the Cardiac Center of the General Medical School Hospital in Prague: a 1-year retrospective study].

UNLABELLED: Direct percutaneous transluminal coronary angioplasty (d-PTCA) in patients with acute myocardial infarctions (AIM) has become an alternative of thrombolytic treatment. If the involved department has adequate experience the success rate of the procedure is high and the immediate and long-term results are better than those of thrombolysis. Moreover contrary to thrombolytic treatment successful percutaneous coronary intervention in AIM is more beneficial for patients also later than 6 hours after the development of infarction pain. In the Cardiocentre of the General Faculty Hospital (GFH) patients with AIM are constantly attended, i.e. those indicated for reperfusion therapy are treated solely by the d-PTCA method. OBJECTIVE AND METHOD: Retrospective analysis of d-PTCA in AIM made during the annual period from Jan. 1 2000 to Dec. 31 2000. Into the observation study patients were included with clinical and/or ECG signs of AIM when the period from the onset of pain to the beginning of intervention did not exceed 12 hours. All patients were given before the procedures 500 mg of acetylsalicylic acid and 10,000 u. heparin. Cardiac catheterization was implemented by the percutaneous Seldinger technique via the a. femoralis l.dx., in exceptional cases from the left femoral artery. An approach via the a. radialis and/or a. brachialis was not used in any of the patients. From the investigation patients were excluded who had before the percutaneous coronary intervention (PCI) a thrombolytic preparation (so-called rescue-PTCA). RESULTS: During the mentioned period in the Cardiocentre of the GFH a total of 673 PTCA were performed, incl. 127 (18.9%) d-PTCA in patients with AIM. In the mentioned group of 127 patients subjected to intervention were 87 (68.5%) men and 40 (31.5%) women. The mean age of the men was 59.1 +/- 12 years and the mean age of the women 68.2 +/- 12 years. As to the main risk factors of coronary atherosclerosis arterial hypertension was present in 48%, smoking in 42%, diabetes in 23% and hyperlipoproteinaemia in 31% of the treated patients. More than one third of the patients had a history of myocardial infarction (38%). The infarcted artery was the r. interventricularis anterior (LAD) in 51 (40.2%), the right coronary artery (RCA) in 54 (42.5%), the r. circumflex (LCX) in 16 (12.6%), the left main coronary artery in 2 (1.6%) and the bypass in 4 (3.1%). Multiple coronary affections were recorded in 80 (63%) patients, affections of one artery in 47 (37%). Primary procedural success (flow TIMI 3/2) was achieved in 121 patients (95.3%). Normal flow through the infarcted artery TIMI 3 was achieved in 118/127 (85.8%) patients. In 91 (71.7%) into the infarcted artery a coronary stent was implanted, during hospitalization no subacute stenosis of the stent developed. The mean period between the onset of infarction pain--injection was 4.4 +/- 2.3 hours. The mean period of the entire procedures was 48 +/- 14.5 minutes. As contrast material only non-ionic contrast substances were used (Iomeron 350) with a mean consumption of 150 ml per patient. The mean skiascopic time was 13.6 +/- 1.8 min. A total of 9 (7.1%) patients were treated with GP IIb/IIIa receptor blockers (abciximab). The total hospitalization mortality of the intervened group was 7.1% (9 patients). In a sub-group of 9 patients who at the onset of the procedure were in cardiogenic shock 3 (33%) died. The hospitalization mortality of the sub-group of patients with AIM without cardiogenic shock, treated with d-PTCA was 5.1% (6/118). During hospitalization the authors did not observe any intracranial haemorrhage. DISCUSSION: The group of subjects with AIM subjected to catheterization who are treated by d-PTCA is relatively numerous in our department. According to a number of clinical studies successful d-PTCA in AIM gives better short-term and long-term results as compared with thrombolytic therapy. The primary success rate of d-PTCA was high and the hospital mortality was low and comparable with contemporary data in the literature. CONCLUSION: Direct PTCA is effective treatment in patients with acute myocardial infarction. The authors results confirm the high procedural success rate and acceptable hospital mortality. These favourable results of an invasive approach to treatment of AIM must be compared in future with bolus thrombolytic treatment by new types of thrombolytic preparations in combination with anti-platelet treatment with blockers of platelet glycoprotein receptors IIb/IIIa with/or without subsequent percutaneous coronary intervention.

Aged↗

PubMed2002

[Cardiovascular stress in laparoscopic surgery].

AIM: To evaluate response of cardiovascular system in laparoscopic surgery. The main aim was: 1--comparison of healthy subjects and morbid obese population, 2--comparison of healthy subjects and cohort of patients with organic cardiopathy. Secondly we compared the influence of the operation position. PATIENTS AND METHODS: Patients (n = 17) were divided into 3 subgroups:--Control group of "normal" subjects: mean age 36.8 +/- 11.2 years; BMI 25.33 +/- 3.62; BSA 1.84 +/- 0.21 m2; two men and four women; op. diagnosis: 3x cholecystectomy, 1x appendectomy, 1x inguinal herniotomy, 1x hiatal hernia operation. --Group of patients with morbid obesity: mean age 38 +/- 8.1 years; BMI 45.82 +/- 7.54!; BSA 2.66 +/- 0.32 m2; one man and five women; all of them were operated for obesity (laparoscopic gastric banding).--Group of patients with severe cardiopathy: mean age 64.0 +/- 11.55; BMI 26.4 +/- 4.09; BSA 1.89 +/- 0.23; three men and two women; card. diagnosis: 2x aortic stenosis, 1x combined aortic valvulopathy, 1x aortic stenosis with secondary mitral regurgitation, 1x secondary mitral regurgitation (both caused by coronary artery disease); op. diagnosis: 4x cholecystectomy; 1x extraction of catheter for peritoneal dialysis. The method of our examination was transesophageal echocardiography with use of omni planar sond with continual monitoring of each patient. Our data are based on repeated measurements (3x minimal for each state and each patient) before and after peritoneal cavity insufflation and third after positioning of patient (in Trendelenburg or Fowler position). Examinations were recorded and data analysed off-line. Following parameters were analysed: mean age, BMI, BSA, heart rate, mean arterial pressure (MAP), ejection fraction of left ventricle (EF), E/A ratio of transmitral flow, cardiac output (CO), cardiac index (CI), systemic (peripheral) vascular resistance (SVR) and pressure-rate-product (PRP). For statistical analysis were used: ANOVA tests, t-tests with Benforroni correction and Friedman's tests. RESULTS AND DISCUSSION: In comparison of normal and obese patients statistically significant differences were found (after exclusion of BMI and BSA) in cardiac output values, after recalculation on body surface (cardiac index) remained only non-significant trend to fall. Differences between control group and group of cardiacs were also non significant with exclusion of E/A ratio of transmitral flow. This result we explain by pseudonormalization. All 17 operations were successfully done without any complication. CONCLUSIONS: Our data were obtained on relatively small cohort of patients but the number of patients was respected by statistics and results might be borderline but significant. Laparoscopic gastric band (operation is done in semi-sitting position) in morbid obese patients is well tolerated without any differences in comparison to healthy population. The population of patients with severe organic cardiopathy needs careful approach. Our data are favourable but significant change in the left ventricle filling together with non-significant hemodynamic disadvantageous trends in EF, CI and MAP requires care. Further investigations are needed and with intraoperative monitoring (transesophageal echocardiography is preferred) can be considered as safe.

Adult↗

PubMed2001

Cardiac manifestations in Fabry disease.

Fabry disease is an X-linked recessive genetic disorder of glycosphingolipid metabolism, due to deficiency of the lysosomal enzyme alpha-galactosidase A. The disease is characterized by the progressive intracellular lysosomal accumulation of neutral glycosphingolipids throughout the body, including the cardiovascular system. It has been reported that cardiac involvement could be the sole manifestation of the disease in some patients. Myocardial abnormalities are characterized mainly by left ventricular (LV) wall thickening without significant cavity dilatation, the most frequent abnormal structural pattern being concentric LV hypertrophy (LVH). In some patients the disease mimics a typical hypertrophic obstructive cardiomyopathy. According to our experience, systolic function is largely preserved in a large majority of affected individuals. In contrast, mild to moderate impairment of diastolic filling is a relatively common finding, representing probably the most important cause of dyspnoea in patients with Fabry disease. However, in a relatively large population of affected patients, severe diastolic dysfunction, typical of restrictive cardiomyopathy, was not found. Valvular structural abnormalities are frequent due to valvular infiltration. In several patients, hypertrophy of papillary muscles and/or systolic anterior motion of the mitral leaflets associated with LV outflow obstruction may aggravate the mitral valve dysfunction. We did not confirm the previously reported high prevalence of mitral valve prolapse. Valvular regurgitation seems to be relatively frequent but mostly non-significant. Electrocardiographic changes in Fabry disease are multiple and include atrioventricular (AV) conduction abnormalities (abbreviation of the P-R interval or AV blocks), signs of LVH and repolarization abnormalities. Our observations suggest that conduction defects and repolarization changes are present predominantly in subjects with LV structural abnormalities. Cardiac symptoms in patients with Fabry disease include shortness of breath on effort (related to LV diastolic dysfunction), vasospastic and/or exertional angina pectoris (due to LVH, endothelial dysfunction and/or fixed coronary artery stenosis) and syncope (related to AV blocks or LV outflow obstruction). The extent of cardiac involvement, in particular LV mass assessment, could represent an ideal surrogate endpoint for evaluating the efficacy of specific therapies.

Electrocardiography↗

PubMed2001

[Takayasu's disease associated with autoimmune thyroiditis and celiac disease. Clinical course and limitations of treatment].

Takayasu's arteritis is a non-specific form of vasculitis involving the aorta, its main branches and pulmonary arteries. It is a rare disease in our country, contrasting with the high prevalence in Southeast Asia, Africa and South America. We discuss the course of the disease in our patient who was a young women who developed Takayasu's arteritis associated with autoimmune thyroiditis and malabsorption syndrome due to celiac sprue. Long-term immunosuppressive therapy contributed to stabilizing the associated diseases, but did not stop the progression of the vascular lesions. The main adverse outcome in our patient was the development of severe renovascular hypertension. Bilateral renal artery stenosis was treated by angioplasty with stent implantation. Based on literature reports, the association of Takayasu's disease with multiple autoimmune disorders is a rare event. However, it would appear that the arteritis was the limiting disease for prognosis in our patient.

Celiac Disease↗

PubMed2001

[Complications in administration of contrast media in the catheterization laboratory: a 5-year retrospective study].

UNLABELLED: Iodine contrast substances (CS) are used since the twenties of the 20th century. From the chemical aspect benzoic acid derivatives are involved which are classified according to their osmolality (high-low- and isoosmolar) tendency of ionization (ionic and non-ionic) and according to the molecular structure monomers and dimers). Side-effects are due to their osmotic, ionic and chemical action on tissues. They include a number of systemic and organ symptoms (cardiovascular, immunological, haemocoagulation, neurological and renal), from the clinical aspect divided into mild, medium severe and severe. The relatively high incidence of undesirable reactions when ionic high-molecular CS are used led to the-development of non-ionic preparations with a lower osmolality, the more extensive use of which is limited by economic factors. Moreover so far convincing clinical data are lacking that the higher incidence of undesirable reactions after the use of CS has a clinical impact. OBJECTIVE: To summarize experience with administration of CS in the catheterization laboratory of the Cardiocentre of the General Faculty Hospital during diagnostic and intervention procedures within 5 years with regard to the occurrence of undesirable effects when comparing ionic and non-ionic CS. METHOD: Retrospective analysis of a group of patients examined in the catheterization laboratory to whom a CS was administered during the period between Jan. 1 1995 and Dec. 31 1999. RESULTS: In 1995-1999 (5 years) in the catheterization laboratory a total of 10,149 procedures where implemented where ionic (ioxitalam-Telebrix 350) and non-ionic (iopromide Ultravist 370, ioversol--Optiray 370 and iomeprol-Iomeron 350) contrast substances were administered. Ionic CS were administered in 4,668 (46%) and non-ionic CS in 5,481 (54%) instances. Undesirable effects were recorded in a total of 107 (1.1%) patients, incl. ventricular fibrillation in 76 (0.75%), cardiac arrest in 12 (0.12%) and in 19 (0.19%) there were other undesirable effects (weakness, nausea, hypotension, flush, urtica etc.). Ionic and non-ionic CS participated equally in complications: ionic CS in 53 (49.5%) cases and non-ionic CS in 54 (50.5%), whereby no difference was observed in the type of complications. No death in conjucntion with administration of CS was observed. CONCLUSION: The use of contemporary contrast substances in the catheterization laboratory for diagnostic and intervention procedures on the heart is relatively safe with a minimal risk of development of serious complications. No difference was observed between the use of ionic and non-ionic CS.

Cardiac Catheterization↗

PubMed2000

[Humoral profile of patients with severe aortic stenosis].

BACKGROUND: While the effect of neurohormones is often studied in congestive heart failure, their role in aortic stenosis needs to be elucidated. METHODS: 54 consecutive patients with symptomatic aortic stenosis without overt heart failure were studied at the age of 64.4 +/- 9.3 yrs with echocardiography, x-ray and catheterization. Levels of circulating atrial natriuretic factor endothelin-1, catecholamines, plasma renin activity, immunoreactive insulin and C-peptide were assessed, related to hemodynamic data and compared to those in 23 healthy controls, aged 59.2 +/- 12.8 yrs. RESULTS: Patients had significantly higher plasma levels of endothelin-1 (z-value 0.64 +/- 1.19, p = 0.019), atrial natriuretic factor (z-value 2.46 +/- 2.46, p < 0.001) and dopamine (z-value 0.91 +/- 2.33, p = 0.02). Levels of endothelin-1 and ANF positively correlated with mean (r = 0.631, p < 0.001) and wedged pulmonary artery pressures and with left atrial diameter index (r = 0.602, p < 0.001). Endothelin-1 levels correlated negatively with aortic valve area (r = -0.306, p = 0.041). No correlation was found between neurohumoral plasma concentrations and left ventricular mass index. CONCLUSIONS: In patients with symptomatic aortic stenosis without overt heart failure, elevated plasmatic levels of endothelin-1, atrial natriuretic factor and dopamine were documented. The increase of ET-1 levels is related to pulmonary hypertension and severity of the disease. Left ventricular hypertrophy is not related to neurohormonal levels. Neither circulating system renin-angiotensin nor noradrenaline are activated in these patients.

Adult↗

PubMed2000

New insights in cardiac structural changes in patients with Fabry's disease.

BACKGROUND: Fabry's disease is an X-linked recessive genetic deficiency of the enzyme alpha-galactosidase leading to the pathologic intracellular deposition of neutral glycosphingolipids. Although cardiac involvement is frequent, there is controversy regarding the character of the associated left ventricular (LV) changes and the severity of valvular involvement. METHODS: Clinical evaluation (disease severity scaling, laboratory tests, and echocardiography) was performed in 13 hemizygous men (mean age 39 +/- 10 years) and 17 heterozygous women (mean age 35 +/- 19 years). RESULTS: LV hypertrophy (LVH) was frequent in subjects older than 30 years, more often in men (61%) than in women (18%, P <.001). The degree of LVH was independently associated with age and the logarithm of alpha-galactosidase activity (r(2) = 0.70, P <.001). The predominant LV geometric patterns were concentric LVH and remodeling, both present in 11 subjects (36%). Three patients had an asymmetric septal hypertrophy mimicking hypertrophic cardiomyopathy. In most subjects with LVH, the systolic function was normal and severe diastolic dysfunction (restrictive pattern) was not noted. Minor structural abnormalities of the mitral valve were found in 17 subjects (57%). The aortic valve was affected in 14 patients (47%). Valvular abnormalities were frequently accompanied by regurgitation of minor to mild degree. The presence of LVH or valvular changes was associated with increased disease severity. CONCLUSIONS: Echocardiographically detectable cardiac involvement is frequent with Fabry's disease, particularly in older subjects, and more pronounced in affected hemizygous men than in heterozygous women. LVH is frequently observed but usually not associated with significant systolic or restrictive diastolic dysfunction. Concentric LVH and remodeling appear to be the major manifestations of LV structural alteration. The frequently noted valvular abnormalities were not associated with a significant degree of regurgitation. Valvular and especially LV structural changes may serve as a useful marker of disease severity.

Adolescent↗

PubMed2000

Pilot study of combined blockade of the renin-angiotensin system in essential hypertensive patients.

BACKGROUND: Additive hemodynamic effects of combined blockade of the renin-angiotensin system by an angiotensin I converting enzyme inhibitor and an angiotensin II antagonist have been observed in sodium-depleted normotensive volunteers and in patients with congestive heart failure. OBJECTIVE: To investigate whether the same additive hemodynamic effects occur in patients with hypertension and to verify the safety of such an approach. DESIGN: Multicenter, randomized, double-blind, parallel-group, pilot study. PATIENTS: 177 patients with mild-to-moderate hypertension [diastolic blood pressure (DBP): 95-115 mmHg after a 4-week placebo run-in period] were included in the study. INTERVENTION: Combination therapy consisting of 50 mg losartan daily and 10 mg enalapril daily was administered for 6 weeks. The effects of this therapeutic regimen was compared with similar groups of patients who received either 50 mg losartan daily or 10 mg enalapril daily. MAIN OUTCOME MEASURES: 24-hour ambulatory mean DBP and clinic DBP measured at trough after 6 weeks of treatment. RESULTS: 24-hour ambulatory mean DBP did not significantly differ between treatment groups although the combination tended to lower BP more. The combination therapy was more effective on clinic DBP measured at trough than was losartan by 3.2 mmHg [confidence interval (95%, CI) 0.7-5.7 mmHg, P = 0.012], and more effective than enalapril by 4.0 mmHg (95% CI, 1.5-6.4 mmHg, P = 0.002). In a subgroup of 28 patients, higher plasma active renin and angiotensin I levels during blockade by the combination therapy were observed. This finding confirmed that the combination of the two agents inhibited the renin-angiotensin system to a greater extent than did either agent alone. CONCLUSION: A combination of 10 mg enalapril daily and 50 mg losartan daily safely induces a supplementary, although modest, fall in clinic DBP in patients with mild-to-moderate essential hypertension.

Adolescent↗