Arterial stiffening contributes to impairment of cerebrovascular reactivity in patients with coronary artery disease without carotid stenosis.

Impaired cerebrovascular reactivity (CVR), an important risk factor for future stroke, is affected by a presence carotid stenosis. However, in some cases CVR can be impaired in the absence of carotid stenosis due to several poorly characterized mechanisms. We hypothesized that arterial stiffening as observed in coronary heart disease (CHD) could be associated with alteration in CVR in CHD patients without carotid stenosis. The study population consisted of patients referred for coronary angiography without significant carotid stenosis (<50 %). CVR was evaluated by breath holding index (BHI) measured with transcranial color code duplex ultrasound. Arterial stiffness was assessed by pulse wave velocity (PWV) measured by the oscillometric method. The extent of coronary atherosclerosis was quantified by Gensini score (GS). Out of 186 subjects, sixty-two patients fulfilled the inclusion and exclusion criteria. BHI decreased with increasing PWV (r = -0.47, p<0.001). Decrease in BHI was significantly inversely associated with GS (r = -0.61, p<0.001). GS was associated with PWV (p<0.001). In conclusion, impaired CVR was associated with increased arterial stiffening in CHD patients in the absence of significant carotid stenosis. Thus, we speculate that increased arterial stiffness may at least partially contribute to the pathophysiology of CVR alteration in coronary artery disease.

[Late complication of surgical repair of aortic coarctation: ruptured pseudoaneurysm of the aorta treated by thoracic endovascular aortic repair]. / Complication tardive d'un traitement chirurgical par interposition prothétique d'une coartation aortique: rupture d'un faux anévrisme anastomotique traitée par implantation endoluminale d'endoprothèse couverte.

Aortic coarctation is a frequent congenital defect requiring early surgical treatment. Late complications of these surgical procedures can be fatal as in the case of a ruptured anastomotic pseudoaneurysm. We present a case of a 49-year-old man presenting with hemorrhagic shock due to this complication who was successfully treated by endovascular techniques with implantation of two stent grafts. This case illustrates the fact that endovascular aortic repair is feasible, certainly less invasive and very efficient for this type of complication when used in an experienced center.

[The isolated form of cardiac amyloidosis in the form of beginning infiltrative cardiomyopathy without restrictive physiology]. / Izolovaná forma srdecní amyloidózy v podobe pocínající infiltrativní kardiomyopatie bez restriktivní fyziologie.

The authors describe an interesting case of isolated cardiac manifestation of AL-amyloidosis manifesting as an incipient infiltrative cardiomyopathy with heart failure symptoms due to moderate left ventricular diastolic dysfunction. Restrictive cardiomyopathy with severe diastolic dysfunction is considered as the characteristic manifestation of fully developed cardiac amyloidosis. However, the organ deposition of amyloid is progressive and left ventricular filling worsens continuously, starting with less advanced forms of diastolic dysfunction; the restrictive physiology is characteristic only for advanced phases of the disease. Therefore, the possibility of the incipient infiltrative cardiomyopathy due to the amyloidosis should be considered in patients with heart failure symptoms and echocardiographic findings of unexplained left ventricular hypertrophy with only mild or moderate diastolic dysfunction.

[Bilateral leg compartment syndrome due to severe myonecrosis caused by inappropriate use of simvastatin]. / Syndrome de compression des loges bilatéral des membres inférieurs et myonécrose sévère due à l'usage inapproprié de la simvastatine.

Bilateral leg compartment syndrome due to myonecrosis caused by inappropriate use of statins is a rare but potentially fatal complication of this lipid lowering medication. We report a case of a 39-year-old woman who presented with suspicious critical lower limb ischemia. Subsequently, bilateral leg compartment syndrome and myonecrosis developed. The primary cause of myonecrosis was due to misuse of simvastatin mistaken by the patient for a weight-reducing drug. Urgent fasciotomies were performed and the patient underwent urgent renal replacement therapy with continuous hemodialysis for acute renal failure due to myoglobinuria. After this complex treatment, the patient was discharged. She almost fully recovered with only a residual paresis of the left fibular nerve. According to literature, this is a unique case of bilateral compartment syndrome and myonecrosis with acute renal failure due to statin overdose leading to acute renal failure and bilateral fasciotomy.

Natural history of the respiratory involvement in Anderson-Fabry disease.

BACKGROUND: Anderson-Fabry disease (AFD) is an X-linked disorder caused by deficient activity of enzyme alpha-galactosidase A, resulting in the accumulation of glycosphingolipids within lysosomes. Pulmonary involvement in AFD has previously been documented, but until now has been studied only in a few series of patients without any longitudinal follow-up. The aim of this study was to compare spirometric changes in AFD patients with a matched control population and to follow the subsequent progression of the disease. MATERIALS AND METHODS: Fifty individuals (27 women, 23 men, mean age 40 +/- 14 years) with AFD from 14 families underwent a static spirometric examination under standard conditions. A set of indices was compared with that of the control population. Out of this cohort, 39 individuals not receiving enzyme replacement therapy were longitudinally evaluated (median follow-up time 24 months). RESULTS: A clinically significant reduction in spirometric parameters, corresponding to mild to severe airway obstruction, was observed in 26% of women and 61% of men. During the serial follow-up, a significant (p < 0.05) age-dependent reduction of predicted %FVC and %FEV1 values was observed in male patients, while the influence of age was not seen in female patients. The %FEF(25-75) values decreased by similar degrees in men and women and in older and younger patients, indicating that progressive bronchial disease affects the small airways first. CONCLUSIONS: We have demonstrated a clinically relevant age- and sex-dependent progressive pulmonary involvement in AFD patients. The effects of enzyme replacement therapy on pulmonary involvement remain to be demonstrated.

[Takotsubo cardiomyopathy: case report and literature review]. / Takotsubo kardiomyopatie: popis prípadu a prehled literatury.

The authors present an interesting case report of 69-year-old caucasian woman with Takotsubo cardiomyopathy. Takotsubo cardiomyopathy is a relatively recently described heart syndrome that probably develops due to the direct toxic effect of excessively released catecholamines on cardiac adrenoceptors during emotional or physical stress. The typical features include reversible left ventricular apical dyskinesis, chest pain with ST-T changes on ECG, minimal myocardial enzymatic release and the absence of coronary stenosis on coronary angiogram. Early coronary angiographic examination is highly recommended as the clinical picture of this syndrome mimics acute myocardial infarction. Betablockers are considered to be the treatment of choice.

How does delaying treatment affect the long-term prognosis for patients with acute myocardial infarction treated with primary coronary angioplasty?

BACKGROUND: The benefit of thrombolysis in patients with acute myocardial infarction (AMI) strongly depends on the time from the onset of symptoms to the initiation of treatment. For AMI patients treated with percutaneous coronary interventions (PCI) this delay of treatment seems to be important only up to a certain time level. AIM: To assess the effects of time to treatment of AMI with PCI on the short- and long-term prognosis. METHODS: We followed 339 consecutive AMI patients treated with PCI from 1995 to 1999 in our centre. Patients were divided into five groups according to the time to treatment and ischaemic time (time from symptom onset to reperfusion). RESULTS: Time to treatment <90 min was achieved in 35 (10.5%) patients; 91-210 min in 105 (31%); 211-330 min in 72 (21%); 331-690 min in 74 (22%); and >691 min in 53 (15.5%) patients. According to ischaemic time, the patients were divided into groups: <2 h, 2-4 h, 4-6 h, 6-12 h, and >12 h. The ejection fraction of the left ventricle 3-5 days after AMI was 50%, 51%, 45%, 40%, and 46%, and the 30 day mortality - 5.7%, 2.9%, 11.1%, 10.8%, and 11.3%, respectively. Compared with patients treated later, patients with time to treatment <3.5 h had a significantly higher rate of TIMI 3 flow (93.6% vs 83.9%, p=0.007), lower 30-day mortality (3.6% vs 11.1%, p=0.012), lower 3-year mortality (8.6% vs 19.1%, p=0.003), lower frequency of heart failure during hospitalisation (11.4% vs 28.1%, p<0.001) as well as lower maximal level of creatine kinase (32+/-29 vs 44+/-39 micro kat/l, p=0.005). CONCLUSIONS: The success rate of primary PCI to achieve normal flow in an infarct-related artery is high, but it decreases when treatment is started later than 3,5 h from AMI onset. The short-term and long-term mortality as well as the incidence of heart failure during the acute phase of MI are the lowest when PCI is started within 3,5 h from the onset of symptoms.

[Main clinical and surgical determinants of in-hospital mortality after surgical revascularization of left main coronary artery stenosis: 2 year retrospective study (1998-1999)]. / Les facteurs cliniques et opératoires associés à la mortalité hospitalière après chirurgie d'une sténose du tronc commun de l'artère coronaire gauche. Analyse rétrospective de janvier 1998 à décembre 1999.

UNLABELLED: Stenosis of left main coronary artery (LMCA) is a common finding on coronary angiographies (7 to 10%). Mortality is very high for conservative therapy in comparison with surgery, which provides very good long-term outcome and is accepted as the standard therapy of this condition. Over the last few years, percutaneous coronary intervention (PCI) has become a new alternative in selected patients. AIM OF THE STUDY: To assess in hospital mortality in subjects referred to our surgical unit for coronary artery bypass grafting (CABG) and to identify the main clinical and surgical determinants of in-hospital mortality. METHODS: Two year retrospective analysis (1998-1999) of in-hospital mortality and morbidity of patients having left main stenosis referred for CABG. RESULTS: Among a group of 1443 patients operated for coronary heart disease, LMCA stenosis was found in 330 patients (22.9%). The mean age was 63 +/- 8.6 years. Elective surgery was done in 173 patients (52.4%), and acute operation (i.e. urgent, emergent or desperate) in 157 patients (47.6%). The total in-hospital mortality was 4.2% (14/330). Elective surgery in-hospital mortality was 1.7% (3/173) and urgent surgery in-hospital mortality was 7% (11/157). The main risk factors of in-hospital mortality were age > 68 years, low ejection fraction (LVEF<0.4), history of myocardial infarction, gender (female) and urgency of the operation. CONCLUSION: The proportion of patients undergoing CABG for left main stenosis is relatively high in our center and their mortality is quite low especially for elective operations. As the mortality of patients treated by PCI for LMCA stenosis is similar to surgery in high-risk patients, we postulate that this approach can be a real alternative treatment for such patients. Because of its potential benefit, combined revascularisation is also an alternative that should be considered in some cases. Furthermore, as a new generation of coated stents has emerged in the treatment of restenosis after PCI, we hypothesize that this method can in the near future be accepted as equivalent to surgery. However several randomized clinical trials must first be conduced prove this point.

Tobacco use amongst high school students in the Czech Republic.

OBJECTIVE: We were interested in the prevalence of smoking amongst teen-age students, its possible causes, and their understanding of its associated health risks. METHODS: We constructed a questionnaire that was responded to by a total of 419 students from 5 high schools in Prague, Czech Republic. Students were classified as non-smokers, mild (1-10 cigarettes daily), moderate (11-20 cigarettes daily), and severe smokers (>20 cigarettes daily). The survey also contained questions about passive smoking, motivation for smoking, the understanding of its associated health risks, alcohol consumption, and drugs. RESULTS: We found that amongst 16-20 years old high school students there are 37.5% smokers (38.0% men, and 37.0% women). The majority are mild smokers (82.3%), 15.8% moderate smokers and 1.9% heavy smokers. 29.0% of non-smokers reported passive smoking; i.e. that 65.7% of students are exposed to harmful effect of tobacco smoke. The average onset of smoking is at 14 years of age. The youngest smoker started smoking at the age of 5 years. Parents of 52.0% of students smoke (69.4% of smokers and 41.6% of non-smokers). Most of students know about the risk of lung cancer and cardiovascular diseases (86-99%). CONCLUSIONS: The prevalence of active and passive smoking among high school students is high. Parents smoking is significantly more frequent in teen-age smokers than in non-smokers. We consider the "teen-age" population together with their parents to be the key target for a successful antismoking campaign.

[Cardiac manifestation of Fabry's disease: current knowledge]. / Kardiální manifestace Fabryho choroby: soucasné znalosti.

Fabry's disease is a rare lysosomal storage disease caused by the X-linked defect of the enzyme alpha-galactosidase A leading to the intracellular accumulation of glycosphingolipids in various organs and tissues. Cardiac involvement is frequent and, in individuals with some residual enzyme activity, may be the sole manifestation of the disease. Hemizygous men are generally more seriously affected than heterozygous women. The dominant cardiac manifestations include myocardial hypertrophy of the left ventricle, which, in some patients, mimics hypertrophic cardiomypathy. Left ventricular systolic function is usually preserved, on the other hand mild to moderate diastolic dysfunction is regularly detected. Valvular abnormalities are frequently noted. However, hemodynamically significant lesions are rare. Conduction system involvement leads initially to the shortening of atrioventricular conduction, in later stages, with a progression of the disease, antrioventricular blocks and various forms of supraventricular and ventricular arrhythmias appear. Myocardial ischemia in Fabry disease has in most cases a functional origin due to endothelial dysfunction of coronary arteries and also due to the increase oxygen demand of hypertrophied myocardium. The results of so far performed studies with enzyme replacement therapy are promising in preventing further deterioration and even improving function of affected organs.

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.

[Comparison of the cost effectiveness of treatment of acute myocardial infarct with primary angioplasty and thrombolysis ]. / Srovnání cenové efektivity lécby akutního infarktu myokardu primární angioplastikou a trombolýzou.

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.

[Takayasu's disease associated with autoimmune thyroiditis and celiac disease. Clinical course and limitations of treatment]. / Association de la maladie de Takayasu avec la thyroïdite auto-immune et la maladie coeliaque. Déroulement de la maladie et limites du traitment.

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.

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.