[The importance of cardiac bio-marker assay for the stratification and monitoring of AL amyloidosis patients -  single center experience]. / Význam stanovení kardiálních bio-markeru ve stratifikaci a sledování nemocných s AL amyloidózou -  zkusenosti jednoho centra.

INTRODUCTION: Cardiac involvement is a dominant prognostic factor in AL amyloidosis patients. A detailed assessment of the presence and degree of cardiac involvement utilizes an array of noninvasive investigation methods, particularly echocardiography and MRI; laboratory parameters include troponins and natriuretic peptides. Cardiac involvement detection aside, cardiac bio-markers are used as a relatively strong stratification and prognostic factor. OBJECTIVE: The presentation of cardiac bio-markers assay applications in AL amyloidosis patients at an individual treatment center. PATIENTS AND METHODS: The monitored patient set consisted of 22 patients with histologically confirmed AL amyloidosis, of whom 18 met the criteria for cardiac involvement. Levels of cardiac bio-markers troponin T (TnT) and Nterminal probrain natriuretic peptide (NT ProBNP) were determined in all patients. Risk stratification of the patients utilized the Mayo staging system which is based on both bio-markers assays; Log Rank Test was applied to survival evaluation. RESULTS: Median survival of patients with cardiac involvement stigmata was 10 months vs 60 months survival of patients without signs of cardiac involvement (p = 0.133). Of the 4 patients without cardiac involvement, 1 has shown positive levels of TnT and 2 positive levels of NT ProBNP. All cardiac involvement patients exhibited abnormal levels of NT ProBNP (median 4,752 ng/ l; 415.7- 35,000) as well as positive levels of TnT (median 0.0815 µg/ l; 0.02- 0.986). The application of the Mayo stratification system to the set had determined 2 patients at stage I, 5 patients at stage II and 15 patients at stage III. The median survival of the Mayo I + II group vs the Mayo III group was 60 vs 6 months (p = 0.015), revealing extremely limited survival of stage III patients. Assessment of TnT and NT ProBNP levels relative to treatment response shows that the degree of decrease in both markers depends on maximum treatment response -  respectively the attainment of a complete hematological remission. CONCLUSION: The results, although obtained from a limited set of patients, confirm a definitive benefit of the application of cardiac bio-markers assay in the diagnostic and therapeutic algorithm of AL amyloidosis patients. The Mayo stratification system utilizing the cardiac indicator values represents a robust tool for risk stratification of AL amyloidosis patients.

[Attainment of complete hematological remission is crucial for extended survival of AL amyloidosis patients with cardiac involvement]. / Dosazení kompletní hematologické remise je zásadní pro prodlouzení prezití nemocných s AL amyloidózou a srdecním postizením.

BACKGROUND: Symptomatic cardiac involvement is the most important prognostic factor in AL amyloidosis patients. Longterm survival is limited not only by cardiac involvement condition, but also by limited choice of treatment with unsatisfactory results. The aim of the present report is to assess the effect of achieved treatment response on survival of AL amyloidosis patients with symptomatic cardiac involvement under conventional treatment. MATERIAL AND METHODS: The monitored patient set consisted of 19 patients with systemic AL amyloidosis and symptomatic cardiac involvement, treated and monitored at the III. Clinic of Internal Medicine between 2004 and 2012. The male : female ratio was 17 : 2, and the age median was 64 (range 48 to 78 years). Thirteen patients died within the monitored period. Functional status was defined according to the NYHA classification, where five patients had class II involvement, 10 patients had class III involvement, and four patients had class IV involvement. Treatment response was assessed by the application of modified IMWG and ISA criteria; all patients were undergoing conventional treatment. Nine patients were treated by a combination of alkylating agents (alkeran, cyclophosphamide), six were treated by a combination treatment with thalidomide, and four were treated by a combination of bortezomib and dexamethasone. Data were analyzed with software SPSS v. 15 (SPSS, Inc., Chicago, USA). Log Rank Test was applied to survival evaluation. RESULTS: The statistical analysis included only 13 patients who underwent at least three months of treatment, where six patients attained complete remission (CR), four patients attained partial remission (PR), and three patients attained only stabilization of disease (SD). Significant difference in patient survival was found to be correlated with attained hematological response, where the patients who attained CR had median survival of 39 months vs 10 months in patients who attained PR or SD (p = 0.005). CONCLUSION: The results indicate that attainment of complete hematological remission is associated with significantly longer survival of AL amyloidosis patients with symptomatic cardiac involvement.

[Risk stratification by cardiac gated SPECT combined with coronary artery calcium score in patients with diabetes mellitus and/or renal failure]. / Stratifikace rizika pomocí zátezového SPECT zobrazení myokardu v kombinaci se stanovenim koronárního kalciového skóre u rizikových pacientu s diabetem a/nebo ledvinným selháním.

AIM: The aim of this study was to evaluate added value of coronary artery calcium score (CAC) measurement as an adjunct to cardiac gated SPECT for risk stratification in population of patients with diabetes mellitus (DM) and/or chronic renal failure on hemodialysis (CHRF-HD). METHODS: Retrospective analysis of 67 patients, who were referred for stress gated myocardial perfusion SPECT and CAC. Characteristics of study population: 40 men, mean age 59 +/- 12 years, DM (n = 28), CHRF-HD (n = 22), DM and CHRF-HD simultaneously (n = 17). Perfusion summed stress and different scores (SSS, SDS), the left ventricle ejection fraction (LVEF) and enddiastolic/endsystolic volumes (EDV/ESV) were automatically calculated using 4D-MSPECT software. The hard cardiac event was defined as sudden cardiac death or myocardial infarction (MI); angina or other symptoms requiring coronary revascularization were also calculated. RESULTS: During the average period of 18 +/- 10 months, we registered 8 cardiac deaths, 4 nonfatal MI and 7 patients underwent revascularization. In the subgroup of 19 patients with cardiac events, the observed parameters were significantly worse concerning perfusion (SSS 9 +/- 11 vs 2 +/- 3 and SDS 6 +/- 9 vs 1 +/- 2, P < 0.05), the left ventricle function (stress LVEF 53% +/- 13% vs 59% +/- 13%, rest LVEF 55% +/- 14% vs 59% +/- 12%, stress EDV/ESV 144 ml/71 ml vs 128 ml/59 ml, P < 0.05), and CAC score (1 965 +/- 1 772 vs 387 +/- 740, P < 0.05) in comparison with patients without cardiac event. In patients without a reversible perfusion abnormality (SDS < 2), we observed lower annual hard event rate (8% vs 19.6%, P < 0.05) and revascularization procedures (4% vs 19.6%, P < 0.05) in comparison with patients with SDS > or = 2. In patients with or without reversible defects, we registered significantly higher annual hard event rate in the setting of post-stress worsening of the LVEF > 5% and/or severe CAC score > or = 709 (23.8% vs 1.9% in patients with SDS < 2, and 26.7% vs 9.5% in patients with SDS > or = 2, P < 0.05). CONCLUSION: The findings of highly elevated CAC score as well as the post-stress left ventricle stunning enable further risk stratification in patients with or without reversible perfusion abnormalities.

[Prognostic significance of stress tomographic scintigraphy of myocardial perfusion in diabetic patients]. / Prognostický význam zátezové tomografické scintigrafie myokardiální perfuze u diabetických pacientu.

OBJECTIVE: To assess the prognostic value of SPECT in patients with diabetes (DM) and without DM. METHOD: A total of 366 patients (104 women, 262 men) were examined by T1 +/- 201 or Tc-99m-MIBI SPECT of the myocardium. DM was recorded in 149 patients, 217 patients did not suffer from DM. The SPECT findings were classified as normal and abnormal perfusion (fixed or reversible perfusion defect). A serious cardiac event was defined as sudden cardiac death or non-fatal myocardial infarction. Also angina pectoris requiring revascularization was recorded. RESULTS: During the average investigation period of 24 +/- 7 months we recorded in 147 patients with a normal load perfusion only one non-fatal myocardial infarction and the risk of a serious cardiac event was low: 0.3% per annum in the whole group. A significantly higher incidence of a severe cardiac event, 5.9% per year, was recorded in 219 patients with an abnormal finding on SPECT (10 deaths and 16 non-fatal myocardial infarctions, P < 0.01). On comparison of the frequency of serious cardiac events in groups of patients with and without DM no significant difference was found as regards normal load perfusion of the heart muscle (1.1% vs. 0%, P = NS) even in patients with an abnormal SPECT finding (5.8% vs. 6.1, P = NS). CONCLUSION: SPECT myocardial perfusion makes it possible to stratify the risk in patients with DM similarly as proved previously in non-selected groups. Similarly as in patients without DM normal perfusion in diabetic patients predicts a benign prognosis.

Determination of arsenate and organic arsenic via potentiometric titration of its heteropoly anions.

Determination of arsenate based on its conversion to molybdoarsenate heteropoly anions followed by potentiometric titration is described. The titration is realized on the ion-pairing principle using cetylpyridinium chloride (or an analogous titrant containing a lipophilic cation), and is monitored by a carbon paste electrode, although other liquid-polymeric membrane-based electrodes can also be used. Calibration plots of the titrant end-point consumption versus concentration of arsenic were constructed and used to evaluate the content of arsenic in aqueous samples. The method could be applied in the analyses of samples with quite low arsenic content (amounts approximately 10 mug As in 50 cm(3) could be titrated). Organic arsenic was determined analogously after the Schöniger combustion of the sample and conversion of its arsenic to arsenate.

Dextrocardia and Marfan's syndrome.

It is described the occurrence of dextrocardia together with the congenital cyanotic heart disease in 20 year old man included in the fruste forme of the Marfan's syndrome. The diagnosis was made by the physical examination with the evidence of the arachnodactyly by the metacarpal indices and confirmed by autopsy with the following results: dextrocardia, large atrial septal defect, common ventricle, atresia of the pulmonary artery with the collateral lung perfusion from the descending aorta. There were found neither ocular manifestations, nor unambiguous manifestations of the aortic lesions. The ultrastructural examinations showed only greater accumulation of the PAS positive substances. Dextrocardia as the cardiovascular manifestation of the Marfan's syndrome has not yet been reported in the available literature.

Short-term heart rate variability changes after exercise training in subjects following myocardial infarction.

Heart rate variability reflects the activity of autonomic nervous system. The aim was to evaluate the value of short-term spectral analysis of heart rate for monitoring the effects of exercise training on the autonomic nervous system in subjects following myocardial infarction. Short-term spectral analysis of heart rate variability was performed during standardised supine-standing-supine test in a group of 29 clinically stable patients after myocardial infarction and the same test was repeated after 2 months of exercise training (ergometry or rapid walking). Each subject exercised at 60-80% of his maximal performance for 30 minutes once a day. Short-term spectral analysis of heart rate variability was assessed in the frequency range of 0.02-0.5 Hz involving very low frequency (VLF, 0.02-0.05 Hz), low frequency (LF, 0.05-0.15 Hz) and high frequency (HF, 0.15-0.5 Hz) spectral bands. The influence of exercise training on the autonomic nervous system was registered in the physically active group (n = 16) as a decrease in sympathetic response to standardised orthostatic load. In conclusions the regular exercise influenced an altered neural autonomic regulation of heart rate in post-infarction patients shown by a decrease in sympathetic activity in the short-term heart rate variability. This method enables monitoring of the effects of physical training on the autonomic nervous system in patients following myocardial infarction.

Dynamics of some biochemical indices in myocardial infarction treated with thrombolysis and creatine phosphate.

There was studied the effect of intravenously administered creatin phosphate (Neoton-drug with cardioprotective effect, Alfa Schiapparelli Wassermann) in the group of 98 patients affected by their first attack of myocardial infarction. In the course of the treatment there were observed the serum level of many biochemical indices, including the cardioselective enzyme activity. We concluded that the patients treated with Neoton, compared with the control group, revealed the significantly higher elevation of the CK, AST and LDH activity levels and the shift of the CK peak activity towards to the earlier hours after the onset of anginal pain.