Mastocytosis is a clonal disease of the hematopoietic stem cell. The condition consists of a heterogeneous group of disorders characterized by a pathological accumulation of mast cells in tissues including the skin, bone marrow, liver, spleen and the lymph nodes. Mastocytosis is a rare disease which occurs both in children and adults. Childhood onset mastocytosis is usually cutaneous and transient while in adults the condition commonly progresses to a systemic form. The heterogeneity of clinical presentation of mastocytosis is typically related to the tissue mast cell burden, symptoms due to the release of mast cell mediators, the type of skin lesions, the patient's age at the onset and associated haematological disorders. Therefore, a multidisciplinary approach is recommended. The present article provides an overview of clinical symptoms, diagnostic criteria and treatment of mastocytosis to facilitate the diagnosis and management of mastocytosis patients in clinical practice.

IntroductionThe septomarginal trabecula is a constant element of the anatomy of the human heart, which connects the interventricular septum and the anterior wall of the right ventricle. Considering the diversity of opinions about the structure and numerous studies suggesting its important role in haemodynamics and conduction of electrical impulses in the heart, we decided to study this element in detail.Material and methodsThe research was conducted on 220 human hearts. Attention was mainly paid to the structure and topography of the trabecula. Its relation to the anterior papillary muscle was also a part of the study.ResultsThe presence of this morphologically diverse element was confirmed in each of the studied hearts. In most cases the trabecula originated from the upper part of the interventricular septum, separating at an angle increasing proportionally to the number of branches of the crista supraventricularis as well as the number of secondary trabeculae. The criteria established for the study, which included the course of the trabecula in the lumen of the right ventricle and its relation to the anterior papillary muscle, let us distinguish 4 types of septomarginal trabecula (I, II, III, IV). The most common was type III, the undivided trabecula, tightly connecting with the anterior papillary muscle.ConclusionsBased on the results of the following study we propose a hypothesis on the genesis of respective parts of the septomarginal trabecula and a plausible sequence of changes they undergo during human ontogenesis and phylogenesis of the primates.

The morphology of myocardial bridges (MB) in the heart of the domestic pig remain an open issue. Despite numerous analyses of the subject, many controversies still exist. Opinions also differ when the influence of the MB on haemodynamic processes in the coronal vessel system is concerned. In the examined group of 150 domestic pig's hearts, the length of the detected MB varied from 1.8 to 39.7 mm while their thickness amounted to 0.8 -4.7 mm. Both the longest and the thickest bridges were connected with the posterior interventricular branch. It was noticed that the MB muscle bands cross the long axis of the vessels located in the grooves mostly at almost a right angle. Three forms of perivascular space were educed using the criterion of the distance of the vessel from the surrounding muscularis externa.

Atrial fibrillation (AF) is the most common arrhythmia encountered in clinical practice. Despite the common association of AF with cardiovascular disease, some patients can be classified as 'lone AF'. The latter is essentially a diagnosis of exclusion, and should be preceded by careful evaluation, including thorough collection of medical history, physical examination, blood pressure measurement, laboratory tests, ECG, echocardiography and, possibly, chest x-ray and exercise testing. Lone AF patients were initially thought to have a good prognosis with respect to thromboembolism and mortality, compared with the general AF population, but more recent data suggest otherwise. This review focuses on the clinical epidemiology and management aspects of lone AF, as well as various associated novel risk factors, such as familial, genetic and socioeconomic factors, alcohol, sports activity and biochemical markers.

We report seven patients with both myelodysplastic syndrome (MDS) and inflammatory bowel disease (IBD): Crohn's disease in six cases, ulcerative colitis in one case. We describe their characteristics, and those of 10 previously published similar cases are presented here. Median age at diagnosis of IBD (61 years) was high, as compared to the usual age at diagnosis of IBD. IBD was diagnosed first in nine cases, MDS first in one patient, and both diseases were diagnosed simultaneously in seven cases. Concerning IBD, there was a strong predominance of Crohn's disease (15/17 cases), with an unusually high frequency of colonic involvement (11/15 cases). MDS, in 12/17 cases, showed no excess of marrow blasts. Cytogenetic analysis was abnormal in five of the 13 evaluable cases. These observations suggest that the association between MDS and IBD may not be fortuitous in some cases, and that, in particular, patients with IBD and anemia of nonobvious origin should be evaluated for MDS. The pathogenesis of those associations, however, remains unclear.

IntroductionFalse chordae tendineae are fibrous-muscular bundles which do not interconnect with right atrioventricular valves. The structures have occasionally been described in the right ventricle. There are reports suggesting their influence on electromechanical processes taking place in the heart, in thromboembolic events as well as in the course of cardiac invasive procedures. The objective of the study was to perform a macroscopic evaluation of false chordae tendineae in the right ventricle.Material and methodsThe research specimens consisted of 100 hearts of adult humans, aged from 18 to 59 years, fixed in a solution of 10% formaldehyde and 98% ethanol. The ratio of false chordae tendineae to individual elements of the right ventricle, such as its walls, papillary muscles, septomarginal trabecula and the apex of the ventricle, was examined.ResultsDuring examination, six types of chordae tendineae were described based on the criterion of the type of structures they connected. The most common were false chordae connecting ventricle walls within its apex, while the least common were individual segments of papillary muscles. The research proved that the examined structures are morphologically extremely diverse. Substantial clinical implications of their presence seem very probable.ConclusionsThe present work is the first of a scheduled series devoted to the problem of false chordae tendineae. Further analyses will cover the subject of morphological aspects in a microscopic perspective.

he traditional site for ventricular lead placement-the right ventricular apex (RVA), produces an abnormal pattern of ventricular depolarization, and there is growing evidence that pacing from this site is associated with detrimental functional and structural changes in the heart which might lead to an adverse clinical outcome. [1][2][3] These observations have increased interest in pacing at sites alternative to the RVA, mainly in the area of the right ventricular outflow tract (RVOT). It has been hypothesized that pacing in the RVOT, owing to its proximity to the His-bundle, results in a more physiological depolarization pattern and better hemodynamics and might reduce detrimental effects of long-term ventricular pacing. Results of a meta-analysis comparing RVA to RVOT pacing have suggested acute benefit from pacing at the RVOT. 4 However, poor definition of the outflow tract area and the non-randomized character of most trials confound the data. Longterm evaluation of the effects of chronic pacing are limited, although some mid-term observations show equivalency between apical and outflow tract pacing. 5,6 Other studies indicate that, in contrast to RVA, RVOT pacing preserves left ventricular (LV) function. 7 However, it is still not clear whether RVOT pacing provides better long-term outcome than RVA pacing.In this study we investigated RVOT pacing in comparison to RVA pacing in patients with standard indications for permanent ventricular pacing and preserved LV systolic function to determine whether RVOT pacing would provide better all-cause and cardiovascular survival. MethodsThe present study was a single-center randomized study performed in a tertiary care university hospital. The first patient was enrolled on 7 th September 1995 and the last patient on 24 th November 1997. After the 10-year follow-up visit the long-term survival in the studied population was evaluated. PatientsPatients were eligible for the study if they were at least 21 years old, had preserved LV ejection fraction (LVEF) There were no differences in the all-cause or cardiovascular mortality between the pacing sites after adjustment for age, gender, arterial hypertension, atrial fibrillation, New York Heart Association class and left ventricular end-diastolic diameter. Conclusions:The RVOT provides no additional benefit in terms of long-term survival over RVA pacing. (Circ J

Day-by-day variability should be taken into account when using spontaneous BRS measurements to detect treatment effects in individual patients. The observed substantial to good relative reliability, as assessed by the ICC, indicates that spontaneous BRS measurements are suitable to detect differences between individuals, which is a prerequisite for proper diagnosis and prognosis.