Ventricular and supraventricular arrhythmias and heart failure in a patient with left ventricular noncompaction and Brugada syndrome

We report a 47 year-old male patient with coexistence of left ventricular noncompaction and Brugada syndrome. He presented malignant ventricular arrhythmias followed by cardioverter- -defibrillator implantation, atrial fibrillation and flutter and progressive heart failure. This case could be an example of the coexistence of two rare diseases of various genetic patterns that only partially showed overlapping symptomatology and complications, particularly ventricular arrhythmias. (Cardiol J 2011; 18, 3: 310–313

Two histologically different tumours in a neonate born from an assisted reproductive technology pregnancy

The first case of a female neonate born from an in vitro fertilization with embryo transfer (IVF-ET) and intracytoplasmic sperm injection (ICSI) (IVF-ET (ICSI) with two histologically different tumours (craniopharyngioma and hepatoblastoma) is described. Anti-neoplasmatic therapy was abandoned due to the significant extent of the disease (craniopharyngioma, 15×12 cm in diameter with active internal hydrocephalus; and right liver lobe hepatoblastoma, 5 cm in diameter) and the severely impaired general condition of the neonate. The neonate died on the 30th day of life due to cerebellar and brainstem herniation, followed by circulatory and respiratory failure

Skuteczna ablacja ustawicznego, nasierdziowego częstoskurczu komorowego pod kontrolą ograniczonego mappingu stymulacyjnego w czasie operacji kardiochirugicznej. Strategia z wyboru?

A patient presented with incessant ventricular tachycardia (VT) of suspected epicardial origin. The earliest activation of the LV apex, however with negative entrainment and pace-mapping was detected. Diagnosis of cardiac tamponade lead to the repair of the right ventricle apex laceration with an epicardial radiofrequency ablation following limited pace-mapping. Ablation lesions covering left ventricle apical segment rendered the patient VT free.U chorego z ustawicznym częstoskurczem komorowym (VT) podejrzewano nasierdziowe pochodzenie zaburzeń rytmu.Stwierdzono obszar wczesnej aktywacji w okolicy koniuszka lewej komory, jednak z ujemnym rezultatem stymulacjisprzęgania (entrainment) i mappingu stymulacyjnego. W związku z rozpoznaniem tamponady serca naprawiono rozdarcie wierzchołka prawej komory, stosując ablację prądem o częstotliwości radiowej pod kontrolą ograniczonego mappingu stymulacyjnego. Blizny po ablacji pokrywające segment koniuszkowy LV spowodowały ustąpienie VT u chorego

Śluzak prawego przedsionka u pacjenta z twardziną układową

The myxoma makes 80–90% of benign cardiac neoplasms. The first symptom in 50% of patients is an embolism resulting from relocation of tumor fragments or blood clots into the bloodstream. Elevated values of acute phase proteins in these patients are results of non-specific immune response to an antigen causing the disease. Interleukin 6 (IL-6) plays a role in this reaction, modifying inflammatory response by: influence on lymphocyte T differentiation, lymphocyte B to plasmocyte transformation, and stimulation of the liver to produce acute phase proteins. Elevated IL-6 is found in 80% of patients with diagnosed myxoma, which causes it to be an important marker in diagnostic and post-operational monitoring. The role in non-specific inflammatory response played by IL-6 in myxoma and autoimmune disorders was a cause of many diagnostic mistakes. Available literature does not suggest a coincidence of cardiac myxoma and systemic sclerosis. This is why we would like to present a case report, with special regard to correlation between IL-6 values and activity/stage of diagnosed disorders.Śluzak stanowi 80-90% łagodnych nowotworów serca. Pierwszym symptomem choroby u 50% pacjentów ze śluzakiem jest incydent zatorowy fragmentami guza lub skrzeplinami. Występujący wzrost wartości białek ostrej fazy u tych pacjentów jest związany z niespecyficzną reakcja immunologiczną. Często stwierdza się podwyższone stężenia białek ostrej fazy i immunoglobulin. Odpowiedzialną za to w głównej mierze jest interleukina 6, cytokina modyfikująca odpowiedź zapalną poprzez wpływ na różnicowanie się limfocytów T, transformację limfocytów B w plazmocyty oraz stymulacje białek ostrej fazy. Wzrost IL-6 obserwuje się u 80% pacjentów z rozpoznanym śluzakiem. Stanowi on w ten sposób ważny marker w dalszej obserwacji pooperacyjnej tej grupy pacjentów. Wspólne ogniwo jakim jest IL-6 w nieswoistej odpowiedzi immunologicznej o charakterze ogólnoustrojowym śluzaka i chorób o podłożu immunologicznym stał się źródłem pomyłek diagnostycznych. Nie opisywano do tej pory współistnienia twardziny układowej i śluzaka serca. Przedstawiamy przypadek chorego z twardziną układową i śluzakiem serca oraz korelację stężenia IL-6 z aktywnością rozpoznanych chorób

A comparison of the outcome in diabetic and non-diabetic patients with prolonged ST-segment elevation myocardial infarction subjected to primary coronary angioplasty on the basis of the Western Pomerania Database for the year 2003

Background: Of all patients with acute coronary syndrome with prolonged ST-segment elevation (STEMI) 13-25% suffer from diabetes mellitus. Despite the introduction of fibrinolytic therapy, patients with STEMI and diabetes have worse prognosis than those without diabetes. The introduction of primary angioplasty as the preferred method of treatment in this group of patients has improved the prognosis somewhat. However, the problem has not been totally solved and diabetic patients still show worse prognosis when compared to non-diabetics, especially in long-term follow-up. The aim of our study was to compare short-term and long-term outcomes in diabetic and non-diabetic patients treated with primary angioplasty for STEMI in the Western Pomeranian Region of Poland between January and December 2003. Methods: The medical files of patients treated for acute coronary syndromes in centres in the Western Pomeranian Region of Poland were analysed. The inclusion criteria were hospitalisation between January 1st and December 31st of 2003 as a result of a first or subsequent STEMI recognised according to European Cardiological Society guidelines. The study enrolled 329 patients including 60 diabetics (18.2%). The data obtained were categorised according to the unified scheme including risk factors, coexisting diseases and a previous history of myocardial infarction. The following parameters were analysed: complications of the acute phase of myocardial infarction, 30-day mortality and one-year mortality. Results: Significantly higher 30-day mortality was noted in diabetic patients who underwent percutaneous revascularisation. Similar results were seen in one-year mortality, with values almost four times as high as those for the non-diabetic group (p = 0.00023). Conclusions: An analysis of the medical files of STEMI patients revealed a higher 30-day mortality and a higher one-year mortality in those patients with coexisting diabetes. These results cannot be attributed to delayed reperfusion therapy, TIMI flow in the related artery or adjuvant pharmacological therapy. The data obtained confirm the observation that diabetes is an important factor in a poor prognosis in patients with acute coronary syndrome with prolonged ST-segment elevation

Safety and feasibility of Lin- cells administration to ALS patients : a novel view on humoral factors and miRNA profiles

Therapeutic options for amyotrophic lateral sclerosis (ALS) are still limited. Great hopes, however, are placed in growth factors that show neuroprotective abilities (e.g., nerve growth factor (NGF), brain-derived neurotrophic factor (BDNF), and vascular endothelial growth factor (VEGF)) and in the immune modulating features, in particular, the anti-inflammatory effects. In our study we aimed to investigate whether a bone marrow-derived lineage-negative (Lin-) cells population, after autologous application into cerebrospinal fluid (CSF), is able to produce noticeable concentrations of trophic factors and inflammatory-related proteins and thus influence the clinical course of ALS. To our knowledge, the evaluation of Lin- cells transplantation for ALS treatment has not been previously reported. Early hematopoietic Lin- cells were isolated from twelve ALS patients’ bone marrow, and later, the suspension of cells was administered into the subarachnoid space by lumbar puncture. Concentrations of selected proteins in the CSF and plasma were quantified by multiplex fluorescent bead-based immunoassays at different timepoints post-transplantation. We also chose microRNAs (miRNAs) related to muscle biology (miRNA-1, miRNA-133a, and miRNA-206) and angiogenesis and inflammation (miRNA-155 and miRNA-378) and tested, for the first time, their expression profiles in the CSF and plasma of ALS patients after Lin- cells transplantation. The injection of bone marrow cells resulted in decreased concentration of selected inflammatory proteins (C3) after Lin- cells injection, particularly in patients who had a better clinical outcome. Moreover, several analyzed miRNAs have changed expression levels in the CSF and plasma of ALS patients subsequent to Lin- cells administration. Interestingly, the expression of miR-206 increased in ALS patients, while miR-378 decreased both in the CSF and plasma one month after the cells’ injection. We propose that autologous lineage-negative early hematopoietic cells injected intrathecally may be a safe and feasible source of material for transplantations to the central nervous system (CNS) environment aimed at anti-inflammatory support provision for ALS adjuvant treatment strategies. Further research is needed to evaluate whether the observed effects could significantly influence the ALS progression

Key genetic variants in the renin-angiotensin system and left ventricular mass in a cohort of Polish patients with heart failure

Background: Heart failure (HF) is a complex disease that is under the control of different physiological systems. Left ventricular mass (LVM) is a strong predictor of HF. The renin-angiotensin system (RAS) may contribute to the pathogenesis of HF and LVM.Aims: The aim of this study is to examine the association between RAS genetic variants and HF and LVM in the cohort of Polish patients with HF.Methods: The study included 401 patients with HF. Two-dimensional M-mode echocardiography was used to assess LVM. Genomic DNA was extracted from blood, and genotyping of the angiotensin-converting enzyme (ACE) (rs4646994), angiotensinogen (AGT) (rs5051), and angiotensin II receptor type 1 (AGTR1) (rs5186) polymorphisms was carried out using polymerase chain reaction (PCR).Results: A significant association was found between HF and the genotypes of G(–6)A AGT, and the homozygotes AA of AGT were significantly less common in the HF vs control group.The results of this study did not confirm the relationship between AGT, ACE and AT1R genetic variants with LVM in Polish patients with HF.Conclusions: Our results suggested that AGT polymorphism may play a protective role in the development of HF

Przygotowanie farmakologiczne pacjentki z ASD II i nadciśnieniem płucnym przed operacją kardiochirurgiczną

Zaprezentowano opis przypadku 36-letniej kobiety skierowanej do kliniki kardiologii z narastającą od 2 lat dusznością wysiłkową. W wyniku diagnostyki u pacjentki stwierdzono obecność ubytku w przegrodzie międzyprzedsionkowej typu II z przeciekiem lewo-prawym oraz nadciśnienie płucne. W badaniu echokardiograficznym uwidoczniono ubytek o średnicy 4,3 cm, ciśnienie w prawej komorze (RSVP) 80 mm Hg i niedomykalność zastawki trójdzielnej. W cewnikowaniu prawego serca (RHC) stwierdzono: średnie ciśnienie w tętnicy płucnej (mPAP) 59 mm Hg, naczyniowy opór płucny 10,22 jednostki Wooda (jW.) oraz ujemny wynik testu wazoreaktywności. Po konsultacji kardiochirurgicznej pacjentkę zakwalifikowano do wstępnego leczenia farmakologicznego i ponownego badania. Do leczenia włączono sildenafil, a następnie macytentan. Obserwowano poprawę tolerancji wysiłku (w teście 6-minutowego marszu z 440 do 526 m; klinicznie z klasy wg New York Heart Association [NYHA] III do NYHA I/II) i zmniejszenie stężenia N-końcowego fragmentu propeptydu natriuretycznego typu B (z 250 do 170 pg/ml). W echokardiografii stwierdzono obniżenie się RVSP do 60 mm Hg. W RHC wykonanym po roku leczenia ujawniono obniżenie się mPAP do 40 mm Hg, zmniejszenie PVR do 3,25 jW. oraz wzrost rzutu serca z 5,57 do 10,44 l/min. Saturacja mieszanej krwi żylnej wzrosła z 64,5% do 72,5%. Po ponownej konsultacji kardiochirurgicznej pacjentkę zakwalifikowano do operacji. Zamknięcie ASD II wykonano łatą z osierdzia i uzupełniono plastyką zastawki trójdzielnej. Okresy około- i pooperacyjny przebiegały bez powikłań, a w kontroli klinicznej utrzymuje się dobry efekt kliniczny i echokardiograficzny zastosowanego leczenia

Estimating brain volume loss after radiation therapy in children treated for posterior fossa tumors (Corpus callosum and whole brain volume changes following radiotherapy in children).

Background More than half of pediatric tumors of central nervous system (CNS) primarily originate in the posterior fossa and are conventionally treated with radiation therapy (RT).Objectives The objective of this study was to establish whether corpus callosum volumes (CCV) and whole brain volumes (WBV) are correlated and to determine the impact of whole-brain lowvs high-dose RT on brain parenchymal volume loss as assessed using each technique.Material and methods Of the 30 identified children (6-12 years) with newly diagnosed posterior fossa tumors treated with cranial RT, including focal and whole-brain RT, suitable imaging was obtained for 23. Radiotherapy regimens were the following: no whole-brain RT (Group 1, n = 7), low-dose whole-brain RT (30 Gy, Group 3, n = 7) in addition to focal boost. Magnetic resonance images (MRIs) were analyzed at baseline and follow-up (median 14 months). The CCVs were manually segmented on midline sagittal slice (n = 23), while WBVs were segmented semi-automatically using Freesurfer (n = 15). This was done twice (6-month interval) for all baseline CCV measurements and 5 randomly selected WBV measurements to establish measurement reproducibility. Correlations between CCV and WBV were investigated and percentage of children demonstrating reduction in CCV or WBV noted.Results Correlation between baseline CCV and WBV was not significant (p = 0.37). Measurement reproducibility was from 6% to -9% for CCV and from 4.8% to -1.2% for WBV. Among the children studied, 30.4% (7/23) had >9% reduction in CCV at follow-up, while 33.3% (5/15) had >1.2% reduction in WBV. Five of 7 patients with CCV loss were not picked up by WBV measurements. Similarly, 3 of 5 patients with WBV loss were not picked up by CCV measurements.Conclusions The CCV and the WBV are unrelated and may indicate different brain parenchymal losses following RT. Up to a third of posterior fossa tumors treated with RT have measurable CCV or WBV loss; incidence was equivalent in lowvs high-dose whole-brain RT