Recurrent ventricular fibrillation during a febrile illness as the first manifestation of Brugada syndrome--a case report

A case of a 33-year-old male who was admitted to the hospital due to recurrent ventricular fibrillation during a febrile illness is presented. Initially, the patient was diagnosed with acute myocardial infarction and received thrombolytic treatment. Echocardiography and coronary angiography were normal. Right precordial ECG leads recorded one and two intercostal spaces higher than normal as well as ECG obtained following ajmaline administration revealed a typical Brugada pattern.     

Implantable cardioverter-defibrillators in children

BACKGROUND: Implantable cardioverter-defibrillators (ICD) have been increasingly used in adult patients for the prevention of sudden cardiac death (SCD). The usefulness and feasibility of ICD implantation in children have been less well established. AIM: To analyse indications, results and safety of ICD therapy in children. METHODS: ICDs were implanted in seven children, aged from 6 to 17 years. All patients underwent cardiological evaluation which included analysis of medical history, physical examination, chest X-ray, standard ECG, 24-hour Holter ECG monitoring and echocardiography. RESULTS: In five children devices were implanted due to aborted sudden death (ventricular fibrillation) whereas in the remaining two - as a primary prevention of SCD. Three children had hypertrophic cardiomyopathy, one - dilated cardiomyopathy, one - mitral valve prolapse and QT prolongation, one - congenital long QT syndrome and the remaining patient - idiopathic ventricular tachycardia. Single-chamber devices were implanted in six children, and dual-chamber system - in one patient. In all patients endocardial leads were implanted and ICD pocket was formed under the greater pectoral muscle. During follow-up ranging between four months to 5.4 years, four children developed ventricular fibrillation or ventricular tachycardia which were terminated by appropriate ICD discharges. CONCLUSIONS: 1. ICD implantation in children is effective in the prevention of SCD. 2. In our population, the most frequent indications for device implantation were life-threatening ventricular arrhythmias occurring in patients with cardiomyopathy. 3. Cardiac arrest due to ventricular fibrillation may occur in children without a history of aborted SCD. 4. ICD implantation in children is feasible and safe.     

Eleven-years long follow-up in a patient after myocardial infarction, with low ejection fraction and recurrent ventricular tachycardia. The role of implantable cardioverter defibrillator and selective ablation

The selective ablation of the recurrent ventricular tachycardia (VT) in a 75-year old patient after extensive inferior myocardial infarction (24 years ago), with low ejection fraction was performed. In 1995 the cardioverter-defibrillator was implanted due to recurrent, symptomatic VT. The coronary angiography in 1995 and in 2006 revealed the occlusion of the right coronary and the circumflex arteries. One year after implantation, he had electrical storm caused by proarrhythmic effect of amiodarone with prolongation of QT/QTc interval. During follow up episodes of VT (approximately 5/year) were successfully terminated by ATP and rarely by cardioversion. Recently, the patient was admitted to the hospital because of the very frequent (25/day) episodes of slow (500-560 ms), sustained ventricular tachycardia. The pharmacological treatment was unsuccessful. CARTO mapping and entrainment pacing revealed VT circuit around mitral annulus. A few applications at the paraseptal part of the mitral isthmus terminated VT, which was no longer inducible. During following days there were no VTs requiring ICD interventions.     

From implantable cardioverter-defibrillator to cardiac resynchronization therapy with the use of epicardial left ventricular lead. The evolution of the treatment of post inflammatory heart failure--a case report

The authors present a case of a 77-year-old man with heart failure in the course of dilated cardiomyopathy (DCM) and atrial fibrillation (AF), after implantation of an automatic cardioverter-defibrillator (ICD) due to recurrent symptomatic ventricular tachycardia (VT). Addition of cardiac resynchronization therapy (CRT) was decided due to the heart-failure dependent intensification of the arrhythmia and poststimulation enlargement of QRS. CRT was led to withdraw patient's arrhythmia and to improvement of the general condition of the patient for approximately one year. After the arrhythmia reoccurred due to dislocation of the electrode in the coronary sinus with loss of left ventricle stimulation. Multiple attempts at restoration of resynchronization function via a transvenous approach failed. The patient was qualified for implantation of an epicardial left ventricle electrode. The surgery was combined with a planned exchange of ICD-CRT. Basing on a 6-month observation period an improvement heart performance and general state of health have been observed. No arrhythmic event has been noted in device memory. Performed procedures are picturing the evolution of in pacing techniques and automatic defibrillation in Poland over recent years.     

The role of BCL11B in regulating the proliferation of human naive T cells

The effect of the B-cell chronic lymphocytic leukemia/lymphoma 11B gene (BCL11B) on human T-cell regulation remains unclear. To characterize the functions of BCL11B, recombinant BCL11B and BCL11B siRNA were transfected into human naive T cells to overexpress or knock down BCL11B expression, respectively. After BCL11B overexpression, the proliferation ability and the T-helper (Th) subset were increased, whereas no significant alteration in the expression pattern and clonality of the T-cell receptor Vβ subfamilies was observed. After BCL11B knockdown, a similar distribution of Vβ subfamilies was detected in the naive T cells; however, the proliferation capacity substantially decreased. Global gene expression profiling revealed that the dysregulated genes were mainly involved in T-cell activation and proliferation. BCL11B could selectively promote Th-cell differentiation because of increased CXCL10 and CXCL11 expression. BCL11B suppression may inhibit proliferation and induce apoptosis, which may relate to changes in the expression of CFLAR-CASP8-CASP10 in the mitochondrial pathways. In conclusion, BCL11B is required for T-cell survival; its overexpression could effectively increase the T-cell activation and proliferation abilities and Th-cell differentiation as well.     

Thrombo-embolic pulmonary hypertension--do not spoil a chance for effective surgery!

Chronic thromboembolic pulmonary hypertension (CTEPH) can be defined as pulmonary hypertension with persistent pulmonary perfusion defects causes by unresolved thrombi. All symptomatic CTEPH patients with documented pulmonary vascular resistance > 300 dyn*sec*cm(-5) and proximal lesions should be considered for surgical treatment--pulmonary endarterectomy. The role of pharmacological treatment remains controversial and should be restricted to inoperable cases and persistent pulmonary hypertension after pulmonary endarterectomy. Every year about 30 procedures is performed in two specialised centers in Poland with 1 year mortality at 8-9%. Number of procedures done gives the frequency of pulmonary endarterectomy at 0.7/million of population/year. Current data from UK indicate the actual ratio of surgical treatment of CTPH at 2/million/year. The article discusses reasons for CTEPH is underdiagnosed and why rate of surgical therapy in Poland is too low.     

The use of extracorporeal membrane oxygenation (ECMO) in the treatment of acute respiratory distress syndrome due to pandemic influenza

Pandemic influenza particularly often is associated with symptoms of acute respiratory failure, and in case of refractory hypoxemia patients may have indications for the extracorporeal membrane oxygenation (ECMO). The paper presents a case of a pandemic influenza virus infection, where classical indications for veno-venous ECMO occured. Practical aspects of this kind of treatment in the intensive care unit are discussed.     

Down regulation of BCL11B expression inhibits proliferation and induces apoptosis in malignant T cells by BCL11B-935-siRNA

To screen the highly efficient and specific B-cell chronic lymphocytic leukemia/lymphoma 11B (BCL11B) small interfering RNA (siRNA) which are able to downregulate the BCL11B gene expression in human T-cell acute lymphoblastic leukemia, thereby inhibiting the leukemic T-cell proliferation and inducing apoptosis, four BCL11B-siRNAs and the scrambled non-silencing siRNA control (sc) were designed and obtained by chemosynthesis. After nucleofection, BCL11B expression in the mRNA and the protein levels were measured by qRT-PCR and immunoblotting, respectively. The biological consequences based on the highly efficient and specific BCL11B-siRNA were demonstrated by CCK-8 kit, morphological changes (Hoechst 33258 staining), high-resolution imaging, and flow cytometry. Reduction in the BCL11B mRNA level was observed at 24 or 48 hours in molt-4 T cells with BCL11B-935-siRNA, BCL11B-434-siRNA, or BCL11B-748-siRNA, respectively. BCL11B protein expression levels were reduced by 34·77% and 41·73% in the BCL11B-935-siRNA- and BCL11B-434-siRNA-treated cells, compared with the control level at 72 hours. In comparison with BCL11B-434-siRNA treatment group, the Molt-4 cells transfected with the BCL11B-935-siRNA showed significantly inhibited proliferation and effectively induced apoptosis (P<0·05). When highly efficient and specific BCL11B-935-siRNA was used to analyze the inhibition of BCL11B mRNA level in primary T-cell acute lymphoblastic leukemia (T-ALL) cells, similar result was obtained. In conclusion, siRNAs targeting the different exon domains resulted in different silencing effects and biological consequences. Suppression of BCL11B by RNA interference could inhibit the proliferation and induce the apoptosis effectively in leukemic T cells, which might be considered as a new target therapeutic strategy in T-cell malignancies.     

Detection of β-Herpesviruses in Polish Adult Cord Blood Stem Cell Recipients by Real-Time PCR: Single Centre Study

Umbilical cord blood transplantation (UCBT) is known to be associated with increased risk of infections, compared to bone marrow or peripheral blood stem cell transplantation. In viral diseases for which specific treatment is available, real-time PCR assays are reliable diagnostic tools for timely initiation of appropriate therapy and for rapid assessment of the efficacy of antiviral treatment strategies. A retrospective review of samples from a group of seven adult cord blood stem cell recipients was made. Serum samples taken up to 180 days after transplantation were examined with quantitative real-time PCR for measurement of viral load (CMV, HHV-6, and HHV-7). Cytomegalovirus (CMV) DNA was detected in samples taken from four patients (57%) in the period of 20–80 days after transplantation. Products of amplification of human herpesvirus 6 (HHV-6) DNA were found in samples taken between days 25 and 37 following UCBT from only one patient (14%). On the other hand, the majority of patients (n = 6, 86%) had HHV-7 DNA detected in the period 15–58 days after transplantation. Co-infection with HHV-7 was demonstrated at onset of all episodes of microbiologically confirmed CMV or HHV-6 infection. Our observations indicate that real-time PCR is not only useful for monitoring herpesviral infections in transplant recipients, but is also a powerful method for clarifying the relationships between the viral load and clinical symptoms. Further investigation with a much larger group of patients will be needed to confirm these observations and translate them into a clinical approach.