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Background Atrial fibrillation (AF) is the most common sustained arrhythmia, the most common cause of supraventricular tachycardia in the global population and the most common arrhythmia requiring treatment in an emergency department. Objectives To systematically review recent literature and quantify the correlation between the choice of pharmacological cardioversion (PCV) drug and the national or international guidelines. Material and methods A systematic review was performed in accordance with the PRISMA statement methodology. The PubMed search engine was used to search for articles regardless of type or language and published in the last 6 years (May 2014-May 2020). In addition, we searched for AF guidelines and recommendations published online by cardiology and emergency medicine societies. Results The search strategy returned a total of 2615 abstracts. A total of 2598 full texts were screened; 2540 full texts were excluded with reasons and 58 articles from 32 countries were included in the analysis. In 17 of the 58 articles (29%), we noted discrepancies with the AF guidelines, specifically regarding the PCV drug used, the patients' comorbidities and the contraindications associated with the PCV drug. The most common clinical situation for the use of a contraindicated drug was when ibutilide was administered to patients with heart failure. The analysis did not reveal any statistically significant correlations, although the correlation between the sample size and guideline adherence was close to statistical significance (p Conclusion Our systematic analysis revealed substantial non-adherence to AF treatment guidelines.

Background The aim of this study was to define the alterations in the activity of alcohol dehydrogenase (ADH) isoenzymes and aldehyde dehydrogenase (ALDH) in normal and cancerous lung cells. Materials and methods Lung tissues were taken from 36 patients during surgical resection of cancer. The activities of tested enzymes were measured by spectrofluorometric method (ADH I, ADH II, total ALDH) and photometric method (ADH III, ADH IV, total ADH). Results The activities of class II and III ADH were significantly lower in lung cancer cells compared to histologically normal lung tissue. Conclusion Reduced activity of isoenzyme class II ADH may affect disorders in retinoic acid biosynthesis, leading to its deficit. Lower ADH III activity may result in depletion of glutathione, and in initiation of oxidative stress, leading to cancer progression. These data suggest that alterations in ADH isoenzyme activities can contribute to carcinogenesis in human lungs.

Background: Radiofrequency catheter ablation (RFCA) of atrial fibrillation (AF) can be curative. There are conflicting data on whether AF associated atrial and ventricular structural remodeling reverses after ablation. The aim of this study was to evaluate the hemodynamic effect of AF ablation in patients with preserved left ventricular ejection fraction (LVEF). Methods: Forty three AF patients were studied (aged 56 ± 11 years; 29 male, 23% persistent AF, LVEF ≥ 50%) in whom RFCA was performed. Echocardiographic evaluation of atrial and ventricular diameters, volumes and strain imaging by two-dimensional speckle tracking were performed before and at least 6 months after RFCA. Nine patients had AF during baseline examination. Results: A significant decrease in the left (LA) and right (RA) atrial volume and an increase in the LA strain were observed 15 ± 7 months after RFCA. In the subgroup with baseline sinus rhythm, the increment in LA strain was only borderline significant. An increase in RA, right ventricular (RV) and Biatrial strain was noticed (p < 0.05). LVEF and global longitudinal strain of the left ventricle (LV), however, did not improve substantially. Conclusions: Radiofrequency catheter ablation of AF in patients with preserved LV systolic function results in significant improvement in RA and RV function with a substantial reduction in LA and RA size. No deleterious impact of AF ablation on LA function was revealed.

Background: Beta-blockers are one of the most important classes of cardiovascular agents and have been considered a cornerstone therapy in heart diseases, such as heart failure (HF) and atrial fibrillation (AF). Among different beta-blockers, metoprolol is a selective beta(1)-adrenergic antagonist, which has been extensively used since the 1970s. Areas of Uncertainty: Although current guidelines include recommendations for the use of controlled-release metoprolol succinate in specific HF and AF indications, and despite extensive clinical experience with metoprolol, comparative evidence on the use of metoprolol succinate compared with other beta-blockers in these indications is limited. Data Sources: We systematically reviewed the data from head-to-head studies directly comparing this compound with other beta-blockers in the treatment of HF or AF. Only clinical trials and observational studies were considered; no other limits were applied. The quality and relevance of retrieved articles were reviewed. Results: A total of 18 articles of the 353 articles identified were selected for inclusion; 12 HF articles and 6 for AF. Additional references were identified from the bibliographies of retrieved articles. The studies show that oral prophylaxis with an appropriate dose of metoprolol may reduce new incidents of AF in high-risk patients. Furthermore, metoprolol succinate is associated with significant mortality and morbidity benefits in the treatment of HF. Conclusions: Despite the introduction of newer beta-blockers with differing clinical characteristics since its introduction, metoprolol succinate remains a useful drug in both HF and AF.

Background. The right ventricular outflow tract (RVOT) is located above the supraventricular crest and reaches the level of the pulmonary valve. Detailed knowledge of the RVOT spatial structure and its morphology is extremely important for cardiac invasive therapeutic procedures. Objectives. To examine the spatial structure of the RVOT using virtual models of the right ventricle (RV) interior obtained post mortem. Material and Methods. The study was carried out using 40 adult hearts from both sexes fixed in formalin. Donors had a negative history of cardiovascular diseases. Silicone models of the interior of the RV were made and then subjected to a digital modelling procedure using the photogrammetry technique. For each 3D model of the RV, the RVOT was extracted and measurements were performed. Results. Statistical analysis demonstrated that the dimensions of the transverse (p < 0.001) and sagittal (p = 0.002) axis at the level of the upper and lower border of the RVOT differed significantly. There was also a significant difference between the right and left height of the RVOT (p = 0.009). A clear correlation was found between the volume of the RVOT and the volume of the entire RV (r = 0.718, p < 0.001). Conclusion. The obtained 3D models of the RVOT can help standardize the data related to RVOT architecture. Furthermore, they can extend knowledge about the RVOT in the field of cardiology and improve the procedures in cardiac surgery.

Congenital disorders of glycosylation (CDG) are a growing, heterogeneous group of genetic disorders caused by a defect in the glycoprotein synthesis. The first and still widely used method for routine CDG screening was isoelectric focusing (IEF) of serum transferrin. Dried blood spot (DBS) testing is commonly used in newborn screening procedures to detect inborn errors of metabolism. The aim of this study was to demonstrate the reliability of the IEF method in DBS testing. Dried blood spot testing can help in the postmortem diagnosis of CDG disorders when other material is unavailable. The patterns and concentrations of transferrin isoforms in serum and DBS are comparable, and slight differences do not affect interpretation of results.

Aim Children with ultra‐short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, using citrulline serum concentration and cholestasis. Methods We studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015. Results The median standard deviation score (SDS) was −1.2 for body mass according to chronological age, −1.72 according to height and −0.59 according to height for age. Patients requiring seven days per week parenteral nutrition had a citrulline concentration below 10 μmol/L. Decreased bone‐mineral density was observed in 87% of the patients. Low values of 25‐hydroxyvitamin D were found in 53% of the children. None of the patients had elevated conjugated bilirubin levels above 34.2 μmol/L. Conclusion Children with USBS were growth deficient according to their chronological age, with frequent abnormal bone mineralisation and vitamin D deficiency. Children requiring parenteral nutrition seven days a week had citrulline concentrations below 10 μmol/L. Cholestasis was not seen.

Primary heart tumours in the paediatric population are very rare and they range from 0.01% to 0.04%. Most are benign lesions of which about half are rhabdomyomas. Rhabdomyoma tumour diagnosis is associated with a 75-80% risk of tuberous sclerosis complex (TSC). TSC are characterised with numerous changes of hamartoma-type located in the brain, kidneys, skin and other organs including the heart. More than two-thirds of newborns with TSC present rhabdomyomas in the heart. These changes may be asymptomatic, but in some cases they may cause heart failure, arrhythmias and death. We present a case report of an infant with giant rhabdomyoma tumours in the course of TSC.

Background: Autonomic imbalance is associated with poor prognosis of patients with systolic dysfunction. Most of the previous data were written several years ago and constituted to cardiovascular or arrhythmic mortality. Current treatment of these patients has improved substantially over the last decades, and thus, the population at risk of death may have altered as well. Consequently, data on high-risk patients with systolic dysfunction in the modern era are sparse and those from previous trials may no longer be applicable. The aim herein, was to verify whether well-known autonomic indices — baroreflex sensitivity (BRS) and heart rate variability (HRV) — remain accurate predictors of mortality in patients with systolic dysfunction. Methods: Non-invasively obtained BRS and HRV were analyzed in 205 clinically stable patients with left ventricular ejection fraction (LVEF) ≤ 40%. 28 patients died within 28 ± 9 month follow-up. Results: Baroreflex sensitivity, low-frequency (LF) in normalized units, LF to high-frequency ratio and standard deviation of average R-R intervals were significantly associated with mortality; cut-off values of the highest discriminatory power for abovementioned parameters were ≤ 3.0 ms/mmHg, ≤ 41, ≤ 0.7 and ≤ 25 ms, respectively. In bivariate Cox analyses (adjusted for LVEF, New York Heart Association [NYHA] or absence of implantable cardioverter-defibrillator [ICD]) autonomic indices remain significant predictors of death. Conclusions: Baroreflex sensitivity and HRV — may still be helpful in identifying patients with left ventricular systolic dysfunction at the highest risk of all-cause mortality, independently of LVEF, NYHA class and ICD implantation.

Background: The left ventricular (LV) hypertrophy increases the risk of heart failure. Hypertension and infiltrative cardiomyopathies are the well-known reasons of LV hypertrophy. The growing interest of scientists in this issue affects hereditary haemochromatosis (HH), which is characterised by the excess deposition of iron mostly due to HFE gene mutation. The aim of our study was to investigate the possible influence of HH on LV parameters in patients with early-diagnosed (early HH) and long-lasting and long-treated (old HH) disease. Materials and methods : Thirty nine early HH and 19 old HH patients were prospectively enrolled in the study; age- and sex-matched healthy volunteers constituted the appropriate control groups. All participants had echocardiography performed (including three-dimension volume and mass analysis); the iron turnover parameters were measured at the time of enrolment in every HH patients. Results : Echocardiographic parameters regarding to left atrium (LA), LV thickness, mass and long axis length were significantly higher, whereas LV ejection fraction was lower in early HH in comparison to healthy persons. In old HH patients the differences were similar to those mentioned before, except LV ejection fraction. The presence of hypertension in both HH groups did not influence echo parameters, as well as diabetes in old HH. The strongest correlation in all HH group was found between the time from HH diagnosis and LA, LV thickness and volumes parameters, but the correlations between iron turnover and echo parameters were non-existent. Conclusions: Hereditary haemochromatosis, not only long-lasting, but also early-diagnosed, could lead to exacerbation of LV wall thickness and cardiac hypertrophy. This effect is not simply connected with hypertension and diabetes that are frequent additional diseases in these patients, but with the time from HH diagnosis.

This document is a statement from Cardiovascular Pharmacotherapy Section of the Polish Cardiac Society regarding the practical approach to therapy with novel oral anticoagulants — thrombin antagonists and Xa antagonists, based upon most recent scientific information including European Society of Cardiology guidelines 2016 and recommendations from European Heart Rhythm Association.

Introduction: Methylmalonic Aciduria (MMA) is a heterogeneous group of rare diseases leading to accumulation of methylmalonic acid in body fluids. One of the causes of the disease is the methylmalonic aciduria, cblA type (cblA – type MMA), conditioned by a mutation in the MMAA gene, which is essential for the proper functioning of a cofactor of the methylmalonyl-CoA mutase. The symptoms of the disease, depending on the cause, may manifest themselves at different ages. Most patients are sensitive to high doses of hydroxycobalamin, which is associated with better prognosis. Material and method: The purpose of the study was to retrospectively analyze the clinical picture and effects of treatment of patients with methylmalonic aciduria related to mutation in the MMAA gene. Results: Five patients with diagnosed cblA – type MMA were presented. At the time of diagnosis the median of age was 18.8 months, but the symptoms had already appeared since infancy, as recurrent vomiting and delayed psychomotor development. Significant excretion of methylmalonic acid in urine and metabolic acidosis traits with significantly increased anionic gap were observed in all patients. All of them were sensitive to the treatment with vitamin B12. The median of therapy duration and observation is 12.2 years. During the treatment, good metabolic control was achieved in all patients, but their cognitive development is delayed. Three patients have renal failure and pharmacologically treated arterial hypertension. Conclusions: Patients with a mutation in the MMAA gene are sensitive to treatment with hydroxocobalamine, but the inclusion of appropriate treatment does not protect against neurodevelopmental disorders and chronic kidney disease. Keywords: MMAA, CblA, Hydroxocobalamin, Renal failure

BACKGROUND Hereditary haemochromatosis (HH) is a common inherited disease. Abnormally increased intestinal iron absorption and accelerated recycling of iron by macrophages lead to progressive body iron accumulation and the generation of oxidative stress. In the late stages, iron overload can lead to dysfunction of the left ventricle (LV). It is believed that two-dimensional speckle tracking echocardiography (2D STE) can evaluate LV dysfunction more accurately than conventional echocardiography. This accurate evaluation seems to be clinically important in the early stages of HH before substantial damage of the LV. Evaluation of this assessment was the purpose of this paper. METHODS We prospectively enrolled 24 patients with early diagnosed HH and without any history of cardiovascular diseases; 23 healthy age- and sex-matched volunteers constituted the control group. Standard echocardiographic parameters and LV rotation and strain parameters were assessed and compared between the groups. RESULTS All echocardiographic parameters were within normal ranges, and there were no differences between the groups. 2D STE revealed significantly worse basal and apical rotation, twist and torsion values in HH patients. The peak systolic longitudinal strain was decreased in HH patients, even though the LV ejection fraction (LVEF) was normal. There were weak correlations between the iron turnover and 2D STE parameters. CONCLUSIONS 2D STE seems to be more sensitive than traditional echocardiography for detecting LV abnormalities in HH patients who are diagnosed early. The results of this study may be clinically useful, but their relevance and therapeutic implications remain to be confirmed by further studies.

INTRODUCTION Inadvertently induced ventricular fibrillation (VF) by radiofrequency (RF) energy delivery for premature ventricular complexes (PVCs) is a rare phenomenon; nevertheless, it is crucial to assess long‑term risk of sudden cardiac death in these patients. OBJECTIVES The aim of our study was to define the long‑term prognosis in patients with normal ejection fraction (EF), in whom VF was inadvertently induced by RF energy application during ablation of symptomatic idiopathic PVCs originating from the right ventricular outflow tract (RVOT). PATIENTS AND METHODS Among over 20 000 RF catheter ablations performed at 5 tertiary centers (2008-2016), 6 patients (5 men) had VF induced by RF application to the RVOT. The mean (SD) age of patients was 35.2 (16.8) years. All patients had normal EF (≥60%). We analyzed the risk of malignant ventricular arrhythmias and assessed heart function during follow‑up. RESULTS After ablation, baseline contrast‑enhanced magnetic resonance imaging was performed in 4 of the 6 patients; no area of late gadolinium enhancement was observed. One patient received an implantable cardioverter‑defibrillator (ICD). Exercise tests revealed only rare PVCs. All patients completedthe follow‑up (mean [SD] duration of follow‑up, 64.0 [34.9] months). All patients were alive, with no cases of syncope, documented ventricular tachycardia, or VF. The patient with an ICD received 2 inappropriate high‑voltage therapies. CONCLUSIONS Patients with inadvertently induced VF via RF energy application during ablation of PVCs from the RVOT, who have normal left ventricular function and no electrocardiography abnormalities have good prognosis and low VF risk during long‑term follow‑up. Therefore, ICD placement seems to be not indicated for these patients.

INTRODUCTION Inadvertently induced ventricular fibrillation (VF) by radiofrequency (RF) energy delivery for premature ventricular complexes (PVCs) is a rare phenomenon; nevertheless, it is crucial to assess long‑term risk of sudden cardiac death in these patients. OBJECTIVES The aim of our study was to define the long‑term prognosis in patients with normal ejection fraction (EF), in whom VF was inadvertently induced by RF energy application during ablation of symptomatic idiopathic PVCs originating from the right ventricular outflow tract (RVOT). PATIENTS AND METHODS Among over 20 000 RF catheter ablations performed at 5 tertiary centers (2008-2016), 6 patients (5 men) had VF induced by RF application to the RVOT. The mean (SD) age of patients was 35.2 (16.8) years. All patients had normal EF (≥60%). We analyzed the risk of malignant ventricular arrhythmias and assessed heart function during follow‑up. RESULTS After ablation, baseline contrast‑enhanced magnetic resonance imaging was performed in 4 of the 6 patients; no area of late gadolinium enhancement was observed. One patient received an implantable cardioverter‑defibrillator (ICD). Exercise tests revealed only rare PVCs. All patients completedthe follow‑up (mean [SD] duration of follow‑up, 64.0 [34.9] months). All patients were alive, with no cases of syncope, documented ventricular tachycardia, or VF. The patient with an ICD received 2 inappropriate high‑voltage therapies. CONCLUSIONS Patients with inadvertently induced VF via RF energy application during ablation of PVCs from the RVOT, who have normal left ventricular function and no electrocardiography abnormalities have good prognosis and low VF risk during long‑term follow‑up. Therefore, ICD placement seems to be not indicated for these patients.