[The significance of N-terminal pro-B natriuretic peptide in the management of adult patients with congenital heart disease - Results from the CSONGRAD Registry]. / Az N-terminális pro-B natriureticus peptid jelentosége a felnott korú congenitalis szívbetegek gondozásában ­ Eredmények a CSONGRAD Regiszterbol.

INTRODUCTION: The prognostic role of B-type natriuretic peptide (BNP) level was confirmed in chronic heart failure and congenital heart diseases irrespective of the aetiology. AIM: The aim of this study was to compare NT­proBNP measured in the clinical practice and important clinical and echocardiographic parameters in patients with adult congenital heart diseases under our care. METHOD: Data of a total of 70 patients were analysed; 34 patients had corrected tetralogy of Fallot and 19 patients had corrected transposition of the great arteries. In 17 further cases, patients with other congenital vitiums have been involved in the study. In all cases, out-patient examination was performed with electrocardiography, echocardiography and NT-proBNP measurement. The New York Heart Association (NYHA) stages of the patients were determined and the patients filled in a questionnaire regarding their quality of life. RESULTS: Elevated NT-proBNP level resulted in a worse quality of life (visual analogue scale, VAS) and an increased right ventricular end diastolic diameter; the incidence of arrhythmia increased as well. Negative correlation was found between the NT-proBNP level and the VAS value (r = -0.45, p = 0.0001) and the left ventricular ejection fraction (LV-EF) (r = -0.67, p = <0.0001). ROC analysis showed that NT­proBNP≥668.1 pg/ml was the cut-off value that most accurately predicted NYHA class III-IV (sensitivity 93%, specificity 63%, area under the curve 80%, p = 0.001). Similarly, NT-proBNP≥184.7 pg/ml was found to be the cut-off value most accurately predicting LV-EF below 55% (sensitivity 66%, specificity 67%, area under the curve 77%, p = 0.02). CONCLUSIONS: NT-proBNP has a role in the evaluation of adult patients with congenital heart diseases and in determining the prognosis of these patients, and in addition to other examinations used in the follow-up of these patients, NT-proBNP may help in determining the time of reoperation or potential heart transplantation. Orv Hetil. 2018; 159(4): 141-148.

[Long-term follow-up of patients with transposition of the great arteries following Senning or Mustard operations. Results from the CSONGRAD Registry]. / A teljes nagyér-transzpozíción átesett betegek Senning- és Mustard-féle mutéti megoldásának hosszú távú utánkövetése. Eredmények a CSONGRAD Regiszterbol.

INTRODUCTION: Transposition of the great arteries is one of the most common cyanotic congenital heart diseases. AIM: The present study aimed to examine and compare long-term survival, functional grading, arrhytmologic and quality of life control in patients with transposition of the great arteries following Senning- and Mustard-operations. METHODS: The present study comprised 85 patients with transposition of the great arteries, of whom Senning-operation was performed in 37 cases and Mustard-operation in 48 subjects. Follow-up study was performed in all cases. RESULTS: The success rate of long-term follow-up proved to be 74%. Twelve out of the 31 Senning-operated and 16 out of 32 Mustard-operated patients died during the follow-up (39% vs. 50%, p = 0.45). Neither features of heart failure, nor those of arrhythmias showed differences between the groups, but parameters of quality of life and functional capacity proved to be favourable in Senning-operated patients. CONCLUSIONS: There is no significant difference in mortality and morbidity of patients with transposition of the great arteries following Mustard- and Senning-operations. Regarding to long-term follow-up quality of life and functional capacity of Senning-operated patients were more favourable.

[More than 50 years' experience in the treatment of patients with congenital heart disease at a Hungarian university hospital]. / Több mint 50 év tapasztalat a congenitalis szívbetegek ellátásában egy magyar egyetemi központban. A CSONGRÁD Regiszter alapadatai.

Improvements in surgical techniques and technical advancements have made possible for several patients with congenital heart disease to grow up to adulthood. It has been decided to create a registry for their more precise treatment. This registry now includes 2770 patients with data on 3043 operations, with almost 30 different diagnoses. The purpose of this paper is to review the facts and the basics leading to the establishment of this registry.

Impact of single nucleotide polymorphisms of cytarabine metabolic genes on drug toxicity in childhood acute lymphoblastic leukemia.

BACKGROUND: Cytarabine (cytosine arabinoside, ara-C) is a chemotherapeutical agent used in the treatment of pediatric acute lymphoblastic leukemia (ALL). Adverse drug reactions, such as interpatient variability in sensitivity to ara-C, are considerable and may cause difficulties during chemotherapy. Single nucleotide polymorphisms (SNPs) can play a significant role in modifying nucleoside-drug pharmacokinetics and pharmacodynamics and thus the development of adverse effects. Our aim was to determine whether polymorphisms in genes encoding transporters and enzymes responsible for the metabolism of ara-C are associated with toxicity and clinical outcome in a patient population with childhood ALL. PROCEDURE: We studied 8 SNPs in the CDA, DCK, DCTD, SLC28A3, and SLC29A1 genes in 144 patients with childhood acute lymphoblastic leukemia treated according to ALLIC BFM 1990, 1995 and 2002 protocols. RESULTS: DCK rs12648166 and DCK rs4694362 SNPs were associated with hematologic toxicity (OR = 2.63, CI 95% = 1.37-5.04, P = 0.0036 and OR = 2.53, CI 95% = 1.34-4.80, P = 0.0044, respectively). CONCLUSIONS: Our results indicate that DCK polymorphisms might be important genetic risk factors for hematologic toxicity during ALL treatment with ara-C. Individualized chemotherapy based on genetic profiling may help to optimize ara-C dosing, leading to improvements in clinical outcome and reduced toxicity.