Long QT syndrome (LQTS) is a rare (1:2500–1:10,000) inherited disorder characterized by the onset of arrhythmogenic syncope, polymorphic ventricular tachycardia, and sudden cardiac death. The aim of this article was to describe an unexpected success with an unusual therapeutic modality of a patient diagnosed with LQTS syndrome (suspected Romano–Ward syndrome) during an 8-year period. A 59-year-old female patient was admitted to the hospital due to chest pain and nausea, and after diagnostic and therapeutical approach, a permanent dual-chamber rate-modulated (DDDR) pacemaker was implanted instead of the implantable cardioverter defibrillator (ICD). During the 8-year period, the patient remained stable, without rhythm disorder. Romano–Ward syndrome as a congenital LQTS carries a high risk of sudden cardiac death and presents an indication for ICD. In this patient, for objective reasons, this could not be performed. Implantation of a DDDR with an appropriate pharmacological therapy, including propranolol, in this case, proved to be a successful therapeutic modality.

Introduction: The head-up tilt table test is noninvasive diagnostic procedure, which is used in the diagnosis of syncope. Syncope presents a benign short-term disorder of cerebral circulation with the sudden loss of consciousness and muscle tone. Aim: The aim is to present not only the role and importance of orthostatic tests in the daily clinical practice of pediatric cardiology, neuropediatrics but also pediatrics in general. Patients and Methods: This study has retrospective descriptive character and included the period from April 1997 to June 2020, during which the registration and analysis of orthostatic stability tests (head-up/tilt table test, tilt table test) was performed. Medical documentation of outpatient and hospitalized patients on the Paediatric Clinic of Clinical Center University of Sarajevo (Register of Tilt Table Test) was used. Results: During this period, 1029 tests were registered and analyzed. Modification test (head-up) was performed in 132 (12.8%) patients, and since 2008, classic tilt table test was performed in 897 (87.1%) patients. Patients were 6.5–19 years old, with a predominance of female patients 611 (59.4%). There were 519 (50.4%) patients who were 15–19 years old and 510 (49.6%) patients who were under 15 years of age. Indications were syncope or suspected syncope in 671 (65.2%) patients, cardiovascular etiology (arrhythmias, chest pain, congenital heart defects [CHDs], surgically corrected CHDs, hypotension, and hypertension) in 195 (19%) patients, neuropediatric pathology (epilepsia, suspected epilepsia, headache, vertigo) in 101 (9.8%) patients, and other indications in 62 (6.03%) patients. From the total number of tests, 862 were first tests (83.4%) and 167 were control tests (16.3%). The positive test was found in 538 (52.3%) patients, most often vasovagal syncope (473 patients or 87.9%). Conclusion: Tilt table test is a reliable diagnostic tool in examining the etiology of syncope, primarily vasovagal, and is an extremely important method primarily in cardiopediatric and neuropediatric daily diagnostics.

Introduction: A Holter blood pressure monitoring is a basic method for the diagnosis and evaluation of hypertension therapy. Hypertension in children and adolescents is defined as an increase of systolic/diastolic pressure, which is equal, or above 95th percentile blood pressure for sex, age, and height. Aim: The aim of this study is to analyze the etiology of arterial hypertension (AH) in the pediatric population. Methods: Research had descriptive and retrospective character. During the period from March 2006 to April 2020, 1527 registered continuous Holters of blood pressure were analyzed. Data were taken from the medical documentation of patients that were hospitalized on Pediatric Clinic (register of continuous Holter of blood pressure). Results: Out of the total number of registered and analyzed patients 833 were male (54.5%) with dominant age 15–19 years of life 774 (50.6%), school-age children 660 (43.2%), preschool children 93 (6.1%). We had 902 (59%) first registrations and 626 (41%) control registrations. AH was verified in 52 patients (387 records of continuous Holter of blood pressure were performed to them). Primary AH was verified in 27 patients and secondary AH in 25 patients. Forty patients (76.9%) were treated with monotherapy while combined therapy was used in 12 (23.1%) of cases. Renal cause was in 28% patients, endocrine in 24%, cardiovascular in 24%, neurological in 16%, and rheumatic in 8% of patients with secondary AH. Conclusion: Continuous Holter of blood pressure represents useful diagnostic method and method of control of high blood pressure in children and adolescents. It should be routine method in everyday pediatric clinical practice especially in pediatric cardiology.

Introduction: Emergency cerclage in the second trimester is aestablished treatment for a dilated cervix. Aim: To report a case of a successful cerclage performed in a 33-year old woman in her secondpregnancy, after 5 years of non-successfulpregnancy outcomes. Case report: In her fourth month of pregnancy, the patient was hospitalized because of suprapubic pressure. After complete laboratory results, ultrasound and vaginal examination the patient was diagnosed with cervical shortening, cervical canal was opened 3cm, with prolapse and bulging of the fetal membranes in vagina. An amount of 120 ml of clear amniotic fluid was removed transabdominally under ultrasound guidance, and sent to the microbiological and genetical analysis. McDonald emergency cerclage of the cervical canal was performed. Patient was monitored few days on department and released home with advice of strict reduction of activity to minimum, and prescribed therapy due to that: antithrombotic, progesterone and antibiotic therapy. Conclusion: Amnioreduction at the time of emergency cerclage placement is associated with a lower rate of extreme prematurity and related neonatal morbidity. Successful outcome is not impossible, along with adequate antibiotic regimen, bed rest and regular obstetrical control/checkup.

Objective – The aim of the paper is to present a rare and complex congenital heart defect (CHD), congenitally corrected transposition of the great arteries of the heart (ccTGA) with associated anomalies, including ventricular septal defect (VSD), valvular and subvalvular pulmonary stenosis, dysplasia of the tricuspid valve, and atrial septal defect (ASD) with first-degree atrioventricular block, which was diagnosed, monitored and successfully treated with heart surgery in an infant. Case Report – A female infant was born with 3350 grams in weight, 50 cm in length, oxygen saturation of 98%, and heart rate of 170 beats per minute. The antenatal and perinatal period was normal. CHD was verified by ultrasound at the age of 3 days. Angiotensin-converting-enzyme inhibitors (ACE inhibitors) and diuretics were introduced in therapy after one month. Cardiac surgery (Senning-Rastelli procedure with placement of an 18-mm Contegra conduit) was performed at the age of 9 months. After the operation, the infant was stable on therapy with diuretics, antiaggregants, beta blockers, and antianemic therapy with vitamin D in prophylaxis. Conclusion – ccTGA with associated anomalies is a rare, life-threatening, congenital heart disease. After birth it demands correct diagnosis, adequate follow-up, and cardiac surgery in infancy.

Background: The aim of the study was to highlight the importance of adequate anticoagulant therapy and the correlation of higher risk of stroke. Methods: This study analyzed data obtained from 103 patients with diagnosis of atrial fibrillation (AF) (39 of them had a stroke). Patients were divided into groups according to the CHADS2, CHA2DS2-VASc, and HASBLED scores. Results: An analysis showed that anticoagulant drugs were more often prescribed to subjects <75 years of age (P = 0.001). Patients with a higher CHADS2 score had a higher CHA2DS2-VASc score and vice versa (rho = 0.513; P = 0.0001). According to the CHA2DS2-VASc, 91.3% of the patients examined were prescribed an anticoagulant medication as a therapy at discharge from the hospital. The result was statistically significant compared to the practice where an anticoagulant was prescribed to 55.9% of high-risk subjects as estimated by the CHA2DS2-VASc score (P < 0.05). Our results also show that rivaroxaban is more commonly prescribed as a discharge therapy than warfarin (χ2 = 12.401; P = 0.0001). Furthermore, a significantly higher number of patients who were being prescribed aspirin (38.5%) had a stroke compared to 12.8% of patients who were being prescribed warfarin (χ2 = 12.259; P = 0.0001). Conclusions: Novel oral anticoagulants (NOACs) seem to be a better choice as a pharmacological therapy in the treatment of AF, due to a lack of adequate monitoring of patients' international normalized ratio (INR) values. CHA2DS2-VASc and HASBLED scores must be used as a part of routine clinical diagnostics when dealing with patients with AF.

Alzheimer's disease represents the most common age-related neurodegenerative disorder and a leading cause of progressive cognitive impairment. Predicting cognitive decline is challenging but would be invaluable in an increasingly aging population which also experiences a rising cardiovascular risk. In order to examine whether plasma measurements of one of the established biomarkers of heart failure, brain natriuretic peptide (BNP), reflect a decline in cognitive function, associated with Alzheimer's disease neurodegeneration, BNP levels were analysed, by using a novel assay called a SOMAscan, in 1. cognitively healthy, control subjects; 2. subjects with mild cognitive impairment, and 3. subjects with Alzheimer's disease. The results of our study show that the levels of the BNP were significantly different between the three types of diagnoses (p < 0.05), whereby subjects with mild cognitive impairment had the lowest mean BNP value, and healthy subjects had the highest BNP value. Importantly, our results show that the levels of the BNP are influenced by the presence of at least one APOE4 allele in the healthy (p < 0.05) and in the Alzheimer's disease groups of subjects (p < 0.1). As the levels of the BNP appear to be independent of the APOE4 genotype in subjects with mild cognitive impairment, the results of our study support inclusion of measurements of plasma levels of the BNP in the list of the core Alzheimer's disease biomarkers for identification of the mild cognitive impairment group of patients. In addition, the results of our study warrant further investigations into molecular links between Alzheimer's disease-type cognitive decline and cardiovascular disorders.

Lafora progressive myoclonus epilepsy (Lafora disease, LD) is a fatal autosomal recessive neurodegenerative disorder (with an onset in teenage years in previously normal adolescents). This paper represents a view of a patient diagnosed with Lafora progressive myoclonus epilepsy, over a course of seven years. A description of the initial manifestation of symptoms, doctors' attempts to combat the symptoms with drug treatment, further attempts towards reaching the correct diagnosis, the final confirmation of the Lafora diagnosis (mutation in the NHLRC1 gene), and the current state of the patient is presented. The absence of a positive family history, the lack of staff specialized in dealing with this or similar pathology, and the diagnostic inability to characterize this type of disorder in Bosnia and Herzegovina have led to a fair delay in diagnosing and beginning of an adequate pharmacological treatment. Overall, recent identification of LD cases in Bosnia and Herzegovina warrants an establishment of a Centre for Genetic Testing in order to ensure more humane counseling of an entire family whose family member(s) might be diagnosed with this devastating and currently an incurable disorder.

1Sarajevo School of Science and Technology, Sarajevo, Bosnia and Herzegovina 2University Clinical Center Sarajevo, Sarajevo, Bosnia and Herzegovina KeYWORdS: natriuretic peptides, brain natriuretic peptide, biomarker of myocardial infarction, Alzheimer’s disease. citAtiON: Cardiol Croat. 2018;13(11-12):435. | https://doi.org/10.15836/ccar2018.435 *AddReSS fOR cORReSpONdeNce: Edin Begic, Sarajevo School of Science and Technology, Sarajevo Medical School, Department of Pharmacology, Hrasnicka cesta 3a, 71000 Sarajevo, Bosnia and Herzegovina. / Phone: +38761303375 / E-mail: edinbegic90@gmail.com ORcid: Edin Begic, https://orcid.org/0000-0001-6842-262X • Suncica Hadzidedic, https://orcid.org/0000-0001-9026-8737 Ajla Kulaglic, https://orcid.org/0000-0003-3410-7079 • Belma Ramic-Brkic, https://orcid.org/0000-0002-8205-0137 Zijo Begic, https://orcid.org/0000-0002-1863-5755 • Mirsada Causevic, https://orcid.org/0000-0002-6099-6415

Introduction: Brugada syndrome (BS) is a dominantly inherited arrhythmogenic disease caused by a mutation in the SCN5A gene. It accounts for 20% of cases of sudden death, without structural heart abnormalities1. Diagnosing the BS is achievable by electrocardiography (ECG), ST segment elevation in V1 to V3, with the right bundle branch block pattern as a hallmark of the syndrome2. BS is divided into three types. However, only type 1 can be verified with an ECG2,3. BS manifests as a syncope that is caused by ventricular tachycardia, which, if converted to ventricular fibrillation, leads to a fatal outcome. An implantable cardioverter defibrilator (ICD) implantation is indicated, while pharmacological therapy on its own is not sufficiently effective. Aim: To present a diagnostic and therapeutic approach towards suspected BS in a younger patient. Case report: 24-years-old patient was admitted to a hospital, after a cardiac arrest and a prolonged cardiopulmonary resuscitation with intubation. ECG findings verified sinus rhythm, with heart rate of 94 beats per minute, normal heart axis with PQ interval of 0.16 s, and right bundle branch block (RBBB) with an ST elevation from V1 to V3. An ajmalin provocation test was performed, and ECG changes (J-wave elevation of >2 mm with ST elevation from V1 to V3 with RBBB) were recorded, but without induced ventricular arrhythmia. Patent foramen ovale was suspicious as a cause, but after transesophageal echocardiography it was excluded. According to electrocardiographic changes, the BS was diagnosed as the cause of malignant ventricular heart rhythm. Genetic testing for Brugada syndrome was not performed and in consultations with the Centre for Electrophysiology in Sarajevo (Bosnia and Herzegovina) and Zadar (Croatia), the implantation of an ICD was indicated, and subsequently performed. The patient was discharged under pharmacological therapy consisting of metoprolol 25 mg twice per day, amiodarone 100 mg per day, with magnesium, and aspirin once per day. Conclusion: In daily clinical work, in all conditions of syncope occurring in younger patients, in order to prevent sudden death, an existence of the BS should be considered. An overall clinical status of a patient, including positive ajmaline test with specific ECG changes, can verify BS, even when information on the presence of the SCN5A gene is not available.

Introduction: A tricuspid annular plane systolic excursion (TAPSE) reflects longitudinal myocardial shortening and represents an echocardio­graphic parameter to assess right ventricular systolic function.Aim: To determine relationship between TAPSE and gestational age and body weight in neonates, establishing method for prediction the normal TAPSE values in neonates based on gestational age and body weight.Patients and methods: The prospective study group consisted of 97 neonates from 24 to 40 weeks of gestation, with a weight of 625-4,340 g and normal echocardiographic results with determination of TAPSE based on gestational age and body weight.Results: The TAPSE range was 6.45-9.80 mm (with average value 8.07 ± 1.89 mm) in male and 6.95-8.50 mm (with average value 7.9 ± 1.86 mm) in female babies, depending on gestational age. There was no statistically significant difference of normal TAPSE values between female and male patients (p = 0.586). TAPSE is affected by increasing birth weight and increasing gestational age. The TAPSE values have had strong and positive correlation with gestational age (p = 0.0001, rho = 0.692) as well with body weight (p = 0.0001, rho = 0.786). Regression equation relating body weight and TAPSE is: TAPSE predicted = 4.738 + (body weight * 0.002); equation relating gestational age and TAPSE is: TAPSE predicted = -4.163 + (gestational weeks * 0.385).Conclusions: It is possible to adequately predict TAPSE based on gestational age. As TAPSE is easy to measure and highly reproducible, we consider it a useful quantitative parameter to assess right ventricular longitudinal function in premature baby.