Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) syndrome is a potentially lethal and rare congenital cardiac anomaly (for approximately 1 in 300,000 live births). ALCAPA syndrome mostly presents in the first few months of life. Left untreated, the mortality rate in the first year of life is 90% secondary to myocardial ischemia or infarction and mitral valve insufficiency leading to congestive heart failure. When pulmonary arterial pressure decreases, it results in reversal of flow so that LCA drains from the right coronary artery through collateral vessels into the pulmonary artery. This phenomenon causes ischemia or eventually infarction of the anterolateral left ventricular wall. Sudden death may occur because of inadequate collateral circulation between LCA and the right coronary artery and/or development of arrhythmia. ALCAPA can be found both in infants and in older individuals, with the absence or presence of strong collateral circulation from the right coronary artery, with different clinical presentations. Clinical diagnosis of ALCAPA can be demanding. Findings can be similar to those in dilatative cardiomyopathy and the diagnosis of ALCAPA has to be excluded, principally in the presence of ischemic ECG findings. Currently, the prognosis for patients with ALCAPA syndrome is dramatically improved as a result of both early diagnosis and improvements in surgical techniques, including myocardial preservation. This paper presents 3 patients with ALCAPA syndrome as well as the variations in their age, clinical presentation, modality of treatment (pharmacological treatment, surgical correction and percutaneous coronary stent implantation after graft obstruction) and outcome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is rare, but life-threat-ening condition. The treatment of choice in patients with ALCAPA is the establishment of a dual coronary artery system with surgical reimplantation of the left coronary artery in the left coronary sinus. Percutaneous coronary intervention is infrequent in the pediatric population but can be a life-saving by promptly restoring flow to an obstructed coronary artery. It is a highly demanding and high-risk procedure in infants due to the technical difficulties and the small coronary artery diameter in infants.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is rare, but life-threatening condition. The treatment of choice in patients with ALCAPA is the establishment of a dual coronary artery system with surgical reimplantation of the left coronary artery in the left coronary sinus. Percutaneous coronary intervention is infrequent in the pediatric population but can be a life-saving by promptly restoring flow to an obstructed coronary artery. It is a highly demanding and high-risk procedure in infants due to the technical difficulties and the small coronary artery diameter in infants.

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.

Introduction: Modern pediatric cardiology mainly deals with congenital heart defects (CHD), as the most common congenital anomalies. In most cases CHD requires surgical or interventional treatment. Goal: The goal of the research was to evaluate CHD treatment at Pediatric Clinic, University Clinical Center (UCC) Sarajevo, Bosnia and Herzegovina (B&H). UCC Sarajevo is the only institution in B&H where cardiac treatment of CHD in pediatric population is performed. Pediatric cardiosurgery has started to develop in Bosnia and Herzegovina in April 1997. Patients and methods: Study included 745 patients (period from April 1997 to January 2017). Results: Cardiac treatment was performed on 745 patients with CHD, 541 (72.6%) of them were acyanotic patients and 204 (27.4%) were cyanotic patients. Reoperation was performed in 49 (6.5%) of patients. Out of total number of patients, 59 (7.9%) died. In 660 children (88.5%) a complete correction and in 85 (11.5%) a palliative operation/correction was performed. Defects with left to right shunt were present in 397 (53.2%) patients, complex heart defects with Tetralogy of Fallot in 173 (23.2%), obstructive heart defects in 106 (14.2%), obstructive heart defects with shunt in 53 (7.1%), and others in 16 (2.1%) of patients. During surgery, extracorporeal circulation was regulated in 554 (74.3%) patients. Peri and early postoperative complications occurred in 180 (24.1%) of patients. During this period 24 pacemakers were implanted and 24 radiofrequency ablations were performed. Conclusion: Results of CHD cardiac treatment in childhood, which took place simultaneously, followed by the contractual joint programs and individual work of the Bosnian and Herzegovinian team has reached the highest level in the last two years, not only by the number, type and complexity of corrected CHD, but also by the age and body weight of the patients who underwent surgery, and development of invasive procedures, arrhytmology, pacemaker placement, intensive peri and postoperative treatment. That led pediatric cardiac surgery of UCC Sarajevo, to a position of leading center in the region.

Objective: To show the place and role of continuous electrocardiographic twenty-four-hour ECG monitoring in daily clinical practice of pediatric cardiologists. Methods: According to protocol, 2753 patients underwent dynamic continuous ECG Holter monitoring (data collected from the “Register of ECG Holter monitoring” of Pediatric Clinic, UCC Sarajevo in period April 2003- April 2015). Results: There were 50,5% boys and 49,5% girls, aged from birth to 19 years (1,63% - neonates and infants, 2,6% - toddlers, 9,95% - preschool children, 35,5% - gradeschoolers and 50,3% children in puberty and adolescence). In 68,1% of patients Holter was performed for the first time. Indications for conducting Holter were: arrhythmias in 42,2% cases, precordial pain in 23,5%, suspicion of pre-excitation and/or pre-excitation in 10%, crisis of consciousness in 8%, uncorrected congenital/acquired heart defects in 4,2%, operated heart defects in 3,7%, hypertension in 3,1% cases, control of the pacemaker in 1,63% and other causes in 3,5% cases. Discharge diagnosis after ECG Holter monitoring were: insignificant arrhythmias in 47,1% cases, wandering pacemaker in 21,3%, pre-excitation in 16,2%, benign ventricular premature beats in 6,3%, atrioventricular block in 3%, sinus pause in 2.2% cases and other arrhythmias in 3,5%. In mentioned period 57 cases of Wolf Parkinson White syndrome were registered, in 4,5% of patients antiarrhythmic therapy was administered. Radiofrequent ablation was performed in 23 cases. Conclusion: The development of pediatric cardiac surgery has initiated development of pediatric arrhythmology as imperative segment of pediatric cardiology. Continuous ECG Holter monitoring has become irreplaceable method in everyday diagnostics and therapy of arrhythmias in children.

This case report presents a four month old infant with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome including clinical presentation of severe cardiomyopathy and left-sided heart failure. Child was operated for reimplantation of ALCAPA to aortic root with satisfactory outcome.