Clinical and Hemodynamic Outcomes with Enalapril Orodispersible Minitablets in Young Children with Heart Failure Due to Congenital Heart Disease.

: The angiotensin-converting enzyme inhibitor (ACEI) enalapril is often administered to infants and young children with heart failure (HF) in various dosing regimens and formulations not adapted for their age. : This prospective, two-center, open-label 8-week study evaluated an age-appropriate formulation of orodispersible minitablets (ODMTs) of enalapril (0.25 mg and 1 mg) in children aged 0 to 6 years with HF due to congenital heart disease.

Immediate results of primary balloon dilation for congenital aortic valve stenosis predict the mid-term outcome.

Background: Balloon valvuloplasty is the primary treatment for congenital aortic valve stenosis in our centre. We sought to determine independent predictors of reintervention (surgical repair or repeated balloon dilation) after primary valvuloplasty.

Methods: We retrospectively studied patients with congenital aortic valve stenosis who underwent balloon valvuloplasty during 2004-2018.

Genetic evaluation of newborns with critical congenital heart defects admitted to the intensive care unit.

Rapid and efficient diagnostics is crucial for newborns with congenital heart defects (CHD) in intensive care unit (ICU) but is often challenging. Given that genetic factors play a role in 20-30% cases of CHD, it is likely that genetic tests could improve both its speed and efficiency. We aimed to analyze the utility of rapid and cost-effective multiplex ligation dependent probe amplification analysis (MLPA) for chromosomal analysis in newborns with critical CHD.

Oral propranolol for infantile hemangiomas: a prospective study on the role of 48-hour Holter monitoring in additional safety assessment.

Purpose: Oral propranolol has been recently approved for infantile hemangiomas (IHs), but potential side effects stay a challenge. We sought to make an additional assessment on oral propranolol safety for this indication.

Materials And Methods: Prospective study included 108 infants consecutively treated for IHs at the University Children's Hospital Tirsova, Belgrade from January 2010 to December 2013.

[The Role of Two-Dimensional Echocardiography in Diagnostics of Coarctation of the Aorta in Newborns].

Introduction: Diagnosis of neonatal coarctation of the aorta (CoA) still presents a challenge in routine practice because of absence of reliable morphologic and functional parameters for early detection of this congenital heart defect in newborns.

Objective: The aim of this study is to identify easy obtainable two-dimensional echocardiographic parameters for detection of the CoA in newborns.

Methods: Echocardiographic evaluation was performed in 30 newborns with CoA and 20 healthy neonates (control group).

[Mid-term results after complete surgical correction of transposition of the great arteries].

Introduction: Arterial switch operation (ASO) is a cardiosurgical method of choice for complete anatomical correction of transposition of great arteries. Improvement of this procedure has made considerably improved the outcome and long-term prognosis of children born with this complex congenital heart disease.

Objective: The aim of this study was to estimate the success rate of ASO through retrospective analysis of mortality and late complications.

[Outcomes of surgery for total anomalous pulmonary venous drainage].

Introduction: Total anomalous pulmonary venous connection (TAPVC) is a rare congenital heart disease and in some variants represents the only true surgical emergency in congenital heart surgery. Basic anatomical characteristic of this anomaly is an abnormal connection of pulmonary veins with systemic venous circulation. Although the results of TAPVC repair in infancy have been markedly improved in recent years, the recurrent pulmonary venous obstruction (RPVO) remains relatively frequent complication of surgical treatment.

Balloon valvuloplasty as a treatment of congenital aortic stenosis in children and adolescents.

Introduction: Balloon valvuloplasty (BVP) is one of the primary therapies for congenital aortic stenosis in children and adolescents. The aim of this interventional procedure is to gain time before possible surgical therapy (aortic valve replacement) until adulthood.

Objective: The aim of this study was to evaluate the efficacy, safety and mid-term results oftranscatheter BVP in children and adolescent in our Center.

Transcatheter stenting of arterial duct in duct-dependent congenital heart disease.

Introduction: Critical congenital heart diseases (CHD) are mostly duct-dependent and require stable systemic-pulmonary communication. In order to maintain patency of the ductus arteriosus (DA), the first line treatment is Prostaglandin E1 and the second step is the surgical creation of aortic-pulmonary shunt. To reduce surgical risk in neonates with the critical CHD, transcatheter stenting of DA can be performed in selected cases.

Anomalous origin of the left coronary artery from the pulmonary artery, scimitar syndrome, and aortic coarctation.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) represents one of the most common causes of myocardial ischemia in infants and if left untreated results in a high mortality rate. When ALCAPA coexists with other congenital malformations, particularly those associated with pulmonary hypertension, the initial presentation can be quite confusing and is often misinterpreted. We report an infant with ALCAPA associated with scimitar syndrome and aortic coarctation whose clinical course illustrates the complexities and difficulties of management with a successful outcome.

Proteinuria in Frasier syndrome.

Introduction: Frasier syndrome (FS) is a genetic form of glomerulopathy, which results from mutations in the Wilms'tumour suppressor gene (WT1). Proteinuria in FS has been traditionally considered unresponsive to any medication and FS inevitably progresses to end stage renal failure.

Case Outline: We present a patient with FS who had atypical clinical manifestation and unusual beneficial antiproteinuric response to renin-angiotensin system (RAS) inhibitors given in combination with indomethacin.

The role of modern imaging techniques in the diagnosis of malposition of the branch pulmonary arteries and possible association with microdeletion 22q11.2.

Malposition of the branch pulmonary arteries is a rare malformation with two forms. In the typical form, pulmonary arteries cross each other as they proceed to their respective lungs. The “lesser form” is characterised by the left pulmonary artery ostium lying directly superior to the ostium of the right pulmonary artery, without crossing of the branch pulmonary arteries.

4q34.1-q35.2 deletion in a boy with phenotype resembling 22q11.2 deletion syndrome.

Unlabelled: Small terminal or interstitial deletions involving bands 4q34 and 4q35 have been described in several patients with a relatively mild phenotype such as mild to moderate intellectual disability and minor dysmorphic features. We present a boy born from unrelated parents with a de novo 4q34.1-q35.

Vomiting as the initial clinical presentation of myocardial infarction in children with anomalous left coronary artery from the pulmonary trunk.

Anomalous origin of the left coronary artery from the pulmonary trunk is a rare condition. The clinical presentation is usually nonspecific and varies from completely asymptomatic form to sudden cardiac death. We report a two-month-old infant with vomiting as a presenting symptom of anomalous origin of the left coronary artery from the pulmonary trunk.

[Early rehabilitation and cardiorespiratory response in children after correction of congenital heart defects].

Introduction: Significant improvement in survival and quality of life in patients after the correction of congenital heart defects was enabled by the introduction of surgical procedures. Due to increased vulnerability in the period after defect correction, early rehabilitation have found its place in recovery. Various studies suggest good effects of a rehabilitation programme especially in the early rehabilitation period.

Ruptured hydatid cyst of the interventricular septum with acute embolic pulmonary artery complications.

Hydatid cysts located in the interventricular septum are especially rare but with the high risk for intracavitary rupture. We report a patient with acute pulmonary embolism caused by an isolated, ruptured hydatid cyst on the right side of the interventricular septum. Early diagnosis was made by echocardiography and positive serology.

[Surgery for coarctation of the aorta in infants younger than three months].

Introduction: Recurrent coarctation is a complication which is seen at a consistent rate following repair for coarctation of the aorta in young infants.

Objective: This retrospective analysis was carried out to compare the results between resection with end-to-end anastomosis (ETE), and resection with extended end-to-end anastomosis (E-ETE), in this age group during late follow-up period. The role of ductus arteriosus is not clearly defined and the second objective of this study was to analyze intimal thickening in aortic coarctation.

[Results of treatment of cyanotic heart defects in children].

Cyanotic heart diseases are relatively rare, but they are severe and heterogeneous congenital heart diseases, which require complex surgery. Development of different advanced surgical procedures, such as arterial switch operation (ASO), Fontan and its modifications, Norwood etc. operations, as well as better perioperative care significantly improved survival rate and quality of life of these children.

[Echocardiographic evaluation of left ventricular size and function in children with arterial hypertension on hemodialysis].

In order to evaluate the effects of hypertension on left ventricular (LV) anatomy and function, we investigated children on long-term hemodialysis using two-dimensional and M-mode echocardiography. The following echocardiographic estimations were made: indexed left atrial diameter (LAi) LV and diastolic diameter (LVDDi), inter ventricular septum (IVSi) and I.V posterior wall (PWi), with calculations of the LV mass index (massi), ejection fraction (EF), fractional shortening (FS), the velocity of circumferential fiber shortening (VCF) and wall stress (WS).

[Continuous blood pressure monitoring over a 24-hour period in children with terminal renal failure treated with hemodialysis].

Recent evidence suggests that circadian blood pressure changes are common in patients with impaired renal function and has excellent correlation with end-organ damage. The aim of this paper was to: 1) evaluate if children with end-stage renal failure have altered circadian blood pressure rhythm; 2) assess whether pre- or postdialytic blood pressure is representative for the average interdialytic blood pressure; 3) assess whether pre- or postdialytic blood pressure is representative for the average interdialytic blood pressure; 3) determine influence of pre-, post and interdialytic blood pressure. Ambulatory blood pressure monitoring was performed in two groups of patients: group A-13 children with end-stage renal failure, aged 15.

[The effect of hemodialysis on endothelin-1 plasma levels in children with terminal renal insufficiency].

We analysed the effect of hemodialysis (HD) on endothelin (ET) plasma level in children with terminal renal failure. Twenty-five patients (pts) and 27 healthy children as controls were included in the study. There was no difference in ET plasma levels in pts before HD (1.