Chronic mild to moderate mitral regurgitation will not have an impact on left atrial strain parameters in the pediatric population.

BACKGROUND: Left atrial strain (LAS) analysis represents a newer non-invasive, sensitive and specific technique for assessing left atrial (LA) function and early detection of its deformation and dysfunction. However, its applicability in mitral regurgitation (MR) in pediatric population remains unexplored, raising pertinent questions regarding its potential role in evaluating the severity and progression of the disease. OBJECTIVE: To investigate the impact of chronic MR in children and adolescents on LA remodeling and function. METHODS: The study included 100 participants. Patients with primary and secondary chronic MR lasting at least 5 years fit our inclusion criteria. The exclusion criteria from the study were: patients with functional mitral regurgitation due to primary cardiomyopathies, patients with artificial mitral valve, patients with MR who had previously undergone surgery due to obstructive lesions of the left heart (aortic stenosis, coarctation of the aorta), patients with significant atrial rhythm disorders (atrial fibrillation, atrial flutter). The echocardiographic recordings were conducted by two different cardiologists. Outcome data was reported as mean and standard deviation (SD) or median and interquartile range (Q1-Q3). RESULTS: The study included 100 participants, of whom 50 had MR and the remaining 50 were without MR. The average age of all participants was 15.8 ± 1.2 years, with a gender distribution of 37 males and 63 females. There was a significant difference in the values of LA volume index (LAVI), which were higher in patients with MR (p= 0.0001), S/D ratio (and parameters S and D; p= 0.001, p= 0.0001, p= 0.013), mitral annulus radius (p= 0.0001), E/A ratio (p= 0.0001), as well as septal e' (m/s), lateral e' (m/s), and average E/e' ratio, along with the values of TV peak gradient and LV global longitudinal strain (%). There was no significant difference in LA strain parameters, nor in LA stiffness index (LASI). CONCLUSION: Our findings revealed significant differences in several echocardiographic parameters in pediatric patients with MR relative to those without MR, providing insight into the multifaceted cardiac structural and functional effects of MR in this vulnerable population.

Left atrial stiffness is in correlation with left atrial reservoir strain in pediatric patients with mitral regurgitation.

BACKGROUND: Left atrial stiffness index (LASI), defined as the ratio of early diastolic transmitral flow velocity/lateral mitral annulus myocardial velocity (E/e') to peak atrial strain, reflects reduced left atrial (LA) compliance and represents an emerging marker that can be used for noninvasive measurement of fibrosis of LA in patients with mitral regurgitation (MR). OBJECTIVE: To investigate the impact of chronic MR in children and adolescents on the remodeling and function of the LA, quantified through strain parameters and diastolic function. METHODS: The study included fifty patients (n= 50) diagnosed with primary and secondary chronic MR lasting at least 5 years. The echocardiographic recordings were performed by a third party, two cardiologists actively engaged in echocardiography on a daily basis. RESULTS: Older participants had higher values of the LASI (r= 0.467, p= 0.001). Participants with higher LASI values had a smaller LA reservoir (r= 0.784, p= 0.0001) and smaller LA conduit values (r=-0.374, p= 0.00). Participants with higher LASI values had a larger LA diameter (r= 0.444, p-value= 0.001) and higher average E/e' ratio (r= 0.718, p= 0.0001). There was a significant difference (p= 0.04) in the LASI among participants based on the MR jet area (< 20.85 cm2/⩾ 20.85 cm2), LASI was higher in participants with an area greater than 20.85 cm2. Differences in other parameters such as LA reservoir, LA conduit, LA contractile were not statistically significant. CONCLUSION: Increased LA stiffness is associated with diminished atrial compliance and reservoir capacity, and LASI has a potential to as an early marker for assessing disease severity and progression in pediatric MR.

Left atrial strain in fetal echocardiography - could it be introduced to everyday clinical practice?

OBJECTIVES: Prenatal cardiology is a part of preventive cardiology based on fetal echocardiography and fetal interventional cardiology, which facilitates treatment of congenital heart defects (CHD) in pediatric patients and consequently in adults. Timely prenatal detection of CHD plays a pivotal role in facilitating the appropriate referral of pregnant women to facilities equipped to provide thorough perinatal care within the framework of a well-structured healthcare system. The aim of this paper is to highlight the role of left atrial strain (LAS) in prenatal evaluation of fetal heart and prediction of structural and functional disorders. METHODS: We conducted a comprehensive literature review searching PubMed for articles published from inception up until August 2023, including the search terms "left atrial strain", "fetal echocardiography", and "prenatal cardiology" combined through Boolean operators. In addition, references lists of identified articles were further reviewed for inclusion. RESULTS: Our review underscores the significance of LAS parameters in fetal echocardiography as a screening tool during specific gestational windows (starting from 11 to 14 weeks of gestation, followed by better visualization between 18 and 22 weeks of gestation). The left atrial strain technique and its parameters serve as valuable indicators, not only for identifying cardiac complications but also for predicting and guiding therapeutic interventions in cases of both cardiac and noncardiac pregnancy complications in fetuses. Evidence suggests establishment of second-trimester reference strain and strain rate values by speckle-tracking echocardiography in the healthy fetal cohort is essential for the evaluation of myocardial pathologies during pregnancy. CONCLUSIONS: Finding of LAS of fetal heart is feasible and probably can have potential for clinical and prognostic implications.

Left atrial strain analysis in the realm of pediatric cardiology: Advantages and implications.

BACKGROUND: Left atrial (LA) strain analysis has emerged as a noninvasive technique for assessing LA function and early detection of myocardial deformation. Recently, its application has also shown promise in the pediatric population, spanning diverse cardiac conditions that demand accurate and sensitive diagnostic measures. OBJECTIVE: This research endeavors to explore the role of LA strain parameters and contribute to the growing body of knowledge in pediatric cardiology, paving the way for more effective and tailored approaches to patient care. METHODS: A comprehensive literature review was conducted to gather evidence from studies using echocardiographic strain imaging techniques across pediatric populations. RESULTS: LA strain parameters exhibited greater sensitivity than conventional atrial function indicators, with early detection of diastolic dysfunction and LA remodeling in pediatric cardiomyopathy, children with multisystem inflammatory syndrome, rheumatic heart disease, as well as childhood renal insufficiency and obesity offering prognostic relevance as potential markers in these pediatric subpopulations. However, there remains a paucity of evidence concerning pediatric mitral valve pathology, justifying further exploration. CONCLUSION: LA strain analysis carries crucial clinical and prognostic implications in pediatric cardiac conditions, with reliable accuracy and sensitivity to early functional changes.

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. The following risk factors were analysed: aortic valve insufficiency after balloon valvuloplasty >+1/4, post-procedural gradient across the aortic valve ≥35 mmHg, pre-interventional gradient across the valve, annulus size, use of rapid pacing, and balloon/annulus ratio. Primary outcome was aortic valve reintervention. RESULTS: In total, 99 patients (median age 4 years, range 1 day to 26 years) underwent balloon valvuloplasty for congenital aortic valve stenosis. After a mean follow-up of 4.0 years, 30% had reintervention. Adjusted risks for reintervention were significantly increased in patients with post-procedural aortic insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg (HR 2.55, 95% CI 1.13-5.75, p = 0.024). Pre-interventional gradient, annulus size, rapid pacing, and balloon/annulus ratio were not associated with outcome. CONCLUSION: Post-procedural aortic valve insufficiency grade >+1/4 and/or residual gradient ≥35 mmHg in patients undergoing balloon valvuloplasty for congenital aortic valve stenosis confers an increased risk for reintervention in mid-term follow-up.

Protein-losing enteropathy managed with percutaneous enlargement of a restrictive atrial septal defect.

Protein-losing enteropathy is one of the most feared complications of the Fontan circulation. The diagnosis of protein-losing enteropathy in this setting should prompt a thorough investigation for the presence of a treatable hemodynamic impairment. In this report, we describe a complete reversal of protein-losing enteropathy following percutaneous enlargement of a restrictive atrial septal defect in a patient with a fenestrated lateral tunnel Fontan and severe mitral stenosis.

Glucose and lipid metabolism disorders in children and adolescents with spinal muscular atrophy types 2 and 3.

We aimed to estimate the prevalence of glucose and lipid metabolism disorders in children and adolescents with spinal muscular atrophy (SMA) types 2 and 3. A cross-sectional study was conducted. Medical history, anthropometric measurements, pubertal status, blood chemistry (glucose and insulin levels, lipid profile, aminotransferases, and hemoglobin A1c [HbA1c]), and liver ultrasound were obtained in all patients. Oral glucose tolerance test was performed in those with body mass index (BMI) >25th percentile or glucose or HbA1c levels in the prediabetic range. A total of 37 patients with SMA (22 type 2, 15 type 3) with a median age of 8.5 years (range 2-18.9 years) were included. Eleven patients (29.7%) met the criteria for prediabetes, but none had overt type 2 diabetes. Dyslipidemia was detected in 11 patients (29.7%), and 4 (10.8%) had hepatic steatosis on ultrasound. Sixteen patients (43.2%) had at least one abnormal finding (prediabetes, dyslipidemia, or hepatic steatosis); all but one were non-ambulatory and 12 (75%) had BMI ≥85th percentile. One young child developed fasting hypoglycemia. Our results suggest that non-ambulatory overweight/obese SMA patients are particularly prone to abnormalities in glucose and lipid metabolism. Young underweight patients might develop fasting hypoglycemia.

Cardiac findings in pediatric patients with spinal muscular atrophy types 2 and 3.

BACKGROUND: It is unclear whether the heart is affected in pediatric patients with milder forms of spinal muscular atrophy (SMA). Therefore, we aimed to determine the presence of any cardiac abnormalities in these patients. METHODS: We conducted a cross-sectional study of children and adolescents with SMA types 2 and 3 between July 2018 and July 2019. All patients underwent a comprehensive cardiac evaluation, including history-taking, physical examination, electrocardiography, echocardiography, measurement of cardiac biomarkers (cardiac troponin T [cTnT] and N-terminal pro-brain natriuretic peptide [NT-proBNP]), and 24-hour Holter monitoring. RESULTS: In total, 42 patients were enrolled (27 and 15 with SMA type 2 and 3, respectively). No patient had structural heart disease, except for one with mitral valve prolapse. None had signs of ventricular dysfunction on echocardiography. Both cTnT and NT-proBNP levels were normal in all patients. Electrocardiography showed sinus tachycardia in seven patients (16.7%), and prolonged P-R interval in one (2.4%). Holter monitoring detected benign ventricular arrhythmias in two patients (4.8%), and rare supraventricular premature beats in one. The mean 24-hour heart rate was elevated in six patients (14.3%), whereas both the minimum 24-hour heart rate and the maximum R-R interval were increased in 23 (54.8%). DISCUSSION: The prevalence of cardiac disease in pediatric patients with SMA types 2 and 3 is low; however, these patients may have increased resting heart rates. A complete cardiac history and physical examination are a useful screen. Additional cardiac investigations may be performed as needed.

Isolated nocturnal and isolated daytime hypertension associate with altered cardiovascular morphology and function in children with chronic kidney disease: findings from the Cardiovascular Comorbidity in Children with Chronic Kidney Disease study.

INTRODUCTION: Prevalence of isolated nocturnal hypertension (INH) and isolated daytime hypertension (IDH) is around 10% in adults. Data in children, especially in chronic kidney disease (CKD), are lacking. The aim of this cross-sectional multicenter cohort study was to define the prevalence of INH and IDH and its association with cardiovascular morphology and function, that is, pulse wave velocity (PWV), carotid intima-media thickness (cIMT), or left ventricular mass index (LVMI) in children with CKD. METHODS: Ambulatory blood pressure (BP) monitoring profiles were analyzed in 456 children with CKD stages III-V participating in the Cardiovascular Comorbidity in Children with Chronic Kidney Disease Study (64.3% males, 71.3% congenital anomaly of the kidney and urinary tract, age 12.5 ±â€Š3.2 years, estimated glomerular filtration rate 29 ±â€Š12 ml/min per 1.73 m). Baseline PWV, cIMT, and LVMI were compared in normotension, INH, IDH, or sustained 24-h hypertension. RESULTS: Prevalence of sustained hypertension was 18.4%, of INH 13.4%, and of IDH 3.7%. PWV SDS (SD score) and cIMT SDS were significantly higher in sustained hypertension and INH, and PWV SDS was significantly higher in IDH, compared with normotension. LVMI was significantly increased in sustained hypertension, but not in INH or IDH. Determinants of INH were smallness for gestational age, older age, higher height SDS and parathyroid hormone, and shorter duration of CKD. In logistic regression analysis, day/night-time hypertension or ambulatory BP monitoring pattern (normal, INH, IDH, sustained hypertension) were independently associated with cardiovascular outcome measures: elevated night-time BP was associated with increased cIMT, PWV, and left ventricular hypertrophy; INH was associated with cIMT. CONCLUSION: INH is present in almost one out of seven children with predialysis CKD; INH and nocturnal hypertension in general are associated with alterations of arterial morphology and function.

Symptomatic myocardial bridging: a frequently occurring coronary variation can cause severe myocardial ischaemia in affected children with underlying cardiac conditions.

Myocardial bridging is a congenital coronary artery anomaly in which the coronary artery has a partly "tunnelled" intramyocardial course. This tunnelling leads to compression of the affected vessel segment during ventricular systole. It is considered to be a benign variation of the norm in about 25% of the population caused by an aberrancy of embryologic coronary development. The bridging is also thought to cause severe cardiac conditions in a few of those affected. The series of six young patients presented here is the largest series so far to report on symptomatic myocardial bridging in children with different underlying heart diseases. All patients recently presented to our centre with signs of myocardial ischaemia. They subsequently underwent coronary angiography, which revealed myocardial bridging of the ramus interventricularis anterior. In all patients, therapy with ß blockers was started to reduce heart rate and myocardial contractility. ß Blocker treatment was also given in order to prolong diastole and improve coronary artery blood flow. Two patients underwent surgical exposure of the involved coronary segment: a 2-year-old boy because of recurrent, severe myocardial ischaemia in combination with a reduction of general health, changes in ST-segments, and the presence of a dilative cardiomyopathy; and a 13-year-old girl because of evidence of myocardial ischaemia during exercise testing after surviving sudden cardiac death. Surgery was successful and recovery was complete and uneventful. The presented series shows that myocardial bridging can be symptomatic and may require urgent treatment and even surgical intervention in early childhood in rare cases.

Transcatheter closure of a residual aorto-left ventricular tunnel: report of a case with a 6-year follow-up.

Aorto-left ventricular tunnel is an exceedingly rare congenital cardiac defect. Early surgical closure is the treatment of choice. Residual or recurrent tunnel and aortic valve insufficiency are well-recognised complications after surgical repair. In this article, we report on successful transcatheter closure of a residual aorto-left ventricular tunnel using an Amplatzer duct occluder in a 7-year-old boy. The outcome after 6 years of follow-up is encouraging.

Improving the Diagnosis of Children with 22q11.2 Deletion Syndrome: A Single-center Experience from Serbia.

OBJECTIVE: The incidence of the 22q11.2 microdeletion among children who have at least two out of five major clinical criteria for 22q11.2 deletion syndrome. DESIGN: Prospective study. SETTING: University Childrens Hospital in Belgrade, Serbia between 2005 and 2014. PARTICIPANTS: 57 patients with clinical characteristics of 22q11.2 deletion syndrome. METHODS: Standard G-banding cytogenetic analysis was performed in all children, and the 22q11.2 genomic region was examined using fluorescence in situ hybridization (FISH). For patients with no deletion detected by FISH, multiplex ligation-dependent probe amplification (MLPA) analysis was also done in order to detect cryptic deletions of this region and to analyze other genomic loci associated with phenotypes resembling the syndrome. A selected group of patients diagnosed to have 22q11.2 microdeletion by FISH underwent MLPA testing in order to characterize the size and position of deletion. OUTCOME MEASURES: The frequency of 22q11.2 microdeletion among children with at least two of the five major characteristics of 22q11.2 deletion syndrome (heart malformations, facial dysmorphism, T-cell immunodeficiency, palatal clefts and hypocalcemia/hypoparathyroidism). RESULTS: Typical 22q11.2 microdeletion was detected in 42.1% of patients; heart malformation were identified in all of them, facial dysmorphism in 79.2%, immunological problems in 63.6%, hypocalcemia in 62.5% and cleft palate in 8.3%. CONCLUSION: A higher detection rate compared to one-feature criterion is obtained when at least two major features of 22q11.2 deletion syndrome are taking into consideration. The criteria applied in this study could be considered by centers in low-income countries.

Multiple Major and Minor Anomalies Associated With Klippel-Feil Syndrome: A Case Report.

Klippel-Feil syndrome is defined as congenital fusion of two or more cervical vertebrae. In this article, we report a 55-year-old male patient with one-year history of neck pain, headaches, and one episode of syncope after a severe trauma. X-rays and magnetic resonance imaging of cervical spine revealed fused vertebral bodies of C2-C5. The major anomalies associated with Klippel-Feil syndrome (small stature, thoracic kyphoscoliosis, lumbar scoliosis, restricted opening mouth, and bilateral sensorineural hearing loss) as well as multiple minor anomalies (mild face asymmetry, high arched palate, rhinoscoliosis, high nasal bridge, inclined septi nasi, and thin upper lip) were detected. This is a rare case describing the anomalies of the nose in Klippel-Feil syndrome patients. Our patient had no central cord impairment following a severe trauma.

[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). Measurements of the proximal transverse arch (PTA), distal transverse arch (DTA), isthmus, distance between the left common carotid artery (LCCA) at the origin of the left subclavian artery (LSA), were obtained by two-dimensional echocardiography. Aortic arch hypoplasia was defined using Mouleart, Karl and Mee criteria, and Z-value. Index 1 was calculated as a ratio of DTA and distance between origins LCCA-LSA, Index 2 was calculated as a ratio of the ascending aorta and the distance between LCCA-LSA origins, and Index 3 was calculated as a ratio of PTA and distance between LCCA-LSA origins. RESULTS: Index 1 was significantly lower in patients with CoA in comparison with control group (0.50 vs. 1.39; p≤0.01). A cut-off point at 0.39, for Index 1, showed a sensitivity of 92% and specificity of 99% for the diagnosis of neonatal CoA, while cut off points at 0.69 and 0.44, for Index 2 and Index 3, showed the highest sensitivity and specificity for the diagnosis of CoA in newborns. CONCLUSION: By using these echo indexes, two-dimensional echocardiographic aortic arch measurement becomes a simple, reliable noninvasive method for the evaluation of aortic coarctation in newborns and may lead to earlier diagnosis and subsequent surgical correction.

Doppler parameters of the maternal hepatic artery blood flow in normal pregnancy: maternal hepatic artery blood flow in normal pregnancy.

OBJECTIVE: Objective of our study was to evaluate changes in Doppler resistance indices in the common hepatic artery during normal pregnancy. STUDY DESIGN: Cross-sectional study included 210 healthy pregnant women gestational age 6-40 weeks, 40 healthy non-pregnant women and 30 women after delivery. We divided all pregnant women by pregnancy trimester. We registered pulsatility index (PI) and resistive index (RI) in the common hepatic artery and compared the evaluated values among non-pregnant women and women in first, second and third trimester and post partum and tested correlation of both parameters with gestational age. Statistical analysis was done by Chi square test, one-way ANOVA followed by post-hoc test and two-tailed Pearson and Spearman correlation. The difference was considered to be significant if p<0.05. RESULTS: We found lower values of PI and RI in the third trimester compared to control group and first and second trimester (p<0.01). There is negative correlation between the values of PI and RI with the gestation (p<0.01). CONCLUSION: Hepatic artery resistance indices decrease during the third trimester of pregnancy. This decrease may be the result of systemic arterial vasodilatation in normal pregnancy. The arterial resistance indices may be more useful for the evaluation of liver blood flow over the total blood flow as they are more reliable, being angle independent, easier to obtain, reflect vascular changes and might help in quick orientation about liver blood flow in pregnancies complicated by preeclampsia and HELLP syndrome. Our study is a pilot one, and further studies are needed to establish nomograms for the PI and RI during the gestation.

[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. METHODS: This study included 57 children operated from 1st January 2005 until 31st December 2009. Parameters that could influence the outcome of surgery were investigated. The following late complications were investigated: neopulmonary artery stenosis, neoaortic stenosis and regurgitation, as well as clinical signs of heart failure. RESULTS: Early postoperative mortality was 15.8% (9/57 patients). During follow-up (8 to 72 months, average 36.5 months) there were no lethal outcomes. On the last echocardiography examination, 73.2% patients had neoaortic regurgitation and 67.4% patients had neopulmonary regurgitation, but all of them were mild in intensity. Neopulmonary stenosis had 32.6% of patients, but only two had moderate or severe stenosis. No one had ischemic ECG changes. Three reinterventions were performed due to serious residual problems: surgical correction of neoaortic stenosis, surgical correction of neopulmonary stenosis and transcatether balloon dilatation for aortic recoarctation. At the end of the follow-up period, only one of 46 consistently followed patients had signs of heart failure which required therapy (2.2%), while the majority of patients were without any symptoms and with good effort tolerance. CONCLUSION: Arterial switch operation has been successfully performed at our institution, with acceptable perioperative mortality and excellent late outcome.

[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. OBJECTIVE: The aim of this study was a retrospective evaluation of TAPVC repair at a single institution, identifying the risk factors associated with the increased mortality and morbidity. METHODS: Between January 2001 and January 2010, 43 consecutive patients underwent repair of TAPVC at the University Children's Hospital, with median weight of 3.8 kg (1.8-13 kg). Median age at surgery varied from 5 days to 5 years. Distribution of TAPVC types was as follows: supracardiac 19 (44%), cardiac 12(28%), infracardiac 9 (21%), and mixed 3 (7%). Eleven patients (26%) were emergencies due to obstructed drainage. RESULTS: Early mortality was 9.30% (4/43). An average time of followup/survival for 95% interval of confidence was 101.6 +/- 6.7 months. Kaplan-Meier cumulative survival was 83.7 +/- 5.7%. Freedom from reintervention after 10 years was 87.2 +/- 0.5%. The principal reason for reintervention was RPVO. CONCLUSION: Preoperative obstruction is not a risk factor of early mortality and RPVO. Low body mass (below 2.5 kg) is the only identified risk factor of early mortality. Complex morphology of the confluens, particularly in a mixed type of TAPVR, is the main risk factor of RPVO development.

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. METHODS: From 2004 to 2011, 50 patients, aged 18 days to 18 years (mean 6.3 years) underwent BVP. Retrospective analysis of the echocardiographic and hemodynamic parameters were performed before and after procedure, especially peak pressure gradient (PG) across the aortic valve, semiquantification of the aortic regurgitation (AR) after the BVP as well as the left ventricle dimensions and functions. RESULTS: The mean peak PG in the whole group decreased from 74.80 +/- 27.72 mm Hg to 27.86 +/- 3.04 mm Hg (p < 0.001) after BVP. In 39 patients (78%), residual PG was lower than 30 mm Hg just after dilation. At the end of follow-up period, 25 patients (50%) had PG above 50 mm Hg, measured by Doppler technique, and four of them underwent re-dilation. Eight patients (16%) had severe AR. During the follow-up period (12-80 months, mean 51 months), six patients (12%) were referred to cardiac surgeons for aortic valve replacement or Ross procedure. CONCLUSIONS: This retrospective study analyzes our first experience of BVP as primary therapy of the congenital aortic stenosis. The results confirmed that BVP effectively postponed the need for surgery in children and adolescents toward the adulthood.