[B-type natriuretic peptide as a marker of cardiac dysfunction in children with chronic kidney disease]. / Peptyd natriuretyczny typu B jako marker uszkodzenia serca u dzieci z przewlekla choroba nerek.

Left ventricular hypertrophy is the most common organ damage in children with chronic kidney disease (CKD). AIM: The aim of the study was to assess the usefulness of B-type natriuretic peptide (BNP) as a marker of heart injury in children with CKD. MATERIALS AND METHODS: We included 66 children (41 boys and 25 girls) aged 0.7 to 18.6 (median 11.6) years with CKD stage 1-5. The concentrations of urea, creatinine, cystatin C and BNP in blood serum were assessed, and the estimated glomerular filtration rate (eGFR) was calculated from the Schwartz and Filler formulas. Patients were divided into groups depending on the CKD stage [group 1: CKD stages 1 + 2 (GFR> 60 ml/min/1.73 m2), group 2: stage 3 (GFR = 30-59 ml/min/1.73 m2), group 3: CKD stage 4 (GFR 15-29 ml/min/ 1.73 m2), group 4 - stage 5 (dialyzed children)]. On the basis of echocardiography, the left ventricular mass (LVM) was calculated, which was indexed for height (left ventricular mass index, LVMI). Left ventricular hypertrophy (LVH) was diagnosed if the LVMI value was > 95th percentile for sex and age. RESULTS: Depending on the CKD stage the median BNP concentrations for group 1, group 2, group 3, and group 4 were 2.5 pg/ml, 6.0 pg/ml, 9.3 pg/ml and 18.0 pg/ml, and the LVH prevalence 27.3%, 33.3%, 60.0% and 63.6% , respectively. Significant correlations between BNP concentration and LVH expressed by LVMI (R=0.256, p=0.038), creatinine (R=0.453, p<0.001), cystatin (R=0.494, p<0.001) and eGFR (R=-0.473, p<0.001) were found. CONCLUSIONS: In children with chronic kidney disease, BNP is an indicator of heart failure correlating with renal function parameters and left ventricular mass index.

[Therapeutic results in children with hypoplastic left heart syndrome after Norwood procedure]. / Wyniki leczenia dzieci z zespolem niedorozwoju lewego serca po operacji metoda Norwooda.

UNLABELLED: Hypoplastic left heart syndrome (HLHS) encompasses hypoplasia or atresia of the left ventricle, stenotic or artretic aortic and mitral valves and hypoplasia of the ascending aorta. The aim of the 1-st stage operation called the modified Norwood procedure is reconstruction of the systemic flow, providing an adequate pulmonary flow and relieving interatrial restriction. The aim of the study was the analysis of treatment results after the Norwood operation in children hospitalized at Cardiology and Cardiac Surgery Departments in the period of 2009-2012. The material consisted of 65 children (42 males, 23 females). The following factors were analyzed: the age on admission, general condition, pre-operative clinical course. RESULTS: The mean age of children on admission was 4.4 ± 4.1 days and mean body weight--3.3 ± 0.5 kg. The general condition was good in the majority of patients (74%). The remaining children were found to have early symptoms of congestive heart failure (CHF). Cardiological and/or surgical interventions were necessary in 33 (50.8%) patients, among them three children required both procedures. In the majority of patients (n = 21, 32%), the main cause of intervention was re-coarctation of the neo-aortic isth- mus treated with balloon plasty and/ or stenting. In the early post-operative period, six children died due to CHF (n = 4) and infections (n = 2), while the remaining six children died due to various complications while waiting for the 2nd stage. CONCLUSIONS: Despite the progress in diagnosis and treatment of children with HLHS the mortality is still high.

Coronary artery abnormalities in Kawasaki disease.

INTRODUCTION: Kawasaki disease is the number one cause of acquired heart disease among children in developed countries. AIM: The aim of the study was a retrospective analysis of the factors that may influence the persistence of coronary artery abnormalities in patients with Kawasaki disease. MATERIALS AND METHODS: Analyzing the medical records of patients hospitalized in the University Children's Hospital of Krakow in the years 2005-2011 we collected the data of 28 patients diagnosed with Kawasaki disease. The group was divided into two subgroups, depending on the duration of the persistence of changes in the coronary arteries - A (n = 17) for up to 6 months, B (n = 11) - for more than 6 months. Both groups were analyzed for the presence of factors that may influence the course of the disease. RESULTS: There were more boys in group A (11 boys (65%), 6 girls (35%)), whereas in group B the distribution was more uniform (6 boys (55%), 5 girls (45%)). The age of onset in group A was 37.9 months (SD 30.8), in group B 39.5 months (SD 16.7). 17.6% of patients in group A and 36.4% in group B were treated with glucocorticoids. CONCLUSIONS: In the group of patients in which coronary artery abnormalities disappeared more quickly, male and slightly older children dominated. The only difference observed between the 2 groups related to the frequency of the use of glucocorticoids, they were used more often in children, in whom coronary artery abnormalities persisted longer.

Hypoplastic left heart syndrome: from the prenatal to the postnatal period.

OBJECTIVES: To analyse a population of foetuses with prenatally diagnosed hypoplastic left heart syndrome (HLHS). MATERIAL AND METHODS: Retrospective study of foetuses diagnosed with HLHS between 2013 and 2017 in a referral centre. RESULTS: HLHS was found in 9.7% (65/665) of foetuses with cardiovascular abnormalities (CVA). As an isolated anomaly, HLHS was present in 40% of cases; in 24.5% other CVA were detected; in 14%, CVA and extracardiac anomalies; and in 21.5% only extracardiac malformations. Genetic disorders were present in 18.4% (12/65) of foetuses. 42% of cardiovascular and 25% of extracardiac anomalies were diagnosed postnatally. There were 10 (15.4%) elective terminations, 1 (1.5%) spontaneous foetal demise. Two newborns died after birth before surgery. Of the 52 children who underwent Norwood surgery, 13 (25%) died (9 with additional anomalies, and 4 with isolated HLHS). Of the 38 children who underwent stage II surgery, 2 (5.2%) with isolated HLHS died, and 1 (2.6%) with CVA. CONCLUSIONS: A diagnosis of HLHS is an indication for a detailed examination of cardiac and noncardiac structures. It is advisable to consider genetic testing, together with the microarray assessment. The prognosis depends on underlying cardiac and extracardiac anomalies and coexisting genetic defects.

The Impact of COVID-19 Pandemic on Children with Pulmonary Arterial Hypertension. Parental Anxiety and Attitudes. Follow-Up Data from the Polish Registry of Pulmonary Hypertension (BNP-PL).

The COVID-19 pandemic has impacted healthcare systems worldwide. Little is known about the impact of the pandemic on medical and psycho-social aspects of children with rare diseases such as pulmonary arterial hypertension and their parents. The study is based on children registered in The Database of Pulmonary Hypertension in the Polish Population and a parent-reported survey deployed during the first 6 months of the pandemic. The questionnaire consisted of six question panels: demographic data, fear of COVID-19, General Anxiety Disorder-7 (GAD-7), social impact of pandemic, patients' medical status, and alarming symptoms (appearance or exacerbation). Out of 80 children registered, we collected 58 responses (72.5% response rate). Responders (parents) were mostly female (n = 55; 94.8%) at a mean age of 40.6 ± 6.9 years. Patients (children) were both females (n = 32; 55%) and males with a mean age of 10.0 ± 5.1 years. Eleven (19%) children had symptoms of potential disease exacerbation. Eight parents (72.7%) decided for watchful waiting while others contacted their GPs or cardiologists (n = 6; 54.5%). Three children had to be hospitalized (27.3%). Most planned hospitalizations (27/48; 56.2%) and out-patient visits (20/35; 57.1%) were cancelled, delayed, or substituted by telehealth services. Among the participating parents, the study shows very high levels of anxiety (n = 20; 34.5%) and concern (n = 55; 94.8%) and the need for detailed information (52; 89.6%) regarding COVID-19 and medical service preparedness during the pandemic. The COVID-19 pandemic has influenced child healthcare and caused high levels of anxiety among parents.

Amiodarone-induced thyroid dysfunction in the developmental period: prenatally, in childhood, and adolescence - case reports and a review of the literature.

INTRODUCTION: Amiodarone is an important antiarrhythmic drug used in paediatric practice, mainly in children with complex congenital cardiac diseases and/or severe arrhythmias. One of the side effects of amiodarone therapy is thyroid dysfunction, which is observed in about 20% of patients. The thyroid dysfunction may present with various forms: from subclinical changes in hormone levels to amiodaroneinduced thyrotoxicosis (AIT) and amiodarone-induced hypothyroidism (AIH). MATERIAL AND METHODS: We reported six patients in the age range from two weeks to 14 years, with complex congenital cardiac diseases and severe arrhythmias, who developed amiodarone-induced thyroid dysfunctions: thyrotoxicosis or hypothyroidism or both together. The clinical signs and symptoms of all thyroid dysfunctions were atypical, most patients presented with an aggravation of heart insufficiency. Our patients with thyrotoxicosis were treated with combined therapy including thionamides and corticosteroids due to the presentation of mixed-identified type of AIT. RESULTS: Currently, five patients (one patient's status is unknown) are in biochemical and clinical euthyreosis; however, in one of them it was impossible to discharge amiodarone treatment. Three of them are still treated with levothyroxine, and two do not need thyroid treatment. CONCLUSIONS: Amiodarone-induced thyroid dysfunction is usually atypical; therefore, monitoring of thyroid status before, during, and after amiodarone is demanded. AIH could significantly influence the development of the child, while AIT could significantly deteriorate the clinical status of children with complex cardiac diseases. Early and proper diagnose of AIT and AIH allows the introduction of immediate and appropriate treatment considering the cardiac condition of the young patient.

[The assessment of heart function in children with chronic kidney disease (CKD)]. / Ocena funkcji serca u dzieci z przewlekla choroba nerek (PChN).

THE AIM OF THE STUDY: The echocardiography evaluation of cardiac function in children with CKD. PATIENTS AND METHODS: 30 children (17 males, 13 females), aged 2-20 yrs with CKD 2-5. Left ventricular (LV) dimensions, LV ejection fraction (EF) and LV mass index (LVMI), as well mitral inflow velocity (E/A waves) and isovolumetric relaxation time (IRT) were evaluated. RESULTS: Impaired diastolic heart function were ascertained in 12 patients (in 2 from 5 with CKD 3, in 3 from 10 with 4 and 7 from 14 with 5). Left ventricular hypertrophy was observed in 13 children with CDD 3-5 (4, 4, 5), and decreased ejection fraction in 2. CONCLUSIONS: The vast majority of children with chronic kidney disease demonstrate an impairment of diastolic cardiac function.

[Cardiac syncope in children]. / Omdlenia kardiogenne u dzieci.

INTRODUCTION: Syncope are serious problems in paediatrics. Approximately 15% of children and adolescents at the age of 8-18 years experience at least one episode of syncope. Neurocardiogenic syncope are the most common (60-80%). Cardiogenic syncope are rare (6%), although they require special attention due to their background. AIM OF THE STUDY: Evaluation of incidence, causes, methods of treatment and prognosis in cardiogenic syncope in children and adolescents. MATERIAL AND METHODS: Retrospective analysis was made in 18 patients with syncope and different pathology of cardiovascular system. Patients were chosen among 766 children treated in the University Hospital for Children in Cracow from January 2005 to June 2006 due to syncope or pre-syncope. The patients were divided into 2 groups depending on the main cause of syncope: Gr I n = 6 due to arrhythmias without structural heart disorders and Gr II n = 12 due to heart diseases or defects. In all patients physical exam and additional test were analysied carefully (ECG, Holter study, chest X ray, ECHO, angio-TK in 1 patient, and catheterisation with angiography in 3 children). RESULTS: Cardiogenic syncope appeared more frequently in children with structural abnormalities in the heart (Gr II--12 pts/ 66%), rather than in children with arrhythmias (Gr I--6 pts / 33%). Family history was positive in both groups in 17%. Median age at the moment of first episode was 10.3 y +/- 5.0 in Gr I and 12.8 y +/- 3.0 in Gr II. Syncope in Gr II were frequently (33%) initiated by an effort or stress. In Gr I physical examination did not reveal serious symptoms except for irregularity of heart rate, and general condition of children was good (NYHA I). In 5 pts (58%) in Gr II heart murmur and prominent impulse of the left ventricle were found. Symptoms of congestive heart failure were observed in 4 pts (33%) and their condition was poor (III/IV class according to NYHA). In ECG and/or Holter study in Gr I arrhythmias and/or long QTc were found. Pharmacotherapy was sufficient in treatment of serious arrhythmias and prophylaxy of syncope. In Gr II in most cases hypertrorepolarization abnormalities were found. In these children treatment was multidirectional and included both antiarrhythmic drugs, implantation of cardioverter defibrillator (ICD), operation and even heart transplantation. CONCLUSIONS: Cardiogenic syncope are rare causes of loss of consciousness and indicate on serious pathology in the cardiovascular system. Heart structural diseases or defects influence severity on clinical course, complex treatment and serious prognosis while in children with arrhythmias pharmacotherapy is effective and prognosis is usually good.

[Preliminary assessment of heart function in chronically dialysed children]. / Wstepna ocena funkcji serca u dzieci przewlekle dializowanych.

UNLABELLED: Renal replacement therapy has become a recognized treatment modality of children with chronic renal failure (CRF). Despite of unquestionable progress in heamodialysis treatment quite high morbidity and mortality still remain a serious problem among pediatric patients. The most common cause of death in haemodialyzed and transplanted patients are cardiovascular complications. The main aim of the study was an echocardiographic (ECHO) assessment of selected cardiac parameters in heamodialyzed children. METHODOLOGY: 16 chronically dialyzed (6 HD, 10 PD) children participated in the study (10 M, 6 F), aged 5-18,5 yrs (x=12.2 +/- 3.8 yrs). Echocardiography examinations were carried out with HP 5500 device and S4 ultrasound probe of variable frequency. Diastolic and systolic LV dimension, ejection fraction (EF) and LV mass index (LVMI) were evaluated. By means of pulsating Doppler method mitral flow peak E and A velocity and isovolumetric relaxation time (IRT) were assessed. RESULTS: On the basis of ECHO examinations 3 groups were singled out: A (n=3) of normal heart function, B (n=3) of impaired systolic and diastolic heart function and C (n=10) of normal systolic and impaired diastolic heart function. In group of children with severe cardiac lesion (B group) a higher LV mass (A vs B vs C: 74.7 vs 119.9 vs 73.5 g/m2) and statistically significant lower ejection fraction (68.1 vs. 33.7 vs. 65.9%) were ascertained. These children were anuric (996 vs. 0 vs. 1112 mild), their systolic (102.1 vs. 118.4 vs. 117,9) and diastolic (64,4 vs. 84.8 vs. 77.9) blood pressure were significantly higher, so was the number or hipotensive medications (0.33 vs. 1.72 vs. 1,44). CONCLUSIONS: The great majority of chronically dialyzed children demonstrates an impairment of cardiac function mainly of diastolic parameters. Anuria and hypertension stand for a significant risk factor of cardiac lesion.

Oxidative Stress Biomarkers and Left Ventricular Hypertrophy in Children with Chronic Kidney Disease.

Cardiovascular diseases remain the most frequent cause of morbidity and mortality in patients with chronic kidney disease (CKD). The aim of the study was to assess the association between oxidative stress biomarkers and cardiovascular risk factors and left ventricular hypertrophy in children with CKD. Material and Methods. The studied group consisted of 65 patients aged 1.4-18.6 (mean 11.2) years with stages 1 to 5 CKD. Serum oxidized low-density lipoprotein (oxLDL), protein carbonyl group, creatinine, cystatin C, albumin, lipids, high-sensitivity C-reactive protein, intercellular adhesion molecule-1, insulin, plasma renin activity, and aldosterone levels were measured. Patients were divided into groups depending on CKD stage. Anthropometric measurements, ambulatory blood pressure (BP) measurements, and echocardiography with left ventricular mass (LVM) calculation were performed. Results. Serum oxLDL strongly correlated with creatinine (R = 0.246; p = 0.048), cystatin C (R = 0.346; p = 0.006), total cholesterol (R = 0.500; p < 0.001), triglycerides (R = 0.524; p < 0.001), low-density lipoprotein concentrations (R = 0.456; p < 0.001), and 24 hour BP values of systolic (R = 0.492; p = 0.002), diastolic (R = 0.515; p < 0.001), and mean arterial pressure (R = 0.537; p < 0.001). A significant correlation between oxLDL levels and LVM z-scores (R = 0.299; p = 0.016) was found. Conclusions. Hypertension and dyslipidemia correlated with lipid oxidation in children with CKD. oxLDLs seem to be valuable markers of oxidative stress in CKD patients, correlating with left ventricular hypertrophy.

[The influence of increased preload on left ventricular systolic function in infants with congenital heart disease]. / Wplyw zwiekszonego obciazenia wstepnego na funkcje skurczowa lewej komory u niemowlat z wrodzonymi wadami sercabadania echokardiograficzne.

UNLABELLED: The aim of the study was to assess the systolic function of the volume overloaded left ventricle (LV) in infants with congenital heart disease. MATERIAL: Seventy five consecutive patients (36 males, 39 females) with following breakdown were examined: 14 (18.7%) newborns, 24 (32%) infants under 3 months, 16 (21.3%) between 4 and 6, and 21 (28%) infants above 6 months of age. VSD was recognized in 60 (80%), PDA--6(8%), DORV--7 (9.3%), and CT (type I) in 2 (2.7%) of the patients. METHODS: Based on ECHO studies LV end diastolic volume (LVedv), end diastolic muscle mass (LVedm), LV shortening fraction (FS), ejection fraction (EF), LV fractional area changes (FAC), and heart rate (HR) versus pulmonary blood flow (Qp), which reflected the LV preload systemic blood flow (Qs), Qp/Qs, Qp-Qs (left-to-right shunt) and indexed for body surface area (BSA) and age, were assessed by statistical analysis. RESULTS: With a gradual increase of BSA with age (cc = 0.82, p < 0.001), a significant decrease in Qp-Qs (cc = -0.27, p = 0.035) and Qp/Qs (cc = -0.31, p = 0.013) were noted. Qp for the whole group ranged from 3.3 to 21.7 l/min/m2 (m = 8.9, x = 9.7, SD = 4.1), significantly increased with the rise of Qp-Qs (cc = 0.9, p < 0.0001) and influenced the increment of Qp/Qs (cc = 0.59, p < 0.001). Qs for the whole group ranged from 2.1 to 11.7 l/min/m2 (m = 4.3, x = 4.6, SD = 1.7) correlating with the rise of Qs (cc = 0.45, p < 0.001), and being lower than 2.5 l/min/m2 in only 5 (6.7%) patients but in only 5 (6.7%) patients (all newborns). The heart rate gradually decreased with age (r = -0.26, p = 0.04), from mean 145/min in newborns to 123/min in infants over 6 months, and with BSA (cc = -0.34, p = 0.006). Heart rate also correlated well with Qp (cc = 0.31, p = 0.014). The increase of LVedv was related to age (cc = 0.37, p = 0.003), BSA (cc = 0.43, p = 0.0004) and Qp (cc = 0.33, p = 0.009). However, only in newborns was the mean LVedv within normal limits for age. LVedm only correlated with BSA (cc = 0.26, p = 0.04), except in newborns where its mean values exceeded the upper limits for age in other subgroups of patients. LV systolic function indicators (FS, EF, FAC) were diminished in only 2 cases and did not correlate either with age, BSA, nor with hemodynamic parameters. CONCLUSIONS: 1. The reactions to increased preload of the systemic ventricle in children below one year of age were different. In newborns, the adrenergic response predominated, while infants manifested increased end-diastolic volume and left ventricular muscle mass. 2. The systolic function of the overloaded left ventricle in infants was normal regardless of age, body surface area and intensity of hemodynamic disturbances.

[Echocardiographic assessment of early and midterm results of transcatheter closure of a narrow patent ductus arteriosus in children]. / Echokardiograficzna ocena wczesnych i srednio odleglych wyników interwencyjnego zamykania droznego przewodu tetniczego u dzieci.

This paper presents the echocardiographic assessment of early and midterm results of transcatheter closure of a narrow patent ductus arteriosus (PDA) in children using Jackson and Gianturco coils. The preliminary criterion for qualifying patients for the procedure was the PDA diameter (< or = 4 mm) at the narrowest site, as assessed echocardiographically. The criterion was met by 22 of 116 children with PDA treated at the Department of Pediatric Cardiology, Faculty of Medicine, Jagiellonian University, between January 1, 1996 and December 31, 2000. Angiography revealed, that in one case, the PDA diameter was larger (6.5 mm) and the patient required two Gianturco coils. Of 22 patients (8 males and 14 females) aged 2.7-16 years (x = 6.5 +/- 3.8), the procedure was successful in 21 children (95.5%). One patient with PDA was referred to the surgical department due to complications (the coil was displaced to the periphery of the left pulmonary artery and required removal by an Amplatzer device). The analysis of the follow-up echocardiography performed within 48 hours after the procedure and 1, 6, 12, 24 and 48 months later, revealed immediate total PDA closure in 29% of children. In 33% of patients, the PDA closed within 24 months. One child did not report to follow-up, however, the others are asymptomatic and show residual negligible shunts. The authors determined that the incidence and time of closure of residual shunts is dependent on the magnitude of the left-to-right shunt before the procedure.

Impact of right ventricle-pulmonary artery conduit placement on pulmonary artery development after the Norwood procedure in hypoplastic left heart syndrome.

OBJECTIVES: The right ventricle-to-pulmonary artery (RV-PA) shunt in the Norwood procedure (NP) for children with hypoplastic left heart syndrome (HLHS) provides stable haemodynamics and improves interstage survival. The aim of the study was to find the effect of RV-PA placement on pulmonary artery development after the NP. METHODS: A prospective, randomized study of 60 children with HLHS was carried out between 2008 and 2010. All children underwent the NP in the neonatal period and survivors underwent the hemi-Fontan operation (at a mean age of 4.78 ± 2.8 months). RV-PA was left side to the neo-aorta in 32 children (the first group) and right side to the neo-aorta in 28 children (the second group). Echocardiography and angiograms were used to asses the pulmonary artery size. RESULTS: There was a significant difference between right pulmonary artery (RPA) and left pulmonary artery (LPA) diameters in both groups before the NP (first: 4.94 ± 0.27 vs. 4.26 ± 0.22, P = 0.04; second: 4.97 ± 0.23 vs. 4.14 ± 0.17, P = 0.003). This difference was not significant when z-scores were taken into account. The dynamics of the pulmonary artery development was similar in both groups comparing pre-Norwood and pre-hemi-Fontan periods. A slight increase in the LPA and the RPA diameter with a significant decrease in the z-scores was noted. At the pre-hemi-Fontan stage, there was no significant difference in the diameter and the z-score between LPA and RPA in the second group, whereas in the first group, the z-score for LPA was significantly lower compared with RPA (-1.34 ± 1.6 vs. -0.86 ± 1.4, P = 0.016). CONCLUSIONS: Placement of the RV-PA conduit on the right side to the neo-aorta ensures more equal distribution of the blood to the pulmonary arteries and better development of the LPA.

[Hybrid, perventricular closure of muscular ventricular septal defects]. / Hybrydowe, przezkomorowe zamknieciemiesniowych ubytków miedzykomorowych.

Muscular ventricular septal defects (VSD) located below the trabecula saepto-marginalis are difficult to approach for surgical closure through the tricuspid valve. We present the hybrid technique of perventricular closure of VSD in 2- and 5-year old children with complex congenital heart defects, employing an Amplatzer septal occluder, dedicated to muscular type of VSD. The procedures were performed during cardiopulmonary bypass in one patient and on beating heart in the other one. The perventricular device technique may be the method of choice for closing hard to reach muscular VSD.

[Origin of right pulmonary artery branch from descending aorta. Case report of the successful treatment]. / Nieprawidlowe odejscie galezi prawej tetnicy plucnej od aorty zstepujacej. Opis pomyslnego przebiegu leczenia.

This report describes an extremely rare case involving anomalous origin of part of the right pulmonary branch from the thoracic aorta in a 3 month-old boy and its successful repair.

Significance of the residual aortic obstruction in multistage repair of hypoplastic left heart syndrome.

OBJECTIVE: The population of children who received right ventricle-to-pulmonary artery shunt (RV-PA) at Norwood procedure (NP) is growing, but limited data are available regarding the long-term outcome. The aim of this study was to present operative outcomes and mid-term hemodynamics and to assess the impact of the residual aortic obstruction on the results in patients undergoing three-staged surgery with RV-PA application at NP. METHODS: Between June 2001 and June 2009, 229 children with hypoplastic left heart syndrome (HLHS) and variants underwent NP with RV-PA; 172 have proceeded to stage II and 95 to stage III. The medical records (clinical data, echocardiographic records, cardiac catheterization reports, electrocardiograms, and surgical notes) were retrospectively reviewed. RESULTS: The later era of NP was associated with significantly better outcome (2001-2004: 1- and 5-years' survival were 64.3% and 59.8%, respectively; 2005-2009: 1- and 4-years' survival were 93.1% and 86.9%, respectively) (p<0.001). There was no association between long-term survival and diagnosis (HLHS/HLHS variant) (p=0.39). The incidence of depressed ventricular function and moderate or severe systemic atrioventricular valve regurgitation among the children who required balloon aortoplasty (BA) before stage II surgery was significantly higher than in children without aortic arch obstruction (p=0.027, p=0.008, respectively). In midterm follow-up, BA had no significant influence on the actuarial survival (p=0.089). No ventricular arrhythmias were noticed at any stage. CONCLUSIONS: The RV-PA shunt is a safe technique that does not seem to impair systolic or electrical ventricular function; its outcomes continue to improve with growing experience. Combined cardiologic interventional and surgical procedures are required to optimize the outcomes.

Can early echocardiographic findings predict patent ductus arteriosus?

BACKGROUND: Prophylactic treatment with prostaglandin synthetase inhibitors (PSI) is potentially harmful. Moreover, long-term benefits of prophylactic use of indomethacin or ibuprofen are not proven. Early treatment of a high-risk population is alternative to the routine prophylactic use of PSI, but it remains unclear which newborn is at greatest risk for patent ductus arteriosus (PDA). OBJECTIVE: Evaluation of the prognostic value of early echocardiographic studies with respect to PDA in later life. METHODS: Sixty preterm infants with a mean birth weight of 1,087 g and mean gestational age of 28.5 weeks were included in a prospective study. Cardiac scans were performed in all newborns on entry into the study (within 12-48 h after birth) and further in case of clinical suspicion of PDA or obligatorily on the 7th and 28th days of life. There was no prophylactic or treatment use of any PSI during the study period. Newborns were divided into 2 cohorts: with significant left to right shunt requiring surgical ligation of PDA (n = 16) or without significant PDA during follow-up (control group, n = 44). RESULTS: On entry, the mean internal diameter of the ductus arteriosus (2.6 vs. 0.91 mm/kg; p < 0.01), mean cardiac index across aortic valve (2.96 vs. 2.37 l/min/m(2); p < 0.01) and early filling peak velocity (43.1 vs. 33.7; p = 0.01) were significantly higher in babies who later needed surgical ligation of PDA. There was no difference in the mean values of the other echocardiographic parameters studied. An early ductal diameter of >1.5 mm/kg predicted symptomatic PDA with a sensitivity of 94% and a specificity of 73%, and its positive predictive value equaled 57% and negative predictive value amounted to 97%. CONCLUSIONS: Early echocardiographic studies possess negative predictive value and may decrease unnecessary surgical ligation of PDA in very low birth weight infants.

Right atrial extension of Wilms' tumor.

BACKGROUND: Right atrial involvement in children with Wilms' tumor (nephroblastoma) is a very rare phenomenon. CASE REPORT: The authors present four children with nephroblastoma, in whom the tumor involved the inferior vena cava and the right atrium. In two children the intracardiac tumor extension was asymptomatic, while the other two had Budd-Chiari syndrome. Therapeutic management included initial three-drug chemotherapy in three patients, and primary tumor excision in one child, since the venous involvement had been not diagnosed. In each child, the therapeutic strategy was individualized and tailored to the course of the disease. Two patients were cured. The child with bilateral Wilms' tumor died due to disease progression. Another child died suddenly at home in the course of an intermission between consecutive cycles of successful chemotherapy. CONCLUSIONS: The extension of Wilms' tumor to the great vessels and the right atrium indirectly affects the final outcome. Preoperative chemotherapy in children with Wilms' tumor invading the inferior vena cava and the right atrium is the method of choice. The extent of surgery depends on the preliminary chemotherapy results.