Who is at higher risk of hypertension? Socioeconomic status differences in blood pressure among Polish adolescents: a population-based ADOPOLNOR study.

UNLABELLED: In Poland, there is no data on parental socioeconomic status (SES) as a potent risk factor in adolescent elevated blood pressure, although social differences in somatic growth and maturation of children and adolescents have been recorded since the 1980s. This study aimed to evaluate the association between parental SES and blood pressure levels of their adolescent offspring. A cross-sectional survey was carried out between 2009 and 2010 on a sample of 4941 students (2451 boys and 2490 girls) aged 10-18, participants in the ADOPOLNOR study. The depended outcome variable was the level of blood pressure (optimal, pre- and hypertension) and explanatory variables included place of residence and indicators of parental SES: family size, parental educational attainments and occupation status, income adequacy and family wealth. The final selected model of the multiple multinomial logistic regression analysis (MLRA) with backward elimination procedure revealed the multifactorial dependency of blood pressure levels on maternal educational attainment, paternal occupation and income adequacy interrelated to urbanization category of the place of residence after controlling for family history of hypertension, an adolescent's sex, age and weight status. Consistent rural-to-urban and socioeconomic gradients were found in prevalence of elevated blood pressure, which increased with continuous lines from large cities through small- to medium-sized cities to villages and from high-SES to low-SES familial environments. The adjusted likelihood of developing systolic and diastolic hypertension decreased with each step increase in maternal educational attainment and increased urbanization category. The likelihood of developing prehypertension decreased with increased urbanization category, maternal education, paternal employment status and income adequacy. Weight status appeared to be the strongest confounder of adolescent blood pressure level and, at the same time, a mediator between their blood pressure and parental SES. CONCLUSION: The findings of the present study confirmed socioeconomic disparities in blood pressure levels among adolescents. This calls for regularly performed blood pressure assessment and monitoring in the adolescent population. It is recommended to focus on obesity prevention and socioeconomic health inequalities by further trying to improve living and working conditions in adverse rural environments.

Frequency of 22q11.2 microdeletion in children with congenital heart defects in western poland.

BACKGROUND: The 22q11.2 microdeletion syndrome (22q11.2 deletion syndrome -22q11.2DS) refers to congenital abnormalities, including primarily heart defects and facial dysmorphy, thymic hypoplasia, cleft palate and hypocalcaemia. Microdeletion within chromosomal region 22q11.2 constitutes the molecular basis of this syndrome. The 22q11.2 microdeletion syndrome occurs in 1/4000 births. The aim of this study was to determine the frequency of 22q11.2 microdeletion in 87 children suffering from a congenital heart defect (conotruncal or non-conotruncal) coexisting with at least one additional 22q11.2DS feature and to carry out 22q11.2 microdeletion testing of the deleted children's parents. We also attempted to identify the most frequent heart defects in both groups and phenotypic traits of patients with microdeletion to determine selection criteria for at risk patients. METHODS: The analysis of microdeletions was conducted using fluorescence in situ hybridization (FISH) on metaphase chromosomes and interphase nuclei isolated from venous peripheral blood cultures. A molecular probe (Tuple) specific to the HIRA (TUPLE1, DGCR1) region at 22q11 was used for the hybridisation. RESULTS: Microdeletions of 22q11.2 region were detected in 13 children with a congenital heart defect (14.94% of the examined group). Microdeletion of 22q11.2 occurred in 20% and 11.54% of the conotruncal and non-conotruncal groups respectively. Tetralogy of Fallot was the most frequent heart defect in the first group of children with 22q11.2 microdeletion, while ventricular septal defect and atrial septal defect/ventricular septal defect were most frequent in the second group. The microdeletion was also detected in one of the parents of the deleted child (6.25%) without congenital heart defect, but with slight dysmorphism. In the remaining children, 22q11.2 microdeletion originated de novo. CONCLUSIONS: Patients with 22q11.2DS exhibit wide spectrum of phenotypic characteristics, ranging from discreet to quite strong. The deletion was inherited by one child. Our study suggests that screening for 22q11.2 microdeletion should be performed in children with conotruncal and non-conotruncal heart defects and with at least one typical feature of 22q11.2DS as well as in the deleted children's parents.

Mutation of the MYH7 gene in a child with hypertrophic cardiomyopathy and Wolff-Parkinson-White syndrome.

Familial hypertrophic cardiomyopathy (HCM) displays autosomal dominant inheritance with incomplete penetration of defective genes. Data concerning the familial occurrence of ventricular preexcitation, i.e. Wolff-Parkinson-White (WPW) syndrome, also indicate autosomal dominant inheritance. In the literature, only a gene mutation on chromosome 7q3 has been described in familial HCM coexisting with WPW syndrome to date. The present paper describes the case of a 7-year-old boy with HCM and coexisting WPW syndrome. On his chromosome 14, molecular diagnostics revealed a C 9123 mutation (arginine changed into cysteine in position 453) in exon 14 in a copy of the gene for beta-myosin heavy chain (MYH7). It is the first known case of mutation of the MYH7 gene in a child with both HCM and WPW. Since no linkage between MYH7 mutation and HCM with WPW syndrome has been reported to date, we cannot conclude whether the observed mutation is a common cause for both diseases, or this patient presents an incidental co-occurrence of HCM (caused by MYH7 mutation) and WPW syndrome.

Environmental variation in the prevalence of hypertension in children and adolescents - is blood pressure higher in children and adolescents living in rural areas?

INTRODUCTION: Despite considerable progress in diagnostic methods and treatment options, cardiovascular diseases constitute a serious health issue in Poland, and hypertension is considered one of the main risk factors in the incidence of the diseases. OBJECTIVES: The aim of this study is to assess the values of arterial blood pressure among adolescents in the context of urban and rural environments. MATERIALS AND METHOD: The study was carried out on a representative, randomly selected group of youths aged 10-18 years, participating in the ADOPOLNOR project. Prior to blood pressure measurement, each pupil was assessed according to anthropomorphic principles. The BP measurements strictly followed the guidelines of the Fourth Protocol of the American Working Group of High Blood pressure in Children and Adolescents. RESULTS: Mean values of systolic blood pressure (SBP) in boys in rural areas were higher in every age group than in the boys in urban areas. Higher values of systolic and diastolic blood pressure were observed also in girls in rural areas. BP values sufficiently high for the diagnosis of hypertension were observed in 12.8% of the rural boy population but only in 6.7% of the boys from the urban environment. The difference is statistically significant. The girls in rural areas (13.3%) had twice the incidence of elevated BP values in comparison with the girls in urban areas (7.2%) and, as in boys, the difference is statistically significant. CONCLUSIONS: The high proportion of pupils with elevated arterial blood pressure observed in the rural areas of Wielkopolska calls for regularly performed BP assessment and monitoring in this populatio.

[The antioxidant therapy modulates intracellular lymphokine expression in children on dialysis]. / Leczenie antyoksydacyjne moduluje wewnatrzkomórkowa ekspresje limfokin u dzieci dializowanych.

UNLABELLED: Oxidative stress (SOX) is believed to be responsible for functional disabilities of lymphocytes in end-stage renal disease (ESRD). Therefore, we investigated the effect of antioxidant therapy with vitamin E and N-acetylcysteine (NAC) on SOX and cytokine synthesis in T cells in dialyzed children. Eighteen children (aged 2-20, mean 10.9 yr) treated with hemodialysis (n=5) and peritoneal dialysis (n=13) were enrolled into the study. Vitamin E and NAC were given orally for six months. Throughout the study, intracellular lymphokines [interleukin (IL)-2, interferon (IFN)-gamma, IL-4, IL-6] and SOX in T cells were measured by means of flow cytometry. In dialyzed children, mean fluorescence intensity (MFI), which reflected intracellular SOX, was significantly higher than in the controls in both CD3+ and CD3+CD4+ cells (p<0.05). We also found a cytokine dysregulation with a trend toward a predominant T helper (Th)-1 response compared to the controls. After 6 months of treatment with antioxidants, a significant reduction in MFI was noted compared to baseline values in CD3+ and CD3+CD4- cells (p<0.001). Interestingly, the therapy led to a decrease in IFN-gamma as well as an increase in IL-4 and IL-6 production. In addition, a gradual decline in IFN-gamma/IL-4 ratio in Th cells was noted. CONCLUSIONS: Vitamin E and NAC used in combination are effective in reducing the intracellular SOX, and besides their action on cellular redox state, they modulate the cytokine profile in children on dialysis.

[Immunohistochemical analysis of podocytopathy with immature glomeruli and glomerulosclerosis in children with nephrotic syndrome]. / Immunohistochemiczna analiza podocytopatii z cechami niedojrzalosci.

Idiopathic nephrotic syndrome in children may be complicated by resistance to steroids with constant proteinuria in diffuse mesangial proliferation (DMP) and focal segmental glomerulosclerosis (FSGS). In our observation, sometimes in children with steroid-resistant nephrotic syndrome, the presence of immature renal glomeruli can be detected (hypercellularity and presence of a constant layer of cubical epithelial cells on the surface of glomerular tufts, without sclerosis, resembling M-stage of glomerulo-genesis). The aim of this study was immunohistochemical analysis of the podo-cyte-associated proteins, particularly ezrin, podocalyxin, synaptopodin and nephrin in glomeruli with and without signs of immaturity in children. In DMP with signs of immaturity podo-cytes situated in the central region of the glomerulus were immunohistochemically negative. The positive reaction was observed exclusively in the most superficial continuous 'layer' of podo-cytes. The unfavourable clinical course of nephrotic syndrome with signs of glomerular immaturity may be a consequence of decreased immunohistochemical expression of cytoskeleton-specific proteins.

[New, successful treatment of urinary tract infection caused by Pseudomonas aeruginosa]. / Nowy, skuteczny model leczenia zakazenia ukladu moczowego spowodowanego Pseudomonas aeruginosa.

Urinary tract infection (UTI) is common in pediatric practice and an important cause of morbidity and mortality in children. Escherichia coli remains the predominant uropathogen (80%) isolated in acute community-acquired uncomplicated infections, followed by Staphylococcus saprophyticus (10% to 15%) and Pseudomonas aeruginosa (9%) The pathogens traditionally associated with UTI are changing many of their features, particularly because of antimicrobial resistance. Reinfections and relapses of urinary tract infections caused by PA are very frequent. The aim of the study was to evaluate the efficacy of combined clarithromycine and ceftazidime in terms of eradication of PA infection. We analyzed 20 out of 264 children with UTI where PA infection was confirmed with urine culture. Those children were treated for at least 14 days with the protocol used for PA infection in patients with mucoviscidosis. Short-term eradication was achieved in all patients. Long-term study revealed relapse in 25% of children, all with serious congenital malformations. 75% of children were treated with success. No side effects were observed. Conclusion. We conclude that an empirical combination treatment of clarithromycine and ceftazidime is appropriate and effective in children with UTI caused by PA. This therapy was clinically efficacious, well tolerated, and cost effective, and should prevent unnecessary development of antimicrobial resistance.

Evaluation of medical and psychological parameters of quality of life in supraventricular tachyarrhythmia children. A comparison with healthy children.

INTRODUCTION: There are only a few available studies evaluating quality of life (QoL) in pediatric patients with cardiac arrhythmia. The aim of the study was to evaluate medical and psychological parameters of the QoL in children with a diagnosed supraventricular tachyarrhythmia (SVT) and to compare the obtained data with a group of healthy children (HC). MATERIAL AND METHODS: Inclusion criteria: children aged 7-18 with SVT, treated at Poznan University of Medical Sciences, Department of Pediatric Cardiology. The evaluation tools were the WHOQOL-BREF instrument and a questionnaire related to the patient's feelings and observations concerning arrhythmia (Pediatric Arrhythmia Related Score - PARS), developed by the authors and adjusted to the group of arrhythmia patients. RESULTS: The study included 180 SVT children and 83 HC. On the basis of WHOQOL-BREF the SVT group was found to have lower assessment values of QoL within the physical domain (Phd) (mean ± SD: 65.7 ±15.8 vs. 81.6 ±12.8; p < 0.0001) and psychological domain (Psd) (mean ± SD: 75.8 ±15.2 vs. 81.3 ±14.1; p < 0.005). No significant differences were found within the social relationships domain or the environment domain. On the basis of PARS in the SVT group the patients reported significantly increased symptoms within Phd (mean ± SD: 2.3 ±0.7 vs. 1.6 ± 0.3; p < 0.0001) as well as increased negative feelings within Psd (mean ± SD: 2.3 ±0.7 vs. 2.1 ± 0.6; p < 0.005). CONCLUSIONS: Medical and psychological parameters of the QoL in SVT children are significantly lower in comparison with HC. A diagnosis of SVT has no influence on the social and environmental areas of QoL. The PARS appears to be a useful tool to supplement the generic questionnaire for QoL evaluation in SVT children.

Abnormal cytokine synthesis as a consequence of increased intracellular oxidative stress in children treated with dialysis.

BACKGROUND/AIM: End-stage renal disease (ESRD) induces a clinical state of immunodeficiency with a higher incidence of infections and higher mortality due to infectious complications compared with the normal population. The definite mechanism responsible for the host defense alterations is not well understood. The aim of the study was to investigate intracellularly the relationship between cytokine synthesis and oxidative stress in peripheral blood lymphocytes in children with ESRD. METHODS: Twenty-one children (age 11.7 +/- 5.8 years) with ESRD treated with hemodialysis (HD; n = 10) and peritoneal dialysis (PD; n = 11) were studied. Nine healthy children of comparable age formed the control group. To determine intracellular oxidative stress we used dihydrorhodamine-123 (DHR), which after oxidation to rhodamine-123 (RHO) emitted a bright fluorescent signal. Intracellular oxidation of DHR in T lymphocytes reflected intracellular oxidative stress. The intracellular synthesis of cytokines (IL-2, IFN-gamma, IL-4, IL-6) was also measured. Both parameters were detected at a single-cell level by flow cytometry. Lymphocyte subsets were evaluated using the monoclonal antibodies conjugated with fluorochromes. RESULTS: We found that in T lymphocytes the mean fluorescence intensity (MFI), which reflected intracellular oxidative stress, was increased in ESRD patients compared to the controls (CD3+: 34.77 +/- 11.55 vs. 22.55 +/- 4.97, p < 0.01; CD3+CD8+: 34.31 +/- 12.17 vs. 20.77 +/- 4.89, p < 0.01; CD3+CD4+: 36.06 +/- 6.98 vs. 24.44 +/- 7.68, p < 0.001). HD patients showed slightly higher MFI compared to PD patients in CD3+ cells (39.32 +/- 11.70 vs. 30.63 +/- 10.20, NS), in CD3+CD8+ cells (37.90 +/- 14.32 vs. 31.06 +/- 9.34, NS) and in CD3+CD4+ cells (40.10 +/- 2.28 vs. 29.33 +/- 7.06, p < 0.001). The intracellular synthesis of IL-2 was higher in ESRD patients compared to the controls, both in CD3+ cells (31.34 +/- 9.80 vs. 20.49 +/- 15.26%, p < 0.05) and in CD3+CD4+ cells (36.10 +/- 8.69 vs. 24.03 +/- 16.95%, p < 0.05). The intracellular synthesis of IFN-gamma, IL-4 and IL-6 was significantly lower in the ESRD group compared to the controls. Interestingly, in patients treated with HD, negative correlations between the degree of intracellular oxidative stress and intracellular cytokine synthesis in CD3+ lymphocytes were found. CONCLUSION: Our results show that patients with ESRD, especially those treated with HD, present increased oxidative stress in T lymphocytes, which may lead to decreased cytokine synthesis and abnormal immune response.

[Ross operation using "contegra" conduit in a 5 year old girl with severe aortic valve insufficiency in Kawasaki disease coexisting with infective endocarditis--a case report]. / Ciezka niedomykalnosc aortalna w chorobie Kawasaki wspólistniejaca z bakteryjnym zapaleniem wsierdzia u 5-letniego dziecka. Operacja Ross'a z zastosowaniem Conduitu "Contegra".

A case of a 5-year-old girl with severe dysfunction of aortic valve in Kawasaki disease coexisting with endocarditis, is described. The role of Ross operation in the treatment of this condition is discussed. The 18-months follow-up showed good function of aortic valve and "Contegra" conduit (bovine jugular vein), but long-term follow-up of patients with "Contegra" conduit remains unknown. In conclusion, a Ross operation using "Contegra" conduit in pulmonary position could be effective method in the treatment of dysfunction of aortic valve in Kawasaki disease coexisting with endocarditis in children.

Aortoventriculoplasty and left ventricle function: long-term follow-up.

OBJECTIVE: Depressed cardiac function after aortoventriculoplasty is well known during the postoperative period. Little data exist concerning the long-term follow-up. The aim of this study is to determine whether septal incision has any permanent effect on the left ventricle function. METHODS: From 1988 to 2002, 45 patients received aortic mechanical prosthesis. These patients were divided into two groups. Group A consisted of 26 patients 5-18 years old, who underwent simple aortic valve replacement. Group B consisted of 19 patients 4-20 years old, who underwent the Konno procedure. Systolic and diastolic functions of the left ventricle were analyzed using echocardiography. For the systolic function, the following parameters were assessed: pressure gradient between left ventricle and ascending aorta, shortening and ejection fraction of the left ventricle. For the diastolic function, left ventricle-filling parameters were assessed: ratio of early to late filling velocity, deceleration slope of the early filling velocity and left ventricular isovolumetric relaxation time. Furthermore the percentage fraction of the aortic valve index (AOVI%) was calculated and compared between these two groups. RESULTS: After the surgery in group A, AOVI% dropped from 110+/-21 to 98+/-11%, while in group B it increased from 82+/-16 to 114+/-11%. As a result a higher residual pressure gradient across the aortic valve was noted in group A: 21.26+/-15 as compared to 11.17+/-5 mmHg in group B. A mean pressure above 30 mmHg appeared in group A 2 years after the surgery, while in group B this was obtained after 6 years. As for the diastolic function no significant difference was noted between these two groups. Overall there was one late death in group A, and in group B two early deaths, two reoperations because of excessive drainage and in two patients permanent pacemakers had to be implanted. CONCLUSIONS: Improvement of the systolic function after the surgery was noted in both groups. In patients with low AOVI%, postsurgical pressure gradient, either residual or recurrent, appeared during the follow-up. As for the septal incision, it may have some transient effects on the left ventricle function in the postoperative period, but no permanent sequelae were observed in the long-term follow-up.

Arterial switch operation in neonates with complex congenital heart defects.

The aim of the study was to analyze short-term results of the arterial switch operation in 29 neonates with simple transposition of the great arteries (group A) and 18 (group B) with complex heart defects: transposition with a ventricular septal defect (10), coarctation of the aorta (5), or Taussig-Bing anomaly (3). The operations were usually performed on the 7th day of life (2nd-30th day), after a Rashkind procedure when necessary. The mean weight was 3,530 +/- 780 g, body surface area was 0.219 +/- 0.032 m(2). Delayed sternal closure was necessary in 7 patients from group A (24%) and 8 from group B (44%) because of hemodynamic instability after weaning from extracorporeal circulation; these neonates had significantly lower body weights and smaller body surface areas. Perioperative mortality was 13.8% (4 patients) in group A and 27.8% (5 patients) in group B. Correction of complex transposition tends to be associated with a higher operative risk than simple transposition, but the difference was not significant.

[Pregnancy and labour in a patient after Fontan operation - a case report]. / Ciaza i poród u pacjentki po operacji metoda Fontana.

We present a case a 25-year-old pregnant female who had Fontan surgery performed 20 years ago due to a common ventricle of the left ventricular morphology. She had a history of three miscarriages and one successful pregnancy. This was a fifth pregnancy and she delivered normal healthy child. This is a second patient after Fontan procedure who had two successful deliveries. Clinical aspects of pregnancy in patients after Fontan procedure are described.

Blood pressure, left ventricular mass and function in adult patients after successful repair of coarctation of the aorta.

BACKGROUND: In spite of surgical correction, arterial hypertension may persist in a significant proportion of patients with aortic coarctation. AIM: To asses arterial blood pressure (BP) and left ventricular (LV) mass and function in adult patients who underwent surgical repair of aortic coarctation. METHODS: The study group consisted of 56 patients (27 females, 29 males, mean age 31.2+/-10 years), who underwent surgery due to aortic coarctation at the mean age of 11.53+/-8 years. The control group consisted of 61 healthy subjects. The following parameters were analysed: BP values, end-diastolic interventricular septum (IVS) and posterior wall (PW) thickness, end-diastolic LV diameter, residual stenosis of descending aorta, LV ejection fraction, LV mass (LVM), LVM index (LVMI) and parameters of diastolic LV function. RESULTS: Hypertension was diagnosed in 55% of patients, particularly in those who underwent surgery in more advanced age. In 21 patients the residual pressure gradient through the descendent aorta exceeded 20 mmHg; 14 of them had hypertension. Echocardiography showed significantly higher values of PW thickness in patients than in controls, and significantly higher values of PW, IVS, LVM and LVMI in patients with rather than without hypertension. The most pronounced differences in these parameters were noted between controls and patients with hypertension. In patients with residual trans-aortic gradient, all the above mentioned parameters were similar to those obtained in patients without the gradient or controls. Also, both systolic and diastolic LV function were similar in patients and controls, in patients with or without hypertension, and in patients with or without residual aortic gradient. In the whole study group, a positive linear correlation between age at surgery and BP values was found (p=0.01) whereas no such correlation was found between BP and time which elapsed after surgery. There was a significant negative correlation between E/A and age at surgery (p=0.004) and a positive correlation between time from surgery and IVRT (p=0.025). IVRT positively correlated with BP (p=0.024). BP values correlated positively with PW (p=0.024) and IVS (p=0.04). Among patients with hypertension, a significant positive correlation between age at surgery and LVM (p=0.034) as well as LVMI (p=0.013) was noted. Diastolic LV function in the subgroup of patients with hypertension was characterised by a negative correlation between age at surgery and E/A (p=0.001) and a positive correlation between time from surgery, BP values and IVRT (p=0.045 and p=0.024, respectively). In patients without hypertension no significant correlation between the analysed parameters was found. CONCLUSIONS: 1. Hypertension is present in more than half of adult patients after surgical repair of aortic coarctation, is more frequent in patients who underwent surgery at a more advanced age, and in the majority of patients is not associated with the presence of residual trans-aortic gradient. 2. LV thickness, mass and index increase due to hypertension, regardless of the presence of gradient. 3. LV diastolic parameters are similar to those in healthy subjects, however, they become impaired as the time from surgery increases, particularly in patients who underwent repair at an advanced age and who have hypertension.

Aortic regurgitation in adults after surgical repair of tetralogy of Fallot.

BACKGROUND: Total surgical repair of tetralogy of Fallot is usually associated with a favourable long-term outcome. However, complications or consequences of the defect such as dilatation of aortic root and aortic regurgitation are present in some patients. AIM: To assess the prevalence of aortic regurgitation in relation to the timing of surgery and preceding palliative procedures as well as to examine its effects on left ventricular (LV) dimensions in adults after surgical repair of tetralogy of Fallot. METHODS: The study group consisted of 63 patients in the mean age of 25.6+/-6.5 years who underwent surgery at the mean age of 7.4 years (mean 17.2 years ago). Echocardiographic assessment of the thickness of the inter-ventricular septum, posterior LV wall, end-diastolic LV diameter, left atrial dimension and aortic root diameter was performed. Aortic incompetence was examined using a colour Doppler method. RESULTS: Aortic incompetence was found in 13 (20%) patients. Only four of them had undergone a palliative procedure prior to the repair of the defect. Patients with aortic regurgitation had significantly greater aortic root dimension (p=0.001), LV diameter (p=0.05) and were significantly older (p=0.005) than patients without aortic incompetence. A significant correlation was found between aortic root diameter and current age (p=0.02). A significant correlation was also observed between LV diameter and time elapsed after surgery (p=0.001), and between left atrial dimension and patients' current age (p=0.02) as well as time from the repair of the defect (p=0.003). CONCLUSIONS: 1. Minor aortic regurgitation and LV dilatation are present in 20% of adults who underwent repair of tetralogy of Fallot. 2. Aortic regurgitation is present in those who have dilated aortic root. 3. Aortic regurgitation is not associated with palliative surgery performed in the past. 4. Aortic regurgitation is more frequent in the elderly but is not associated with patient's age at surgery or time elapsed since repair.

Transcatheter closure of perimembranous ventricular septal defect using an Amplatzer occluder--early results.

BACKGROUND: Transcatheter closure of perimembranous ventricular septal defect with the use of an Amplatzer occluder was introduced in 1999, however, clinical experience with this method remains limited. AIM: To assess short-term safety and efficacy of this technique in children. METHODS: Since 2001, nine children underwent transcatheter closure of perimembranous ventricular septal defect in the cardiac centre in Bratislava (Slovak Republic) or in our centre. Patients were selected for the procedure based on the results of transesophageal echocardiography (TEE). Transcatheter closure using the Amplatzer occluder was guided by both TEE and angiography. The follow-up duration ranged from 3 to 22 months, mean 11.5 months. Control angiography was performed in order to detect any leakage through the aortic valve. The end-diastolic left ventricular diameter before and after the procedure as well as the function of the aortic, tricuspid and mitral valves were also assessed. RESULTS: The procedure was effective in all patients. No damage to atrio-ventricular nor aortic valves was observed. Tricuspid regurgitation, present prior to the procedure, markedly decreased following the closure of the defect. The left ventricular end-diastolic diameter significantly (p=0.001) decreased after the procedure. CONCLUSIONS: (1) Perimembranous ventricular septal defect can be safely and effectively closed using a transcatheter Amplatzer occluder. (2) Appropriate selection for the procedure as well as TEE and angiographic guidance during the procedure enable the avoidance of complications. (3) Tricuspid regurgitation is not an absolute contraindication to perform this procedure.

Echocardiographic parameters of left ventricular systolic and diastolic function in infants, children and adolescents before and after surgical correction of secundum atrial septal defect.

BACKGROUND: Left ventricular (LV) dysfunction has been described in adults with secundum atrial septal defect (ASD II) and heart failure (CHF). This anomaly has been rarely regarded as a cause of CHF in pediatric patients with ASD II. AIM: To assess LV systolic and diastolic function in patients with ASD II before and after cardiosurgery as well as to establish the prognostic value of these parameters in infants, children and adolescents with ASD II. METHODS: LV systolic (LVEF) and diastolic function parameters (E/A, DCT, IVRT) were studied using Doppler echocardiography in 104 patients aged between 1-18 years with ASD II before cardiosurgery and 4 years afterwards. These parameters were compared with similar variables in 150 healthy infants, children and adolescents. RESULTS: Before surgery LVEF was significantly lower only in infants with ASD II and CHF when compared with healthy controls. Relaxation abnormalities of LV diastolic function were observed before surgery in 40.4% of patients, especially in infants and adolescents with CHF. These disturbances were still present in about 20% of patients after surgery, especially in infants. Before and after surgery both LV and RV end-diastolic volumes were normal in about 6-10% of patients with LV diastolic dysfunction. CONCLUSIONS: In patients with ASD II and CHF diastolic dysfunction of LV is more common than systolic dysfunction. RV volume overload mainly affects LV diastolic function but it is not the only cause of CHF in patients with ASD II, especially in infants and adolescents. After surgery, normalisation of LV diastolic function is not observed in about 20% of patients with ASD II, especially in those who had CHF before operation. Special monitoring of the circulatory system is necessary in patients with ASD II operated in infancy and adolescence.

[Influence of autonomic nervous system on electrolyte abnormalities in children with mitral valve prolapse]. / Wplyw autonomicznego ukladu nerwowego na wystepowanie zaburzen elektrolitowych u dzieci z wypadaniem platków zastawki dwudzielnej.

Measurement of the heart rate variability enables to assess the activity of the autonomous nervous system. The aim of the study was to evaluate the possible relationship between clinical status, serum sodium (Na), potassium (K), calcium (Ca), magnesium concentrations and HRV in children with mitral valve prolapse. The study group consisted of 151 children with MVP (age +/- SD: 12.2 +/- 3.1 years). Thirty patients out of the MVP group were symptomatic and 121 were asymptomatic. The total number of 165 healthy children (age +/- SD: 12.3 +/- 3.7 years) were included into the control group. We assessed power spectrum of HRV (low frequency component--LF, high frequency component--HF, and LF/HF ratio) from 5-minute recording during daytime and night. Serum Mg (p < 0.00001) and K (p < 0.03) concentrations were significantly lower in MVP group as compared to healthy children. A marked decrease in Mg concentration was observed in symptomatic children with MVP as compared to asymptomatic patients (p < 0.0001). A significant positive correlation was found between Mg concentration and HF and HF n.u. components (daytime and night) and significant negative correlation was observed between Mg concentration and LF and LF n.u. (daytime and night) parameters and LF/HF ratio (daytime and night). Symptomatic children with MVP had significantly higher LF n.u. and LF/HF ratio and a lower HF n.u. as compared to asymptomatic patients. The autonomic changes in MVP children (reduction in parasympathetic tone with sympathetic predominance) were associated with decreased serum Mg concentration and higher prevalence of clinical symptoms. Mg supplementation seems to be a prudent approach in symptomatic children with MVP.

[Evaluation of systolic and diastolic function of the left ventricle in children with acute lymphoblastic leukaemia before treatment]. / Ocena czynnosci skurczowej i rozkurczowej lewej komory serca u dzieci z ostra bialaczka limfoblastyczna przed rozpoczeciem leczenia.

Between 1995 and 2001 echo-cardiography was performed in 244 children (128 boys, 116 girls) with acute lymphoblastic leukaemia (ALL) before the beginning of therapy with anthracyclines (medium 5.4 days after the diagnosis). The mean age at diagnosis was 5.4 years (range 9 months to 17.7 years). 189 children (97 boys and 92 girls) were included into the standard and medium risk groups and 55 (31 boys and 24 girls) into the high risk group. 29% of ALL children had disturbances in ECG. Changes in the thickness of the intraventricular septum (%IVSTh) and left ventricular posterior wall (%LVPWTh) were statistically lower, especially in children under 7 years of age. Some children showed lowering of shortening fraction (%FS - 8.6%), ejection fraction (%EF - 10.2%) and corrected velocity of fibber-shortening (Vcfc - 25.8%). Children with decreased shortening fraction (%FS) had left ventricular posterior wall thickness (%LVPWTh) impairment. Changes in diastolic function indicate impaired relaxation and compliance of the left ventricle. Decreased peak early filling velocity (E) was found. There were also longer deceleration time (EDecT) and decreased deceleration from peak E velocity (E/Dec) and longer isovolumetric relaxation time in children in standard and medium risk groups. Shorter acceleration time (EAccT) was seen in the high risk group. Evaluation of cardiac function before anthracycline chemotherapy will allow to select patients with pre-existing cardiac impairment for whom cardioprotective treatment is absolutely necessary.

[Hybrid treatment of interrupted aortic arch in a newborn with contraindications for extracorporeal circulation: case report including 1.5 year follow-up]. / Leczenie hybrydowe noworodka z przerwanym lukiem aorty i przeciwwskazaniami do krazenia pozaustrojowego: opis przypadku z uwzglednieniem 1,5-rocznej obserwacji.

Despite marked improvement in the cardiosurgery, total repair of interrupted aortic arch with coexisting risk factors in neonatal or early infancy is associated with high mortality. We present a patient treated by an alternative hybrid procedure without exposing the critical ill neonate to the risk of cardiopulmonary bypass. At the 1.5 year of life a successful arch reconstruction, repair of associated anomalies and de-banding of pulmonary arteries with a stent cut out was done.