Metabolic syndrome as risk factor for left ventricular hypertrophy in children with chronic kidney disease.

Background: The metabolic syndrome (MS), a cluster of clinical and biochemical abnormalities including insulin resistance, dyslipidemia and hypertension, is often diagnosed in chronic kidney disease (CKD) children. Left ventricular hypertrophy (LVH) is a major target organ damage in hypertension and an important cardiovascular risk factor in CKD patients. We aimed to identify the most significant risk factors of LVH in children with CKD. Methods: Children with CKD stage 1-5 were enrolled in the study. MS was diagnosed according to De Ferranti (DF) as ≥3 from 5 criteria. Ambulatory blood pressure measurements (ABPM) and echocardiographic evaluation were performed. LVH was defined as ≥95th percentile of LV mass index related to height and age. Clinical and laboratory parameters included: serum albumin, Ca, HCT, cystatin C, creatinine, estimated glomerular filtration rate (eGFR) based on Schwartz formula, triglycerides, high-density lipoprotein (HDL), proteinuria, BMI standard deviation score (SDS), height SDS, waist circumference, ABPM data. Results: 71 children (28 girls/43 boys) with median age 14.05 (25%-75%:10.03-16.30) years and median eGFR 66.75 (32.76-92.32) ml/min/1.73m2 were evaluated. CKD stage 5 was diagnosed in 11 pts (15.5%). MS (DF) was diagnosed in 20 pts (28.2%). Glucose ≥ 110 mg/dL was present in 3 pts (4.2%); waist circumference ≥75th pc in 16 pts (22.5%); triglycerides ≥ 100 mg/dL in 35 pts (49.3%); HDL < 50mg/dL in 31 pts (43.7%) and BP ≥ 90th pc in 29 pts (40.8%), respectively. LVH was detected in 21 (29.6%) children. In univariate regression the strongest risk factor for LVH was CKD stage 5 (OR 4.9, p=0.0019) and low height SDS (OR 0.43,p=0.0009). In stepwise multiple logistic regression analysis (logit model) of the most important risk factors for LVH in CKD children, only three were statistically significant predictors: 1)MS diagnosis based on DF criteria (OR=24.11; 95%CI 1.1-528.7; p=0.043; Chi2 = 8.38,p=0.0038); 2), high mean arterial pressure (MAP SDS) in ABPM (OR=2.812; 95%CI 1.057-7.48; p=0.038;Chi2 = 5.91, p=0.015) and 3) low height SDS (OR=0.078; 95%CI 0.013-0.486;p=0.006; Chi2 = 25.01, p<0.001). Conclusions: In children with chronic kidney disease LVH is associated with the cluster of multiple factors, among them the components of MS, hypertension, stage 5 CKD and growth deficit were the most significant.

Recent achievements in transcatheter closure of ventricular septal defects: a systematic review of literature and a meta-analysis.

BACKGROUND: Ventricular septal defect (VSD) is one of the most common congenital heart defects. Currently, surgery remains the treatment of choice. However, transcatheter techniques for closing of various types of VSDs have become an alternative. AIMS: The objective of our study was to present the outcomes of transcatheter closure of various types of VSD based on a systematic review of recent publications. METHODS: A systematic review of studies published in English between January 2014 and March 2020 was performed using the PubMed database (MEDLINE) independently by 2 reviewers. Data on success and complication rates were extracted. Studies including fewer than 5 patients and those with acquired VSD were excluded from the analysis. RESULTS: Finally, 44 studies were included for analysis, with a total number of 4050 patients. The pooled estimate of the overall success rate based on the random effects model was 97.96% (95% CI, 97.37-98.56; Q test P 0.99; I 2 = 0%) for permanent VSD. CONCLUSIONS: Transcatheter closure of selected VSDs appears to be an effective and safe method of treatment. Recent studies have shown high rates of successful interventions with a low incidence of complications.

Echocardiography and Newer Imaging Techniques in Diagnosis and Long-Term Follow-Up of Primary Heart Tumors in Children.

BACKGROUND: Primary heart tumors (PHTs) in the pediatric population are very rare and do not manifest any characteristic symptoms. METHODS: A retrospective analysis of 61 cases was undertaken. Data from three centers for the years 2003-2018 were gathered. The tumors' clinical course, location, number, hemodynamic, treatment, and follow-up were evaluated. Echocardiography was complemented with magnetic resonance imaging, computer tomography, and histopathological examination. RESULTS: Out of 61 PHT diagnoses, 56 (91.8%) were circumstantial including all 16 (26.2%) prenatal tumors. The reasons for cardiological consultations were arrhythmia, syncopes, lowered physical performance, and murmurs. Only five patients (8.2%) were suspected of tumors based on previous symptoms of sclerosis tuberosa. Rhabdomyoma was the most frequently found PHT (60.7%). The tumors were predominantly located in the ventricles (49.1%) and intraventricular septum (14.9%) and tended to be single (70.5%). About 37.7% of patients suffered from coexistent multi-organ problems, two (3.28%) from congenital heart defects and one (1.64%) from Carney's syndrome. Tumor resection was performed on 26 (42.7%) patients, of which 16 (61.5%) underwent total and 10 (38.5%) partial tumor resection. During the follow-up (mean 4.3 years), 54 patients (88.5%) have improved or were stable, while seven (11.5%) died. CONCLUSIONS: Primary pediatric heart tumors are diagnosed completely circumstantially, and the most common is rhabdomyoma, although arrhythmia may suggest fibroma. Diagnosis of a heart tumor in children is not synonymous with fatal prognosis, and most of them require only constant observation. Life-saving operation allows improvement, while the prognosis for malignant tumors in children is definitely unfavorable.

[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.

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.

Atypical cardiac myxoma in the course of Carney complex in the child. Case Report.

Cardiac tumors in infants and children are rare. Myxomas are the second (after rhabdomyomas) most common primary cardiac tumors in pediatric patients. Cardiac, cutaneous and mucous myxomas are likewise the second most frequent manifestation of the Carney complex, an autosomal dominant multi neoplasia syndrome, which consists of myxomas in different locations, spotty skin pigmentation and endocrine overactivity. We present a case of 13-years-old boy send to our department from a district hospital because of the large tumor in the right atrium discovered in the echo study. On admission he presented discrete signs of Cushing's syndrome and scarse pigmented nevi on the face and trunc. The detailed echo examination showed the large right atrial tumor with features of myxoma, protruding across the tricuspid valve into the right ventricle during diastolic period. Atypical location of cardiac myxoma as well as the signs of Cushing's syndrome suggested Carney's complex. Detailed endocrine studies confirmed the hypothesis. Thus two-step bilateral adrenalectomy was planned. The histopathologic study confirmed primary pigmented nodular adrenocortical disease.

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.

Percutaneous balloon valvuloplasty for the treatment of pulmonary valve stenosis in children - a single centre experience.

BACKGROUND: Isolated pulmonary valve stenosis (PVS) is a common heart defect (6-9%); the preferred treatment is balloon pulmonary valvuloplasty (BPV). AIM: To assess BPV results in children with isolated PVS treated between 1988 and 2004, with a mean follow-up of 6.1 +/- 3.4 years. METHODS: The studied group consisted of 137 children (76 males and 61 females), aged 5.4 +/- 4.8 years. The diagnosis was based on physical examination, ECG, chest radiograph, echo, haemodynamic and angiocardiographic studies. The patients were divided into three groups, depending on the ratio of right ventricular systolic (RVSP) to systemic pressure (SP): I (n = 58) - RVSP < or = 75% of SP, II (n = 41) - RVSP = 76-100% of SP, III (n = 38) - RVSP > 100% of SP. In 7.3% of patients, dysplastic pulmonary valve (DPV) was seen. The balloon diameter to pulmonary valve annulus ratio was 1.29 +/- 0.1, and 1.42 +/- 0.1 in DPV children. RESULTS: Immediately post-BPV, the patients showed significantly (p < 0.001) decreased pressure gradient across PVS (I: 49.3 +/- 11.1 - 12.5 +/- 7.6, II: 75.6 +/- 12.3 - 17.0 +/- 13.0, III: 117.3 +/- 28 - 17.9 +/- 15.5 mmHg), decreased RVSP (I: 65.3 +/- 10.3 - 28.6 +/- 7.6, II: 91.7 +/- 11.6 - 35.0 +/- 14, III: 133.0 +/- 27.3 - 38.4 +/- 19.2 mmHg) and end-diastolic RV pressure (I: 6.2 +/- 3.0 - 5.6 +/- 7.6, II: 6.3 +/- 3.0 - 5.5 +/- 2.9, III: 8.5 +/- 3.0 - 7.2 +/- 2.3 mmHg), non-significant (p > 0.05) increase in pulmonary artery pressure in group I (15.8 +/- 1.1 - 16.8 +/- 0.9 mmHg) and II (15.8 +/- 1.2 - 17.8 +/- 1.3 mmHg) and a significant (p < 0.003) rise in group III (14.5 +/- 1.3 - 19.4 +/- 2.1 mmHg). The procedure was ineffective only in one (2.4%) child in group II, who required surgery. Complications were seen in five (3.6%) patients, including one case of a balloon being lodged in the iliac vein (surgical repair). Follow-up echo showed similar to immediate post BPV values of pressure gradients across PVS. Pre-BPV subpulmonary stenosis was seen in 5.1%, post-BPV - 15.3%, and end of follow-up - only 3.6% of children, mainly from group III. Pre-BPV tricuspid insufficiency > IIo was noted in 8.8%, significantly more frequently in group III; while in late follow-up, it was seen in 7.2%, e.g. twice as often in group III vs. groups I and II. Pulmonary regurgitation > IIo increased from 2.2% before BPV to 25.5%, i.e. 17.2%, 24.4% and 39.5%, respectively in groups I-III. Restenosis was observed in eight (5.8%) patients (group I - 1, III - 7), of whom five had re-BPV, two were operated on and one was disqualified due to insignificant restenosis. CONCLUSIONS: These long-term follow-up data confirm efficacy and safety of BPV performed in children with isolated PVS.

[Focal atrial tachycardia originating in the right atrial appendage]. / Czestoskurcz ogniskowy z uszka prawego przedsionka.

We present a case of 14-year-old boy with incessant atrial tachycardia from right atrial appendage, resistant to pharmacotherapy and with early signs of the left ventricle tachyarrhythmic dysfunction. The P-wave was positive in leads I, II, III, aVF, negative in aVR, aVL. Moreover, P waves configuration specific for this localization: negative in V1-V2 that become positive in V3-V6 was present. After first ablation session a recurrence was observed after 3 weeks, due to inadequate power delivery resulting from trabeculation/anatomy that limited conventional ablation catheter cooling. Second ablation session with the use of an active electrode cooling and 3D mapping system was successful.

[Syncope in children and adolescents]. / Omdlenia u dzieci i mlodocianych.

The report presents a definition and causes of syncope in children. Syncope differs from other states with loss of consciousness by causes leading to decreased perfusion and resultant transient cerebral dysfunction with decreased muscle tone. The most common causes of syncope noted in almost 15% of children are neurocardiogenic. This group includes vasovagal, carotid sinus reflexive, situational (coughing, dysphagia, micturation and defecation disturbances) and post-exercise syncope. Another group is represented by orthostatic syncope that may be triggered by primary and secondary dis-autonomy, decreased blood volume (hemorrhage, diarrhea, Addison's disease), some medications and substances of abuse (alcohol). An important group, accounting for 2%-6% of all cases, are cardiogenic syncope, caused mainly by congenital/acquired obstructive cardiac sub- and valvar heart defects, various cardiomyopathies, some heart tumors (e.g. myxoma), exudative pericarditis, pulmonary embolus and hypertension, congenital and acquired coronary anomalies, various significant brady-tachyarrhythmias (sick sinus syndrome, supra- and ventricular tachycardias, congenital and acquired atrio-ventricular blocks). Subclavian steal syndrome as the cause of syncope is exceptional in children. Syncope does not include loss of consciousness due to neurological and metabolic (hypoglycemia) causes, hypoxia, hyperventilation with hypocapnia or CO intoxication. Differential diagnosis should also include pseudo-syncope (hysteria). Preliminary diagnostic management should include a detailed medical history, including family history, on the frequency and circumstances of syncope, sudden deaths, a physical exam with orthostatic assessment of peripheral blood pressure and standard ECG (heart rate, intraventricular and atrioventricular conduction defects, cardiac hypertrophy, arrhythmias, L-QT, changes in ST-T). Further specialist tests depend on preliminary findings.

[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.

[Infections as a preoperative problem in patients with tetralogy of Fallot (TOF) associated with complete atrioventricular canal septal defect (AVC)]. / Infekcje jako problem przedoperacyjny u pacjentów z zespolem Fallota (TOF) skojarzonym z calkowitym kanalem przedsionkowo-komorowym (AVC).

UNLABELLED: Tetralogy of Fallot (TOF) coexisting with atrioventricular canal septal defect (AVC) is a rare combination of anomalies. Additionally, in cases with concomitant Down syndrome, recurrent infections can be a serious problem in patients (pts.) waiting for cardiosurgery treatment. The purpose of the study was an analysis of types of infections and other factors complicating the preoperative period in those patients. MATERIAL AND METHODS: The study group consisted of 17 pts. with TOF and AVC aged from 1 day to 9 years (mean 9.4 month). In this group there were 11 pts. with Down syndrome. All of them were subjected to physical examinations, blood analysis, ECG, chest X-ray and echocardiographic study. Additionally, in 8 pts. we performed catheterization. The signs of different types of infection were analyzed and results were compared in two groups: with and without Down syndrome. RESULTS: The differences were observed in the frequency of recurrent or chronic infections (21 v/s 4), time of hospitalization before surgery (17 v/s 9 days), necessity (11 v/s 3) and duration of antibiotic therapy (19 v/s 7 days) in the two studied groups. Elevated body temperature of unknown etiology was noted in 8 cases with Down syndrome, compared to 1 patient without trisomy 21. Also, the children with Down syndrome had to wait 11 days longer (19 v/s 8) for discharge after operation. CONCLUSIONS: Infections in children with TOF, AVC and Down syndrome significantly complicate the natural course of this anomaly. Prolonged preoperation time is characteristic of Down syndrome pts. compared to patients without chromosomal abnormalities.

[Evaluation of surgical treatment results of coarctation of the aorta in neonates and young infants]. / Ocena wyników operacyjnego leczenia zwezenia ciesni aorty u dzieci ponizej pierwszego roku zycia.

UNLABELLED: The authors present the analysis of surgical results obtained for coarctation of the aorta (CoA) in children less than 1 year of age. The material consisted of 103 infants (62 Males and 41 Females) aged 0.33-10.5 months (x = 3.3 +/- 2.6 months) treated between January 1, 1985 and December 31, 1999. All the patients were subjected to a detailed physical examination, ECG, chest X-ray and comprehensive echocardiography, while 12 children had additional hemodynamic studies and angiocardiography. Two groups were distinguished among the investigated children: Below 3 months of life (Group 1, N = 65) and above 3 months of life (Group 2, N = 38). In 69 infants, (including 55 from Group 1 and 14 from Group 2), the recommendation for surgery was circulatory failure, while in 34 infants (10 from Group 1 and 24 from Group 2), the recommendation for surgery considerable systemic hypertension with absent femoral pulses. In 76 patients, the Waldhausen procedure was performed, while 24 were subjected to aortic isthmus angioplasty using a Gore-Tex patch and 3 were subjected to end-to-end anastomosis. Six patients died, including 5 from Group 1 and 1 from Group 2. The remaining 97 infants (60 from Group 1 and 37 from Group 2) were followed-up for a mean period of 96.2 +/- 48.2 months. Postoperative recoarctation was encountered in 12 patients (12.4%); the condition was more predominant in Group 1 (16.6%) than in Group 2 (5.4%), but, no statistical significance was noted (Chi 2 = 2.677, p = 0.102). Despite the repair of aortic coarctation, systemic hypertension was noted in 17 children (17.5%) and it was equally common in both groups, but, twice as frequent in children with recoarctation. CONCLUSIONS: 1. CoAo correction in children below 3 months of age is associated with an increased risk of recoarctation. 2. Even when surgical treatment of CoAo is attempted very early in life, the risk of elevated systemic blood pressure is not completely eliminated.