Diastolic function evaluation in children with ventricular arrhythmia.

Premature ventricular contractions (PVC) are frequently seen in children. We evaluated left ventricular diastolic function in PVC children with normal left ventricular systolic function to detect whether diastolic function disturbances affect physical performance. The study group consisted of 36 PVC children, and the control group comprised 33 healthy volunteers. Echocardiographic diastolic function parameters such as left atrial volume index (LAVI), left atrial strains (AC-R, AC-CT, AC-CD), E wave, E deceleration time (Edt), E/E' ratio, and isovolumic relaxation time (IVRT) were measured. In the cardiopulmonary exercise test (CPET), oxygen uptake (VO2 max) was registered. Evaluation of diastolic function parameters revealed statically significant differences between the patients and controls regarding Edt (176.58 ± 54.8 ms vs. 136.94 ± 27.8 ms, p < 0.01), E/E' (12.6 ± 3.0 vs. 6.7 ± 1.0, p < 0.01), and IVRT (96.6 ± 19.09 ms. vs. 72.86 ± 13.67 ms, p < 0.01). Left atrial function was impaired in the study group compared to controls: LAVI (25.3 ± 8.2 ml/m2 vs. 19.2 ± 7.5 ml/m2, p < 0.01), AC-CT (34.8 ± 8.6% vs. 44.8 ± 11.8%, p < 0.01), and AC-R-(6.0 ± 4.9% vs. -11.5 ± 3.5%, p < 0.01), respectively. VO2 max in the study group reached 33.1 ± 6.2 ml/min/kg. A statistically significant, moderate, negative correlation between VO2 max and E/E' (r = -0.33, p = 0.02) was found. Left ventricular diastolic function is impaired and deteriorates with the arrhythmia burden increase in PVC children. Ventricular arrhythmia in young individuals may be related to the filling pressure elevation and drive to exercise capacity deterioration.

Electrocardiographic Parameters Associated with Adverse Outcomes in Children with Cardiomyopathies.

Cardiomyopathies have a low prevalence in children and thus may lead to malignant ventricular arrhythmias or the progression of heart failure, resulting in death. In adults, the QRS-T angle derived from ECG has been associated with adverse outcomes in patients with hypertrophic and dilated cardiomyopathies. We aimed to assess the electrocardiographic parameters, including QRS-T angle, associated with adverse cardiac events in children with cardiomyopathies. Forty-two children with cardiomyopathies were included in this study: 19 with dilated cardiomyopathy, 17 with hypertrophic cardiomyopathy, and 6 with left ventricular non-compaction. Additionally, 19 control subjects were recruited. In terms of ECG parameters, the QRS-T angle was significantly greater among patients with adverse outcomes compared to patients without the end points of the study (133° vs. 65°, p < 0.001). On Kaplan−Meier survival curves, QRS-T angle > 120°, increased serum concentrations of NT-proBNP and troponin I levels as well as greater NYHA or Ross scale were associated with the greatest risk of unfavorable outcome. The QRS-T angle appears to be a valuable component of 12-lead ECG interpretation, and might be helpful in outlining patients with the greatest cardiovascular risk. Additionally, serum biomarkers such as NT-proBNP (p = 0.003) and troponin (p < 0.001) are useful in outlining patients with the worst survival.

Subcutaneous Implantable Cardioverter Defibrillators for the Prevention of Sudden Cardiac Death: Pediatric Single-Center Experience.

BACKGROUND: The subcutaneous implantable cardioverter defibrillator (S-ICD) was developed as an alternative to the transvenous ICD, to prevent lead-related complications associated with the latter. The absence of intravascular or intracardiac components offers potential advantages to pediatric patients. AIMS: The aim of the study is to present an overview of our experience with S-ICDs in the pediatric center that, currently, has performed the largest number of implantations in children in Poland. METHODS: Retrospective analysis of data from medical history, qualification, implantation procedure, and S-ICD post-implantation observations in 11 pediatric patients were performed. RESULTS: S-ICDs were implanted in 11 patients, 8 boys and 3 girls, aged 12-17 years. The S-ICD was implanted for primary prevention in seven patients: four with hypertrophic cardiomyopathy (HCM), two with dilated cardiomyopathy (DCM), and one with arrhythmogenic right ventricular cardiomyopathy (ARVC). It was implanted for secondary prevention in four patients: two with sudden cardiac arrest (SCA) in the course of idiopathic ventricular fibrillation (IVF), one with long QT syndrome (LQTS) after probable SCA, and one with Brugada syndrome after SCA. In all patients, the device was implanted intramuscularly. One patient did not have a defibrillation test performed due to the presence of an intracardiac thrombus. In one patient, during screening, it was decided to implant an electrode on the right side of the sternum. There were no early or late complications with any of the procedures. So far, no inadequate discharges have been observed. CONCLUSIONS: Our results prove the efficacy of the S-ICD treatment option along with technically simple surgery, which supports its further and more widespread application in children.

Image quality and radiation doses of volumetric 320-row computed tomography angiography with prospective electrocardiogram-gating in the assessment of coronary arteries in neonates and infants.

BACKGROUND: Diagnostic imaging of coronary arteries is required in neonates and infants suspected of congenital or acquired coronary artery anomalies and in pre- and postoperative assessment of complex congenital heart diseases (CHD). AIM: Our study aimed to evaluate the image quality of volumetric 320-row computed tomography angiography (CTA) with prospective electrocardiogram (ECG)-gating for coronary arteries in neonates and infants with heart diseases, analyze factors influencing image quality and assess a radiation dose related to the procedure. METHODS: The study included 110 CTA performed in neonates and infants with CHD. RESULTS: CTA was performed in 37 girls and 73 boys at a median (interquartile range [IQR]) age of 3.0 (0.5-5.0) months, median (IQR) body weight of 5 (3.66-6.5) kg, and median heart rate (HR) of 133 (92-150) beats per minute. The orifices of the left coronary artery were visible in 100% of CTA, the orifices of the right coronary arteries were visible in 96%, whereas all coronary segments were assessable in 45% of CTA. Patients with non-diagnostic segments were significantly younger, median (IQR) age of 2.0 (0.21-5.00) months, had lower body weight of 4.6 (3.45-6.07) kg and faster HR of 136.5 (120-150) beats per minute (P <0.05) than patients with diagnostic image quality in all segments (4.0, 2-6 months, 6.0, 4.2-7 kg, and 130; 110-150 beats per minute, respectively; P <0.05). CONCLUSIONS: CTA performed with volumetric 320-row prospective ECG-gating allows for good visibility of the coronary arteries with an acceptable radiation dose. Children aged >15 days, with body weight >4.85 kg and HR <130 beats per minute are good candidates for excellent quality non-invasive CTA of all segments of coronary arteries.

A Scoping Review of Galectin-3 as a Biomarker of Cardiovascular Diseases in Pediatric Populations.

Galectin-3 as a cardiac biomarker has proven to be a significant prognostic tool in adults. However, it has not yet been established in the pediatric population as a biomarker in daily clinical practice. The aim of the study was to summarize the current knowledge on galectin-3 as a biomarker in children with cardiac conditions by reviewing the literature. Bibliographic databases such as PubMed, Web of Science and Embase were searched, and consequently twelve articles met the inclusion criteria. Supplemental handsearching of references delivered one additional source. These prospective studies concerning galectin-3 as a cardiac biomarker present analyses performed in cohorts composed of healthy children and children with cardiovascular diseases. The results, despite being based on small cohort studies, inform that galectin-3 could serve as a potential biomarker in cardiovascular risk stratification in children with heart failure, arrhythmia, Kawasaki disease or in patients undergoing cardiac surgery. The evidence for the usefulness of galectin-3 in the assessment of such pathologies as idiopathic dilated cardiomyopathy, coarctation of the aorta, functionally univentricular heart or tetralogy of Fallot were not completely confirmed. Galectin-3 seems to be a promising biomarker; however, there is a need for further research to establish its use in daily clinical practice.

Treatment of Cardiac Rhabdomyomas with mTOR Inhibitors in Children with Tuberous Sclerosis Complex-A Systematic Review.

BACKGROUND: Cardiac rhabdomyomas (CRs) are the earliest sign of tuberous sclerosis complex (TSC). Most of them spontaneously regress after birth. However, multiple and/or large tumors may result in heart failure or cardiac arrhythmia. Recently, the attempts to treat CRs with mTOR inhibitors (mTORi) have been undertaken. We reviewed the current data regarding the effectiveness and safety of mTORi in the treatment of CRs in children with TSC. METHODS: The review was conducted according to the PRISMA guidelines. Medline, Embase, Cochrane library, and ClinicalTrial.gov databases were searched for original, full-text articles reporting the use of mTORi (everolimus or sirolimus) in the treatment of CRs in children with TSC. RESULTS: Thirty articles describing 41 patients were identified (mostly case reports, no randomized or large cohort studies). Thirty-three children (80.5%) had symptomatic CRs and mTORi therapy resulted in clinical improvement in 30 of them (90.9%). CRs size reduction was reported in 95.1%. Some CRs regrew after mTORi withdrawal but usually without clinical symptoms recurrence. The observed side effects were mostly mild. CONCLUSIONS: mTORi may be considered as a temporary and safe treatment for symptomatic CRs in children with TSC, especially in high-risk or inoperable tumors. However, high-quality, randomized trials are still lacking.

Intracardiac tumor as a rare manifestation of genetic syndromes-presentation of a family with Gorlin syndrome and a literature review.

Intracardiac tumors in children are relatively rare, but their clinical consequences may include severe outflow tract obstruction, embolism, cardiac insufficiency, or rhythm disturbances. In some cases, the tumor may constitute part of a genetic condition and prompt additional investigations, as well as a modification of therapeutic management. Herein, we present a molecularly confirmed familial case of Gorlin syndrome with an early cardiac tumor as a presenting sign. We provide detailed clinical characteristics of the affected individuals and a useful review of syndromic causes of pediatric cardiac tumors in clinical practice.

Long-term results of percutaneous balloon aortic valvuloplasty in children with aortic stenosis: a single-center experience.

BACKGROUND: Percutaneous balloon aortic valvuloplasty (BAV) is an effective method of treatment for aortic stenosis in children. AIMS: This study aimed to assess the long­term results of BAV in various age groups in the pediatric population. METHODS: This retrospective study included 68 patients (newborns, infants, and children older than 1 year). We analyzed their demographic data and measurements performed with the use of cardiac catheterization, angiography, and echocardiography at 3 time points: before, directly after, and at late follow­up after BAV, including the invasive aortic valve peak­to­peak systolic pressure gradient (PGpeak), maximum systolic pressure gradient (PGmax) by Doppler echocardiography, and the severity of aortic regurgitation (AR). Long­­term follow­up data were available for 57 patients. RESULTS: The BAV procedure led to a significant reduction in the PGpeak values. Late follow­up revealed a progression of AR. Aortic regurgitation of grade 2 or higher directly after BAV was the strongest risk factor for severe AR at late follow­up. After 5, 10, and 18 years after BAV, surgical treatment was not required in 90%, 77%, and 59.5% of patients, respectively. Severe AR and a residual PG less than 35 mm Hg at late follow­up were stronger risk factors for aortic valve surgery than moderate AR and PG higher than or equal to 35 mm Hg. CONCLUSIONS: Balloon aortic valvuloplasty is an effective method of treatment for aortic stenosis in children, which delays the need for surgery. The progression of AR occurs at late follow­up. A greater reduction in PGmax is a risk factor for at least moderate AR directly after BAV, which results in the progression of AR at late follow­up.

Initial experience of catheter ablation for cardiac arrhythmias in children and adolescents at a newly built ablation centre.

BACKGROUND: Catheter ablation (CA) therapy is the first-choice treatment in adults with heart rhythm disturbances. Arrhythmias in adults are mainly conditioned by coronary artery disease. Aetiology of arrhythmias in children is mostly associated with inherited heart disorders. According to the current guidelines, CA is widely used in children, indicating the need to make it more achievable in the paediatric population. AIM: To assess the efficacy and safety of CA in children with different types of arrhythmias on the initial learning curve at a newly built Ablation Centre in the Independent Paediatric Hospital of the Medical University of Warsaw, Poland. METHODS: The study population comprised 32 children with supraventricular tachycardias, asymptomatic pre-excitation syndrome, or ventricular ectopic beats undergoing CA. The mean age of the study population was 14.1 ± 2.4 years. In all patients, electrophysiological study (EPS) and CA were performed. Analysis with respect to procedure duration, fluoroscopy exposure duration, location of accessory pathways (AP), success rate, recurrences, and complications was performed. RESULTS: The mean procedure duration was 105.4 ± 41.4 min (range 40-175 min). The mean fluoroscopy duration was 8:34 ± 5:01 min (range 1:28-21:01). The mean exposure to ionising radiation was 4.7 ± 3.2 mcG/kg. EPS revealed significantly more frequent presence of AP in the left side (57.1%). The radiofrequency ablation procedure was successful in 26 of 32 (81.3%) children, and cryoablation was successful in two of four patients. In two (6.3%) children minor complications occurred. CONCLUSIONS: Catheter ablation may be effectively performed without major complications in the initial phase of the learning curve if a reasonable approach with a gradual increase of the procedural complexity is taken.

Ultrasonography of the brain, abdomen, and heart in neonates born to liver or renal transplant recipient mothers.

BACKGROUND: Pregnancies in graft recipients are associated with increased risk of a number of pathologies. The aim of the study was to analyze results of brain and abdominal ultrasonography and echocardiography (ECHO) in neonates born to liver (LTx) or renal recipients (RTx). MATERIAL/METHODS: The study group consisted of 82 neonates born to transplanted women (46 neonates of liver recipients and 36 neonates of renal recipients), enrolled in a retrospective study. The control group consisted of 74 neonates from the general population. Sonographic examination of the brain was performed to check for the presence of intra-/periventricular hemorrhage (IVH/PVH) according to Papile, and periventricular leukomalacia (PVL).The results of abdominal ultrasonography and 2-dimensional echocardiography (ECHO) were compared between the groups. The immunosuppressive therapy used during pregnancy was also analyzed. RESULTS: No significant differences were observed between the frequency of IVH in LTx and RTx groups and LTx, RTx, and control groups. Abdominal ultrasonography revealed 1 case of suprarenal hemorrhage, 1 case of cystic kidney, and 3 cases of pyelocalyceal system dilatation in the study group. There were no abnormalities in the echocardiography in 97.8% of children born to mothers after LTx and in 94.4% after RTx. There were significant differences in the immunosuppressive therapy between the pregnant women after LTx and RTx. CONCLUSIONS: The risk was not increased in intra-/periventricular hemorrhage and congenital abnormalities of the gastrointestinal tract and heart in neonates of mothers after organ transplantation, regardless of the immunotherapy used, and risk was similar to that of the general population.

Assessment of left ventricular diastolic function in children after successful repair of aortic coarctation.

The purpose of the study was an assessment of left ventricular diastolic function in children after the successful repair of aortic coarctation (CoA). The prospective study concerned 32 pediatric patients after the CoA surgery. Tissue Doppler imaging parameters including strain and strain rate and the conventional echocardiographic indexes were analyzed in patients and healthy controls. Analysis of mitral annulus velocities, E-E' ratio, strain, and strain rate of left ventricular mid-cavity segments and conventional indexes of mitral inflow showed the worsening of left ventricular diastolic mechanics in the study group compared to healthy controls. The E/E' ratio was significantly higher in the study group compared to the control group (8.30 ± 3.24 vs. 6.95 ± 1.36; p < 0.05). The early diastolic strain rate to late diastolic strain rate ratio as well as early to late diastolic strain ratio of the left ventricular mid-cavity segments were significantly lower in the study group compared to healthy controls (1.81 ± 0.63 vs. 3.74 ± 1.53; p < 0.001 and 1.20 ± 0.49 vs. 3.41 ± 1.26; p < 0.001). No differences of the pulmonary venous flow parameters between those two groups were observed. The left ventricular diastolic mechanics in hypertensive patients after CoA repair did not differ from normotensive subjects. Hypertensive and normotensive children after surgical repair of CoA are found to have worsening of the left ventricular diastolic mechanics suggesting the impairment of the active myocardial relaxation.

Assessment of left ventricular systolic function using tissue Doppler imaging in children after successful repair of aortic coarctation.

UNLABELLED: SUMMARY AIM: Assessment of left ventricular systolic function in children after the successful repair of aortic coarctation using tissue Doppler imaging (TDI). METHODS: The study group consisted of 32 patients (mean age 12.0 +/- 4.2 years) after the aortic coarctation repair. The TDI parameters and the conventional echocardiographic endocardial and midwall indices of the left ventricular systolic function were analysed and compared with the results obtained from 34 healthy children. RESULTS: The systolic mitral annulus motion velocity, systolic myocardial velocity of the medial segment of the left ventricular septal wall, left ventricular strain and Strain Rate (SR) in the study group were significantly higher than in the control group, respectively: 6.92 +/- 0.75 cm s(-1) versus 6.45 +/- 0.83 cm s(-1); 5.82 +/- 1.03 cm s(-1) versus 5.08 +/- 1.11 cm s(-1); -28.67 +/- 6.04% versus -22.53 +/- 6.44% and -3.20 +/- 0.76 s(-1) versus -2.39 +/- 0.49 s(-1). Except midwall shortening fraction the conventional endocardial and midwall echocardiographic indices in the study group were significantly higher in comparison to the healthy controls. The left ventricular systolic meridional fibre stress and end-systolic circumferential wall stress did not differ between the examined groups. There were no differences of the TDI or conventional parameters between hypertensive and normotensive patients. CONCLUSIONS: Left ventricular systolic performance in children after the surgical repair of aortic coarctation reveals tendency to rise in late follow-up despite a satisfactory result after surgery. Higher systolic strain and SR in children treated due to coarctation of the aorta may suggest the increased preserved left ventricular performance despite normalization of afterload.

[Mitral valve insufficiency in a 2.5-year-old girl as a result of endocarditis? A case report]. / Niedomykalnosc zastawki dwudzielnej u 2.5-letniej dziewczynki--stan po przebytym zapaleniu wsierdzia?

We present a case of a 2.5-year-old girl with a history recurrent pneumonia, followed by decreased physical capacity. Echocardiography showed severe (grade IV) mitral regurgitation with anterior leaflet perforation. The patient was selected for cardiac surgery. The possibility of endocarditis as a cause of mitral valve insufficiency is discussed.

[Cardiac tamponade in a neonate with cardiac tumour]. / Tamponada osierdzia u noworodka z guzem serca.

A case of a neonate with a life-threatening cardiac tamponade due to cardiac tumour is presented. Pericardiocentesis was performed and 300 ml of purulent fluid was evacuated. Pericardial drainage was undertaken. Six months later cardiac tamponade recurred and was again successfully treated with pericardial drainage. A subsequent 10-month follow-up was uneventful. Diagnosis and treatment of cardiac tumours associated with pericardial effusion are discussed.