Transcatheter Ventricular Septal Defect Closure with Lifetech™ Konar-MF Occluder in Infants Under 10 kg with Only Using Venous Access.

Transcatheter closure of VSD remains a complex procedure in infants with technical challenges and carries the risk of significant complications, due to its complex anatomical morphology and closed proximity to the atrioventricular valves and the conduction system. In this article, we presented transcatheter VSD closure in infants under 10 kg using the Lifetech Konar-MF device via only venous route without TEE guidance and arterial access. Between January 2021 and May 2023, a total of 34 patients weighing less than 10 kg who underwent transcatheter VSD closure antegradely with Lifetech™ Konar-Multifunctional (MF) occluder were included in the study. The mean age of the patients was 8.1 (3.5-35) months. Average weight was 6.5 kg (range 4.5-10 kg). VSD was perimembranous in 27 patients (79.4%). Successful device placement was achieved in all 34 patients. However, device embolization occurred in three patients. One of the patients was successfully implanted with a one size larger device, the surgical closure was performed other two cases. TR occurred in seven patients (20.6%) after releasing devices. None of the patients developed complete heart block. Right bundle branch block developed in two patients. Residual shunt was observed in 9 patients (six small, two moderate, and one large). During follow-up, residual shunt disappeared in six of these patients and only mild residual shunt remained in the other four patients which have not required any further intervention. Transcatheter closure of VSD with Lifetech Konar-MF device is safe and effective in infants less than 10 kg via only venous access with a high success rate and low complication rate. In these patients, transcatheter VSD closure can be performed by excluding the risk of complications that may occur due to AV loop formation, arterial intervention, endotracheal intubation and TEE use.

Adaptation of adult right ventricular scoring systems to pediatric patients undergoing continuous LVAD implantation: Feasible or not?

PURPOSE: The aim of this study is to investigate the efficacy of adaptation of right ventricular (RV) risk scores used in adult patients to pediatric age group undergoing LVAD implantation. METHODS: Twenty-two pediatric patients who underwent LVAD implantation were retrospectively reviewed from January 2014 to September 2018. Preoperative patient characteristics, hemodynamic parameters, and echocardiographic data were collected. Adult RV risk scores were calculated for all patients. Effects of all the parameters on RV function were also investigated. Study endpoints were RVF and in-hospital mortality. RESULTS: Eleven (50%) of 22 patients were male. The mean age of the patients was 13.4 ± 3.8 years. The mean body surface area of the patients was 1.4 ± 0.4 m2. In five patients BiVAD implantation was performed. Of these five BiVAD patients two underwent successful heart transplant; two weaned from temporary RVAD and last patient died due to multi-organ failure. Four patients showed signs of early RVF; one patient was transplanted successfully while on medical support. Three patients developing RVF did not respond medical therapy necessitating ECMO and died in the early postoperative period. All risk scores and potential predictive factors were evaluated individually and in combination of several parameters. No significant predictor for RVF in pediatric patients that underwent LVAD implantation was found (p > 0.05). CONCLUSION: Neither an adult risk score nor a predictive factor was successful in predicting RVF, alone or in combination due to limited number of patients and events. Large further investigations are needed to identify the predictors or scoring system in pediatric population.

A case series of intracardiac thrombi and vascular involvement in pediatric Behçet's disease.

To evaluate the general characteristics of pediatric Behçet's disease (BD) patients with thrombus and to present the clinical features, treatment responses and prognosis of patients with intracardiac thrombus. The clinical characteristics and outcomes of 15 patients with thrombus among 85 pediatric BD patients followed in the Department of Pediatric Rheumatology were evaluated retrospectively. Of the 15 BD patients with thrombus, 12 (80%) were male, 3 (20%) were female. The mean age at diagnosis was 12.9 ± 1.1 years. Thrombus was present at the time of diagnosis in 12 patients (80%), while thrombus developed in three patients within the first three months after diagnosis. The most common site of thrombus was the central nervous system (n = 9, 60%), followed by deep vein thrombus (n = 6, 40%) and pulmonary artery thrombus (n = 4, 26.6%). Three male patients (20%) developed intracardiac thrombus. The overall intracardiac thrombus rate in the 85 patients was 3.5%. Two of the three patients had thrombus in the right, and one had thrombus in the left heart cavity. In addition to steroids, 2 of the 3 patients received cyclophosphamide, while the patient with thrombus localized in the left heart cavity was given infliximab. In the follow-up, the two patients with thrombus in the right heart cavity were switched to infliximab because of resistance to cyclophosphamide. Complete resolution was observed in 2 of the 3 patients on infliximab; a significant reduction in the thrombus of the other patient was achieved. Intracardiac thrombus is a rare presentation of cardiac involvement in BD. It is usually observed in males and in the right heart. Although steroids and immunosuppressive agents such as cyclophosphamide are recommended as first-line treatment, favorable outcomes can be achieved with anti-TNFs in resistant cases.

The Relationship between Arterial Elasticity Parameters of Ascending Aorta, Abdominal Aorta and Carotid Arteries with Carotid Intima Media Thickness in Children with Bicuspid Aortic Valve.

BACKGROUND: Bicuspid aortic valve is a congenital cardiac malformation that affects not only the valve and ascending aorta but also the abdominal aorta and large central arter-ies like carotid arteries by damaging the elasticity of the vessel resulting in increased stiffness and reduced distensibility. Deterioration of aortic compliance disturbs functions of the left ventricle and triggers atherosclerosis determined with carotid intima-media thickness. The aim of this study was to assess the effect of the bicuspid aortic valve on the elastic properties of these parts of the arterial system in children. METHODS: Thirty-four children with bicuspid aortic valves with normal valvular functions or mild valvular dysfunction and a control group of 34 individuals with tricuspid aortic valves were included in the study. Echocardiographic measurements of the left ventri- cle, ascending aorta, and ultrasonographic measurements of the abdominal aorta and carotid arteries were performed, and elasticity indexes were calculated. RESULTS: The bicuspid aortic valve group had higher stiffness and lower distensibility in ascending aorta, abdominal aorta, and carotid arteries with higher carotid intima- media thickness values than the tricuspid aortic valve group. Aortic valvular z scores and ascending aorta and abdominal aorta stiffness were higher in patients with bicus- pid aortic valves irrespective of valvular functions than in controls. Valvular dysfunction affected stiffness in carotid arteries. Dilatation of ascending aorta increased stiffness in the abdominal aorta. Distensibility was lower in ascending aorta and left carotid artery, with increased carotid intima-media thickness independent from ascending aorta dila- tation. Stiffness of abdominal aorta revealed a positive correlation with the stiffness of the ascending aorta and the carotid arteries (P < .05, for all). CONCLUSIONS: Elasticity indexes of children with bicuspid aortic valves were impaired in ascending aorta, abdominal aorta, and carotid arteries with an increase in carotid intima-media thickness.

Echocardiographic Findings and Correlation with Laboratory Values in Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19.

Cardiac involvement is a common and serious problem in multisystem inflammatory syndrome in children (MIS-C). Echocardiographic evaluation of systolic and diastolic function by traditional, tissue Doppler and three-dimensional (3D) echocardiography was performed in consecutive 50 MIS-C patients during hospitalization and age-matched 40 healthy controls. On the day of worst left ventricular (LV) systolic function (echo-1), all left and right ventricular systolic function parameters were significantly lower (p < 0.001), E/A ratio was significantly lower, and averaged E/e' ratio was significantly higher (median 1.5 vs. 1.8, p < 0.05; 8.9 vs. 6.3, p < 0.001 respectively) in patients compared to control. Patients were divided into 2 groups according to 3D LV ejection fraction (LVEF) on the echo-1: Group 1; LVEF < 55%, 26 patients, and group 2; LVEF ≥ 55%, 24 patients. E/e' ratio was significantly higher in group 1 than group 2 and control at discharge (median 7.4 vs. 6.9, p = 0.005; 7.4 vs. 6.3, p < 0.001 respectively). Coronary ectasia was detected in 2 patients (z score: 2.53, 2.6 in the right coronary artery), and resolved at discharge. Compared with group 2, group 1 had significantly higher troponin-I (median 658 vs. 65 ng/L; p < 0.001), NT-pro BNP (median 14,233 vs. 1824 ng/L; p = 0.001), procalcitonin (median 10.9 vs. 2.1 µg/L; p = 0.009), ferritin (median 1234 vs. 308 µg/L; p = 0.003). The most common findings were ventricular systolic dysfunction recovering during hospitalization, and persisting LV diastolic dysfunction in the reduced LVEF group at discharge. Coronary artery involvement was rare in the acute phase of the disease. Also, in MIS-C patients, the correlation between LV systolic dysfunction and markers of inflammation and cardiac biomarkers should be considered.

Transcatheter closure of post-operative type 1 Gerbode defect by Amplatzer Duct Occluder 2.

Acquired and congenital left ventricular to right atrial communication is rare, but nowadays, the frequency of the iatrogenic subgroup is increasing. Successful transcatheter closure of these defects with different devices has been reported. Herein, we presented successful closure of left ventricular to right atrial communication with Amplatzer Duct Occluder 2 after attempting to close with a failed Amplatzer Vascular Plug II device in a 7-year-old girl. This report supports that transcatheter closure of iatrogenic Gerbode defect with Amplatzer Duct Occluder 2 device is safe and effective.

Therapeutic plasma exchange: A potential management strategy for critically ill MIS-C patients in the pediatric intensive care unit.

This study aimed to evaluate the effectiveness and the role of therapeutic plasma exchange (TPE) in treatment of children with severe MIS-C. In addition, we assessed demographic data, clinical features, laboratory abnormalities, underlying conditions, treatments, and outcomes. Patients with severe MIS-C who were admitted to the pediatric intensive care unit (PICU) between September 01 and October 05, 2020 were included in this observational, descriptive, retrospective study. The data collected included the patients' demographic data, presenting symptoms, clinical features, laboratory parameters, diagnostic investigations, and medications. Of 27 children with MIS-C, 63 % were male. The median age of the patients was nine years. Intravenous immunoglobulin and corticosteroids were used for treatment in 100 % of the patients, anakinra in 51.8 %, vasopressors in 85.1 %, noninvasive mechanical ventilation in 25.9 %, and invasive mechanical ventilation in 18.5 %. Ten of the 27 patients (37 %) underwent TPE. In the patients who underwent TPE, the median PELOD score was 21 (IQR: 11-30.25) before TPE and 10 (IQR: 10-11) after TPE (p < 0.001). Moreover, their median left ventricular ejection fraction (LVEF) was 52 % (IQR: 49.25 %-55 %) before TPE and median LVEF was 66.5 (IQR: 58 %-68.5 %) after TPE (p = 0.012). The median number of TPE sessions was three (IQR: 2-4.75). The mortality rate of the patients with severe MIS-C admitted to the PICU was 7.4 %. We suggest that TPE should be considered as a therapeutic option in children with severe MIS-C. Early initiation of TPE followed by immunomodulatory therapy in critically ill children with MIS-C may help improve clinical and laboratory outcomes.

Comparison of baseline laboratory findings of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis and multisystem inflammatory syndrome in children.

AIMS: Recently, multisystem inflammatory syndrome in children (MIS-C) has been recognized in association with coronavirus disease 2019 as a cytokine storm syndrome. MIS-C presents with symptoms similar to Kawasaki disease and macrophage activation syndrome (MAS). We aimed to better understand this cytokine storm syndrome by comparing the initial laboratory findings of MIS-C and MAS. METHODS: Patients who were diagnosed with MAS due to systemic juvenile idiopathic arthritis in our clinic between March 2002 and November 2020 and with MIS-C between 20 September and 20 October 2020 were enrolled into the study. The medical files of all patients were reviewed retrospectively. RESULTS: A total of 13 MAS (9 boys, 4 girls) and 26 MIS-C (16 boys,10 girls) patients were included in the study. Hemoglobin, absolute neutrophil and lymphocyte counts, C-reactive protein (CRP), ferritin, fibrinogen and lactate dehydrogenase (LDH) levels showed significant differences between the two groups (P < 0.05). Patients with MAS had lower hemoglobin (10.10 g/dL) and fibrinogen (2.72 g/dL), but higher ferritin (17 863 mg/dL) and LDH (890.61 U/L) at the time of diagnosis. Patients with MIS-C had higher absolute neutrophil count (12 180/mm3 ) and CRP (194.23 mg/dL) values, but lower absolute lymphocyte count (1140/mm3 ) at the time of diagnosis. Left ventricle ejection fraction was significantly lower in the MIS-C group in echocardiographic evaluation (P < 0.001). CONCLUSION: Ferritin, hemoglobin, LDH, and fibrinogen levels were significantly changed in MAS compared with MIS-C. However, patients with MIS-C have more severe signs than MAS, such as cardiac involvement.

Midodrine treatment in children with recurrent vasovagal syncope.

BACKGROUND: Vasovagal syncope is the most common cause of syncope in childhood and its treatment is not at a satisfactory level yet. We aimed to investigate patients who were diagnosed with vasovagal syncope, did not benefit from conventional treatment, received midodrine treatment, and to evaluate their response to midodrine treatment. METHODS: Files of 24 patients who were diagnosed with recurrent vasovagal syncope, did not benefit from non-pharmacological treatments, and received midodrine treatment during June 2017-October 2019 were retrospectively analysed. RESULTS: In total, 24 patients received a treatment dose of midodrine at 5 mg/day (2.5 mg BID) included in the study. The mean number of syncope was 5.75 ± 2.67 prior to treatment. Following treatment, the mean number of syncope was 0.42 ± 0.89. It was observed that syncope episodes did not recur in 17 patients, but it recurred in 4 out of 7 patients in the first 3 months of the treatment and did not recur in the following months. The episodes improved in two patients with an increase in the treatment dose, but the syncope episodes continued in only one patient. CONCLUSION: It was concluded that midodrine treatment was effective and safe in adolescents with recurrent vasovagal syncope.

Assessment of Cardiac Arrhythmic Risk in Children With Covid-19 Infection.

Coronavirus disease of 2019 (COVID-19) is a cause of significant morbidity and mortality worldwide. Although COVID-19 clinical manifestations are mainly respiratory, major cardiac complications are being reported. The mechanism of cardiac injury and arrhythmias is unclear. Also, drugs currently used to treat the COVID-19 may prolong the QT interval and may have a proarrhythmic propensity. The study aims to investigate the effects of COVID-19 infection with asymptomatic and mild symptoms on trans-myocardial repolarization parameters in children without treatment. A total of 105 COVID-19 patients were compared with 40 healthy children. The patient and control group data were compared by calculating the QT interval, corrected QT (QTc), QT dispersion (QTd), QTc dispersion (QTcd), Tp-e, Tp-e dispersion, Tp-e/QT ratio, and Tp-e/QTc ratio on the 12-lead surface electrocardiogram. The mean age was determined as 11.2 ± 0.3 years in the patient group, and 10.8 ± 2.1 years in the control group. In the COVID-19 group, QTd, QTcd, Tp-e, Tp-e dispersion, Tp-e/QT ratio and Tp-e/QTc ratio were statistically higher than the control group. The ventricular repolarization was impaired even in asymptomatic children with COVID-19 infection. These results suggest the need to further assess the long terms risks of prolonged QT dispersion in the setting of COVID-19 infection.

The evaluation of right ventricular systolic function in patients with repaired Tetralogy of Fallot by conventional echocardiographic methods and speckle tracking echocardiography: Compared with the gold standard cardiac mangenetic resonance.

BACKGROUND: Right ventricular (RV) function is one of the important prognostic factors in patients with repaired Tetralogy of Fallot (TOF). We aimed to assess RV function by conventional echocardiography and two-dimensional speckle tracking echocardiography (2D-STE) in patients with repaired TOF. METHODS: Twenty-seven (male 17) adolescents and young adult patients (mean age 22.7 ± 6.7 years) operated on for TOF and age- and gender-matched healthy controls were included. RV systolic function in both groups were evaluated by fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), RV myocardial performance index (RVMPI) and tricuspid annular peak systolic velocity by pulsed tissue Doppler (tricuspid S'), and also RV global longitudinal strain (RV GLS) obtained from STE. These results were compared with RV ejection fraction (RVEF) measured by cardiac magnetic resonance imaging (CMRI) performed within 3 months in patient group. RESULTS: Systolic RVGLS values were significantly lower in patients compared to controls (-17.4 ± 3.1% vs -25.6 ± 3%). Among the echocardiographic parameters, RVGLS had the best correlation with RV EF derived from CMRI (r: -.6). By receiver operating characteristics analysis (ROC), an RV GLS cutoff value of -17.4% had 75% sensitivity and 68.4% specificity in identifying RVEF <45% with an area under curve 0.743 (P < .05). The intra- and inter-observer agreement for RV GLS was excellent. CONCLUSION: RVGLS is an easy, effective, feasible, and reproducible tool in the evaluation of RV systolic function. So, RVGLS measurement by STE may be one of the routine echocardiographic parameters in the evaluation of RV systolic function in patients with repaired TOF.

Transesophageal and invasive electrophysiologic evaluation in children with Wolff-Parkinson-White pattern.

BACKGROUND: Risk stratification for Wolff-Parkinson-White (WPW) pattern either by noninvasive or invasive tests is important to determine whether an ablation is necessary or not. The aim was to compare noninvasive tests and invasive studies in a pediatric WPW population. METHODS: A total of 71 WPW patients (median age 14 years [interquartile range, 11-16 years]; 43 male) underwent Holter monitoring, exercise stress test (EST), and transesophageal electrophysiological study (TEEPS). In the case of a ≤270-ms effective refractory period of accessory pathway or induction of supraventricular tachycardia using TEEPS, patients were classified as high risk and underwent invasive electrophysiological study (EPS). RESULTS: Nine of 23 patients with low risk by Holter and eight of 26 patients with low risk by EST had high risk in TEEPS. Risky TEEPS results were detected in 42 of 71 patients and those patients underwent EPS. High-risk characteristic in EPS was detected in seven of nine patients with low risk by Holter and in seven of eight with low risk by EST. Both tests yielded moderate specificity (52-69%) and negative predictive value (61-69%) according to TEEPS. CONCLUSION: Holter and EST have low predictive value in WPW risk stratification. However, TEEPS and EPS are extremely valuable in WPW risk stratification. TEEPS may reveal important and useful results for WPW risk determination, especially in small children not having undergone EPS in order to avoid its complications.