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.

Changing face of acute rheumatic fever in childhood and our clinical results.

OBJECTIVE: This study aims to evaluate the demographic and clinical findings of acute rheumatic fever (ARF) patients followed up in our clinic, their responses to treatment, and prognoses and to determine the clinical utility of echocardiography (ECHO) in the diagnosis of ARF. METHODS: We retrospectively evaluated the data of 160 patients with ARF (6-17, mean 11.7±2.3 years, F/M: 88/72) that was diagnosed according to the Jones criteria and followed up in the pediatric cardiology clinic between January 2010 and January 2017. RESULTS: About 29.4% (n=47) of 104 patients with rheumatic heart disease (RHD) had subclinical carditis. It was observed that subclinical carditis was most common in patients with polyarthralgia (52.2%); in contrast, clinical carditis was most commonly observed together with chorea (39%) and polyarthritis (37.1%). It was found that 60% (n=96) of the patients with rheumatic fever were between the ages of 10-13 and 31.3% (n=50) presented arthralgia most frequently in the winter months. The most common concomitant major symptoms were carditis + arthritis (35%) and carditis + chorea (19.4%). In patients with carditis, the most affected valves were mitral (63.8%) and aortic (50.6%) valves, respectively. The prevalence of monoarthritis, polyarthralgia, and subclinical carditis increased in cases diagnosed during and after 2015. The cardiac valve involvement findings of 71 of 104 patients (68.2%) with carditis improved during the approximately 7 years of follow-up. The regression of heart valve symptoms was significantly higher in patients with clinical carditis and those that complied with prophylaxis compared to patients with subclinical carditis and those that did not comply with prophylaxis. CONCLUSION: We conclude that ECHO results should be included in the diagnostic criteria of ARF, and that subclinical carditis is associated with a risk of developing permanent RHD. Secondary prophylaxis non-compliance is significantly associated with recurrent ARF, and early prophylaxis can reduce the prevalence of RHD in adults and potential associated complications.

Multisystem inflammatory syndrome in children: clinical presentation, management, and short- and long-term outcomes.

OBJECTIVE: In this study, it was aimed to evaluate the demographic, clinical and laboratory characteristics of MIS-C patients in our hospital, to share our treatment approach, and to assess the outcomes of short- and long-term follow-up. METHODS: MIS-C patients who were admitted and treated in our hospital between July 2020 and July 2021 were evaluated. Demographic, clinical, laboratory, and follow-up data were collected from patient records retrospectively. RESULTS: A total of 123 patients with MIS-C (median age, 9.6 years) were included the study. Nineteen (15.4%) were mild, 56 (45.6%) were moderate, and 48 (39%) were severe MIS-C. High CRP, ferritin, pro-BNP, troponin, IL-6, and D-dimer values were found in proportion to the severity of the disease (p < 0.001, p < 0.001, p < 0.001, p < 0.001, p = 0.005, p < 0.001), respectively. Two (1.6%) patients died. The mean follow-up period was 7.8 months. Valve failure, left ventricular dysfunction/hypertrophy, coronary involvement, and pericardial effusion were the most common cardiac pathologies in the short- and long-term follow-up of the patients. In the long-term follow-up, the most common reasons for admission to the hospital were recurrent abdominal pain (14.2%), cardiac findings (14.2%), pulmonary symptoms (8%), fever (7.1%), neuropsychiatric findings (6.2%) and hypertension (3.5%). Neuropsychiatric abnormalities were observed significantly more common in severe MIS-C patients at follow-up (p = 0.016). In the follow-up, 6.2% of the patients required recurrent hospitalization. CONCLUSION: MIS-C is a serious and life-threatening disease, according to short-term outcomes. In addition to the cardiac findings of patients with MIS-C, long-term outcomes such as neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms should be monitored. Key Points • In MIS-C patients, attention should be paid not only to cardiac findings, but also to symptoms related to other systems. • Patients should be followed up in terms of neuropsychiatric findings, persistent gastrointestinal symptoms, fever and pulmonary symptoms that may occur during follow-up.

Role of Biological Agents in the Treatment of SARS-CoV-2-Associated Multisystem Inflammatory Syndrome in Children.

OBJECTIVES: The aims of this study were to evaluate the role of biological agents in the treatment of severe multisystem inflammatory syndrome in children (MIS-C) and to assess the current application, outcomes, and adverse effects in patients who are followed up in a pediatric intensive care unit (PICU). PATIENTS AND METHODS: This observational, descriptive, medical records review study was performed on patients with MIS-C admitted to the PICU between September 1 and November 1, 2020. Through medical records review, we confirmed that patients were positive for current or recent SARS-CoV-2 infection or for COVID-19 exposure history within the 4 weeks before the onset of symptoms. RESULTS: A total of 33 patients with severe MIS-C were included (21 male) with a median age of 9 years. The most common signs and symptoms during disease course were fever (100%) and abdominal pain (75.5%). Clinical features of 63.6% patients were consistent with Kawasaki disease/Kawasaki disease shock syndrome, and 36.4% were consistent with secondary hemophagocytic lymphohistiocytosis/macrophage activation syndrome. Myocardial dysfunction and/or coronary artery abnormalities were detected in 18 patients during the PICU stay. Intravenous immunoglobulin and corticosteroids were given to 33 patients. Anakinra was administered to 23 patients (69.6%). There was a significant increase in lymphocyte and platelet counts and a significant decrease in ferritin, B-type natriuretic peptide, and troponin levels at the end of the first week of treatment in patients who were given biological therapy. Two patients were switched to tocilizumab because of an insufficient response to anakinra. The mortality rate of MIS-C patients admitted in PICU was 6.0%. CONCLUSIONS: Management of systemic inflammation and shock is important to decrease mortality and the development of persistent cardiac dysfunction in MIS-C. The aggressive treatment approach, including biological agents, may be required in patients with severe symptoms and cardiac dysfunction.

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.