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.

Early outcomes of the treatment of aortic coarctation with BeGraft aortic stent in children and young adults.

We report our experience and early outcomes of using the BeGraft aortic stent in children, adolescents, and young adults. BeGraft aortic stent (Bentley InnoMed, Hechingen, Germany) requires a smaller long sheath compared to other covered stents, and it has a low profile and adequate radial power. With these features, it can overcome some limitations in the treatment of coarctation, especially in children. This is a single centre retrospective analysis of 11 implanted BeGraft aortic stents in coarctation of the aorta between July 2020 and November 2021. The eleven stents were successfully implanted in 11 patients (10 males). The median age of the patients was 13.7 years (interquartile range 12-16 years), and the median weight was 43 kg (interquartile range 35-62 kg). In five patients, after the stents were opened completely by the first balloon, they were exchanged with a Z-MED II™ balloon, 1-3 mm larger in diameter, and the stents were redilated. The median catheter-derived systolic peak-to-peak pressure gradient was 23 mm Hg (interquartile range 16-37 mmHg) before the procedure and 3 mm Hg (interquartile range 1-5 mm Hg) after the procedure. Except for the partial femoral artery thrombosis in two patients, no other procedural complications were observed in our study. The median follow-up duration was 5 months (interquartile range 2-12 months). During follow-up, only one patient (9%) had stent narrowing that required dilation. Our initial results and short-term follow-up showed that the BeGraft aortic stent implantation and redilation can be performed effectively, safely, and successfully in the treatment of coarctation of the aorta.

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.

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.

Association of vitamin D deficiency with ventricular repolarization abnormalities.

BACKGROUND: Vitamin D is involved in cardiac contractility and myocardial calcium hemostasis, and vitamin D deficiencies are known to cause various cardiovascular disorders and have been linked with sudden cardiac death. AIM: The aim of the study was to evaluate repolarization distribution, represented by QT interval, corrected QT interval (QTc), QT dispersion, Tpeak­to­Tend (Tp­e) interval, Tp­e/QTc ratio, JT interval, JT dispersion, and Tp­e/JTpeak ratio in children with vitamin D deficiency. Moreover, we aimed to determine the relationship between ventricular repolarization anomalies and vitamin D deficiency. METHODS: The study included 50 adolescent patients with vitamin D deficiency (vitamin D <20 ng/ml), 50 adolescent patients with vitamin D insufficiency, and 50 age­matched controls (vitamin D level >30 ng/ml). QTc duration, QT dispersion, JTpeak duration, JT dispersion, Tp­e, Tp­e/JTpeak ratio, and Tp­e/QTc ratio were recorded on electrocardiogram. RESULTS: Patients with vitamin D deficiency or insufficiency had longer Tp­e interval (P <0.001), while Tp­e/QTc and Tp­e/JTpeak ratios were found to be increased in the same group of patients (P = 0.005 and P <0.001, respectively). QT dispersion and JT dispersion were higher in the deficient group when compared with the other groups (P = 0.045 and P = 0.02, respectively). CONCLUSION: The present study, conducted in a pediatric population, is the first in the current literature to assess the relationship between ventricular repolarization anomalies and vitamin D deficiency.

Syncope due to acute rheumatic fever with pacemaker-like syndrome.

Bagrul D. Syncope due to acute rheumatic fever with pacemakerlike syndrome. Turk J Pediatr 2019; 61: 810-814. The prolongation in the PR interval on the electrocardiogram is one of the minor criteria of Jones. Abnormal increases in the PR interval lead to hemodynamic impairments caused by atrioventricular asynchrony and is called pseudo-pacemaker or pacemaker-like syndrome in the literature. A 13-yearold boy who had polyarthralgia for six weeks was referred to the emergency room because of syncope while exerting effort. In the electrocardiography, the P wave was regularly seen at the onset of the T wave immediately after the QRS wave with extremely prolonged PR interval and mild tachycardia. Also, 24 hours rhythm Holter recording showed atrioventricular dissociation. The echocardiography revealed findings of severe carditis. Diagnosis of acute rheumatic fever accompanying pacemaker-like syndrome was made. Although the recommendation for marked first-degree atrioventricular block that causes hemodynamic impairment is pacemaker implantation, a significant improvement in the PR prolongation was observed in the short term with anti-inflammatory treatment because the impairment of conduction in the patient was due to inflammation. The pacemaker-like syndrome in a child is being reported for the first time in the literature.

Gastrointestinal involvement in Kawasaki disease: a case report.

Kawasaki disease is an acute febrile multisystem vasculitis. The term Incomplete Kawasaki disease is used in the presence of a minimum of two diagnostic criteria of clinical Kawasaki syndrome accompanied by at least 5 days of fever, the absence of any other reasons characterising the disease, and the presence of severe systemic inflammation findings. Gastrointestinal symptoms, notably diarrhoea, abdominal pain, and vomiting, frequently occur, and elevated serum aminotransferases, gallbladder hydrops, and rarely other forms of gastrointestinal involvement such as ischaemic colitis, intussusception, hepatic necrosis, splenic infarct, intestinal pseudo-obstruction, colitis, and colon oedema are also reported. In this paper, we present an incomplete and atypical Kawasaki case that explicitly shows gastrointestinal involvement. Progressive bowel oedema was detected in the patient presenting with severe abdominal pain and distension. We determined an aneurysm in the right coronary artery and diffuse dilatation in the left main coronary artery despite administration of early intravenous immunoglobulin. In addition to the cardiac problem, hypoalbuminaemia, electrolyte imbalance, sterile pyuria, hepatosplenomegaly, and hydrops of the gallbladder were observed in the case. All findings, including progressive bowel oedema accompanying abdominal distension, improved markedly after the second dose of intravenous immunoglobulin.

Clinical improvement and no further need of transplant after closure of PDA with transcatheter approach in an end-stage heart failure patient with hypertrabeculation.

Although heart failure is managed medically most of the time, heart transplantation is still last resort for selected end-stage heart failure patients with noncompaction cardiomyopathy. Presently described for the first time is case of pediatric patient with noncompaction cardiomyopathy who was initially referred to our hospital for heart transplant but underwent PDA repair and improved clinically without need for heart transplant.

Cardiac nodal and cardiac autonomic functions in children with vasovagal syncope.

In the present study, cardiac nodal (sinoatrial node and atrioventricular node) and cardiac autonomic functions have been investigated in children with vasovagal syncope (VVS). Thus, the effect of existing autonomic status on the characteristics of cardiac conduction system has been demonstrated in children with VVS. The study included 51 pediatric patients (the mean age was 14.01±2.79 years, range 7 to 18 years; 30 females), who were evaluated for syncope using ECG, Holter monitoring, and echocardiography, all of which showed normal findings. All patients underwent head-up tilt testing (HUTT), and the parameters of heart rate variability (HRV) (SDNN, SDANN, SDANNi, rMSSD, pNN50, HF, LF, VLF) were evaluated with the analysis of 24-hour Holter testing. Corrected sinus node recovery time (CNRT) and Wenckebach point (WP) were measured in all patients using transesophageal atrial stimulation. The patients were divided into groups according to HUTT results and evaluated accordingly. Cardiac nodal functions were normal in all patients. HRV parameters (HF, rMSSD, pNN50) indicating parasympathetic effect were higher in patients with positive HUTT results. CNRT did not differ significantly between patients with positive HUTT results and patients with negative HUTT results. However, WP was found to be higher in patients with positive HUTT results. The studies evaluating the parameters of HRV has demonstrated increased parasympathetic tonus in children with VVS. Impairment in cardiac nodal functions may not be expected in children with VVS. However, these patients may exhibit prolonged WP due to increased autonomic tonus in favor of parasympathetic activity.

Infantile malignant osteopetrosis: a rare cause of neonatal hypocalcemia.

Infantile malignant osteopetrosis (IMO; OMIM 259700) is a rare inherited bone disease characterized by reduced or dysregulated activity of osteoclasts, resulting in generalized osteosclerosis. The disease usually presents within the first few months of life with anemia, hepatosplenomegaly, frontal bossing, nystagmus, blindness, deafness, and bone fractures. Children with IMO are at risk of developing hypocalcemia, with attendant tetanic seizures. We report the case of a baby boy who presented with neonatal hypocalcemia. Skeletal radiographs demonstrated sclerotic bones and a dense base of the skull with typical "space alien" face confirming the diagnosis of IMO. Pancytopenia developed at 2 months of age. Visual evoked potential showed severe bilateral optic nerve damage. Genetic mutation study revealed a new mutation in exon 13 of the TCIRG1 gene. Neonatal hypocalcemia can occur as result of IMO, which is easily missed out by clinicians. This causes delay in establishing the diagnosis and starting necessary treatment. Therefore, osteopetrosis should be kept in mind as a rare cause of neonatal hypocalcemia.