Exercise capacity and muscle strength in patients who have undergone the Fontan procedure: a retrospective follow-up study.

BACKGROUND: Due to their relationship with clinical progression, follow-up of exercise capacity and muscle strength is important for optimal disease management in patients who have undergone the Fontan procedure. We aimed to retrospectively analyze exercise capacity and muscle strength trajectory over approximately 2 years. METHODS: Exercise capacity was assessed using an exercise stress test with the modified Bruce protocol on a treadmill, hand grip and knee extensor strength using a hand dynamometer, and body composition using a bioelectrical impedance device. Exercise capacity, muscle strength, and body composition follow-up data recorded between 2020 and 2022 were compared. RESULTS: Fifteen patients [median age from 17 (first assessment) to 18 years (last assessment), 5 females)] with a 20-month median follow-up time were analyzed retrospectively. There was an increase in weight, height, body mass index, and body fat weight (p<0.05). There was a tendency for increased handgrip strength (%) (p=0.069), but no significant difference was observed in the knee extensor strength of patients during the follow-up period (p>0.05). The changes in heart rate (HR) and oxygen saturation were higher in the last test than in the first test (p<0.05). Maximum HR (HRmax), % predicted HRmax and HR reserve recorded during the test and HR 1 minute after the test were similar between the first and last tests (p>0.05). CONCLUSIONS: After 20 months of follow-up, exercise capacity and muscle strength did not decline; instead, the body mass index and fat weight increased. Patients who have undergone the Fontan procedure may not be experiencing a decline in exercise capacity and muscle strength over relatively short time periods during childhood, adolescence, and early adulthood.

Acute kidney injury in children with moderate-severe COVID-19 and multisystem inflammatory syndrome in children: a referral center experience.

BACKGROUND: Data on the characteristics of acute kidney injury (AKI) in pediatric COVID-19 and MIS-C are limited. We aimed to define the frequency, associated factors and early outcome of AKI in moderate, severe or critical COVID-19 and MIS-C; and to present a tertiary referral center experience from Türkiye. METHODS: Hospitalized patients ≤ 18 years of age with confirmed COVID-19 or MIS-C at Ihsan Dogramaci Children's Hospital, Hacettepe University, between March 2020-December 2021 were enrolled. The characteristics of AKI in the COVID-19 group were investigated in moderate, severe and critically ill patients; patients with mild COVID-19 were excluded. RESULTS: The median (Q1-Q3) age in the COVID-19 (n = 66) and MIS-C (n = 111) groups was 10.7 years (3.9-15.2) and 8.7 years (4.5-12.7), respectively. The frequency of AKI was 22.7% (15/66) in COVID-19 and 15.3% (17/111) in MIS-C; all MIS-C patients with AKI and 73.3% (11/15) of COVID-19 patients with AKI had AKI at the time of admission. Multivariate analyses revealed need for vasoactive/inotropic agents [Odds ratio (OR) 19.233, p = 0.002] and presence of vomiting and/or diarrhea (OR 4.465, p = 0.036) as independent risk factors of AKI in COVID-19 patients; and need for vasoactive/inotropic agents (OR 22.542, p = 0.020), procalcitonin and ferritin levels as independent risk factors of AKI in the MIS-C group. Age was correlated with lymphocyte count (r = -0.513, p < 0.001) and troponin level (r = 0.518, p < 0.001) in MIS-C patients. Length of hospital stay was significantly longer in both groups with AKI, compared to those without AKI. Mortality was 9.1% in the COVID-19 group; and was associated with AKI (p = 0.021). There was no mortality in MIS-C patients. AKI recovery at discharge was 63.6% in COVID-19 survivors and 100% in MIS-C patients. CONCLUSIONS: Independent risk factors for AKI were need for vasoactive/inotropic agents and vomiting/diarrhea in moderate, severe or critical COVID-19 patients; and need for vasoactive/inotropic agents and severe inflammation in MIS-C patients. Our findings suggest that inflammation and cardiac dysfunction are associated with AKI in MIS-C patients; and the association with age in this group merits further studies in larger groups. Early outcome is favorable; long-term follow-up for kidney functions is needed.

Percutaneous Transcatheter Retrieval of Central Venous Port Fragments in Pediatric Patients; A Single-center Experience From the Pediatric Cardiology Department.

BACKGROUND: Split/fracture and embolization of central venous/shunt catheters are rare but serious complications in children. Percutaneous retrieval of intravascular foreign bodies is an important minimal invasive treatment. This study is aimed to represent our largest pediatric sample experience till now of 17 years from a single institution. Another aim is to compare the results regarding the removal or leaving in place of embolized or ruptured intravascular or cardiac venous catheter parts in children. PATIENTS AND METHODS: A total of 26 cases were included in this study. Any pediatric patient with normal coagulation parameters and a fractured catheter fragment was included in this study. Other intravascular foreign bodies related to interventional devices and/or pacemaker/implantable cardiac defibrillator leads were excluded from this study. RESULTS: Twenty-six patients, of whom 25 had oncologic diseases and 1 had a ventriculoatrial shunt, were included. The median age was 83.5 months (between 20 mo and 18 y) at treatment.Superior vena cava (9 cases), followed by the right atrium (5 cases), were the most two common sites of embolization for cardiovascular foreign bodies. The success rate of percutaneous retrieval was 92.3% in all patients. There were neither complications nor deaths. The retrieval technique revealed a predisposition for extraction through the femoral vein (96.1%) and using snare techniques (100%). Additional catheters like pigtail, National Institutes of Health, or ablation catheters were used for stabilization in selective cases in which the permanent central venous fragments stuck to the vessels. A tractional maneuver and capturing the ruptured material in the middle were other trick points for successful retrieval. Patients were asymptomatic in 76.9% of cases (20/26). CONCLUSION: Percutaneous retrieval of cardiovascular foreign bodies is a reasonable, safe, and effective way in children when the catheter fragments are free and mobile. It should be considered the preferred treatment option instead of surgery. In patients where catheter fragments are stuck and are adherent to vessels, it could be left, and followed up by anticoagulation. Novel techniques accompanied by an experienced team could be helpful in difficult cases.

The risk of cardiovascular comorbidity in children with Behçet's disease.

OBJECTIVE: Patients with Behcet disease (BD) may experience long term morbidity caused by different forms of cardiovascular diseases. This study aimed to assess the risk for cardiovascular comorbidity in pediatric BD patients with and without vascular involvement, independent of the contribution of traditional risk factors. METHODS: Pediatric patients classified as BD according to the 2015 International Pediatric BD criteria were included in the study. Twenty-four-h ambulatory blood pressure monitoring (ABPM), transthoracic echocardiography, and carotid intima media thickness (cIMT) measurements were performed. Patients with an active disease and have other known risk factors for cardiovascular disease were not included in the study. RESULTS: Thirty-one children and adolescents with pediatric BD (16 female, 51.6%; F/M: 1.06) were enrolled in the study. Among all BD patients 10 patients (34.4%) had abnormal ABPM. Carotid IMT values, mean arterial pressure, systolic and diastolic blood pressure by ABPM and the prevalence of abnormal ABPM, non-dipping, and ambulatory hypertension were similar between patients with and without vascular involvement. The echocardiography measurements showed that BD patients with vascular involvement had significantly higher velocity and velocity time integral of the left ventricle outflow tract which may point out increased stiffness of the aorta. CONCLUSION: Pediatric BD patients with vascular involvement may tend to have more cardiovascular risk factors. However, cardiovascular assessment should be considered in all BD patients regardless of the involved systems. We suggest that ABPM may accurately define hypertension and cardiovascular risk in BD.

Cardiac rhabdomyomas: clinical progression, efficacy and safety of everolimus treatment.

BACKGROUND: Primary cardiac tumors are extremely rare. Cardiac rhabdomyoma is the most common primary cardiac tumor. 50-80% of solitary rhabdomyomas and all multiple rhabdomyomas are associated with tuberous sclerosis complex. Due to spontaneous regression, surgery is necessary only in severe hemodynamic compromise and persistent arrhythmias. Everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, can be used in the treatment of rhabdomyomas seen in tuberous sclerosis complex. We aimed to evaluate the clinical progression of rhabdomyomas followed-up in our center between the years 2014-2019 and evaluate the efficacy and safety of everolimus treatment on tumor regression. METHODS: Clinical features, prenatal diagnosis, clinical findings, tuberous sclerosis complex presence, treatment and follow-up results were evaluated retrospectively. RESULTS: Among 56 children with primary cardiac tumors, 47 were diagnosed as rhabdomyomas, 28/47 patients (59.6%) had prenatal diagnosis, 85.1% were diagnosed before one year of age and 42/47 patients (89.3%) were asymptomatic. Multiple rhabdomyomas were present in 51% and median diameter of tumors was 16mm (4.5 - 52 mm). In 29/47 patients (61.7%) no medical or surgical treatment were necessary while 34% of these had spontaneous regression. Surgery was necessary in 6/47 patients (12.7%). Everolimus was used in 14/47 patients (29.8%). Indications were seizures (2 patients) and cardiac dysfunction (12 patients). Regression in size of rhabdomyomas was achieved in 10/12 patients (83%). Although, in the long-term, the amount of tumor mass shrinkage was not significantly different between patients who received everolimus and untreated patients (p=0.139), the rate of mass reduction was 12.4 times higher in patients who received everolimus. Leukopenia was not detected in any of the patients, but, hyperlipidemia was noted in 3/14 patients (21.4%). CONCLUSIONS: According to our results, everolimus accelerates tumor mass reduction, but not amount of mass regression in the long term. Everolimus may be considered for treatment of rhabdomyomas which cause hemodynamic compromise or life-threatening arrhythmias before surgical intervention.

Fetal cardiac interventions: First-year experience of a tertiary referral center in Turkey.

AIM: To present the first-year experience of fetal cardiac interventions (FCIs) in a tertiary referral hospital and to evaluate the outcomes. METHODS: This retrospective study consisted of four pregnant women who underwent fetal pulmonary or aortic balloon valvuloplasty between November 2020 and June 2021. The procedures were performed with a percutaneous cardiac puncture under the ultrasonography guidance. Gestational age at intervention, procedural success, complications, and perinatal outcomes were evaluated. Procedural complications defined as fetal bradyarrhythmia requiring treatment, pericardial effusion requiring drainage, balloon rupture, and fetal death. The procedure was considered technically successful if the valve was dilated with a balloon catheter. Ultimately successful procedure was defined as the discharge of infants alive with biventricular circulation. RESULTS: A total of 5 FCIs attempted between 26 + 3 and 28 + 2 gestational weeks. While the procedure was technically successful in 2 cases with pulmonary stenosis, both attempts were unsuccessful in the fetus with pulmonary atresia. Although the procedure was technically successful in the patient with critical aortic stenosis, it ultimately failed. No fetal death occurred in our series and there were no procedure-related significant maternal complications. However, three interventions were complicated by fetal bradycardia and pericardial effusion necessitating treatment, and balloon rupture cropped up in one case. CONCLUSION: FCIs may lead to improving the likelihood of a biventricular outcome for selected fetuses. Careful selection of patients and centralization of experience are essential for obtaining favorable outcomes. Operators should be aware of procedural complications. Improved procedural techniques with a lower complication rate will be achieved through advanced medical technology and special balloon catheters.

Assessment of exercise capacity using field walking tests in patients after the Fontan procedure: A case-control study.

BACKGROUND: Despite the frequent use of the 6-minute walk test (6MWT), exercise capacity has not been assessed with the incremental shuttle walk test (ISWT) in patients who have undergone the Fontan procedure. It is unclear whether these tests cause clinically relevant cardiorespiratory responses in these patients. OBJECTIVES: We aimed to assess cardiorespiratory responses to the 6MWT and ISWT in Fontan patients, compare the responses with those in the controls, and examine the agreement between the two field tests. METHODS: Submaximal exercise capacity was assessed using the 6MWT, maximal exercise capacity using the ISWT, quadriceps isometric muscle strength with a hand dynamometer, and body composition using a bioelectrical impedance device. RESULTS: Twenty-one Fontan patients (16.42±6.63 years, 5F/16M) and 21 controls (16.57±4.30 years, 7F/14M) were included. While body composition was similar between the groups (p>0.05), quadriceps isometric muscle strength and 6MWT and ISWT distance were lower in the Fontan patients than in the controls (p<0.05). In both the 6MWT and ISWT, pre- and post-test heart rate (HR), oxygen saturation (SpO2), dyspnea, and leg fatigue differed significantly between the Fontan patients and the controls (p<0.05). In addition, the ISWT resulted in a more significant change in HR, SpO2, and leg fatigue than the 6MWT in the Fontan patients (p<0.05). Bland-Altman plots for the 6MWT vs. the ISWT indicated agreement between the two tests. CONCLUSION: There were remarkable changes in HR, SpO2, dyspnea, and leg fatigue in both tests. With similar safety to the 6MWT but with more caution applied for adverse events, the ISWT can also be performed as a field test to evaluate exercise capacity and identify more pronounced exercise-induced responses (especially oxygen desaturation) in Fontan patients.

An unexpected intracardiac location of yolk sac tumor.

PURPOSE: In the pediatric population, intracardiac tumors are rare, usually benign, and mostly diagnosed as rhabdomyoma. Yolk sac tumors (YSTs) are a rare malignant type of germ celltumor that typically occurs in gonads. It can also be seen in midline locations but the intracardiac location is extremely rare. METHODS: The case herein comprises an asymptomatic 2.5-year-old girl with a murmur detected under general examination. RESULTS: Echocardiography showed a 3 × 3-cm mass in the right ventricle. Cardiac magnetic resonance imaging revealed a smooth contoured mass in the right ventricle lumen, which was compatible with rhabdomyoma. After surgical resection, the histopathological results showed a YST. This diagnosis was supported by high values of subsequent serum alpha feto-protein. There was no evidence for any other primary location. CONCLUSION: When an intracardiac mass is observed, a YST should be considered. The increase in the alpha feto-protein level can help in the differential diagnosis.

Interventional cardiac catheterization in neonates and premature infants with congenital heart disease: a single center experience.

BACKGROUND: The increased survival of patients with congenital heart disease over the last three decades has been associated with improvements in diagnosis and treatment. This study aimed to evaluate therapeutic interventional catheterization, outcomes and complications of these procedures in neonates and premature infants. METHODS: In this study, therapeutic catheterization procedures performed on neonates and premature infants with congenital heart disease at a university hospital between February 2000 and October 2019 were retrospectively evaluated. RESULTS: A total of 322 procedures were performed on 279 neonates and 26 premature infants. Of the patients, 217 (67.4%) were male. The median age of the patients was 8 days (interquartile range [IQR] 2-20) and the median body weight was 3050 g (IQR 2900-3600). The most common procedures were balloon atrial septostomy, balloon aortic angioplasty, balloon pulmonary valvuloplasty and balloon aortic valvuloplasty (35.4%, 20.8%, 18.3% and 12.4% respectively). The most common diagnoses were transposition of the great arteries, coarctation of the aorta, pulmonary stenosis and aortic stenosis (26.7%, 19.3%, 15.2% and 11.5% respectively). Most procedures, 274 (85.1%), were successful. Complications were observed in 74 procedures (23%). Of these complications, 45 (14%) were minor and 29 (9%) were major. The most common complication was transient dysrhythmia (6.9%). There was no significant relationship between body weight, age and the rate of complications. However, longer procedure time and fluoroscopy time were associated with higher complication rates (p < 0.05). Four procedurerelated deaths were observed. CONCLUSION: Procedure-related complications are higher in the neonatal period. Although the complication rate varies according to the type of procedure, longer fluoroscopy time and procedure duration are associated with an increased complication rate. Procedures performed with the right indications, appropriate equipment and by experienced teams will play a key role in reducing complication rates.

Early is superior to late plasma exchange for severe multisystem inflammatory syndrome in children.

BACKGROUND: Multisystem inflammatory syndrome in children (MIS-C) can be life threatening in severe cases because of uncontrolled inflammation and multi-organ failure. In this study, we report the effect of plasma exchange in the treatment of MIS-C and to emphasize the effect of its early application on outcome. METHOD: In this retrospective observational study, the medical records of children with severe MIS-C admitted to pediatric intensive care unit (PICU) between April 2020 and January 2021 were reviewed. Severe MIS-C patients were treated according to protocol consisting of plasma exchange (PE), intravenous immune globulin, steroids, and anakinra which we called the "PISA" protocol referring to the initials. The patients were divided into two groups as early plasma exchange (E-PE) and late plasma exchange (L-PE) according to the elapse time between hospital admission and the administration of PE. Groups were compared in terms of outcome variables. Primary study outcome was 28-day mortality. Secondary outcome variables were acute phase response time, length of immunomodulatory treatment, frequency of patients requiring mechanical ventilation (MV) and inotropic support, length of inotropic support and MV, length of hospital and PICU stays. RESULTS: Eighteen pediatric patients with MIS-C were included in the study. Seventeen (95%) of the patients presented with decompensated shock and required inotropic support. One of the 17 patients needed extracorporeal membrane oxygenation support (ECMO) PISA protocol was used in all patients. There was no mortality in the E-PE group while the mortality rate was 20% in the L-PE group. Acute phase reactant response was faster in the E-PE group and immunomodulatory treatments could be reduced earlier; the frequency of patients requiring inotropic and mechanical ventilation (MV) support was lower in the E-PE group; the duration of inotropic support, duration of MV, and length of stay in hospital and PICU were significantly shorter in the E-PE group. CONCLUSION: We suggest that in selected cases, timely administration of PE is a beneficial rescue therapy for MIS-C related hyperinflammation presenting with severe cardiovascular collapse.

Comparison of IVIG resistance predictive models in Kawasaki disease.

BACKGROUND: We aimed to compare the ten different scores (by Kobayashi, Egami, Harada, Formosa, Sano, Piram et al., Wu et al., Yang et al., Tan et al., and Kanai et al.) to assess their performance in predicting IVIG resistance in Turkish children. METHODS: Complete and incomplete KD patients diagnosed with KD at Hacettepe University between June 2007 and September 2019 were evaluated retrospectively. RESULTS: A total of 129 patients, 79 boys (61.2%), with a median age 36 (IQR 19.5-57.0) months were evaluated. Sixteen patients (12.4%) had IVIG resistance. Sensitivity was low for all the ten scores. Tan, Sano, and Egami predictive models had the highest specificity (97.3, 89.4, 86.7%, respectively). Almost all scoring systems distinguished the group of patients with low risk for IVIG resistance but could not differentiate IVIG-resistant patients. Multivariate analysis for the laboratory features showed that platelet count <300 × 109/L and GGT serum levels were independent risk factors for IVIG resistance (OR: 3.896; 95% CI: 1.054-14.404; p = 0.042 and OR: 1.008; 95% CI: 1.001-1.015; p = 0.050). CONCLUSIONS: The current scoring systems had a low sensitivity for predicting the risk for IVIG resistance in Turkish children. On the other hand, increased serum GGT levels and low platelet count were risk factors for predicting IVIG resistance. IMPACT: Intravenous immunoglobulin (IVIG) resistance may be observed in 10-20% of patients diagnosed with Kawasaki disease. Coronary artery involvement is more frequent in IVIG-resistant patients. It is important to predict the patients who might develop IVIG resistance to improve prognosis. The performance of the IVIG resistance predictive models in Kawasaki disease in our population is limited due to the low sensitivity.

Two decades of experience on ablation in children with Ebstein's anomaly.

INTRODUCTION: Accessory pathways are commonly seen due to delamination of tricuspid valve leaflets. In addition to accessory pathways, an enlarged right atrium due to tricuspid regurgitation and incisional scars creates substrates for atrial re-entries and ectopic tachycardia. We sought to describe our experience with catheter ablation in children with Ebstein's anomaly. METHODS AND RESULTS: During the study period, of 89 patients diagnosed with Ebstein's anomaly, 26 (30.9%) of them who underwent 33 ablation procedures were included in the study. Accessory pathways were observed in the majority of procedures (n = 27), whereas atrial flutter was observed in five, atrioventricular nodal reentrant tachycardia in five, and atrial tachycardia in two procedures. Accessory pathways were commonly localised in the right posteroseptal (n = 10 patients), right posterolateral (n = 14 patients), septal (n = two patients), and left posteroseptal (n = one patient) areas. Multiple accessory pathways and coexistent arrhythmia were observed in six procedures. All ablation attempts related to the accessory pathways were successful, but recurrence was observed in five (19%) of the ablations. Ablation for atrial flutter was performed in five patients; two of them were ablated successfully. One of the atrial tachycardia cases was ablated successfully. CONCLUSIONS: Ablation in patients with Ebstein's anomaly is challenging, and due to nature of the disease, it is not a rare occasion in this group of patients. Ablation of accessory pathways has high success, but also relatively high recurrence rates, whereas ablation of atrial arrhythmias has lower success rates, especially in operated patients.

Femoral venous haemostasis in children and young adults using the 'figure-of-eight' suture technique.

AIM: The aim of our study was to evaluate the safety and efficiency of the 'figure-of-eight' suture among children and young adults with congenital heart defects who underwent interventional procedures, in patients with structurally normal hearts who underwent electrophysiological study and in haemodynamically impaired children and newborns. We also reported a novel femoral haemostasis method in patients with a central catheter by modifying the 'figure-of-eight' suture around the catheter for haemorrhage control. METHOD: Between 2015 and 2018, a total of 100 'figure-of-eight' sutures were performed in 90 patients (48 males, 42 females) where the median age was 12.5 years (minimum 3 days, maximum 22 years). The procedures were diagnostic angiography (n = 6), radiofrequency and/or cryoablation (n = 7) and interventional procedures (n = 87). RESULT: Haemostasis was achieved in 89 of 90 patients. Haemostasis could not be achieved in one malnourished patient due to lack of subcutaneous tissue. There were no major complications. A bullous skin lesion and minor bleeding were the only complications seen in two patients. A central catheter was inserted in eight patients using the modified 'figure-of-eight' suture technique. CONCLUSION: The 'figure-of-eight' suture is a safe and effective method for femoral venous haemostasis in patients who require large sheaths for procedures, in those using high-dose heparin and in haemodynamically unstable children who need cardiac catheterisation.

Multiple Ablation Targets in Children: Multiple Accessory Pathways and Coexistent Arrhythmia.

The coexistence of different mechanisms of arrhythmia and multiple accessory pathways (MAPs) leading to multiple ablation targets is rarely seen in children, and data regarding these patients in the literature are limited. Herein, we aimed to evaluate patients who required multiple ablation applications, focusing on different targets during the procedures in children, and evaluating the characteristics of coexistent arrhythmia and MAPs, and the results of these procedures in children. Ablation procedures conducted between March 2009 and December 2018 were evaluated retrospectively, and patients with MAPs and/or coexistent arrhythmia who had undergone ablation procedures were included in the study. Among the 1210 patients who underwent ablation procedures, 52 patients (26 male, 26 female) were ablated for multiple targets. Of the 456 patients with APs, 21 had MAPs (4.6%) and of the 1210 patients who underwent ablation procedures, 31 patients had coexistent arrhythmia (2.5%). The patients had a mean age of 12.24 ± 3.4 (4-18) years and mean body weight of 45.17 ± 14.12 (17-74) kg. A total of 110 APs or foci were identified as quaternary in one patient, while it was triple in four patients. The procedures were unsuccessful in six targets of six patients. Although recurrence was observed in four patients, none were ablated for MAPs. Two complications were encountered, comprising ST segment depression that developed in one patient with Wolf-Parkinson-White syndrome, atrioventricular nodal re-entry tachycardia, and a temporary atrioventricular block during atrioventricular nodal re-entrant tachycardia ablation. The overall success rate according to the pathway/foci number was 94.5% (104/110), with a recurrence rate of 4.5% (5/110), and a complication rate of 1.8% (2/110). The patient success, recurrence, and complication rates were 88.4% (46/52), 7.6% (4/52), and 3.8% (2/52), respectively. In conclusion, the incidence of multiple arrhythmogenic foci and MAPs were not as low as expected in children. A structured and stepwise approach is mandatory for the diagnosis of the different mechanisms of tachycardia, even after successful ablation procedures. The success, recurrence, and complication rates were comparable with those of patients who had a solitary arrhythmogenic focus or solitary AP.

Empiric slow-pathway ablation results for presumed atrioventricular nodal reentrant tachycardia in pediatric patients.

BACKGROUND: In pediatric patients with documented narrow QRS tachycardia that is suggestive of atrioventricular nodal reentrant tachycardia (AVNRT) and not inducible in electrophysiological study (EPS), empiric slowpathway ablation (ESPA) may be considered. There is limited data in children about this topic. METHODS: Seventy-nine patients who underwent cryoablation and/or radiofrequency ablation (RFA) for presumed AVNRT between January 2010 and January 2020, with no inducible tachycardia and no other tachycardia mechanisms during EPS, were included in this study. RESULTS: The age was between 6 and 18 years. All patients had no structural heart disease. Preablation exhibited sustained SP conduction for all patients. In all cases, the ablation end points were prolongation in wenckebach cycle length (WBCL) with loss of cross and/or jump, and/or echo beat. The end points were not achieved in two patients. Overall, the mean basal WBCL increased to 351 ms (240-500 ms) from 301.3 ms (180-420 ms), evident in the non-recurrence group. Nine patients had a transient AV block that improved. We followed the patients without medication for about 46.9 months (8 months to 10 years). Palpitations occurred again in 9 of 77 patients (clinical recurrence rate 9/79 - 11.3%). The documented ECG recurrence rate was 1.2% (1/79). In the non-recurrence group, WBCL prolongation was higher and mean age was lower than in the recurrence group (13.075 vs. 15.33 years). CONCLUSION: In cases with presumed AVNRT, ESPA seems to be a reasonable and safe way. In our study, we found our procedural success rate as 97.4% and follow-up recurrence rate as 12.6% (9+1/79).

Is Takayasu's arteritis more severe in children?

OBJECTIVES: Takayasu's arteritis (TAK) is a chronic vasculitis, affecting predominantly the aorta and/or its major branches. The aim of this study was to compare the differences between childhood and adult onset TAK. METHODS: We retrospectively evaluated 179 TAK patients followed between August 2005 and July 2019. Demographic characteristics, laboratory features, disease activity, echocardiographic data at diagnosis and treatment regimens in the disease course were compared between the paediatric and adult onset patients. RESULTS: Twenty-five paediatric-onset (<18 years of age at diagnosis) and 154 adult-onset patients (≥18 years of age at diagnosis) were enrolled. The mean age at diagnosis for children and adults were 13.6±4 and 35.6±13, respectively. Paediatric onset TAK patients had more intense inflammation at the time of diagnosis reflected in their clinical findings. Acute phase reactants were high in all paediatric patients and significantly higher in patients with paediatric-onset TAK (p=0.006 and p=0.005, respectively). Abdominal predominant disease was more common in the paediatric group, in contrast, focal disease and aortic arch predominant disease were more common in the adult group. Ascending aortic dilatation, left ventricular hypertrophy and moderate-severe aortic insufficiency were more frequent in echocardiography findings of paediatric onset TAK patients. In comorbidities, hypertension was more common in paediatric TAK patients during follow-up, whereas cerebrovascular disease was more common in adult patients. CONCLUSIONS: Our paediatric onset TAK patients presented with a more severe inflammation and more widespread vascular involvement. Multicentre studies from different geographic areas are needed to verify our observation and understand the underlying causes.

Kawasaki-like disease in children with COVID-19.

Children with Coronavirus disease 2019 (COVID-19) are being reported to have manifestations of hyperinflammatory states and/or Kawasaki-like disease. In this study, we investigated children with typical and atypical Kawasaki disease (KD) likely to be associated with COVID-19. We have reported four children with Kawasaki-like disease probably associated with COVID-19. The clinical features were consistent with incomplete KD in three patients. SARS-CoV-2 RT-PCR was positive in one and the serology was positive in one patient with negative RT-PCR. Corticosteroids, anakinra, intravenous immunoglobulin (IVIG), and acetylsalicylic acid were used in the treatment. Three patients recovered after the treatment while one patient died. The literature review revealed 36 articles describing 320 children with Kawasaki-like disease associated with COVID-19. SARS-CoV-2 RT-PCR was negative in 120 (65.5%) of 183 patients while the serology was positive in 130 (83.8%) of 155 patients. The therapeutic options have included IVIG, acetylsalicylic acid, tocilizumab, anakinra, enoxaparin, and methylprednisolone. Pediatric COVID-19 cases may present with atypical/incomplete Kawasaki-like disease. Thus, pediatricians need to be aware of such atypical presentations resembling KD for early diagnosis of COVID-19.

Intrapericardial inflammatory myofibroblastic tumour in a 3-month-old infant associated with Coronavirus OC43 presenting with pericardial tamponade.

Inflammatory myofibroblastic tumour usually has a benign course and is very rarely associated with the heart. It can have life-threatening consequences, depending on its position or the presence of aggressive and metastatic complications. A 3-month-old boy presented with pericardial tamponade and was diagnosed with intrapericardial inflammatory myofibroblastic tumour associated with Coronavirus OC43. A large tumour attached to the left ventricle was completely removed by surgical resection and he made a full recovery. ABBREVIATIONS: ALK: anaplastic lymphoma kinase; CMV: cytomegalovirus; CRP: C-reactive protein; EB: Epstein-Barr virus; ESR: erythrocyte sedimentation rate; IM: inflammatory myofibroblastic tumour; NSAI: non-steroidal anti-inflammatory drugs; PTFE: polytetrafluoroethylene; SMA: smooth muscle actin.