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.

Resuscitating the resuscitation: A single-centre experience on extracorporeal cardiopulmonary resuscitation.

AIM: Extracorporeal cardiopulmonary resuscitation (ECPR) is the rapid deployment of venoarterial extracorporeal membrane oxygenation (ECMO) during active cardiopulmonary resuscitation or in patients with intermittent return of spontaneous circulation. This study aimed to describe the demographic characteristics and outcomes of patients undergoing ECPR to identify survival-associated factors. METHODS: The study was conducted in an extracorporeal life support centre of a tertiary hospital in Turkey and included all patients who underwent ECPR for in-hospital cardiac arrest between April 2013 and June 2021. Complications included bleeding, neurological injury, renal failure, hepatic failure, limb ischemia and bloodstream infections. The primary outcomes were survival of ECMO and survival to discharge. Neurological outcomes were assessed using the Pediatric Cerebral Performance Category Scale for children and the Category of Cerebral Performance Scale for adults. RESULTS: The study included 26 patients (24 paediatric, 2 adults), 22 (85%) of them had cardiac pathology. Bleeding was the most common complication. Twelve (46%) patients survived ECMO, 9 (35%) survived to discharge. Sex, age, primary diagnosis, cardiac arrest rhythm and ECMO duration were not significantly associated with the primary outcomes. Bleeding, neurological injury and renal failure were associated with poorer survival to discharge. The neurological outcomes of all survivors to discharge were good. CONCLUSIONS: ECPR is not commonly accessible. Sharing the experience of the few treating centres to date is crucial to accumulating sufficient knowledge about its efficiency and raising clinician awareness. This limited single-centre experience demonstrated the utility of ECPR.

Evaluation of changes in physician behavior after introduction of pediatric syncope approach protocol in the emergency department.

OBJECTIVES: Syncope is a common presenting symptom in the pediatric emergency department (PED). The etiology of pediatric syncope is generally benign. However, differentiating cardiac and other serious causes from benign causes of syncope are crucial. The aim of this study was to evaluate the change of physicians' behavior after introduction of an institutional protocol designed to act as a guide in the assessment and management of syncope patients in the PED. METHODS: This was a retrospective study, conducted at the PED of our University Children's Hospital. A "pediatric syncope approach protocol" was introduced on March 1, 2019 for quality improvement purposes. Documented information in the medical records, laboratory investigations and the patients' final diagnoses during the six-month periods before and after the protocol introduction were compared. RESULTS: 268 patients were included in the study (131 pre-protocol, 137 post-protocol patients). When compared with the pre-protocol group, there was a significant improvement in the documentation of syncope characteristics (prodromal findings, predisposing factors, association with exercise), cardiac, neurological and electrocardiographic (ECG) warning signs and ordering of cardiac markers in the post-protocol group. CONCLUSIONS: The introduction of the syncope approach protocol in PED has enabled an evidence-based and systematic evaluation and management of syncope patients, and reduced the rates of low-yield investigations. Sustained implementation of this protocol can have long-term benefits in the care of children with syncope at the emergency department.

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.

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).

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.

Bilateral thoracoscopic sympathectomy for cardiac denervation in pediatric population: Does Kuntz nerve cauterization have an impact on success?

INTRODUCTION: Cardiac sympathetic denervation (CSD) is an effective procedure for the treatment of inherited channelopathies. Left CSD has traditionally been recommended as neuromodulation to prevent arrhythmia episodes; however, it is thought that bilateral sympathectomy in combination with Kuntz ablation may have additional effects effective due to the anatomical variability of preganglionic sympathetic fibers. The aim of the study was to share our single-center clinical experience with bilateral thoracoscopic sympathectomy for cardiac denervation in different groups of pediatric patients with malignant arrhythmias. METHODS: Fourteen patients (seven with CPVT, five with LQTS, one with resistant ventricular tachycardia as a sequela of myocarditis, and one with cardiomyopathy and atrial tachycardia) underwent bilateral thoracoscopic sympathectomy for cardiac denervation. RESULTS: In all patients, arrhythmia episodes persisted despite medical therapy, and patients with implantable cardioverter-defibrillator received appropriate therapies. The rate of appropriate therapies was 3.25 per year (1-5 per year) in the year before sympathectomy. No major complications related to the procedure were observed in any of the patients. The QTc interval of LQTS patients decreased from 506.2 ± 16.9 ms before the procedure to 476 ± 28.8 ms after the procedure. The mean duration of follow-up after the procedure was 23.3 months (11-47 months). Only two patients received single episodes of therapy 12 and 22 months after CSD, and one patient had arrhythmic events due to noncompliance to medical therapy. CONCLUSION: Due to the anatomical variability of preganglionic fibers bilateral CSD with Kuntz nerve ablation effective treatment and is a safe option.

Giant diverticulum of coronary sinus with multiple accessory pathways treated with catheter ablation.

Coronary sinus abnormalities are usually associated with arrhythmia disorders when symptomatic. We report a 5-year-old 14 kg patient with a giant diverticulum of coronary sinus and Wolff-Parkinson-White syndrome. Catheter ablation therapy was decided during follow-up due to inadequate response to multidrug therapy. Posteroseptal and left posterolateral accessory pathways were established and radiofrequency ablation was performed successfully through coronary sinus.

Fluconazole-associated QT interval prolongation and Torsades de Pointes in a paediatric patient.

"Torsades de pointes", a life-threatening rhythm disorder, is a polymorphic ventricular tachycardia that usually develops in association with a prolonged QT interval. Fluconazole, an anti-fungal drug, may also induce QT prolongation, in some cases subsequent torsades de pointes. Herein, we report a 16-year-old female presenting "torsades de pointes" after administration of fluconazole and rapidly improved upon cessation of the drug.

Impact of Pacemakers and Implantable Cardioverter Defibrillators on the Psychosocial Functioning of Paediatric Patients.

Although implanted cardiac devices improve patients' physical health, long-term psychosocial effects especially in the paediatric population are still unknown. The aim of this study was to evaluate the psychosocial effects of cardiac devices in a paediatric population.Pediatric Quality of Life Questionnaire (PedsQoL) was used to evaluate life quality, Connor-Davidson Resilience Scale was used to evaluate resilience and Brief Symptom Inventory was used to evaluate psychiatric symptoms in a paediatric population with cardiac devices.Seventy-one patients were enrolled in the study. Fifty of them (70.5%) had a cardiac pacemaker and 21 of them (29.5%) had implantable cardioverter defibrillator. When compared to the control group both implantable cardioverter defibrillator and pacemaker using patients had lower quality of life (79.5 ± 12.4 versus 86.7 ± 12.1, p = 0.001) but no difference was observed in resilience and psychological pathologies. Age, gender, family size, and education level had no effect on quality of life. Implantable cardioverter defibrillator bearing patients had higher levels of anxiety than pacemaker patients (0.58 versus 0.30 p = 0.045), and implantable cardioverter defibrillator patients who had received shock in the last year had higher levels of somatisation than the group that did not receive device shock (0.17 versus 0.44 p = 0.022).In conclusion study showed that cardiac devices have negative effects on the psychosocial health of children. Cardiologist working with these patients should be aware of these pathologies and monitor not only physical health but also psychosocial health too.

Levels of Physical Activity and Physical Fitness in Pediatric Pacemaker Patients: A Cross-Sectional Study.

Children with heart diseases have reduced physical activity (PA) levels relative to their peers, which in turn increases cardiovascular risk. To the best of our knowledge, physical fitness and objectively measured PA levels have not been previously studied in children with pacemakers. We evaluated PA levels and physical fitness in pediatric pacemaker patients compared to their healthy peers. Twenty-eight pediatric patients with pacemakers (15 female, 13 male; mean age 13.43 ± 3.68 years) and 24 healthy subjects (14 female, 10 male; mean age 13.08 ± 3.67 years) were included. Physical fitness was assessed using the Munich Fitness Test (MFT). SenseWear Armband metabolic Holter device was used to record the PA for 7 consecutive days. MFT total and sub-parameter scores were significantly lower in the patient group (p < 0.05). Patients' total and active energy expenditure, PA level, total distance, number of steps, and vigorous PA were significantly lower than those of healthy children (p < 0.05). Sedentary activity and light, moderate, and very vigorous PA durations were similar in both groups (p > 0.05). Duration of mean moderate to vigorous PA was higher than 60 min/day recommended in PA guidelines in both patients and healthy subjects. These results provide initial data on PA and fitness in children with pacemakers and suggest that physical fitness and activity levels in children with pacemakers are lower than in healthy peers. Appropriate exercise programs may improve PA levels in pediatric pacemaker patients. Awareness of the importance of PA should be raised among the parents and families of these children.

Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension: extended study results.

BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term study. AIM: We have recently reported that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension in a 24-week prospective study and well tolerated without adverse events. We now aimed to evaluate clinical efficacy, safety of switch in a larger patient population, in a 24-month prospective study. METHODS: This is a single-institution, 24-month prospective study. Patients ≥12 years with idiopathic/heritable, pulmonary arterial hypertension, or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan treatment were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors/inhaled prostanoids was allowed. Outcome measures included change from baseline to 24 months, in the 6-minute walk distance, functional class, oxygen saturation at rest/after walk distance test, and natriuretic peptide levels. Safety end points included adverse events, laboratory abnormalities. RESULTS: Twenty-seven patients (19 adults/8 children, mean age: 21.1 ± 6.3 years (12-36), weight: 53.1 ± 15.7 kgs (26-87)) were included. Mean duration of macitentan treatment: 22.3 ± 3.9 months (9-24). Six-minute walk distance significantly improved from baseline (mean: 458 ± 79 m (300-620)) at 6 months (mean: 501 ± 73 m (325-616) + 43 m) (p < 0.05), at 12 months (mean: 514 ± 82 m (330-626) + 56 m) (p < 0.05), and at 24 months (mean: 532 ± 85 m (330-682) + 74 m) (p < 0.05). We observed a significant improvement during the first 6 months but no incremental improvement after 6 months (p > 0.05). Macitentan did not significantly change functional class, oxygen saturation, and natriuretic levels (p > 0.05). None of the patients had anaemia, hepatotoxicity, and peripheral edema. CONCLUSIONS: Our study is the first study which showed that switch from bosentan to macitentan improved exercise capacity in children and young adults with pulmonary arterial hypertension significantly in the first 6 months and compared to baseline in 24 months and well tolerated without adverse events.

The factors affecting the disease course in Kawasaki disease.

The aim of this study was to review the characteristics of patients with Kawasaki disease (KD) from Turkey and to assess the performance of the Kobayashi score (KS), Harada score (HS), Formosa score (FS), Egami score (ES) and other parameters in predicting intravenous immunoglobulin (IVIG) resistance and coronary artery involvement (CAI) in the Turkish population. Patients who were diagnosed as being in the acute phase of KD at Hacettepe University Faculty of Medicine (Ankara, Turkey) between June 2007 and January 2016 reviewed retrospectively, and those between January 2016 and February 2018 reviewed prospectively, were included in this cohort study. A total of 100 patients with KD were included in this study. Statistical Package for Social Sciences for Windows 22.0 (SPSS Inc, Chicago, IL, USA) was used for statistical analysis. Eighty-five patients (85%) responded to IVIG treatment, whereas 15 (5 female, 10 male) were IVIG resistant. CAI was detected in echocardiography at diagnosis in 31 (31%) (9 female; 22 male) patients. For predicting IVIG resistance, KS, ES, FS, and HS had sensitivity of 82.1%, 26.7%, 30.8%, 69.2% and specificity of 35.7%, 94%, 51.2%, 45.8%, respectively. For the association with CAI occurrence, the sensitivities were 17.2%, 3.3%, 35.7%, 70.4% and the specificities were 78.5%, 88.4%, 49.3%, 49.3% for the aforementioned scores, respectively. The multivariate analysis showed white blood cell (WBC) count [Odd's ratio (OR) 4.1; 95% confidence interval (CI) 1.26-13.23; p = 0.019] and hematocrit (OR 3.8; 95% CI 1.15-12.4; p = 0.028), as independent predictors of CAI while gamma-glutamyl transferase (GGT) level (OR 5.7; 95% CI 1.73-27.51; p = 0.018) was detected as the only independent predictor of IVIG resistance. This is the first study from Turkey in KD to evaluate the association of the scoring systems for IVIG resistance and CAI. The risk scoring systems in KD did not predict the risk for IVIG resistance and were not associated with CAI in Turkish population.

Sensitive Cardiac Troponins: Could They Be New Biomarkers in Pediatric Pulmonary Hypertension Due to Congenital Heart Disease?

To analyze the role of sensitive cardiac troponin I (scTnI) and high-sensitive troponin T (hscTnT) in the determination of myocardial injury caused by volume and pressure load due to pulmonary hypertension (PH) and to investigate if these markers may be useful in the management of PH in childhood. Twenty-eight patients with congenital heart disease (CHD) with left to right shunt and PH, 29 patients with CHD with left to right shunt but without PH, and 18 healthy children, in total 75 individuals, were included in the study. All cases were aged between 4 and 36 months. Echocardiographic evaluation was performed in all cases, and invasive hemodynamic investigation was performed in 33 cases. Blood samples were obtained from all cases, for the measurement of brain natriuretic peptide (BNP), pro-brain natriuretic peptide (pro-BNP), sensitive cardiac troponin I (scTnI), and high-sensitive troponin T (hscTnT) levels. The mean BNP, pro-BNP, scTnI, and hsTnT levels were statistically significantly higher in patients with PH than in the patients without PH (p < 0.001). A statistically significant positive correlation was determined between pulmonary artery systolic pressure and scTnI and hscTnT levels (r = 0.34 p = 0.01, r = 0.46 p < 0.001, respectively) levels. Pulmonary hypertension determined in congenital heart diseases triggers myocardial damage independently of increased volume or pressure load and resistance, occurring by disrupting the perfusion via increasing ventricular wall tension and the myocardial oxygen requirement. Serum scTnI and hscTnT levels may be helpful markers to determine the damage associated with PH in childhood.

Clinical efficacy and safety of switch from bosentan to macitentan in children and young adults with pulmonary arterial hypertension.

BACKGROUND: Macitentan is an orally active, potent, dual endothelin receptor antagonist and is the only registered treatment for pulmonary arterial hypertension that significantly reduced morbidity and mortality in a long-term event-driven study. Aim Few studies compared the clinical efficacy and safety of switch from bosentan to macitentan only in adult patients with pulmonary arterial hypertension. We aimed to evaluate the clinical efficacy and safety of switch from bosentan to macitentan in children and young adults. METHODS: This is a single-institution, 24-week prospective study. Patients ⩾12 years of age with idiopathic/heritable pulmonary arterial hypertension or related to CHD or residual pulmonary arterial hypertension due to repaired congenital systemic-to-pulmonary shunts and on bosentan therapy were included. Concomitant treatment with oral phosphodiesterase type 5 inhibitors and inhaled prostanoids was allowed. Outcome measures included change from baseline to week 24, in the 6-minute walk distance, functional class, oxygen saturation at rest/after 6-minute walk distance test, systolic pulmonary artery pressure estimated by echocardiography, and brain natriuretic peptide levels. Safety end points included adverse events laboratory abnormalities. RESULTS: A total of 13 patients - 5 male and 8 female - completed the study. The mean age was 20.3±6.5 years (12-35) and weight was 54.0±14.5 kg (27-75). Five patients were ⩽18 years of age. Macitentan improved 6-minute walk distance from baseline (mean: 466±35 m (300-590)), at 12 weeks (mean: 494±78 m (325-590), +28 m) (p0.05). None of the patients had anaemia, hepatotoxicity, and peripheral oedema. CONCLUSIONS: Our study is the first study that showed that switch from bosentan to macitentan significantly improved exercise capacity in children and young adults with pulmonary arterial hypertension and is well tolerated without any adverse events.

Persistent left superior vena cava accompanying congenital heart disease in children: Experience of a tertiary care center.

BACKGROUND: This study focuses on determining concomitant persistent left superior vena cava (SVC) in patients with congenital heart disease (CHD). METHODS: Between 2005 and 2012, a total of 2.663 patients with CHD, 88 (3.3%) of whom were diagnosed with persistent left SVC, were evaluated retrospectively. The demographic characteristics of patients, clinical and radiographic findings, echocardiography, cardiac catheterization, and angiography results obtained from the patients' records were reviewed. RESULTS: The median age of the patients was 9.5 months, and 46 of the 88 (52.3%) patients were female. The most common concomitant CHD were ventricular septal defect, double outlet right ventricle (DORV), and tetralogy of Fallot (TOF). When the patients were compared according to their CHD, persistent left SVC frequency was significantly higher in those with DORV (P<.001), TOF (P=.04), patent ductus arteriosus (P=.01), and atrial septal defect (P=.03). Eighty-three of the 88 (94.3%) patients with persistent left SVC had right SVC, and 5 (5.7%) had absence of the right SVC. Twenty-seven of the 83 (32.5%) patients with double SVC had connected normal innominate vein. In all cases, right aortic arch association was seen in 14 (15.9%) patients. Eighty-four (95.4%) of the patients were diagnosed by echocardiography prior to catheter angiography. Persistent left SVC drained to the coronary sinus in all cases. CONCLUSION: Increased awareness about the association of certain CHD with persistent left SVC and a careful echocardiographic examination can facilitate the diagnosis of persistent left SVC. In addition, precise prior diagnosis of persistent left SVC can prevent complications during surgery.

Massive Pericardial Effusion and Tamponade Can Be a First Sign of Familial Mediterranean Fever.

Cardiac involvement is a rare initial presentation of familial Mediterranean fever (FMF). We described 2 children with massive pericardial effusion and cardiac tamponade, who were later diagnosed as having FMF based on clinical and laboratory findings. Therefore, in children presenting with massive pericardial effusion, FMF should be considered as one of the differential diagnoses. In addition, massive pericardial effusion is a serious clinical condition that requires emergency therapeutic approach including pericardiocenthesis. For medical treatment, colchicine is the first line therapy, but in resistant cases, other anti-inflammatory drugs can be used for extra anti-inflammatory effect.

Unusual Presentations of Childhood Systemic Lupus Erythematosus to the Emergency Department.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a multisystemic autoinflammatory disease that can involve any organ system; therefore, diagnosis can be challenging. Hereby, we present 4 cases that presented to pediatric emergency department with unusual clinical pictures of SLE. CASES: Case 1 presented with inability to walk or talk for the last 1 week as well as intermittent pain and swelling in her joints. Case 2 presented with generalized edema and severe dyspnea. Case 3 and 4 presented to pediatric emergency department with rashes on the legs. DISCUSSION: Systemic lupus erythematosus may mimic many clinical entities, and differential diagnosis may be difficult, especially if presentation is atypical. In every emergency physician, right diagnosis and prompt treatment are very important especially in life-threatening conditions such as cardiac involvement in SLE.

Midterm Results of Implantable Cardioverter Defibrillators in Children and Young Adults from a Single Center in Turkey.

BACKGROUND: Despite concerns about complications with the implantable cardioverter defibrillator (ICD), it is effective for the prevention of sudden cardiac death (SCD). We aimed to analyze our midterm experience with ICD in children and young adults. METHODS: This retrospective study included patients who were implanted with an ICD between 2001 and 2014. Demographic characteristics, clinical information, shock features, and complications for all patients with ICD were analyzed. The study population was divided into two groups: early-era patients implanted before 2008, and late-era patients implanted after 2008. RESULTS: Sixty-nine patients (median age: 12 years, median follow-up: 52 months) were implanted with an ICD. Diagnostic categories were channelopathy (56.6%), cardiomyopathy (36.2%), congenital heart disease (5.8%), and other (1.4%). We performed implantation for primary prevention in 66.6% (39.3% in early-era patients and 85.4% in late-era patients). Thirty-one (44.9%) received 139 appropriate shocks (66% of total shocks) while 14 (20.2%) received 71 inappropriate shocks. However, there was no statistically significant difference in the use of appropriate shocks in the primary (66.7%) versus the secondary (72.2%) prevention groups. The incidence of appropriate and inappropriate shock was 66.7% and 33.3% in the primary prevention group, and 72.2% and 27.8% in the secondary prevention group, respectively. Two patients died, although only one death was the result of a lead problem. CONCLUSIONS: Although lead integrity problems, inappropriate shocks, and infections are significant issues, ICD therapy appears to be a safe, effective, and necessary option for the prevention of SCD in both children and young adults.