Congenital heart defects and postoperative follow-up of patients with Williams syndrome as a single center experience and review of the cases from Türkiye.

BACKGROUND: Cardiovascular system involvement is quite common and the leading cause of morbidity and mortality in patients with Williams syndrome (WS), most of whom need surgery. The present study aimed to provide a detailed evaluation of the features of surgical procedures and outcomes of patients with WS given as single-center experience, and additionally to make a detailed review from Türkiye. MATERIALS AND METHODS: Thirty-five children with WS diagnosed between the years 1992 and 2021 were evaluated retrospectively including cardiovascular data, surgical treatment features, and outcomes. A total of six articles from Türkiye were evaluated. RESULTS: A total of 35 patients with Williams Syndrome (24 male) with a median age of cardiologic diagnosis of 6 months (range, 2 days-6 years) were evaluated. The cardiac defects of the patients with WS were found as supravalvular aortic stenosis (SVAS) (n=30, 85%) and peripheral pulmonary stenosis (PPS) (n=21, 65%). Additional cardiac anomalies were seen in 71% patients. The rate of SVAS and PPS surgery in all patients with WS was 77.1%. The median surgical age of the patients was 2.5 years (range, 7 months-15.5 years). No patients died due to surgery. But one patient died because of ventricular tachycardia due to anesthesia at the beginning of angiography. A total of 138 (63% male) patients with WS were evaluated from the articles published in Türkiye. Of 138 patients, 64.4% had SVAS, 52.1% had PPS, and 39.8% had additional cardiac anomaly. The median follow-up period ranged from 17 months to 18 years, and six (4.3%) patients died in the early postoperative period. CONCLUSION: Cardiovascular system involvement is extremely common and is the leading cause of morbidity and mortality in patients with WS, often requiring surgical intervention. As seen in our study including 35 patients with WS and in publications from Türkiye, SVAS in patients with WS generally requires surgery, especially in the first year of life. PPS, on the other hand, requires surgery less frequently than SVAS, and pulmonary stenosis appears to decrease over time.

Neurologic Complications After Pediatric Heart Transplant: A Single-Center Experience.

OBJECTIVES: Neurologic complications that can lead to serious mortality and morbidity in pediatric heart transplant recipients have been reported to range from 23.6% to 45%. In this study, the frequency, time, cause, and characteristics of neurologic complications in pediatric heart transplant recipients were evaluated. MATERIALS AND METHODS: We retrospectively reviewed data of 37 pediatric heart transplant recipients aged <18 years who were seen at our hospital between 2007 and 2017. Medical records were reviewed to identify neurologic complications. Clinical features were compared between pediatric heart transplant patients with and without neurologic complications. RESULTS: The rate of posttransplant neurologic complications in pediatric heart transplant was 27% (10/37). Median age of patients with neurologic complications was 12 years (range, 11-18 years). Median time for neurologic complications was 3 days (range, 2-46 days). Primary diagnoses of these 10 recipients were dilated cardiomyopathy (n = 7) and restrictive cardiomyopathy (n = 3). There were no significant differences between recipients with and without neurologic complications (P > .05).The etiologies of neurologic complications were posterior reversible encephalopathy syndrome in 3 patients (8.1%), stroke in 2 patients (5.4%), peripheral neuropathy in 2 patients (5.4%), hypertensive encephalopathy in 1 patient (2.7%), and drug encephalopathy in 1 patient (2.7%). CONCLUSIONS: Neurologic complications may lead to serious mortality and morbidity in pediatric heart transplant patients. Seizures, posterior reversible encephalopathy syndrome, stroke, peripheral neuropathy, transient ischemic attack, and cerebral infections are the most common neurologic complications, which are seen in the perioperative period in particular. Careful follow-up of pediatric heart transplant patients, with detection and early treatment of neurologic findings, will contribute to lower rates of sequelae. To our knowledge, this is the largest study to show a detailed experience of neurologic complications in pediatric heart transplant patients from a single center in Turkey.

Multisystem Inflammatory Syndrome in Children (MIS-C) Associated with COVID-19: A Case Series Experience in a Tertiary Care Hospital of Southern Turkey.

OBJECTIVE: Aim of the study is to assess the clinical characteristics and treatment outcomes of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19. STUDY DESIGN: The study comprised 52 children with MIS-C admitted to University of Health Sciences Adana City Training and Research Hospital pediatric wards from September 2020 to April 2021. Demographic characteristics and clinical data were retrospectively collected from patient files. RESULTS: Median age of patients was 9 (5-13) years. Fever (92.3%), abdominal pain (76.9%), rash (48.1%) and vomiting (48.1%) were the most common presenting symptoms. Fever duration was 8 (4.25-10) days in overall. Depressed left ventricular ejection fraction was found in 17.3% of patients. At admission, elevated levels of C-reactive protein, procalcitonine, erythrocyte sedimentation rate, D-dimer and ferritin were found in 98.1%, 96.2%, 75%, 84.6% and 69.2% of the patients, respectively. Lymphopenia, hyponatremia and hypoalbuminemia were found in 76.9%, 59.6% and 42.3% of the patients. Intravenous immunoglobulin was used in 96.2%, corticosteroids in 71.2% and anakinra in 3.8% of the patients. In total, 28.8% of the patients were admitted to pediatric intensive care unit and 17.3% received vasopressor support. Median duration of hospital length of stay was 12.5 days. Comorbidities were present in 19.2% of the patients. No mortality was recorded. CONCLUSIONS: While being rare and treatable, MIS-C is the ugly and mysterious face of the COVID-19 pandemic for children. The increasing number of MIS-C cases shows that this phenomenon is more common than thought. Comprehensive studies are required to understand the pathogenesis of the disease and determine the treatment regimens clearly. LAY SUMMARY: While being rare and treatable, multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 is the ugly and mysterious face of the COVID-19 pandemic for children. MIS-C is now thought to be a post-infectious (SARS-CoV2) hyperinflammatory disease secondary to an abnormal immune response, rather than a complete obscurity. The increasing number of MIS-C cases and new case series reports from all over the world show that MIS-C is more common than thought. Despite our increasing experience, we may encounter a new finding every day in MIS-C patients. Therefore, we want to contribute to literature by presenting the MIS-C cases we treated in our clinic in detail. We have experienced that MIS-C patients can apply with similar but also different and unique characteristics. In case of delayed diagnosis or treatment, morbidity and mortality rates may increase. Therefore, the level of awareness and knowledge of all physicians, especially those dealing with pediatric patients, about MIS-C should be increased. Although the early effects of MIS-C are known, we don't have enough information about the long-term consequences yet. Comprehensive studies are required to understand the pathogenesis of the disease and determine the treatment regimens clearly.

The assessment of treatment outcomes in patients with acute viral myocarditis by speckle tracking and tissue Doppler methods.

OBJECTIVES: The aim was to evaluate the role of tissue Doppler imaging (TDI) and speckle tracking echocardiography (STE), to identify myocardial dysfunction, and to evaluate myocardial segmental deformation in acute viral myocarditis. METHODS: Twenty-one patients and twelve healthy children were studied prospectively. The TDI and STE were performed before and after treatment. The myocardial velocities (Sm , Em , and Am ) and time intervals (isovolumic contraction, isovolumic relaxation, and ejection times [ET]) at interventricular septum (IVS), left, and right ventricular basal segments were examined by TDI. The left ventricular global longitudinal strain (LVGLS) and strain rate (LVGLSR), left ventricular global circumferential strain (LVGCS) and strain rate (LVGCSR), and right ventricular global longitudinal strain (RVGLS) and strain rate (RVGLSR) were examined by STE. RESULTS: Sm and Em at IVS and at LV, ET at IVS, ET at RV, ET at LV were significantly lower in patients before treatment than controls. LVGLS, LVGLSR, LVGCS, LVGCSR, RVGLS, RVGLSR were significantly decreased in patients before treatment than controls. There was significant improvement for LVGLS, LVGLSR, LVGCS, LVGCSR, and RVGLS in patients after treatment. Sm , Em , and Am at LV were significantly lower in patients before treatment than in patients after treatment. In spite of improvements, Sm , Em , and ET at IVS, LVGLS, LVGLSR, LVGCS, LVGCSR were significantly lower in patients after treatment than controls. CONCLUSIONS: The TDI and STE were useful methods for detection of early myocardial dysfunction and evaluation of treatment outcomes in acute viral myocarditis.

Wolff-Parkinson-White syndrome presenting with steroid-induced bradycardia in a patient with acute rheumatic fever.

Steroids are used in the treatment of acute rheumatic fever with moderate-to-severe carditis. Corticosteroids have several cardiovascular side affects that are more common in adults than in children. Corticosteroid-related bradycardia is a rarely seen side effect. Children with bradycardia following oral corticosteroid use are rarely reported previously. We present a child who developed bradycardia after oral corticosteroid treatment and concurrent Wolff-Parkinson-White pattern.

The pre-participation screening in young athletes: which protocol do we need exactly?

The aim was to assess the utility and feasibility of a comprehensive cardiac screening protocol in young athletes before participation in sports. A total of 380 athletes referring before participation in sports, between April, 2014 and April, 2015, were included in this study. The mean age was 12.4 years. A screening protocol has been applied to all, including personal and family history, physical examination, 12-lead electrocardiography, transthoracic echocardiography, 24-hour rhythm Holter analysis, and treadmill exercise test. The most frequent complaints were chest pain in 19 (5%), dyspnoea in 13 (3.4%), and dizziness and fainting in five patients (1.3%) on exercise. There was sudden death and arrhythmia in 41 patients (10.7%) owing to family history. Heart murmur was present in 20 (5.2%) and hypertension in 10 patients (2.6%) on physical examination. The 12-lead electrocardiography was abnormal in 9 patients (2.4%). The findings of transthoracic echocardiography were insignificant in 47 patients (12.3%) and in five patients (1.3%) a haemodynamically important condition was detected. The 24-hour rhythm Holter analysis was abnormal in six patients (1.5%). There were significant ST changes in two patients (0.5%) on treadmill exercise test with normal findings on myocardial perfusion scans. No significant relation was present between findings of screening protocol and transthoracic echocardiography, 24-hour rhythm Holter analysis, or treadmill exercise test results. Pre-participation screening in young athletes should consist of a targeted personal history, family history, physical examination, and 12-lead electrocardiography. Other tests should be applied only if the screening indicates the presence of a cardiovascular disease.

Recovery of myocardial mechanics in Kawasaki disease demonstrated by speckle tracking and tissue Doppler methods.

AIM: To investigate myocardial deformation and function during treatment for Kawasaki disease (KD) in children. METHODS: We performed speckle tracking echocardiography (STE) and tissue Doppler imaging (TDI) in 15 children with KD and 15 healthy children during treatment for KD. STE was performed for longitudinal and circumferential strain (S) and strain rate (SR) at the left ventricle (LV) and for longitudinal S and SR at the right ventricle (RV). TDI was performed at the base of interventricular septum (IVS), LV, and RV. RESULTS: Among TDI parameters, Em and ejection time (ET) at IVS, ET at LV and ET at RV obtained obtained before treatment were significantly lower in patients with KD compared to controls. After treatment, in spite of improvements, ET at IVS and ET at RV remained significantly lower in patients with KD compared to controls. Left ventricular global longitudinal and circumferential S and SR values obtained before treatment were significantly lower in patients with KD compared to controls. Left ventricular S and SR values were found to be increased after treatment. However, left ventricular global circumferential S value remained significantly lower in patients with KD compared to controls. There were no significant differences in right ventricular global longitudinal S and SR values between patients and controls before treatment. CONCLUSION: During acute phase, patients with KD have reduced global left ventricular S and SR which may be more sensitive indicators of myocardial inflammation. This study showed gradual improvements in left ventricular myocardial function during treatment for KD.

A rare cause of facial nerve palsy in a young infant: Kawasaki disease.

Orgun A, Karagöl C, Pamuk U, Gürsu HA, Çetin I. A rare cause of facial nerve palsy in a young infant: Kawasaki disease. Turk J Pediatr 2018; 60: 433-435. Kawasaki disease (KD) is a vasculitis in which the most common complication is development of coronary aneurysms. Neurological complications rarely occur in KD patients such as facial nerve palsy (FNP). FNP associated with KD may indicate increased risk of coronary artery aneurysm. Infants with facial nerve paralysis and unexplained-prolonged febrile period should be evaluated with echocardiography. Here in, we present a 4-month-old female with FNP and unexplained fever who was diagnosed KD due to echocardiographic findings.

The efficacy and safety of naproxen in acute rheumatic fever: The comparative results of 11-year experience with acetylsalicylic acid and naproxen.

The objective was to compare the efficacy and safety of naproxen (NXN) to acetylsalicylic acid (ASA) in the treatment of acute rheumatic fever (ARF). The data of 338 children were retrospectively analyzed. The patients were grouped according to joint and valve involvement and also drug chosen [methyl prednisolone (mPSL), ASA or NXN]. The treatment results and adverse events in each group were compared. The mean age was 10.3 years and the median follow-up was 62 months. Median time for normalization of acute phase reactants was 1 week in patients given steroids and 2 weeks in patients given ASA or NXN. ASA was replaced with NXN in 18 patients (10.2%) due to hepatic toxicity. The rate of rebound, recurrence and the prevalence of rheumatic valve disease were not different in patients given NXN, ASA or mPSL. In conclusion, NXN is a safe and effective alternative to ASA in the treatment of ARF in children.