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Objective: Extracorporeal membrane oxygenation (ECMO) is a life-saving treatment option providing cardiopulmonary support when standard therapies prove insufficient for reversible diseases. The mean objective of this study was to evaluate our center’s experience with ECMO following pediatric cardiac surgery. Materials and Methods: This retrospective study was conducted in our pediatric intensive care unit (PICU) between November 2014 and March 2021 and included patients who received ECMO following cardiac surgery. Results: Over the 7-year period, 324 patients underwent cardiac surgery, of which 24 (7.4%) required ECMO support. Among them, 13 (54.2%) were female, with a median age of 16.0 (2.0-208) months and a median weight of 7.0 (3.5-70) kg. The mean vasoactive inotrope score (VIS) was 53.9 ± 44.5. Atrioventricular septal defect repair was the most common surgical procedure (n = 8/24, 41.6%). The primary indication for ECMO was low cardiac output syndrome (LCOS) in 14 (58.3%) patients. The median duration of ECMO support was 6.0 (1.0-46.0) days. Nonsurvivors had significantly higher Pediatric Risk Score of Mortality (PRISM) III scores (P = .014) and VIS scores during the pre-ECMO period (P = .004). Early or late neurological complications developed in 12 (50%) patients, with significant differences in lactate levels and pH levels pre-ECMO between those with and without neurological complications (P = .01, P = .02, respectively). We successfully decannulated 16 (66.6%) patients, with a final survival rate of 12 (50%). Conclusion: ECMO plays a crucial role in providing pre- and post-cardiac surgery support for children. LCOS remains the main indication, and high PRISM III and VIS scores are valuable predictors of outcomes. [ABSTRACT FROM AUTHOR]

Objective: Extracorporeal membrane oxygenation is a life-saving treatment for patients with circulatory and respiratory failure refractory to standard therapy. However, safe and timely patient transport to the referral extracorporeal membrane oxygenation center is critical for better patient outcomes in patients with acute cardiogenic shock. This study aimed to describe children’s features who were transferred to our center under extracorporeal membrane oxygenation by aircraft/ground vehicle and demonstrated the importance of extracorporeal cardiopulmonary resuscitation for transported children. Materials and Methods: We report the first Turkish pediatric case series of patients with acute cardiogenic shock transported by aircraft and ground ambulances on extracorporeal membrane oxygenation support to a referral extracorporeal membrane oxygenation center between January 2016 and January 2021. Results: Overall, 6 patients on venoarterial extracorporeal membrane oxygenation support were transported by aircraft and ground vehicles to our pediatric intensive care unit. Transport was achieved by fixed-wing aircraft in 5 patients and commercial aircraft in 1. Our mobile extracorporeal membrane oxygenation team cannulated 3 patients, and 3 patients were cannulated by the team at the hospital they applied to. The median age was 112 (range: 14-204) months and the median weight was 28.6 kg (range: 8.6-57.2 kg). The etiology of acute cardiogenic shock was fulminant myocarditis in 4 patients, dilated cardiomyopathy in 1, and transposition of great arteries and atrial flutter in 1. The median distance of travel for the patients to our hospital was 618 (407-955) km. No adverse events were detected during aircraft or ground vehicle transport. Conclusion: Mobile pediatric extracorporeal membrane oxygenation transport teams may provide safe aircraft and ground vehicle transportation in high-risk patients with acute cardiogenic shock bridging to survival or long-term circulatory support. [ABSTRACT FROM AUTHOR]

The article presents a report which provide an evaluation of the cardiovascular system and management of complications in children who experienced COVID-19 infection without underlying cardiac disease. It mentions the guideline for the management of pediatric patients during the pandemic with an emphasis on cardiac evaluation.

We aimed both to define the characteristics of patients with Kawasaki Disease (KD), and to highlight infrequent and unusual findings of the disease by presenting selected cases. We retrospectively evaluated 35 patients diagnosed with KD in our clinic between January 1994 and January 2013. The male to female ratio was 1.33 and the median age at admission was 22 months (1.5-132 months). Fourteen patients (40%) had coronary artery lesions (CAL). Twenty-five cases (72%) had complete and 10 cases (28%) had incomplete KD; the incidence of CAL in these groups was 36% and 50%, respectively. Two patients had giant coronary aneurysms. Six cases received a second dose of intravenous immunoglobulin, and one patient received pulse methylprednisolone. Seven cases had unusual and/or infrequent presentation patterns and/or follow-up. Physicians should be aware of all symptoms and laboratory findings of KD in order to avoid any delays in diagnosis and decrease the risk of life-threatening complications. [ABSTRACT FROM AUTHOR]

A 2.5-month-old boy admitted to our hospital with irritability, poor feeding and fever of 12 hours' duration. On physical examination, he was febrile and extremely irritable. Initial whole blood count revealed a hemoglobin level of 10.1 g/dl, white blood count of 17,800/mm³ and platelet count of 454,000/mm³. Erythrocyte sedimentation rate was 80 mm/h and C-reactive protein was 3.96 mg/dl. Biochemical examinations of serum, urinalysis, chest X-ray, and analysis of cerebrospinal fluid (CSF) were normal. He was started on intravenous ampicillin and ceftriaxone empirically for provisional occult bacteremia. His blood, urine and CSF cultures were negative. On the 7th day of the treatment, there were no additional symptoms or findings other than fever. Echocardiography revealed aneurysms in both the left and right coronary arteries. Intravenous immunoglobulin (IVIG) and per oral aspirin were administered, and the fever resolved after IVIG infusion. Two years later, the echocardiography showed disappearing of the saccular aneurysm on the right coronary artery, but the dilatation of the left coronary artery was persisting. In conclusion, incomplete Kawasaki disease should always be included in the differential diagnosis of an infant with persistent fever, especially one younger than three months of age, when the conventional work-up fails to reveal the underlying cause. [ABSTRACT FROM AUTHOR]

We hypothesized that decreased sympathetic/parasympathetic balance as a result of the absence of auditory stimuli on the autonomic nervous system might be an explanation for our previous finding of lower mean heart rate in congenitally deaf children. To test our hypothesis, we obtained heart rate variability (HRV) data of 23 children with congenital sensorineural deafness from 24-hour Holter ECG recordings and compared them to data of 18 healthy children. HRV was measured by calculating time-domain and frequency-domain indexes from 24-hour recordings and from 6 hours of recordings obtained while subjects were sleeping. We additionally compared the HRV values obtained from children with and without GJB2 gene mutations. We did not find any significant difference in HRV parameters between deaf children and healthy children. There were also no significant differences in HRV parameters between deaf children with and without GJB2 mutations. We conclude that cardiac autonomic dysfunction does not seem to be present in patients with congenital sensorineural deafness. [ABSTRACT FROM AUTHOR]

Central venous catheter (CVC) fracture with embolization is a serious and rare complication, and few cases have been reported in children. Catheter fragments may cause cardiac perforation, arrythmias, pulmonary embolism, and sepsis. We report the successful retrieval of intracardiac CVC fragments by percutaneous transcatheter technique in two infants, aged 8 and 15 months. Double-lumen 7 French Hickman CVCs were accidentally fractured during their removal. Chest radiographs of the two patients revealed migrated intracardiac catheter fragments extending from the superior vena cava (SVC) to the right atrium and from the SVC to the right ventricle, respectively. The procedure was performed under ketamine anesthesia and fluoroscopic guidance using a percutaneous femoral vein approach. Nitinol Amplatz Goose Neck™ snares (10 mm in diameter) were used to successfully retrieve the catheter fragments without any complication. Percutaneous transcatheter retrieval of catheter fragments using Goose Neck snare is a safe and successful technique and can be chosen before resorting to surgery, which has potential risks related to general anesthesia, thoracotomy and cardiopulmonary bypass. [ABSTRACT FROM AUTHOR]

The aim of this study was to evaluate the incidence and spontaneous closure rate of ventricular septal defects in a randomly selected newborn population, using color flow Doppler echocardiographic screening. Color flow Doppler echocardiographic screening was performed in 1075 neonates within the first 72 hours of life. We also followed-up patients with ventricular septal defect for a year to detect spontaneous closure rate and its timing. The incidences of ventricular septal defect in all neonates, preterm neonates and term neonates were found as 47.4/1,000, 56/1,000 and 46.3/1,000 live births, respectively. All patients with ventricular septal defect except one were asymptomatic. Forty-six cases had a trabecular and five cases had perimembranous ventricular septal defect. With the exception of one case, all had a small ventricular septal defect (≤3 mm). Spontaneous closure was observed in 88.6% of ventricular septal defects within the first year. Closure rate was found as 100% for preterm infants and 87.8% for term infants (p>0.05). The incidence of ventricular septal defect was considerably high in neonates when routine color flow Doppler echocardiographic examination was performed. Despite increased incidence of ventricular septal defect, spontaneous closure rate was remarkably high within the first year of life. These defects may result from delayed physiologic development and have fairly good prognosis. [ABSTRACT FROM AUTHOR]

We report a rare case of interventricular septal hematoma after patch closure of a perimembranous ventricular septal defect in a 10-month-old infant. Intraoperative transesophageal echocardiography was not performed. Routine transthoracic echocardiography at the 1st postoperative hour showed a huge intramural hematoma causing severe thickening of the ventricular septum. The patient's hemodynamics were stable and surgical revision was not required. The patient recovered well without complication. [ABSTRACT FROM AUTHOR]

We report a rare case of interventricular septal hematoma after patch closure of a perimembranous ventricular septal defect in a 10-month-old infant. Intraoperative transesophageal echocardiography was not performed. Routine transthoracic echocardiography at the 1st postoperative hour showed a huge intramural hematoma causing severe thickening of the ventricular septum. The patient's hemodynamics were stable and surgical revision was not required. The patient recovered well without complication. [ABSTRACT FROM AUTHOR]

Left ventricular noncompaction (LVNC) is a rare cardiomyopathy. It usually presents with ventricular dysfunction, thromboembolic events and arrhythmias. An asymptomatic clinical course is also possible. LVNC is frequently associated with other congenital heart diseases including heart valve abnormalities. The coexistence of LVNC with double-orifice mitral valve was observed rarely. The presence of such coexistence in a patient presented with heart failure in the newborn period is reported herein because of its rarity. [ABSTRACT FROM AUTHOR]

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article presents case study of a 5-year-old girl, who was the first child of second-degree consanguineous parents, with no significant medical history, and was admitted with complaints of malaise and abdominal pain. The patient had tachypnea and tachycardia, and after being treated for congestive heart failure (CHF) for 2 years, left ventricular assist device was implanted. Biopsy of the heart revealed widespread multifocal myofibrillar damage and interstitial fibrosis.

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Journal of Pediatric Emergency & Intensive Care Medicine / Çocuk Acil ve Voğun Bakım Dergisi is the property of Galenos Yayinevi Tic. LTD. STI and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The article reports on coronary artery evaluation in Kawasaki disease by dual source multi-detector CT coronary angiography in children. It is stated that Kawasaki disease is a childhood vasculitis of medium-sized vessels. Multi-detector computed tomography coronary angiography is a noninvasive alternative imaging modality. It is stated that it can also provide complementary morphologic data to echocardiography.

Invasive aspergillosis (IA) is a major cause of morbidity and mortality. This study aimed to present our 10-year IA experience at a single center. Fifty-nine pediatric patients with IA were included in this study. The male-to-female ratio was 42/17. The median age was 8.75 years. Hematologic malignancy was present in the majority of the patients (40/59, 68%). The mean neutropenia duration was 18.5 days. Cytosine arabinoside was the most common immunosuppressive therapy directed at T cells during IA diagnosis. IA cases were categorized as proven (27%), probable (51%), or possible (22%) according to the 2008 European Organization for Research and Treatment of Cancer/Invasive Fungal Infections Cooperative Group and the National Institute of Allergy and Infectious Diseases Mycoses Study Group (EORTC/MSG) criteria. The lungs (78%) were the most common site of IA, and nodules were the most frequent radiological findings (75.5%). In 38 patients (64.4%) receiving antifungal prophylaxis, prophylactic agents included fluconazole (30.5%), liposomal amphotericin B (23.7%), posaconazole (8.5%), and voriconazole (1.7%). Initial treatment was most commonly administered as monotherapy (69.5%). The median antifungal treatment duration was 67 days. Eleven deaths (18.6%) were due to aspergillosis. With the increased use of corticosteroids, biological agents, and intensive immunosuppressive chemotherapy, IA will most likely continue to occur frequently in pediatric patients.

Arterial tortuosity syndrome (ATS) is a rare hereditary, autosomal recessive, connective tissue disorder. Herein, we describe a five-year-old boy. He had hyperextensible skin, atypical facial features and inguinal hernia. We present his vascular imaging studies of kinking and tortuosity of the aorta and bilateral multiple peripheral pulmonary artery stenoses determined by conventional and magnetic resonance angiographic (MRA) examination. ATS must be considered in patients with connective tissue condition associated with diffuse arterial changes and involvement of the skin, joints and other organs. Vascular imaging studies, especially MRA, are useful in the screening of these vascular abnormalities.

Neurocardiogenic syncope (NS) is a common clinical problem during childhood. In order to characterize the autonomic profile in syncopal children, we evaluated heart rate variability (HRV) of 27 patients, ages 8-17 years, in an asymptomatic period. HRV was calculated over a 24-hour (h) period for the time-domain indices and frequency-domain indices. The obtained results were compared to values in 27 healthy children, ages 10-17 years. Patients with syncope did not have significantly different HRV values in comparison to healthy children. After behavior modification and education, HRV assessment was repeated six months later in the symptomatic group under follow-up, and we found no significantly different values. We conclude that there was no significant difference in baseline autonomic values between children with NS and the healthy control group. After behavior modification and education, none of the patients with NS under follow-up experienced syncope or presyncope during the follow-up period. The major complaint had disappeared but did not reflect the HRV values. There was no significant difference in baseline HRV values of the patients in follow-up. We conclude that patients are predisposed to faint by a triggering autonomic stress, without having constant autonomic instability.

Rhabdomyoma is the most common pediatric heart tumor. Cardiac rhabdomyomas (CRs) have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). We aimed to evaluate the clinical presentation and outcome of CRs and their association with TSC. Patients with CRs diagnosed in last six years were retrospectively analyzed. A total of 25 tumors were identified in seven patients by echocardiography. Three patients were diagnosed prenatally by fetal echocardiography, three patients in the neonatal period and one patient in early infancy. The median followup period was two years (range: 5 months-6 years). Five patients (71%) had multiple tumors. Three patients had arrhythmias and two patients required surgery. Only 36% (9/25) of the tumors regressed. TSC was diagnosed in four patients during the follow-up. CRs may have different presentations and clinical course. Surgery is only necessary when hemodynamically significant obstruction is present. As CRs are associated with long-term development of TSC and other diagnostic features are not yet typically apparent in the prenatal and neonatal periods, careful evaluation and follow-up are essential to exclude TSC.

Fontan operation is the connection of the systemic venous return to the pulmonary arteries, performed in patients with single ventricle physiology. The Fontan circuit is commonly fenestrated because of early postoperative risks such as high systemic venous pressure and low cardiac output. As it causes progressive cyanosis and increased risk of paradoxical embolism in the follow-up period, occlusion of these fenestrations is generally suggested. Successful closure of extracardiac Fontan fenestration with atrial septal occluder in a 10-year-old girl is reported herein. To our knowledge, this is the first such report from Turkey.

The aims of this study were to evaluate the structure of the mitral valve (MV) and subvalvar apparatus in patients with rheumatic mitral regurgitation (MR) by echocardiography and to compare the differences in morphologic abnormalities between subgroups of patients with and without mitral valve prolapse (MVP). Two-dimensional and color Doppler echocardiographic examinations were performed in 20 consecutive patients with isolated rheumatic MR and in 15 healthy subjects as controls. Annular diameter, left ventricular end-diastolic dimension, anterior leaflet length, and both leaflet thicknesses were greater in MR than those of controls. Anterior leaflet and chordal lengths were greater in severe MR than in mild or moderate MR. Sixty percent of rheumatic MR patients had nodules on the body or tip of the anterior mitral leaflet and MR was more severe in these patients. Nine of 20 patients (45%) had MVP. MR was more severe in the patients with MVP than those without prolapse. Rheumatic etiology should be suspected in patients with MR when irregular focal thickening of MV, relatively immobile posterior leaflet, eccentric regurgitant jet, and anterior MVP are found in echocardiographic study.

Between 1993 and 2002, apical muscular ventricular septal defects were identified in 99 cases by echocardiographic examination. Spontaneous closure time was analyzed in 42 cases followed up at least two years, retrospectively. The ages of 42 cases ranged between 1 day and 13 years old. Initial examination was performed in 22 cases within the newborn period, in 8 cases between 1 and 6 months of age and in 12 cases at more than 6 months of age. Spontaneous closure was seen in 24 of 42 cases (57.1%) between 1 and 36 months of age, and it was most commonly recorded during the first 6 months. It was remarkable that spontaneous closure occurred in 20 of 22 cases (90%) diagnosed in the neonatal period. Closure was seen in 4 of 8 cases (50%) whose initial examination was performed between 1 and 6 months and in none of the patients diagnosed at more than 6 months of age. In conclusion, the frequency of spontaneous closure in cases diagnosed in the neonatal period is higher than previously believed. It is advisable to follow up patients to determine spontaneous closure, especially within the first two years of life.

Scimitar syndrome is a rare congenital abnormality. Clear anatomic definition is an important issue before operation. We report definition of both abnormal pulmonary venous drainage and anomalous systemic arterial supply by gadolinium-enhanced magnetic resonance angiography and three-dimensional computerized tomography in an eight month-old infant with scimitar syndrome. The presented case study confirms that these noninvasive diagnostic tools can successfully be used to provide valuable information about vascular anatomy in infants with scimitar syndrome.