18F-fluorodeoxyglucose positron emission tomography/CT in the diagnosis of right-sided endocarditis in children and adults with infective endocarditis.

OBJECTIVE: Infectious endocarditis poses a diagnostic challenge due to its highly variable clinical presentation. To establish a definitive diagnosis, different imaging modalities are essential. In recent years, positron emission tomography/CT has gained increasing significance in diagnosing infective endocarditis; however, its application in the pediatric age group remains limited. This study encompasses patients definitively or potentially diagnosed with infectious endocarditis at our institution from 2018 to 2023. METHODS: A total of 29 patients underwent 18F-fluorodeoxyglucose positron emission tomography/CT examinations, with 19 of them presenting with right-sided infective endocarditis. RESULTS: Evidence consistent with infective endocarditis was observed in 18 (94.7%) of the patients. Pulmonary septic embolism was identified in 15 (78.9%) cases, and splenic involvement was noted in 12 (57.8%) cases. Transthoracic/transesophageal echocardiography failed to reveal vegetation or provided uncertain results in six patients, whereas fluorodeoxyglucose-positron emission tomography-CT exhibited involvement. Subsequently, the diagnosis of infective endocarditis was confirmed post-surgery based on the fluorodeoxyglucose-positron emission tomography-CT findings. CONCLUSION: Our results, along with our clinical experience, demonstrate that fluorodeoxyglucose-positron emission tomography-CT is a safe and viable method for diagnosing right-sided endocarditis, which is often challenging to visualize using echocardiography.

Rapid reversal of left ventricular enlargement after percutaneous closure of pulmonary sequestration in a newborn with prune belly syndrome.

In this article, we describe a newborn with Prune belly syndrome who presented with left ventricular dilation due to an extensive intralobar sequestration of the left lung. Pulmonary sequestration was combined with congenital cystic adenomatoid malformation and also had coarctation of the aorta. Percutaneous closure of the anomalous aberrant artery feeding the sequestrated lung and balloon angioplasty for coarctation resulted in prompt regression of the left ventricular enlargement in the catheterization lab.

Predictors of prolonged pleural effusion after Fontan operation.

Prolonged pleural effusion is a fairly common condition which has considerable impact on complicated and longer hospital stays after Fontan surgery. Identifying the patient population prone to have pleural effusions is still seeking for an answer. This study is to determine the variables that may predict prolonged pleural effusion according to the data of 69 patients who underwent Fontan operation between June 2018 and December 2020 and survived to date. Prolonged pleural effusion was defined as the need for a chest tube for more than 7 days. Two patient groups, with and without prolonged effusion, were compared in terms of pre-, peri-, and post-operative variables. The patients were subdivided into "high-risk" and "low-risk" groups based on the pre-operative catheterisation data. The most frequent main diagnosis was tricuspid atresia (n: 13, 19%). Among 69 patients, 28 (40%) had prolonged pleural effusion whereas 11 (16%) had effusions that lasted longer than 14 days. Ten patients among prolonged effusion group (35%) had pulmonary atresia coexistent with the main diagnosis. Fontan operation was performed in 6 patients (8.7%) over the age of 10, and 4 of these patients (67%) had prolonged pleural effusion. Among numerous variables, statistical significance between the two groups was achieved in pre-operative mean pulmonary artery pressure, post-operative albumin, C-reactive protein levels, length of hospital stay, duration of chest tube drainage, and amount of effusion per day. Early recognition and treatment strategies with routine medical protocol use remain to be the cornerstone for the management of post-operative prolonged pleural effusions after Fontan surgery.

Effect of aortic arch surgery in newborns' cerebral and gastrointestinal hemodynamics: evaluation by Doppler ultrasonography.

AIM: In this study, we investigated changes in newborns' cerebral and intestinal blood flow who had undergone aortic arch surgery. METHOD: This study was carried out prospectively as a preliminary study in patients younger than 30 days at the time of aortic arch reconstruction between 1 August and 1 December, 2019. Cerebral and gastrointestinal hemodynamics were evaluated with Doppler USG before and 7 days after the operation. The middle cerebral artery (MCA) and celiac artery (CA) were used as measurement sites. Patients' peak systolic velocity (PSV), mean systolic velocity (MV), end diastolic velocity (EDV), resistive index (RI) and pulsatility index (PI) were evaluated. RESULTS: A total of 16 patients enrolled in the study. The patients' median weight was 3.2 kg (2.7-4.5 kg), and age was 21 days (7-30 days). Six of them were female. Seven of the patients who underwent arcus reconstruction had an additional ventricular septal defect. The preoperative Doppler USG values of patients' were as follows: for MCA, the mean PSV was 37 ± 12 cm/s, EDV 12 ± 5 cm/s, MV 22 ± 19 cm/s, RI 0.70 ± 0.03, PI 1.24 ± 0.23, and for CA mean PSV was 67 ± 32 cm/s, EDV 29 ± 14 cm/s, MV 24 ± 9 cm/s, RI 0.79 ± 0.27, and PI 1.63 ± 0.89. Doppler USG values of patients' at the postoperative seventh day were as follows: for the MCA, mean PSV 41 ± 13 cm/s, EDV 13 ± 4 cm/s, MV 25 ± 10 cm/s, RI 0.64 ± 0.05, PI 1.23 ± 0.20, and for the CA mean PSV 70.5 ± 34 cm/s, EDV 32 ± 16 cm/s, MV 26 ± 8 cm/s, RI 0.75 ± 0.1, and PI 1.60 ± 0.38. There was a significant decrease in RI of both MCA and CA on the postoperative 7th day compared to the preoperative period (p < 0.05). CONCLUSION: In newborns, there are significant changes in cerebral and intestinal blood flows after aortic arch surgery. RI decreased significantly, especially in the CA and MCA.

Prevalence of Jervell-Lange Nielsen syndrome in children with congenital bilateral sensorineural hearing loss.

OBJECTIVE: Long QT syndrome (LQTS) is an inherited cardiac ion channel disorder (channelopathy) that is characterized by prolonged QT intervals on the electrocardiography (ECG) and possess the risk of sudden cardiac death (SCD). Jervell-Lange Nielsen syndrome (JLNS) is a specific subtype of LQTS that is accompanied by congenital sensorineural hearing loss, inherited autosomal recessively, and higher risk of SCD. In this study, we aimed to investigate JLNS prevalence in deaf children attending special schools for hearing loss, located in our province. METHODS: An ECG screening program was conducted in 6 special schools for children with hearing loss in Istanbul and a total of 440 students between 6 and 18 years old were included. Corrected QT interval (QTc) was calculated using the Bazett formula. Notably, 51 students, detected with any abnormal finding on ECG, were invited to our center for a comprehensive examination. RESULTS: A total of 8 patients were found with a prolonged QT interval. JLNS was diagnosed in 4 (0.9%) patients. In addition, 2 students had already been diagnosed with JLNS at another center earlier. The other 2 students, being siblings, were newly diagnosed with JLNS; and appropriate treatment was initiated. Genetic testing revealed a pathological homozygous mutation in KCNQ1 gene. The younger sibling (Case 1), who possessed a QTc of greater than 500 ms and a history of syncope, which was very suspicious for SCD, was implanted an implantable cardioverter-defibrillator. Propranolol treatment was initiated for both siblings. CONCLUSION: JLNS should be carefully considered and screened, especially in patients with a history of congenital deafness.

A case of a very large haemorrhagic pericardial effusion in an adolescent patient with COVID-19 infection.

An otherwise healthy 17-year-old adolescent boy presented with upper respiratory tract symptoms and was diagnosed with coronavirus disease 2019 infection. A haemorrhagic pericardial effusion was found in the transthoracic echocardiography. He was treated with pericardiocentesis, ibuprofen, and hydroxychloroquine. A large pericardial effusion may complicate COVID-19 in children and should be considered in acute decompensation.

Univentricular Pulmonary Artery Banding: How Tight is Tight Enough for Successful Progress?

We investigated the effects of intraoperative parameters measured during pulmonary artery banding operations and pre-discharge parameters on the completion of Fontan procedures. Fifty consecutive patients with single-ventricle anomalies and unrestricted pulmonary blood flow who underwent a PAB operation in and were discharged from our hospital were retrospectively analyzed. Patients who underwent a Fontan operation, a Glenn shunt operation, or who were eligible for a Fontan procedure were defined as the "successful group." Patients who needed rebanding prior to a bidirectional Glenn shunt, patients who were not eligible for a Glenn shunt, and those underwent a takedown due to high pulmonary arterial pressure after implantation of a Glenn shunt were defined as the "failure-to-progress group." The successful group included 34 (68%) patients and the failure-to-progress group included 16 (32%) patients. The median age was 2 months (IQR 1-4 months). There was a statistically significant difference between the groups in terms of systolic pulmonary arterial pressure, mean pulmonary arterial pressure, and pulmonary arterial pressure/systemic arterial pressure after PAB (P = 0.01, 0.03, and 0.03, respectively). While the median gradient before discharge was 60 mm Hg (IQR 50-70 mm Hg) in the successful group, it was 47.5 mm Hg (IQR 45-63.7 mm Hg) in the failure-to-progress group (P = 0.05). Mortality was observed in one (2.9%) patient in the successful group and five (31.2%) patients in the failure-to-progress group (P = 0.04). Successful pulmonary arterial banding increases long-term survival. Adequate targets should be determined, efforts should be made to achieve these targets, and patients should be followed up closely in terms of rebanding when the targets are not reached.

Is it possible to reduce radiation exposure during transcatheter atrial septal defect closure in children?

OBJECTIVE: Cardiac catheterization continues to be a major source of radiation exposure for patients with congenital heart disease. As children are more prone to both deterministic and stochastic effects of radiation, every effort should be made to reduce radiation exposure. One way to reduce the radiation dose is to lower the pulse fluoroscopy rate. This study is an examination of the magnitude of radiation exposure with a 3.75 frames per second (fps) pulse fluoroscopy rate and a comparison with the previous 15 fps protocol used for transcatheter atrial septal defect (ASD) closure. METHODS: The radiation dose delivered during ASD device closure procedures performed between 2014 and 2016 (Group 1: 3.75 fps fluoroscopy rate) was compared with that recorded in procedures performed between 2011 and 2014 (Group 2: 15 fps fluoroscopy rate). The radiation dose was quantified as air kerma dose (milligray, mGy) and dose area product (DAP; mGy/m2). RESULTS: There were 80 patients in each group. Baseline demographic characteristics and the body weight and height measurements were similar between groups. The mean fluoroscopy time was significantly longer in Group 2. Since the fluoroscopy time was significantly different between groups, the DAP and air kerma dose were indexed according to fluoroscopy time. In Group 1, the DAP and air kerma indexed to body weight values were statistically lower than those of Group 2 (p<0.001). CONCLUSION: A significant reduction in the radiation dose was observed with the implementation of 3.75 fps pulse fluoroscopy, which is the lowest in use. Novel radiation dose reduction protocols can be easily applied without compromising safety or the effectiveness of transcatheter ASD closure and should be utilized for the safety of patients and healthcare staff.

Parameters affecting pleural drainage and management strategy after Fontan operation.

BACKGROUND: Prolonged pleural drainage is a common complication after undergoing the Fontan procedure. Although various protocols have been described, there is no definitive consensus for how to treat this complication. MATERIALS AND METHODS: Our primary aim was to determine the effect of the management strategy protocol on the duration of drainage and length of hospital stay. Our secondary aim was to determine the parameters affecting the need for prolonged drainage after the Fontan procedure. Ninety-two consecutive patients who underwent the Fontan procedure were retrospectively analyzed. A protocol-based postoperative management strategy was adopted in July 2018. Group 1 (n = 48) consisted of patients that underwent the procedure before the protocol was implemented. Group 2 (n = 44) consisted of patients that underwent the procedure after the protocol was implemented. RESULTS: The mean age was 5 years (interquartile range [IQR], 4.0-6.9); the mean body weight was 17.3 kg (IQR, 15.1-21.8). Statistically significant differences were found between the groups in terms of total drainage, duration of pleural drainage, prolonged drainage, and length of hospital stays (LOHS) (P = .05, P = .04, P = .04, P = .04, respectively). The multivariate analysis results showed that the application of the protocol was the only factor impacting prolonged drainage (OR, 2.46, 95% CI lower-upper: 1.03-5.86, P = .04). CONCLUSION: Standardization and strict application of the medical treatment within a specific protocol without being affected by doctor-, nurse-, or patient-based factors increases the success rate of this procedure. After implementing the changes in the medical management strategy, total drainage and duration of pleural drainage and LOHS decreased, and the costs associated with these factors also decreased.

In newly diagnosed hypertensive children, increased arterial stiffness and reduced heart rate variability were associated with a non-dipping blood pressure pattern.

OBJECTIVES: This study was designed to investigate the differences in pulsatile hemodynamics, echocardiographic findings, 24-h Holter monitoring and heart rate variability parameters of dipper patterns in children with newly diagnosed essential hypertension. METHODS: This study included 30 children with newly diagnosed essential hypertension and 30 healthy controls. The essential hypertension cohort was divided into dippers and non-dippers. Physical examinations, 24-hour ambulatory blood pressure monitoring, 24-h Holter monitoring, 24-h heart rate variability, conventional 2-dimensional and Doppler echocardiography, and tissue Doppler imaging were performed. Pulse wave analysis using an oscillometric monitor was conducted to measure augmentation index (AIx) and pulse wave velocity (PWV). RESULTS: In patients with essential hypertension, left ventricular (LV) wall thickness and LV mass index were increased. There were no significant differences in LV mass index and LV wall thickness based on the dipping patterns. Time domain values and the standard deviation of all RR intervals (SDNN) were substantially lower in the essential hypertension group. SDNN values were considerably lower in the non-dipper group compared with the dipper group. In terms of frequency domain measures, low frequency measured in daytime values was much lower in the essential hypertension group compared with the control. The dipper patterns revealed that low frequency measured in nighttime values was also substantially lower in the non-dipper group. Pulse wave analysis and AIx values were notably higher in the essential hypertension patient group and those with non-dipper status. CONCLUSION: SDNN values, which reflect parasympathetic activity, were markedly lower in children with hypertension and the non-dipper group than healthy controls and the dipper group, respectively. Also, parameters related to arterial stiffness, such as PWV and AIx values were significantly higher in children with hypertension and the non-dipper group.

Cardiovascular assessment after treatment for retinopathy of prematurity: a comparative study between anti-VEGF agent (aflibercept) and laser.

OBJECTIVE: The aim of this study was to compare the cardiac effects and aortic arterial indices following intravitreal aflibercept treatment or diode laser photocoagulation for the treatment of retinopathy of prematurity (ROP) in infants. METHODS: This single-centre, retrospective study was conducted in infants who were administered laser photocoagulation (LPC) or intravitreal aflibercept (IVA) treatment as initial treatment and had completed at least one year of corrected age. The patients were evaluated in terms of aortic elastic parameters, right and left ventricular systolic and diastolic function using conventional, pulsed Doppler and tissue Doppler imaging (TDI) echocardiographic parameters. RESULTS: Fifteen infants were in the LPC group, 16 in the IVA group, and 20 in the control group. Although there were some statistically significant differences in terms of pulsed and TDI echocardiographic parameters between the treatment and control groups, these values could not clearly be adopted as a diastolic dysfunction and myocardial performance indices were not influenced. The aortic elastic parameters were impaired in both LPC and IVA groups compared to the control group. Consequently, we observed only minor differences between the treatment groups, which may suggest subtle changes due to the anti-angiogenic treatment. CONCLUSIONS: Although favourable and promising outcomes were obtained with intravitreal injection of anti-vascular endothelial growth factor agents for the treatment of ROP, concerns have been raised about potential systemic side effects, including potential cardiovascular side effects caused by these agents. The small reduction in right ventricular Doppler velocities could probably be explained by the use of anti-angiogenic or laser treatment in infants.

Renal resistive index significantly increased in hypertensive children and it is independently related to the pulse pressure and left ventricular mass index.

Background: To the best our knowledge there is no data in the literature related to changes in renal resistive index (RRI) values in children who have hypertension (HT). In this study, we aimed to investigate the changes of RRI values and it is usability in children with HT. Methods: A total of 75 children; 48 with newly diagnosed HT and 27 healthy controls were included in the study. Routine history, physical examination, and laboratory examinations were performed. Left ventricular (LV) dimensions and systolic-diastolic functions, septum and posterior wall thickness (IVSd and PWd) and LV mass index (LVMI) were calculated by echocardiography. In addition to routine renal ultrasonography (US), renal pulsatility indexes (RPI), accelerated time and RRI were measured. Increased RRI was accepted as >0.70. Results: Increased RRI was detected in 25 (52%) of HT patients. Systolic and diastolic blood pressure (SBP and DBP), and pulse pressure (PP), low-density lipoprotein cholesterol and triglyceride levels, RRI, RPI, kidney length, IVSd, PWd, and LVMI were significantly higher in patients with HT (both p < 0.05). SBP and PP, PWd, LV ejection fraction, LV fractional shortening, and LVMI were positively correlated with the RRI. High-density lipoprotein cholesterol was negatively correlated with the RRI. Of these measurements, RRI values were found to be independently associated with PP and LVMI (p < 0.05). Conclusions: In hypertensive children, RRI was significantly higher than healthy controls and it is also related with LVMI and PP.

Ivabradine is an effective antiarrhythmic therapy for congenital junctional ectopic tachycardia-induced cardiomyopathy during infancy: Case studies.

Junctional ectopic tachycardia (JET) is a rare form of arrhythmia that is most commonly seen during infancy. JET is continuous and incessant, characterized by persistently high heart rates that may result in impaired cardiac function and tachycardia-induced cardiomyopathy. Despite the availability of multiple antiarrhythmic treatments, including flecainide and amiodarone, management of JET is generally very difficult. Catheter ablation has a high risk of atrioventricular block and it may require the placement of a pacemaker. Ivabradine, also known as a cardiac pacemaker cell inhibitor, is a new-generation antiarrhythmic used to treat sinus tachycardia and angina pectoris in adult patients. In this article, we present three cases of subjects with infantile congenital JET who were admitted to our clinic with a tachycardia-induced cardiomyopathy. The age of the subjects ranged from 52 days to 10 months. Although the cases of tachycardia could not be controlled by multiple antiarrhythmics, including a combination of amiodarone and flecainide combined with either propranolol or digoxin, they were rapidly converted into sinus rhythm with an ivabradine treatment of 0.1-0.2 mg/kg/day. No cardiac or other side effects were observed during ivabradine treatment, and left ventricular functions and rhythms improved within 24 hours. These three cases therefore provide hope that ivabradine may be a suitable standard initial treatment for congenital JET. However, additional research is needed to confirm the validity of these results in other circumstances.

Subclinical reduction in left ventricular function using triplane and 2D speckle tracking echocardiography after anthracycline exposure in children.

OBJECTIVE: Speckle tracking echocardiography (STE) enables global and regional evaluation of the left ventricle (LV); therefore, it is the most useful method for detecting subclinical dysfunction in patients exposed to cardiotoxic agents. A novel technique triplane (3P) echocardiography also allows single beat assessment of LV global longitudinal strain values. We firstly aimed to demonstrate both two-dimensional (2D)- and 3PSTE-derived LV global longitudinal strain measurements in children after anthracycline exposure. METHODS: This study included 23 cross-sectionally enrolled asymptomatic pediatric cancer patients who received anthracycline chemotherapy and 17 healthy controls matched by age, gender, and body surface area. All subjects underwent detailed 2D, Doppler, 2D-STE, and 3P-STE for assessment of LV function. The patients had received a median cumulative dose of 150 mg/m2. RESULTS: 1. From "Pulsed" Doppler-based measurements, only pulmonary vein flow ratio showed a significant difference between the groups. 2. When measurements were taken from the interventricular septum, the patients' ejection time values decreased significantly and their myocardial performance index values increased significantly; when the measurements were taken from the LV free wall, the peak systolic velocities showed a statistically significant difference. 3. Both 2D- and 3P-STE-derived longitudinal myocardial deformation values of LV were lower in the patient group. 4. 2D-STE-derived LV circumferential strain values were decreased in the patient group, whereas radial strain values were not significantly different compared with matched controls. CONCLUSION: Using Doppler and 2D- and 3P-STE methods, this study confirmed the subclinical LV dysfunction in patients after anthracycline exposure.