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.

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.

Left Ventricular Systolic Dysfunction Related to Adrenal Insufficiency in a Case due to Autoimmune Polyendocrine Syndrome Type 1 with a Novel Variant.

Introduction: Primary adrenal insufficiency associated with cardiomyopathy has been rarely reported in children. We report a case of left ventricular (LV) systolic dysfunction related to adrenal insufficiency with autoimmune polyendocrine syndrome type 1 (APS1). Case Presentation: A 7-year-old girl presented with a loss of consciousness. She had hyperpigmentation over joints and enamel hypoplasia. Laboratory tests showed hypoglycemia, hyponatremia, hypocalcemia, and hyperphosphatemia. Endocrine evaluations revealed low serum parathyroid hormone, low cortisol, and high ACTH. Echocardiography showed moderate to severe mitral regurgitation and LV systolic dysfunction. Serum pro-brain natriuretic peptide (pro-BNP) level was high (2,348 pg/mL). Adrenal insufficiency, hypoparathyroidism, and enamel dysplasia suggested APS1. A novel homozygous variant in the AIRE gene, NM_000383, p.Cys322Arg (c.964T>C) confirmed the diagnosis. Calcium, calcitriol, and hydrocortisone treatments were started. Serum pro-BNP level returned to normal, and LV systolic function improved. Conclusion: Here, we present a case of adrenal insufficiency and hypoparathyroidism associated with LV systolic dysfunction whose cardiac findings improved completely with hydrocortisone and calcitriol treatments. Our case is the second reported case of APS1 presenting with LV dysfunction.

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.

Radiation Dose Reduction in Congenital Heart Disease Patients During Cardiac Catheterization by a Novel Protocol.

OBJECTIVE: Cardiac catheterization remains a major source of radiation exposure for patients with congenital heart disease. This study reports the magnitude of radiation with a 3.75 frame per second (fps) pulse fluoroscopy rate and compares the reduction with the previous 15 fps protocol during cardiac catheterization for pediatric and adult congenital heart disease. MATERIAL AND METHODS: All diagnostic and interventional cardiac catheterization procedures from a single tertiary center were analyzed from January 1, 2014 to December 31, 2015, one year before and after implementing lower starting pulse fluoroscopy rates. The radiation dose was quantified as air kerma dose (mGy) and dose-area product (DAP; µGy/m2). Radiation exposure was analyzed for diagnostic and interventional procedures; the diagnostic group was subdivided into cyanotic and acyanotic patients, whereas the interventional group was subdivided according to the most common indications. RESULTS: A total of 786 procedures were analyzed. The median fluoroscopy times and contrast amounts did not show a statistically significant difference between both periods (487 vs. 456 seconds and 42.5 vs. 45.3 cm3). The median air kerma for all procedures showed an 88% reduction after implementing lower pulse fluoroscopy rates (340-41 mGy). The doses were reduced significantly for diagnostic and interventional angiograms from 470 mGy and 162 mGy to 40 mGy and154 mGy. Among all patient groups, the most striking decrease was observed in the diagnostic procedures we use, of which fluoroscopy is more prominent than cine angiography. CONCLUSION: We claim that novel radiation dose reduction protocols could be easily applied without increasing fluoroscopy time or losing image quality.

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.

Percutaneous closure of a left ventricular pseudoaneurysm after transcatheter ventricular septal defect closure.

Left ventricular pseudoaneurysm is very rare in children. Although surgery is conventional treatment, recently, percutaneous closure of pseudoaneurysms has been described. Here, we present the first case where a patient developed left ventricular pseudoaneurysm after percutaneous ventricular septal defect device closure and was treated by a second percutaneous method.

Transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart.

BACKGROUND: This study aims to evaluate the results of transcatheter occlusion of antegrade pulmonary blood flow in children with univentricular heart. METHODS: Medical data of a total of seven patients (4 females, 3 males; median age 11.7 years; range, 1 to 24 years) who underwent transcatheter occlusion of the antegrade pulmonary blood flow following Glenn shunt or Fontan operation between September 2014 and January 2017 were retrospectively analyzed. Data including demographic and clinical characteristics of the patients, type of surgery, echocardiographic and cardiac catheterization findings were recorded. RESULTS: Four patients had a previous pulmonary artery banding operation, while three had pulmonary stenosis. Two patients had facial and upper extremity edema after Glenn operation, one had prolonged pleural effusion, one had prolonged pleural effusion after Fontan operation, and one developed dyspnea and effort intolerance several years after Fontan operation. In two patients, antegrade pulmonary blood flow was occluded to decrease systemic ventricular load before surgery. The Amplatzer Septal Occluder was used in five patients and the Amplatzer Vascular Plug-2 was used in two patients. Two patients developed transient, complete atrioventricular block during the procedure and the procedure was terminated early in one of these patients. Transient hemolysis was observed in one patient following the operation. CONCLUSION: Transcatheter occlusion of antegrade pulmonary blood flow is an effective alternative to surgery in patients with hemodynamic compromise after Glenn shunt or Fontan operation.

The role of intraoperative epicardial echocardiography in pediatric cardiac surgery.

AIM: Our aim was to evaluate the findings and the role of intraoperative epicardial echocardiography (IEE) in the management of pediatric cardiac surgery patients. METHODS: Patients evaluated with IEE between December 2015 and December 2017 were analyzed retrospectively. Demographic data, preoperative transthoracic echocardiography (TTE), and IEE reports were evaluated. RESULTS: A total of 410 patients evaluated by IEE were included in the study. Of these, 52% were women, and 48% were men. The median age was 8.5 months (range: 1 month-7 years), and median body weight was 7.1 kg (range: 3.3-61 kg). The most common diagnoses were tetralogy of Fallot (TOF; n = 148), ventricular septal defect (VSD; n = 117), atrial septal defect (ASD; n = 57), and complete atrioventricular septal defect (AVSD; n = 48). There were minor residual lesions not requiring reestablishment of cardiopulmonary bypass (CPB) in 16.6% (n = 68), while major residual lesions requiring return to CPB were determined in 5.1% (n = 21). Major residual lesions were detected in 7 patients with TOF (4 severe right ventricular outflow tract obstructions, 2 pulmonary artery stenosis, 1 residual VSD shunt), 6 patients with VSD (hemodynamically significant residual shunts), and 5 patients with complete AVSD (3 left atrioventricular valve regurgitations, 1 right atrioventricular valve regurgitation, 1 left ventricular outflow tract obstruction). Transient bradycardia was observed in 5 patients. CONCLUSION: Intraoperative epicardial echocardiography provides good guidance during congenital heart surgery. IEE helps to clarify the surgical planning and decreases morbidity and mortality due to unnecessary invasive procedures, especially for pathologies involving the pulmonary artery and its branches, as well as for apical ventricular septal defects.

Isolated left-sided partial anomalous pulmonary venous connection in a child.

Isolated left-sided partial anomalous pulmonary venous connection with intact interatrial septum is a rare diagnosis in childhood. In these cases, a vertical vein drains the left upper pulmonary lobe into the brachiocephalic vein and finally to the right atrium. Surgical treatment is performed to prevent right ventricular failure and pulmonary artery disease in advanced age. In this report, the rare entity of isolated left-sided anomalous pulmonary venous connection in a 14-year-old girl and successful minimally invasive surgery without cardiopulmonary bypass are described.

Coming from behind to win--a qualitative research about psychological conditions of adolescents who have undergone open-heart surgery for single ventricle between the ages 0-5.

Early recognition of congenital cardiac pathologies and their treatment by means of palliative or corrective surgery at birth or infancy has vital importance. Successful repair of congenital cardiac defects by surgical methods has gained importance especially during the last twenty years. As the scope of the surveillance increased so did the interest in the outcomes of these treatments when the patients had reached puberty and adulthood. The purpose of our research was to study the psychological framework of the adolescents who had experienced these surgeries by listening both the children and the parents talk about their feelings and experiences. Our data was accumulated through interviews with 17 adolescents and their families, using qualitative methods. The main theme at the end of the analysis was "to be strong and resistive". We reached the conclusion that this condition was not a pathological build up but an attitude of coping, as it did not cause loss of functionality. The defensive psychological mechanisms used by these adolescents consisted of repression, compensation and reaction formation. We believe that this information is important to understand the real meaning of the manners displayed when these adolescents and their families pursue their daily lives, communicate and make relationships with their environment and especially professionals in the health services.

Worster-Drought syndrome (congenital bilateral perisylvian syndrome) with posterior pituitary ectopia, pituitary hypoplasia, empty sella and panhypopituitarism: a patient report.

Worster-Drought syndrome (WDS) (congenital bilateral perisylvian syndrome, congenital pseudobulbar paresia) is characterized by neuronal migration defect, pseudobulbar paralysis, epilepsy, neuromotor retardation and perisylvian dysplasia. We report a patient with WDS associated with posterior pituitary ectopia, pituitary hypoplasia, partial empty sella and panhypopituitarism, not previously reported in the literature. The 16.4 year-old female patient had severe growth retardation with height SDS -4.5, delayed puberty, microcephaly, pes equinovarus deformity, developmental delay, speech disorder and epilepsy. Laboratory findings, which revealed abnormal electroencephalography and bilateral perisylvian cortical dysplasia on cranial magnetic resonance imaging (MRI) were consistent with WDS. Endocrinological evaluation revealed secondary hypothyroidism and combined deficiency of adrenocorticotropin, gonadotropin and growth hormone (GH). Sella MRI showed congenital empty sella, anterior pituitary hypoplasia, ectopic neurohypophysis, and stalk agenesis. Appropriate replacement therapy was started. GH treatment resulted in a final height of 150.3 cm, appropriate for her target height. This is the first reported patient with WDS associated with congenital structural hypothalamic-pituitary abnormalities, including empty sella, pituitary hypoplasia, posterior pituitary ectopia, stalk agenesis and panhypopituitarism. GH has been successful in the treatment of her short stature.

Weismann-Netter-Stuhl syndrome in two siblings.

Cases of Weismann-Netter-Stuhl syndrome involving the upper extremities and affecting siblings have rarely been reported. We present the radiological findings of Weismann-Netter-Stuhl syndrome in two siblings, with upper extremity involvement in one of them.