Successful ductal stent implantation for initial palliation in two patients with aortic atresia and interrupted aortic arch.

The coexistence of aortic valve atresia and interrupted aortic arch are an extremely rare condition. In this pathology, blood flow to the ascending aorta and coronary arteries should be provided through the ductus arteriosus or collaterals originating from the descending aorta. In rare cases where bilateral ductus arteriosus is present, they can provide circulation. Here, we report two cases in which coronary arteries and ascending aorta were supplied by one ductus arteriosus and distal systemic circulation is supplied by a second ductus arteriosus in one patient and a collateral artery in the other. Initial palliation was successfully performed by bilateral pulmonary artery banding and transcatheter ductal stent implantation in both cases.

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.

High-density Mapping Catheter (Advisor™ HD Grid) Usage for Intra-atrial Reentrant Tachycardia Ablation in Children and Young Adult Patients with Congenital Heart Disease.

BACKGROUND: We aimed to share our experience of intra-atrial reentrant tachycardia mapping and ablation with a new grid-style multielectrode high-density mapping catheter (Advisor™ HD Grid) in pediatric and young adult patients with operated congenital heart disease. METHODS: All patients with operated congenital heart disease and intra-atrial reentrant tachycardia mapping with the new grid-style catheter between October 2019 and December 2022 were included (group 1), and the results were compared to those patients who operated with conventional catheter methods before this period (group 2). All procedures were performed using the EnSite Precision 3D mapping system (Abbott Laboratories, Abbott Park, Ill, USA) with a limited fluoroscopy approach. Data were evaluated retrospectively. RESULTS: In group 1 (n = 16; 9 male), the median age was 21 years (10-36), compared to 19 years (9-27) in group 2 (n = 10; 5 male). While irrigated radiofrequency ablation was pre-ferred in all patients, the median number of 15 lesions (8-38) in group 1 was significantly less than the median of 30 lesions (8-71) in group 2 (P =.027). The median procedure duration of 159 minutes (110-233) in group 1 was significantly shorter compared to 280 minutes (180-370) in group 2 (P <.05). Acute procedural success was achieved in all patients (16/16; 100%) in group 1 compared to 8/10 patients (80%) in group 2. During the median follow-up of 27 months (11-36), there was only 1 intra-atrial reentrant tachycardia recurrence in group 1 (1/16; 6.2%) and 2 recurrences (2/8; 25%) in group 2 during the median follow-up of 110 months (56-151). No complications related to the mapping catheter itself occurred. CONCLUSION: In the intra-atrial reentrant tachycardia ablation of children with congenital heart disease to increase procedural success and shorten the mapping duration, the utility of Advisor™ HD Grid mapping catheter seems to be a feasible alternative.

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.

Biventricular repair in an infant with transposition of the great arteries and straddling of the tricuspid valve.

Biventricular repair is challenging in patients with transposition of the great arteries and straddling of the atrioventricular valves. Biventricular repair is the preferred option because of its anatomical and physiological advantages. However, in cases where biventricular repair carries operative risks that are too high or cases with unsuitable intracardiac anatomy, univentricular heart repair may have to be chosen. We report a five-month-old male patient with transposition of the great arteries, an inlet ventricular septal defect and anomalous coronary anatomy who had previously undergone a pulmonary banding operation and balloon atrial septostomy. Successful biventricular repair was performed while the patient had straddling of the tricuspid valve.

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.

Evaluation of the left ventricular systolic function and myocardial deformation by real-time three-dimensional (four-dimensional) and speckle-tracking echocardiography in children with type 1 diabetes mellitus.

AIM: This study aimed to evaluate the role of real-time three-dimensional (known as four-dimensional) echocardiography and three-dimensional speckle-tracking echocardiography for the early detection of left ventricular systolic dysfunction in asymptomatic children with type 1 diabetes mellitus. MATERIALS AND METHOD: This cross-sectional study included 38 patients (mean age 15.4 ± 2.9, 42.1% male) and 38 age, gender, and body measurements matched healthy children. Each patient underwent an interview about medical history, a detailed clinical examination, blood laboratory tests, conventional echocardiography, and tissue Doppler imaging. Left ventricular ejection fraction; global longitudinal, circumferential, radial strain; twist; and torsion were measured by real-time three-dimensional and speckle-tracking echocardiography. RESULTS: Conventional echocardiography and tissue Doppler imaging showed normal left ventricular systolic function in the patients. Although left ventricular ejection fraction (61.6 ± 1.4%, 61.8 ± 1.1%, p = 0.386), global longitudinal [-26.6 (-27.7 to -26.1)%, -26.2 (-27.7 to -24.9)%, p = 0.224], and radial strain [44.4 (42.4-45.9)%, 43.9 (41-46.1)%, p = 0.513] were similar to the controls, circumferential strain (-27.3 ± 1.3%, -28.0 ± 1.6%, p = 0.048) and twist (9.5 ± 2.3°, 11.4 ± 3.0°, p = 0.003) were decreased in the patients compared with controls. CONCLUSION: We found that even in children with asymptomatic type 1 diabetes mellitus with normal left ventricular ejection fraction, circumferential function was impaired and rotation pattern was changed. This novel echocardiographic method might be an important tool for detecting left ventricular systolic dysfunction in type 1 diabetes mellitus children before it becomes overt on conventional echocardiography and tissue Doppler imaging.

Evaluation of ventricular function and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography.

AIM: The left and right ventricular dysfunction are important clinical course indicators in patients with repaired tetralogy of Fallot. This study aimed to evaluate ventricular volumes, functions, and myocardial deformation in children with repaired tetralogy of Fallot by real-time three-dimensional (four-dimensional) echocardiography and compared with healthy children. It also aimed to investigate the relationships between ventricular volumes, functions, and myocardial deformation parameters in the patients. MATERIALS AND METHODS: In this cross-sectional study, 35 patients (mean age 15.1 ± 2.8 years, 54% male) and 35 healthy controls of similar age, gender, and body measurements underwent echocardiography. End-diastolic volume index, end-systolic volume index, and ejection fractions of both ventricles; global longitudinal, circumferential, radial strain, twist, and torsion of the left ventricle; the longitudinal strain of the right ventricle free wall and septum were measured. RESULTS: Left ventricular ejection fraction, global circumferential and radial strain, twist and torsion were significantly lower in patients compared with controls. Left ventricular ejection fraction correlated with global circumferential (r = -0.446, p < 0.001) and radial strain (r = -0.433, p < 0.001) in the patients. Right ventricular volumes were significantly higher, and ejection fraction was significantly lower in patients compared with controls. All right ventricular parameters correlated with each other in the patients. CONCLUSION: Left ventricular contraction pattern was changed, circumferential and radial fibres were most affected in the patients. Right ventricular dilatation and dysfunction were detected, and right ventricular ejection fraction correlated well with strain measurements of the right ventricle.

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.

Implantation of the Edwards SAPIEN XT and SAPIEN 3 valves for pulmonary position in enlarged native right ventricular outflow tract.

OBJECTIVE: Percutaneous pulmonary valve implantation (PPVI) into right ventricle-to-pulmonary artery conduits is increasingly being performed, but a few options are available for patients with a dilated native right ventricular outflow tract (RVOT), among which is the off-label use of Ed-wards SAPIEN® valves. This study reviews the results of the SAPIEN XT and SAPIEN 3 (S3) valve implantations in the pulmonary position in patients with a dilated native RVOT. METHODS: Between January 2015 and March 2020, PPVI procedures were performed on 129 patients. Among them, 103 (80%) had dilated native RVOT, 86 of whom were eligible for PPVI prestenting and valve implantation. Retrospective analysis was performed on 84 patients who have undergone successful PPVI implantation using the SAPIEN XT or S3 valves with dilated native RVOT. RESULTS: The procedural success rate was 84/86 (98%). The median age was 18.7 years (8-46 years), and the median weight was 57 kg (22-102 kg). The primary underlying diagnosis was tetralogy of Fallot (n=77/84). Stenting was performed simultaneously with valve implantation in 50/84 (60%) cases-six of which were hybrid procedures-whereas prestenting was performed 3 to 14 weeks earlier in 34/84 cases. Before valve im-plantation, the median right anterior oblique and lateral diameters of the stents were 26 mm (20-32 mm) and 28 mm (21-32 mm). Valve sizes were 26 mm (n=13) and 29 mm (n=64) for XT and 29 mm (n=7) for S3. In 59 patients, an additional 1-5 ml (median 2 ml) volume was added to the valves' balloons for stabilization. In all hybrid procedures, the stent and valve were implanted in the same session. During follow-ups of 1 to 59 months (median 14 months), no deaths were reported, 3 patients developed tricuspid regurgitation secondary to the procedure, and valves continued to function in all patients. CONCLUSION: The Edwards SAPIEN XT and S3 valves may be an alternative to PPVI in patients with dilated native RVOT.

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.

Parvovirus Infection in a Child Presenting with Erythema Multiforme and Vasculitis after a Yellow Jacket Bee Sting.

Parvovirus B19 has a wide spectrum of clinical manifestations. Erythema multiforme and vasculitis are rarely reported with parvovirus B19 infections. Reactions to insect stings can range from local swelling to life-threatening systemic reactions. There have been rare reports of unusual reactions, such as vasculitis, occurring in a temporal relationship with insect stings. We report an 8-year-old patient having Parvovirus B-19-related erythema multiforme and vasculitis after a yellow jacket bee sting.

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.

Is the burden of late hypertension and cardiovascular target organ damage in children and adolescents with coarctation of the aorta after early successful repair different to healthy controls?

OBJECTIVE: Cardiovascular morbidity is high in patients with coarctation of aorta even after successful repair. This study aimed to assess the frequency of late hypertension and the relationship between ambulatory hypertension and cardiovascular target organ damage in children and adolescents after early and successful repair of coarctation of the aorta. METHODS: Twenty-five children and adolescents (mean age 13.5 ± 3.43 years) with repaired coarctation of the aorta (median age at repair 4 months, arm-leg gradient <20 mmHg) and 16 healthy controls were included. Office and ambulatory blood pressure, pulse wave velocity, and left ventricular mass index were assessed. RESULTS: Both day- and night-time systolic blood pressure standard deviation score and left ventricular mass index were significantly higher in patients compared to controls (p ≤ 0.001 for all), whereas pulse wave velocity did not differ. The prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy were 40, 28, and 24%, respectively. Left ventricular mass index was higher in patients with sustained hypertension, masked hypertension, and normotension compared to controls (p < 0.05). In multivariate analysis, higher night-time systolic blood pressure standard deviation score was the only independent predictor of left ventricular mass index. CONCLUSION: The present study reveals a high prevalence of masked hypertension, isolated nocturnal hypertension, and left ventricular hypertrophy in children and adolescents with coarctation of the aorta even after early and successful repair. Ambulatory blood pressure monitoring should be considered to diagnose hypertension. All coarctation of aorta patients should be followed up lifelong and encouraged to establish a healthy lifestyle starting from childhood.

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.

Right-heart catheterization using antecubital venous access in patients with complex congenital heart defects and Glenn anastomosis.

OBJECTIVE: Right-heart catheterization using the antecubital veins has recently regained attention, and studies demonstrating the feasibility and safety of antecubital access in adults have been published. However, no changes have been observed in the preferred entrance sites in right-heart catheterizations in children with congenital heart diseases. This article is a description of the technique and features of the antecubital venous approach in pediatric patients with complex congenital heart defects and a Glenn anastomosis. METHODS: The data regarding a right cardiac catheterization through the antecubital fossa veins performed in 18 patients with various clinical indications between January 2014 and August 2017 were reviewed retrospectively and the results were assessed. RESULTS: Ten patients (55%) were male and 8 patients were female. All of the patients but 1 had a complex congenital heart disease with a Glenn anastomosis. One patient had been operated on for a sinus venosus atrial septal defect and an abnormal pulmonary venous return and had a total occlusion of the superior vena cava. A diagnostic catheterization was performed in all cases. Additional procedures consisted of a balloon test occlusion of the pulmonary valve in 2 patients, a superior vena cava-right pulmonary artery anastomosis dilatation in 1, and abnormal veno-venous collateral occlusion with various devices in 2 patients. CONCLUSION: The antecubital venous approach technique can be performed easily and safely for diagnostic and therapeutic catheterization in patients with complex congenital heart defects. The authors advocate that the antecubital venous approach should be the first site selected for right-heart catheterization, especially in patients with a Glenn anastomosis.

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.