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.

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.

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.

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.

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.

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.

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.

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.

Prenatal diagnosis of left coronary artery to right ventricle fistula.

Prenatal diagnosis of a congenital coronary artery fistula between the left coronary artery and the right ventricle was established at 28 weeks of gestation. Hydrops fetalis developed during follow-up and the baby died on the first day after delivery. It is rare for coronary artery fistulas to become symptomatic during fetal life.

Subclinical cardiovascular disease and its association with risk factors in children with steroid-resistant nephrotic syndrome.

BACKGROUND: The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors in pediatric patients with steroid-resistant nephrotic syndrome (NS). METHODS: Thirty-seven patients with normal renal function were compared with 22 healthy controls regarding the presence of subclinical CVD. Measurements included aortic pulse wave velocity (PWV), carotid intima media thickness (IMT), and left ventricular mass (LVM). Patients were additionally assessed for blood pressure (BP) pattern and the presence of hypertension by 24-h ambulatory blood pressure monitoring. RESULTS: Compared with the controls, patients had significantly higher mean aortic PWV-standard deviation scores (SDS), mean carotid IMT-SDS, and LVM index (p < 0.001 for all). Increased aortic PWV was noted in 5 % of patients, increased carotid IMT in 22 %, and increased LVM index in 19 %. Five patients (14 %) were hypertensive, and mean BP indexes, SDS, and BP loads during nighttime were significantly higher than those during daytime (p < 0.001 for all). Multivariate analysis revealed a significant relationship between PWV-SDS and ferritin (R(2) = 0.269, p = 0.006) and between carotid IMT-SDS and proteinuria (R(2) = 0.141, p = 0.022). The LVM index was independently associated only with higher body mass index SDS (R(2) = 0.317, p < 0.001). In addition, six patients (16 %) had multiple abnormal subclinical CVD markers, and increased subclinical CVD risk was independently associated only with higher low-density lipoprotein cholesterol (R(2) = 0.292, p = 0.044). CONCLUSIONS: Based on these results, steroid-resistant NS children generally are at high risk of cardiovascular complications, but the increased risk is likely to be multifactorial.

Relationships between left heart chamber dilatation on echocardiography and left-to-right ventricle shunting quantified by cardiac catheterization in children with ventricular septal defects.

Left atrium and/or left ventricle dilatation on echocardiography is considered to be an indication for closure of ventricular septal defects (VSD). No study has addressed the accuracy of using dilated left heart chambers when defining significant left-to-right shunting quantified by cardiac catheterization in isolated small or moderate VSDs. In this study, the relation between dilated left heart chambers, measured by echocardiography, and left-to-right ventricle shunting, quantified by cardiac catheterization, was evaluated in patients with isolated VSD. The medical records of all patients with isolated VSD who had undergone catheterization from 1996 to 2010 were examined retrospectively. Normative data for left heart chambers adjusted for body weight (BW) and body surface area (BSA) were used. The pulmonary-to-systemic flow ratio (Qp:Qs) was calculated by an oximetry technique. A total of 115 patients (mean age 7.3 ± 5 years) fulfilled the inclusion criteria. There was a statistically significant difference in terms of Qp:Qs between the patient groups with normal and dilated left heart chambers, when adjusted for BW and BSA (p = 0.001 and p = 0.002, respectively). But the relationships between Qp:Qs and left heart chamber sizes on echocardiography were not strong enough to be useful for making surgical decisions, as left heart chamber dilatation was not significantly associated with Qp:Qs ≥ 2 (p = 0.349 when adjusted for BW, p = 0.107 when adjusted for BSA). Left heart chamber dilatation was significantly associated with Qp:Qs ≥ 1.5 only when it was adjusted for BSA (for BW p = 0.022, for BSA p = 0.006). As a result, left heart chamber dilatation measured by echocardiography does not show significant left-to-right ventricle shunting, as quantified by catheterization. We still advocate that catheter angiography should be undertaken when left heart chambers are dilated in echocardiography in order to make decisions about closing small- to moderate-sized VSD.

Clinically asymptomatic myocardial bridging in a child with familial subaortic stenosis.

Myocardial bridging is usually seen in the setting of hypertrophic cardiomyopathy or left ventricular hypertrophy. It is rarely reported in an asymptomatic patient with an otherwise structurally normal heart. Familial subaortic stenosis is also a rare entity, and its mode of inheritance is still unknown. Here, we described the case of a 13-year-old asymptomatic girl with a positive family history of sudden cardiac death and subaortic stenosis who was diagnosed with severe myocardial bridging concomitant with familial subaortic stenosis.

Evaluation of right ventricular function in patients with tetralogy of Fallot using the myocardial performance index and isovolumic acceleration: a comparison with cardiac magnetic resonance imaging.

BACKGROUND: Assessment of right ventricular function is a key point in the follow-up of operated patients with tetralogy of Fallot. Cardiac magnetic resonance assessment of right ventricular function is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. Myocardial performance index and isovolumic acceleration have recently been studied for the assessment of right ventricular function and are shown to be simple yet powerful tools for assessing patients with right ventricular dysfunction of various origins. METHODS: In this study, the integrity of myocardial performance index and isovolumic acceleration obtained by tissue Doppler imaging echocardiography to quantify right ventricular function was assessed in 31 patients operated for tetralogy of Fallot. Myocardial performance index and isovolumic acceleration measurements were compared with the parameters derived by cardiac magnetic resonance imaging. RESULTS: In this study, a significant correlation has not been detected between cardiac magnetic resonance-originated right ventricular ejection fraction, pulmonary regurgitation fraction and myocardial performance index, isovolumic acceleration obtained by tissue Doppler imaging echocardiography from the lateral tricuspid annulus of the right ventricle. CONCLUSION: We have concluded that when evaluated separately, myocardial performance index and isovolumic acceleration obtained from tissue Doppler imaging echocardiography can be used in the long-term follow-up of patients who have been operated for tetralogy of Fallot, but that they do not show correlation with cardiac magnetic resonance-originated right ventricle ejection fraction and pulmonary regurgitation fraction.

Ambulatory blood pressure and subclinical cardiovascular disease in patients with juvenile-onset systemic lupus erythematosus.

BACKGROUND: The aim of this study was to evaluate the presence of subclinical cardiovascular disease (CVD) and its relation to risk factors, particularly hypertension in juvenile-onset systemic lupus erythematosus (SLE). METHODS: A total of 24 patients with normal renal function were examined for subclinical CVD by using non-invasive methods, including the measurement of carotid intima-media thickness (IMT), carotid distensibility, aortic pulse wave velocity (PWV) and left ventricular mass (LVM). Blood pressure (BP) pattern and the presence of hypertension were assessed by 24-h ambulatory blood pressure monitoring (ABPM). RESULTS: The patients had higher aortic PWV than the controls (p = 0.011). Increased carotid IMT was present in 48 % of the patients and reduced carotid distensibility in 17 %. Left ventricular hypertrophy (LVH) was present in 22 % of the patients. Eight patients were hypertensive; hypertensive patients had a significantly lower distensibility coefficient (DC)-SDS, higher aortic PWV and higher LVM index than the normotensive patients (p = 0.008, p = 0.023 and p = 0.001, respectively). Higher carotid IMT-standard deviation score (SDS) significantly correlated with higher nighttime diastolic BP-SDS (R (2) = 0.204, p = 0.046); lower DC-SDS correlated with higher nighttime systolic BP-SDS (R (2) = 0.290, p = 0.014). Increased aortic PWV was significantly associated with higher daytime systolic BP-SDS and elevated erythrocyte sedimentation rate (R (2) = 0.607, p = 0.003 and p = 0.010, respectively). PWV was the only independent predictor of LVM index (R (2) = 0.396, p = 0.004). CONCLUSION: These results provide additional evidence for the presence of subclinical CVD and its relation to hypertension in juvenile-onset SLE. They also indicate a significant relation between LVH and increased arterial stiffness. It is also important to note that our findings reveal significant relationships between ambulatory BP and cardiovascular changes and underline the importance of ABPM to predict CVD.

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.

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.