Infective Endocarditis in North Africa and the Middle East, 1990‒2019: Updates from the Global Burden of Disease Study 2019.

BACKGROUND: Infective endocarditis (IE), a severe and economically impactful condition, lacks substantial epidemiological data in the North Africa and Middle East (NAME) region. This study focused on analyzing the trends and burden of IE in NAME from 1990 to 2019, taking into account factors like age, gender, and socio-demographic index (SDI). METHODS: The Global Burden of Disease data from 1990 to 2019 was retrieved from the Institute for Health Metrics and Evaluation (IHME) website. RESULTS: Between 1990 and 2019, the age-standardized rates (ASR) for IE incidence increased by 59%, and prevalence and years lived with disability (YLDs) rose by 12% and 9%, respectively, while the ASRs for deaths, disability-adjusted life years (DALYs), and years of life lost (YLLs) saw reductions of 22%, 34%, and 34% in the NAME region. Death rates among children under five declined by 72%. Gender and the SDI did not significantly influence these changes. Saudi Arabia witnessed the most significant increase in ASR of IE incidence since 1990, while Turkey had the highest rates in 2019. The year 2019 also saw the highest death rate among those aged 70 and over, with over 91000 DALYs from IE. DALYs decreased by 71.5% for children under five from 1990 to 2019 but remained stable for individuals in their seventies. Jordan showed the most notable decrease in ASRs for deaths, DALYs, and YLLs among children under five. CONCLUSION: This study highlights the changing epidemiology of IE in the NAME region, recommending the establishment of multidisciplinary IE registries, antibiotic prophylaxis guidelines for healthcare-associated IE, and strategies to control antimicrobial resistance as key mitigation measures.

Correlation between echocardiographic estimation of right atrial pressure and invasive measurement of central venous pressure in postoperative pediatric patients with congenital heart disease: a prospective observational study.

BACKGROUND: Right atrial pressure plays a critical role as a hemodynamic parameter in diagnosing pulmonary hypertension and other cardiac diseases, as well as guiding the treatment and prognosis of various cardiac disorders. If there is no obstruction between the inferior or superior vena cava (SVC) as central veins and the right atrium, the pressures in these veins could be considered equal to the right atrial pressure. This study aimed to examine the correlation between echocardiographic methods for estimating right atrial pressure and invasive measurements of central venous pressure (CVPi) in infants and children with congenital heart disease during the 48 h after cardiac surgery and to establish regression equations for echocardiographic estimation of central venous pressure (CVPe). RESULTS: We prospectively enrolled 43 infants and children, ranging in age from 6 months to 16 years, including 20 males and 23 females. We found a significant correlation between CVPi and the ratio of the maximal diameter of IVC to the maximal diameter of the descending aorta ratio (IVCmax/DAOmax) (r = 0.529, P < 0.001), SVCS/D velocity ratio (SVCS/D) (r = 0.462, P = 0.006), right atrial vertical diameter (RAVD) (r = 0.409, P = 0.01), area (r = 0.384, P = 0.014), and tricuspid valve A wave acceleration rate (TVAAR) (r = 0.315, P = 0.048). Multiple regression analysis yielded an equation for estimating central venous pressure using four parameters related to the IVC, SVC, tricuspid valve, and right atrium. The equation is as follows: estimated CVP = 4.36 + (2.35 × IVCmax/DAOmax) + (1.06 × SVCS/D) + (0.059 × RAVD) + (0.001 × TVAAR). This equation is strongly correlated with CVPi (Pearson r = 0.698, P = 0.002). CONCLUSIONS: The estimation of central venous pressure through a multi-parametric equation that included the ratio of the maximal diameter of the inferior vena cava to the maximal diameter of the descending aorta, the ratio of S to D velocity of the superior vena cava, the vertical diameter of the right atrium, and the acceleration rate of the A wave of the tricuspid valve demonstrated a robust correlation with invasively measured central venous pressure. To assess the accuracy of predicted pressures by this equation, further investigations are required to apply this innovative multi-parametric formula to a prospective population of pediatric patients with congenital heart disease.

Congenital anomalous drainage of the inferior vena cava and hepatic veins into the left atrium associated with long QT syndrome: Multimodality imaging, pitfalls, and caveats.

Isolated congenital anomalous drainage of the inferior vena cava (IVC) and partial hepatic veins (HV) into the left atrium (LA) via an interatrial communication (IAC), associated with the normal connection of the IVC to the right atrium is exceedingly rare. Therefore, there is a dearth of knowledge regarding the management of these cases. To date, there has been no report of abnormal IVC drainage caused by abnormal IVC-IAC alignment. Much more frequently, patients have an abnormal connection or abnormal drainage mediated by a persistent Eustachian valve that allows blood to pass from the inferior vena cava to the left atrium. Herein, we report an 8-year-old boy with anomalous IVC and hepatic vein drainage into the LA due to IVC-IAC malalignment. We describe the findings of multimodality imaging, including transthoracic, transesophageal, contrast, and speckle-tracking echocardiography, cardiac angiography, and cardiac magnetic resonance imaging. We go over the diagnostic and therapeutic pitfalls and caveats of this case that can apply to similar patients.

Revisiting the Management of Pediatric Kidney Transplants, A Multicenter Analysis

The newest Kidney Disease Improving Global Outcomes (KDIGO) guideline recommendations were investigated mainly for the care of adult kidney transplant recipients, but no guideline exists for the management of pediatric transplant recipients. This review provides update recommendations in the management of pediatric kidney transplantation. Four electronic databases, PubMed, EMBASE, Google Scholar, and Web of Science were searched systematically for the last two decades, using Mesh terms in English language. The Grades of Recommendation Assessment, Development, and Evaluation (GRADE) approach was used for grading the quality of the overall evidence and the strength of recommendations for each outcome across the studies. The overall quality of evidence categorized as high (A), moderate (B), low (C), or poor (D). The strength of a recommendation was determined as level 1 (recommended) or level 2 (suggested). The ungraded statements were determined on the basis of common sense to provide general advice. Of the 317 citations which were screened for the evidence review, 62 were included in data extraction. The included studies were randomized controlled trials, prospective cohorts and cross-sectional, descriptive, and review studies. Of the 115 statements, 56 (48.6%) were graded 1 (we recommend), 34 (29.5%) were graded 2 (we suggest), and 25 (21.7%) were ungraded statements. Altogether, only 22 (19.1%) of recommendations reached the "A" or "B" levels of quality of evidence. The pediatric kidney transplant recipients are different from adult recipients regarding the primary kidney diseases, surgical techniques, drug metabolism, adherence to medications, growth and neurocognitive development and immunization needs prior to transplantation.  DOI: 10.52547/ijkd.7179.

Epidemiology, burden, and attributable risks of infective endocarditis in Iran and its provinces: From 1990 to 2019.

BACKGROUND: Endocarditis is a potentially life-threatening infectious disease associated with significant morbidity and mortality and an escalating incidence in recent decades. In this study, as a part of the global burden of disease (GBD) 2019 study, we intend to report endocarditis burden in Iran at national and provincial levels from 1990 to 2019. METHOD: This study was conducted using GBD 2019 study data on endocarditis from 1990 to 2019. We gathered incidence, prevalence, disability-adjusted life years (DALYs), and mortality rates in Iran and its 31 provinces by sex and age groups as epidemiological indices for endocarditis burden. Further decomposition analysis was also performed to delineate the endocarditis new cases trend. RESULTS: On the country scale, age-standardized incidence rate (ASIR), age-standardized prevalence rate (ASPR), age-standardized mortality rate (ASMR), and age-standardized DALYs rate were (16.5 (95% uncertainty interval 13.7 to 19.8), 3.4 (2.9 to 4.1), 0.6 (0.5 to 0.9), and 14.4 (12.0 to 21.1) in 2019, respectively. Decomposition analysis showed that only 59.2% of the overall new cases increase (114.1%) was caused by the incidence rate change. All estimated age-standardized rates were higher in men in 1990 and 2019 with a ratio of 1.1-1.5. CONCLUSION: The ASIR and ASPR of endocarditis increased, and the ASMR and age-standardized DALYs rate declined over the past 30 years in Iran, nearly all the provinces followed the same pattern with North Khorasan having the Highest ASIR, ASPR, ASMR, and DALYs rates in both years. High systolic blood pressure (SBP) had the greatest attributed burden among risk factors.

COVID-19-induced silent myocarditis and newly developed hypertension in a 3-year-old boy.

BACKGROUND: COVID-19 myocarditis occurs in 7-28% of patients admitted in the hospital with or without multisystem inflammatory syndrome. It may present as fulminant myocarditis. Dilated cardiomyopathy as a sequela of COVID-19 myocarditis has been reported in the pediatric population. However, to date, no case of silent COVID-19 myocarditis progressing to dilated cardiomyopathy has been reported in children. Furthermore, although newly developed hypertension as a sequela of COVID-19 infection has been reported in adults, there is no report of newly developed COVID-induced hypertension in children. We report a 3-year-old boy with silent COVID-19 myocarditis progressing to dilated cardiomyopathy and newly developed systemic hypertension. CASE PRESENTATION: A 3-year-old boy was referred to the emergency department because of respiratory distress. The parents gave a history of SARS-CoV-2 infection in the child 5 months ago that was manifested as fever and cough, for which he was treated as an outpatient. Echocardiographic examination revealed a severe decrease in left ventricular systolic function in favor of dilated cardiomyopathy. Cardiac magnetic resonance imaging established the diagnosis of myocarditis. The patient left ventricular systolic function did not improve after 2 weeks of intravenous inotropic support. Therefore, the child was transferred to another tertiary center with extracorporeal membrane oxygenation and pediatric cardiac transplantation facilities. CONCLUSIONS: COVID-19 can induce silent myocarditis with progression to dilated cardiomyopathy and newly developed systemic hypertension. Thus, a thorough examination of the heart and measurement of blood pressure are mandatory in every child with COVID-19 infection. Cardiac MR is an indispensable tool in the diagnosis, follow-up, and prognostication of COVID-19 myocarditis. Moreover, four-chamber speckle tracking strain imaging showed apical rocking in all the four heart chambers in this child with opposite direction in the failed left ventricle compared with other cardiac chambers. Lastly, the presence of septal flash on M-mode echocardiography, apical rocking and prestretch-rebound stretch patterns on longitudinal strain imaging of the failed left ventricle in this child may be of predictive value for response to cardiac resynchronization therapy.

Color tissue doppler imaging of tricuspid annular plane systolic and diastolic excursion in children: A comparison of normal, volume-overloaded and pressure overloaded right ventricles.

BACKGROUND: Tricuspid annular plane (TAP) systolic excursion (TAPSE) is a reproducible M-mode parameter for the measurement of longitudinal shortening of the right ventricle (RV). To date, all attention has been focused on the systolic excursion of TAP and the diastolic excursion of the annular plane back to the base has been ignored. This study aims to compare the quantitative (excursion, slope, and duration) and qualitative (velocity, acceleration, and indentation) characteristics of TAP systolic and diastolic excursion, using color tissue Doppler imaging, in three groups of children with normal RV (NORV), volume overloaded RV (VORV), and pressure overloaded RV (PORV) and normal pulmonary arterial pressure. SUBJECTS AND METHODS: A prospective case-control study was performed in three groups of children with normal heart, VORV and PORV. TAPSE and tricuspid annular plane diastolic excursion (TAPDE) were quantitatively and qualitatively analyzed and compared between the three groups. Statistical analysis was performed using IBM SPSS Statistics for Windows. RESULTS: TAPSE, TAPDE, TAPSE slope, TAPSE slope/TAPDE slope, TAPDE duration and TAPDE duration/RR interval were lower in PORV (TAPSE: PORV: 14.45 ± 4.30, NORV: 20.45 ± 5.46, P = .003, TAPDE:PORV: 14.39 ± 4.61, NORV: 20.28 ± 5.65, P = .004, TAPSE slope:PORV: 4.79 ± 1.40, NORV: 7.15 ± 1.98, P = .001, .001, TAPDE duration:PORV: 201.1 ± 87.9 ms, NORV: 292.1 ± 97.9, P = .006, TAPDE duration/RR interval: PORV: .37 ± .09, NORV: .48 ± .08, P = .0002). CONCLUSION: Pressure-overload on RV produced more impairment of TAPSE and TAPDE patterns than volume overload. Values of TAPSE and TAPDE in patients with VORV and PORV stay in two ends of the normal spectrum. The harmful impact of pre-tricuspid volume overload seems to be less than the post-tricuspid volume overload.

Global, regional, and national burden and quality of care index of endocarditis: the global burden of disease study 1990-2019.

AIMS: Endocarditis accounts for significant morbidity and mortality. Timely diagnosis and prompt treatment are of paramount importance for optimal patient outcome. However, only few studies have assessed quality of care (QoC) in endocarditis. We aimed to describe QoC and changes in epidemiological features of endocarditis from 1990 to 2019. METHODS AND RESULTS: Using primary indices of mortality, incidence, years of life lost, years lived with disability, and disability-adjusted life year, obtained from the Global Burden of Disease Study 2019, we calculated four secondary measures. Principal component analysis was performed to calculate QoC index (QCI), scored on a scale of 0-100 with higher values indicating better QoC, for different locations, age groups, and genders from 1990 to 2019. The all-ages incidence rate of endocarditis was estimated to increase significantly from 1990 to 2019, while mortality rate did not change. The age-standardized QCI was 73.6% globally, with higher values in high-income countries than in low-income countries. High-income North America (82.0%) and Asia Pacific (81.1%) had the highest QCI, whereas Eastern Europe (43.3%) had the lowest. Globally, the 30-49 and 95+ age groups had the highest (91.3%) and the lowest (71.7%) QCI, respectively. In most countries, particularly those with lower socio-demographic index, women had better QCI. CONCLUSION: This is the first global assessment of QCI, shedding light on the current trends and highlighting the necessity of improving the endocarditis QoC, mainly by timely case detection, adherence to antibiotic prophylaxis guidelines, utilizing targeted antibiotics and advanced treatments, in the African region and resolving gender inequality in selected countries.

Multimodality imaging of constrictive pericarditis in H syndrome.

This is the first report of constrictive pericarditis (CP) in a 16-year-old boy with H syndrome with pericardial involvement predominantly over the right ventricle with favorable response to anti-inflammatory treatment. H syndrome, first reported in 2008, is a new auto-inflammatory syndrome with multiorgan involvement due to mutation in the SLC29A3 gene. We described the echocardiographic characteristics of asymmetric pericardial involvement and presented the cardiac computed tomography angiographic and magnetic resonance imaging findings. We reviewed the echocardiographic signs of CP, introduced tricuspid E/A respiratory alternans as a novel echocardiographic sign of right ventricular dominant CP, and explained the underlying mechanism.

DiGeorge syndrome and anomalous right aortic arch with arch-on-arch and figure-of-eight configurations: Aortic sac maldevelopment and left brachiocephalic artery abnormal remodeling.

We report a 6-month-old female infant with deletion of chromosome 22q11.2 (DiGeorge/VFS TUPLE 1), normal atrial arrangement with concordant atrioventricular connection, pulmonary atresia, large subaortic ventricular septal defect, diminutive native pulmonary arteries, a characteristic weird-shape right aortic arch with arch-on-arch appearance and figure of 8 configuration. We presented the cardiac computed tomographic angiographic and cardiac angiographic features. Using Autodesk 3ds Max 2018 software, we explained and illustrated the speculative embryologic etiology of this bizarre aortic archanomaly with the extensive abnormal remodeling of the left brachiocephalic artery, based on a "five-embryonic aortic arches" concept. As to the best of the authors' knowledge, this is the first report of a genetically confirmed case of DiGeorge syndrome and an exceedingly rare type of right aortic arch anomaly with embryologic explanation according to the "five-embryonic-aortic-arches" concept. It seems that the constellation of pulmonary atresia, bizarreshaped right aortic arch due to abnormal development of the aortic sac, and abnormal remodeling of the left brachiocephalic artery may be strongly suggestive of DiGeorge syndrome.

Exercise-induced electrocardiographic changes after treadmill exercise testing in healthy children: A comprehensive study.

Background: Treadmill exercise testing is a crucial diagnostic tool for evaluating congenital and acquired heart disease in the pediatric population. This study aimed to perform a comprehensive evaluation of exercise-induced electrocardiographic (ECG) changes in children. Although there are numerous studies on exercise testing in various cardiac pathologies, studies on exercise-induced ECG changes in normal children with coverage of all ECG parameters of atrial and ventricular depolarization and repolarization are very scant, if any. Aims and Objectives: This study aimed to investigate the exercise-induced ECG changes in healthy children and evaluate the effects of gender and four different formulas of heart rate correction of Bazett, Fridericia, Framingham and Hodges on ventricular repolarization parameters pre-and post-exercise. Materials and Methods: Between April 2019 and April 2020, all children with normal electrocardiogram, echocardiogram and exercise test, high-quality ECG tracings and consent for participation were enrolled in this prospective study. Twenty electrocardiographic parameters were measured and 25 indices were calculated. P-value < 0.05 was considered significant. Results: Seventy-four healthy children were studied. Amplitudes of P, S, and T waves increased significantly after the exercise. All durations, except P wave time to peak and T peak -T end /QT (Tp-e/QT) interval decreased significantly with exercise. Generally, the parameters of ventricular repolarization were not statistically significant between males and females. There were significant differences among the heart-rate corrected values of intervals of QTc, QoTc, JTc, J point to peak T and Tp-e/QTc by various formulas. There was no U wave either at pre-exercise or post-exercise. QT interval was shortened by 24.6 % ± 12.1 % with exercise. The ECG-derived estimated duration of mechanical systole and diastole decreased with exercise. The percentage of decrease in diastole was more than systole (43.79 %± 13.31% versus 33.74% ±15.79 %, respectively, P-value < 0.001). Conclusion: Diastolic time decreased more than systolic time with exercise and systolic time to diastolic time increased with exercise. Hodges' and Fridericia's formulas resulted in the longest and shortest QT and QoT, JT, and JTP, respectively. Thus, using a single value as the cut-off for long QT syndrome can lead to under or over-diagnosis. Nomograms incorporating data on age, heart rate, and heart rate correction formula are indispensable for accurate long QT diagnosis. Furthermore, gender differences in ventricular repolarization parameters are not generally present in 5 to 14-year-old healthy children. The lack of U wave in this study may implicate the need for more careful investigation in the presence of U wave in the treadmill exercise testing of healthy children.

Cardiovascular disease in COVID-19: a systematic review and meta-analysis of 10,898 patients and proposal of a triage risk stratification tool.

BACKGROUND: Coronavirus disease 2019 (COVID-19) pandemic has drastically affected global health. Despite several studies, there is yet a dearth of data regarding the mechanisms of cardiac injury, clinical presentation, risk factors, and treatment of COVID-19-associated cardiovascular disease. This systematic review and meta-analysis is aimed at defining the clinical, electrocardiographic, and pathologic spectrum of cardiovascular disease (CVD), frequency of elevated cardiac and inflammatory biomarkers, and their frequency and relationship with severity of the disease and mortality in COVID-19 patients and to develop a triage risk stratification tool (TRST) that can serve as a guide for the timely recognition of the high-risk patients and mechanism-targeted therapy. We conducted an online search in databases of PubMed and Embase to identify relevant studies. Data selection was in concordance with PRISMA guidelines. Results were presented as pooled frequencies, odds ratio, standardized mean difference (SMD), and forest and funnel plots. RESULTS: We gathered a total of 54 studies and included 35 of them in our meta-analysis. Acute cardiac injury occurred in more than 25% of cases, mortality was 20 times higher, and admission to intensive care unit increased by 13.5 times. Hypertension was the most common pre-existing comorbidity with a frequency of 29.2%, followed by diabetes mellitus (13.5%). The deceased group of patients had higher cardiac and inflammatory biomarkers, with statistically significant SMD, compared with survivors. Pediatric patients were predominantly mildly affected. However, less frequently, the presentation was very similar to Kawasaki disease or Kawasaki shock syndrome. This latter presentation hass been called as multisystem inflammatory syndrome in children (MIS-C). CONCLUSIONS: There is a wide spectrum of cardiac involvement in COVID-19 patients, and hence a Triage Risk Stratification Tool can serve as a guide for the timely recognition of the high-risk patients and mechanism-targeted therapy.

Atrial and ventricular ejection force of the fetal heart: Which of the four chambers is the dominant?

BACKGROUND/AIM: This study aimed to measure and compare the ejection force of the cardiac chambers in healthy singleton fetuses and to investigate the relationship of ejection force of cardiac chambers with gestational age, fetal sex, and fetal heart rate. PATIENTS AND METHODS: A prospective study was performed on 68 singleton fetuses with a gestational age of 17-34 weeks. Atrial and ventricular ejection force was measured. Measurements were repeated in 18 of the fetuses to assess intraobserver reliability. RESULTS: The right atrium had the highest ejection force of all the cardiac chambers. Ejection force of both atria and ventricles increased with gestational age. CONCLUSION: The right atrium is the dominant chamber of the fetal heart in 17-34 weeks of gestation. Comparison of our values with previous studies indicates that left atrial ejection force almost doubles in the 1st month after birth. This study highlights the crucial role of the right atrium in the fetal cardiac function during 17-34 weeks of gestation.

Pulmonary artery-focused contrast echocardiography with supplemental oxygen(PCESO) for echocardiographic diagnosis of anomalous origin of left coronary artery from pulmonary artery: Novel use of an old technique.

Pulmonary artery-focused agitated saline contrast echocardiography unveils tricky cases of ALCAPA by the entry of microbubbles into the left coronary artery (LCA) during systole and retrograde flow from LCA into the main pulmonary artery during diastole. Associated pulmonary hypertension, if present, augments the former flow and supplemental oxygen increases the latter.

Isolation of the left brachiocephalic artery revisited: A 52-year literature review and introduction of a novel anatomic-clinical-prognostic classification.

Isolation of the left brachiocephalic artery (ILBA) is an extremely rare anomaly of aortic arch with diverse manifestations in the neurologic system, heart, and left upper arm. This anomaly is defined as the absence of connection of the left brachiocephalic artery (LBA) to aortic arch and connection of LBA to pulmonary artery (PA) through a patent arterial duct (PAD). However, this definition is not inclusive of all cases. Not only are there inconsistencies in the definition and terminology of this aortic arch anomaly but also there is no classification for this anomaly despite its heterogeneous nature in terms of anatomy, clinical presentation and prognosis. We performed a 52-year comprehensive literature review in the period between 1966 and 2018. Our inclusion criteria were any manuscript that included a case report or case series, with confirmed diagnosis of ILBA. All quantitative data were analyzed using descriptive analysis by SPSS version 21 (IBM SPSS Statistics, USA). Results were presented as mean ± standard deviation and median. Based on the presence or absence of connection of LBA to PA and the number of sources of steal from the LBA, we classified ILBA into three types: single-steal type with no connection of LBA to PA and single source of blood flow steal from LBA through the left subclavian artery (LSCA), double-steal type with connection of LBA to PA through PAD and two sources of steal through LSCA and arterial duct (AD), and triple-steal type with bilateral PADs and therefore, three sources of blood flow steal from LBA including the LSCA and the double ADs. Patients with single-steal type have the best prognosis and present latest with symptoms of cerebrovascular insufficiency or left arm claudication. The oldest reported patient was 69 years of age with symptoms of dizziness and near syncope. No death was reported in these patients. Double-steal type is the most common type and is often associated with genetic syndromes and/or extracardiac anomalies. Triple-steal type is the rarest type with the earliest presentation and worst prognosis. The oldest reported patient was 60 days of age. All reported cases had cardiac symptoms, pulmonary overcirculation, pulmonary hypertension, and fatal outcome.

Circumaortic double left innominate vein: Two novel echocardiographic diagnostic clues for a tricky diagnosis.

We report a 7-year-old boy with unilateral choanal atresia who was referred for cardiac consultation before surgery. On echocardiography, the heart was normal except the presence of a double-circle appearance and a V-shaped structure on the suprasternal notch view. Cardiac computed tomography (CT) angiography confirmed the diagnosis of the double left innominate vein (DLIV). DLIV is a rare systemic venous anomaly, which often produces diagnostic confusion on echocardiography. Correct diagnosis is essential before performing any invasive procedure through this vein. Co-existence of the two echocardiographic signs of the double-circle appearance and the V sign confirms this tricky diagnosis.

Giant congenital right atrial epithelioid-capillary hemangioma with prolonged QT interval: Case report and practical surgical treatment strategy for primary cardiac tumors in children based on 25-year review of 299 cases.

Cardiac hemangioma is very rare and accounts for 2%-3% of the primary cardiac tumors. Cardiac epitheloid-capillary hemangioma has not been reported in the pediatric population so far. We report the fatal outcome of a preterm neonate with a huge congenital right atrial epitheloid-capillary hemangioma and elevated serum alpha-fetoprotein, associated with prolonged QT interval. We describe the echocardiographic, computed tomographic (CT) imaging, microscopic and immunohistochemical features of the tumor. Complete resection of the tumor was done at operation necessitating extensive reconstruction of atrial walls. Intramural infiltration of this tumor into the surrounding myocardial walls is a challenging characteristic of cardiac hemangioma. The temptation to complete resection should be avoided in the setting of extensive intramural infiltration and entrapment of the tumoral cells into atrial walls, particularly in a preterm neonate. None of the current classifications for hemangioma was inclusive of our case. Based on a concise literature review of nine published classification systems from 1996 to 2017 , we discuss the shortcomings of the current classifications for hemangioma. We also performed a 25-year-review of 299 cases of primary cardiac tumors in neonates and children, from 1993 to May 2018. We suggest a stepwise surgical treatment strategy according to the characteristics of the patient and of the tumor, based on this review. The stepwise strategy includes watchful observation, partial resection, complete resection and cardiac transplantation.

Congenital unruptured aneurysm of sinus of Valsalva, quadricuspid aortic valve, and ascending aortic aneurysm associated with genetic leukoencephalopathy in an infant: A new syndrome?

We report a 15-month-old female with unruptured noncoronary sinus of Valsalva anreurysm (SOVA) associated with quadricuspid aortic valve, ascending aortic aneurysm (AAA), moderate aortic insufficiency, mild mitral insufficiency, genetic leukoencepaholpathy, developmental delay and mild mixed aminoaciduria. She was referred for evaluation of a cardiac murmur. Initial echocardiographic examination showed the aneurysm as a round cyst in the right atrium which changed in size during systole and diastole. As to the best of our knowledge, this is the first report of association of SOVA, quadricuspid aortic valve, AAA and genetic leukoencephalopathy in an infant. These associations may suggest a new syndrome. We describe the echocardiographic and computed tomographic angiography findings. We also review 641 cases in the literature and the current six classification systems for SOVA.

Pulsatile blood flow in total cavopulmonary connection: a comparison between Y-shaped and T-shaped geometry.

Single-ventricle anomaly is a hereditary heart disease that is characterized by anatomical malformations. The main consequence of this malformation is desaturated blood flow, which without proper treatment increases the risk of death. The classical treatment is based on a three-stage palliative procedure which should begin from the first few days of patient's life. The final stage is known as Fontan procedure, in which inferior vena cava is directly connected to pulmonary arteries without going through the ventricle. This connection is called total cavopulmonary connection (TCPC). After surgery, the single ventricle supplies adequate and saturated systemic blood flow to the body; however, TCPC contains low pressure and low flow pulsatility. To overcome this problem, a new method is proposed wherein pulsatile blood will be directed to the TCPC through the stenosed main pulmonary artery. In this study, through the use of Computational Fluid Dynamics, T-shaped (MRI-based) and Y-shaped (computer-generated) geometries are compared in order to determine the influence of this modification on pulsation of blood flow as well as energy loss in pulmonary arteries. The results indicate that energy loss in Y-shaped geometry is far less than T-shaped geometry, while the difference in flow pulsatility is insignificant.

Left Ventricular Function after Arterial Switch Operation as Assessed by Two-Dimensional Speckle-Tracking Echocardiography in Patients with Simple Transposition of the Great Arteries.

Background: The late postoperative course for children with transposition of the great arteries (TGA) with an intact ventricular septum (IVS) is very important because the coronary arteries may be at risk of damage during arterial switch operation (ASO). We sought to investigate left ventricular function in patients with TGA/IVS by echocardiography. Methods: From March 2011 to December 2012, totally 20 infants (12 males and 8 females) with TGA/IVS were evaluated via 2-dimensional speckle-tracking echocardiography (2D STE) more than 6 months after they underwent ASO. A control group of age-matched infants and children was also studied. Left ventricular longitudinal strain (S), strain rate (SR), time to peak systolic longitudinal strain (TPS), and time to peak systolic longitudinal strain rate (TPSR) were measured and compared between the 2 groups. Results: Mean ± SD of age at the time of study in the patients with TGA/IVS was 15 ± 5 months, and also age at the time of ASO was 12 ± 3 days. Weight was 3.13 ± 0.07 kg at birth and 8.83 ± 1.57 kg at the time of ASO. Global strain (S), Time to peak strain rate (TPSR), and Time to peak strain (TPS) were not significantly different between the 2 groups, whereas global strain rate (SR) was significantly different (p value < 0.001). In the 3-chamber view, the values of S in the lateral, septal, inferior, and anteroseptal walls were significantly different between the 2 groups (p value < 0.001), and SR in the posterior wall was significantly different between the 2 groups (p value < 0.001). There were no positive correlations between S and SR in terms of the variables of heart rate, total cardiopulmonary bypass time, and aortic cross-clamp time. There were no statistically significant differences between the 2 groups regarding S, SR, TPS, and TPSR in the anteroseptal and posterior walls in the 3-chamber view and in the lateral and septal walls in the 4-chamber view. Conclusion: We showed that between 6 and 18 months after a successful ASO, the parameters of S, SR, and global TPS were normal in our patients with TGA/IVS. However, LV myocardial TPSR did not normalize in this time period.