Showing papers in "Annals of Pediatric Cardiology in 2019"

Indian guidelines for indications and timing of intervention for common congenital heart diseases: Revised and updated consensus statement of the Working group on management of congenital heart diseases

Abstract: A number of guidelines are available for the management of congenital heart diseases (CHD) from infancy to adult life. However, these guidelines are for patients living in high-income countries. Separate guidelines, applicable to Indian children, are required when recommending an intervention for CHD, as often these patients present late in the course of the disease and may have coexisting morbidities and malnutrition. Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on August 10 and 11, 2018, at the All India Institute of Medical Sciences. The meeting was supported by Children's HeartLink, a nongovernmental organization based in Minnesota, USA. The aim of the study was to frame evidence-based guidelines for (i) indications and optimal timing of intervention in common CHD; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for CHD; and (iii) indications for use of pacemakers in children. Evidence-based recommendations are provided for indications and timing of intervention in common CHD, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductus arteriosus, and others), obstructive lesions (pulmonary stenosis, aortic stenosis, and coarctation of aorta), and cyanotic CHD (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein's anomaly, and others). In addition, protocols for follow-up of postsurgical patients are also described, disease wise. Guidelines are also given on indications for implantation of permanent pacemakers in children.

Outcome of pediatric cardiac surgery and predictors of major complication in a developing country.

Abstract: Background: Evaluating outcome and identifying predictors of major complications among children undergoing cardiac surgery are essential to improve care. We evaluated short-term outcomes of postcardiac surgery and predictors of major complications in a national referral hospital in Indonesia. Methods: A prospective cohort study was conducted from April 2014 to March 2015 on all children undergoing cardiac surgery. Participants were followed up from the time of surgery until hospital discharge and 30-day mortality. We performed univariate and multivariate logistic regression using STATA 12-1 to identify predictors of postsurgical major complications. Results: A total of 257 patients (median age: 36 months) were recruited; 217 (84.1%) had complications, including low cardiac output syndrome (19.8%), arrhythmia (18.6%), sepsis (17.4%), and pleural effusion (14.8%). Forty-nine (19%) patients had major complications, including cardiac arrest (5%), need for emergency chest opening (3.9%), and multiple organ failure (7.4%). 12.8% died during hospital stay, and 30-day mortality was 13.6%. Predictors of major complications were cyanotic congenital heart disease (odds ratio [OR]: 4.6, 95% confidence interval [CI]: 1.5–14.2), longer duration of cardiopulmonary bypass (CPB, OR: 4.4, 95% CI: 1.5–13.4), high inotropes (OR: 13.1, 95% CI: 3.2–54.2), and increase in lactate >0.75 mmol/L/h or more in the first 24 h (OR: 37.1, 95% CI: 10.1–136.3). Conclusion: One-fifth of children undergoing cardiac surgery experienced major complications with around 13% mortality. Cyanotic congenital heart disease, longer duration of CPB, high inotropes on leaving operating theater, and increase in blood lactate are associated with major complications in children after cardiac surgery.

Prevalence of hypertension and prehypertension in schoolchildren from Central India.

Abstract: Background: Epidemiological transition with increasing burden of cardiovascular risk factors is evident not only in adults but also in children. The data on the prevalence of prehypertension and hypertension in children show large regional differences in India and such data are not available from Central India. We, therefore, conducted a large cross-sectional study in Indore to determine the distribution of blood pressure (BP) and the prevalence of hypertension and prehypertension among schoolchildren. Methods: A total of 11,312 children (5305 girls, 6007 boys) aged 5–15 years, drawn from 80 government and private schools in equal proportion, were evaluated. Anthropometric measurements were obtained and BPs were measured using The Fourth Report on The Diagnosis, Evaluation, and Treatment of High Blood Pressure in Children and Adolescents as reference standard. BP ≥90th to Results: Prehypertension was detected in 6.9% and 6.5% and hypertension was found in 6.8% and 7.0% of boys and girls, respectively. Height and weight were found to be a significant predictor of systolic and diastolic BP among both boys and girls. Conclusions: Our results show a high prevalence of prehypertension and hypertension in Indore schoolchildren with age and height being significant determinants. This highlights the need for routine BP measurements in children by pediatricians when they treat them for intercurrent illnesses or vaccinate them. It should also be mandatory as a part of school health checkup programs to detect childhood hypertension for further counseling and therapy.

Symptomatic improvement using everolimus in infants with cardiac rhabdomyoma.

Abstract: Background: Cardiac rhabdomyoma (CR) often shows spontaneous regression and needs close follow-up. These tumors may be associated with tuberous sclerosis complex (TSC), caused by the disinhibition of m-TOR protein. Objective: The aim of the study is to observe the efficacy of everolimus in infants with significant CR. Materials and Methods: This is a single-center prospective observational study including infants with significant CR causing either clinical symptoms or obstruction to the blood flow. Everolimus was administered at a dose of 4.5 mg/M2/wk till the symptomatic improvement. Liver and renal function tests were monitored during treatment. Results: There were six cases of suspected CR included in the study. Median age and weight of patients were 5 days (range: 1–90 days) and 3.2 kg (range: 2.2–4.5 kg), respectively. One patient was excluded after surgical excision biopsy during concomitant closure of associated large perimembranous ventricular septal defect confirmed it as a fibroma. The remaining all five cases showed regression of tumor during mean follow-up of 6.1 ± 5.1 months. One child developed varicella infection necessitating temporary discontinuation of medicine during follow-up. One case had sudden death at 4 months of age. Conclusion: Everolimus appears to be useful in selected cases of symptomatic CR. Multicentric studies are needed to determine its safety and efficacy in larger population.

Improvement of cardiopulmonary function after minimally invasive surgical repair of pectus excavatum (Nuss procedure) in children.

Abstract: Background: Severe pectus excavatum in children may result in cardiorespiratory functional impairment; therefore, we evaluated cardiopulmonary response to exercise before and after the Nuss procedure. Methods: Twenty-four physically active pediatric patients aged 9–18 years with severe pectus excavatum (Haller index >3.25) were included in the study. Cardiopulmonary exercise testing using treadmill and modified Bruce protocol was performed before and after the Nuss procedure. Results: Maximal oxygen uptake and oxygen pulse improved by 40.6% (32 ± 13–45 ± 10 ml/kg/min; P = 0.0001) and 44.4% (9 ± 4–13 ± 5 ml/beat; P = 0.03), respectively, after surgical correction of pectus excavatum by Nuss procedure. Significant improvement in maximum voluntary ventilation and minute ventilation after Nuss procedure was also noted. Conclusions: We found that, after repair of pectus excavatum by Nuss procedure, the exercise capacity as measured by maximal oxygen consumption improved significantly primarily due to increase in oxygen pulse, an indirect measurement of stroke volume.

A new low-cost method of virtual cardiac dissection of computed tomographic datasets

Abstract: Computed tomography has an established role in the evaluation of a variety of cardiac disorders, including congenital heart diseases. The current generation of high-speed scanners produces volumetric data at low doses of radiation. The interpretation of cardiac anatomy, however, is generally limited to multiplanar assessment of two-dimensional images. The volume rendering technique provides an excellent three-dimensional demonstration of external morphology but offers little information about the intracardiac anatomy. The alternative approach of virtual cardiac dissection, which is a modification of volume rendering, on the other hand, provides crucial insights regarding the intracardiac anatomy. At present, virtual cardiac dissection requires expensive software packages. These software packages are not available in all countries, thus limiting its use in routine clinical care. We present here the details of a newly developed technique that permits virtual cardiac dissection using a personal computer and open-source software. Our technique involves no additional cost and can be achieved in the comfort of the office or operating room of the cardiologist, radiologist, or cardiac surgeon. This enhanced three-dimensional visualization of intracardiac anatomy will surely improve the understanding of the morphological details of both normal and malformed hearts. In addition, by permitting assessment in projections with which modern-day cardiologists and cardiac surgeons are conversant, it is likely to improve clinical decision-making. We illustrate here its potential utility in the morphologic assessment of the atrial septum and its deficiencies, along with malformations of the ventricular outflow tracts, including common arterial trunk.

Artificial intelligence in pediatric cardiology and cardiac surgery: Irrational hype or paradigm shift?

Abstract: You are in the serene cardiac intensive care pod where real‐time analytics are displayed (rather than the de rigueur vital signs) and deep learning (in the form of recurrent neural network) are now routinely used for personalized intensive care unit decision support and to mitigate the stress of families and physicians/ nurses. There is no longer formal lengthy AM rounds as communication among team members is now continuous. The old electronic record and computers are all no longer omnipresent, and patient information is displayed on the video wall only upon activation or automatically in the case of sudden changes. The conversations are recorded with a tiny microphone on your shirt and analyzed with natural language processing (NLP) in order to capture key information onto the electronic record. You receive notifications about recent advances in diagnostics and therapy that pertain to your patients, and you read these new notifications and answer questions to gain points for individualized continuing medical education (as board examinations have been eliminated finally).

Acetaminophen in low doses for closure of the ductus arteriosus of the premature.

Abstract: Objective: The objective of the study is to report the experience with acetaminophen in low doses as an alternative to the treatment of the ductus arteriosus of the preterm newborn. Materials and Methods: Retrospective study including preterm newborns with patent ductus arteriosus who received oral acetaminophen because treatment with indomethacin had failed or is contraindicated. A dosage consisted of a first dose of 25 mg/kg and maintenance doses of 30 mg/kg/day, for 3 to 7 days. A second cycle was administered in cases of reopening of the ductus arteriosus. The rates of ductal closure and surgery were calculated. Patients were categorized into responder and nonresponder groups for acetaminophen, and the average values of ductal diameter, weight, gestational age, and postnatal age were compared. Results: Eighty-seven preterm newborns, with a postnatal age from 3 to 27 days, with average values of ductus arteriosus equal to 2.5 ± 0.8 mm/kg, gestational age 27.2 ± 1.9 weeks, and birth weight 888.9 ± 241 g, received acetaminophen for 3 to 7 days. A second cycle was administered in 15 preterm newborns. The ductus closure rate, after one or two cycles, was 74.7%, and the recommendations for surgical closure were progressively reduced from 50% in the 1 st year to 6.2% in the past year. Lower ductal closure rate occurred in the group of newborns with the lowest average weight (P = 0.018), the highest average ductal diameter (P = 0.002), and the lowest average gestational age (P = 0.09). Postnatal age at the start of acetaminophen use was shown to be irrelevant regarding the treatment (P = 0.591). Conclusions: Acetaminophen in low doses showed to be an effective alternative for the closure of the ductus arteriosus for preterm newborns in whom treatment with indomethacin or ibuprofen failed or was contraindicated.

Comparison of antistreptolysin O and anti-deoxyribonucleic B titers in healthy children to those with acute pharyngitis, acute rheumatic fever, and rheumatic heart disease aged 5–15 years

Abstract: Background: Acute rheumatic fever (ARF) affects millions of children in the third world countries like India. The diagnosis of rheumatic fever is based on the Jones criteria with serological titers, antistreptolysin O titer (ASO), and anti-deoxyribonucleic B (ADB), taken as evidence of recent streptococci infection. There is a lack of recent data available on ASO and ADB titers in children from the Delhi/NCR and thus adequate geographical area-specific cutoffs for the region are not available. Aims and Objectives: The aim of this study is to determine and compare the ASO and ADB antibody titers in children with acute pharyngitis, ARF, rheumatic heart disease (RHD), and in healthy children of the Delhi/NCR region. Materials and Methods: Twenty-six cases of ARF, 51 cases of RHD, 50 cases of acute pharyngitis, and 84 healthy normal children were included in the study. A single ASO and ADB titer measurement was done in these children. Results: The ASO titers was raised in acute pharyngitis – 303 IU/ml (interquartile range [IQR], 142–520 IU/ml) and ARF – 347.5 IU/ml (IQR, 125–686 IU/ml) children in comparison to healthy controls – 163.5 IU/ml (IQR, 133–246.5 IU/ml) and RHD patients – 163 IU/ml (IQR, 98.250–324.500). The ADB titers were highest in ARF patients – 570.5 IU/ml (IQR, 276–922 IU/ml) followed with RHD – 205 IU/ml (IQR, 113.6–456.5), healthy controls – 78.25 IU/ml (IQR, 53.39–128.15 IU/ml), and acute pharyngitis – 75.12 IU/ml (IQR, 64.5–136 IU/ml). The upper limit of normal (ULN) values of ASO and ADB computed from normal healthy children were 262.4 IU/ml and 134.44 IU/ml, respectively, and these can be used as cutoff values for recent streptococcal infection in this geographical area. Conclusions: The median ASO titers in acute pharyngitis group and ARF were significantly raised compared to that of the control group. The ADB titers were raised in ARF and RHD patients albeit the levels were higher in ARF patients. The derived ULN values can be used as cutoff reference.

Anomalous aortic origin of the pulmonary arteries: Case series and literature review.

Abstract: Anomalous origin of the pulmonary arteries from the ascending aorta is a rare, but severe clinical entity necessitating a scrupulous evaluation. Either the right or the left pulmonary arteries can arise directly from the ascending aorta while the other pulmonary artery retains its origin from the right ventricular outflow tract. Such a finding can be isolated or can coexist with several congenital heart lesions. Direct intrapericardial aortic origin, however, must be distinguished with origin through a persistently patent arterial duct. In the current era, clinical manifestations usually become evident in the newborn rather than during infancy, as used to be the case. They include respiratory distress or congestive heart failure due to increased pulmonary flow and poor feeding. The rate of survival has now increased due to early diagnosis and prompt surgical repair, should now be expected to be at least 95%. We have treated four neonates with this lesion over the past 7 years, all of whom survived surgical repair. Right ventricular systolic pressure was significantly decreased at follow-up. Our choice of treatment was to translocate the anomalous pulmonary artery in end-to-side fashion to the pulmonary trunk. Our aim in this report is to update an Italian experience in the diagnosis and treatment of anomalous direct origin of one pulmonary artery from the aorta, adding considerations on the lessons learned from our most recent review of the salient literature.

Long-term follow-up and outcomes of discrete subaortic stenosis resection in children

Abstract: Background: Studies of long-term outcomes of discrete subaortic stenosis (DSS) are rare. Therefore, we reviewed the long-term outcomes of subaortic membrane resection in children with isolated DSS over 16 years from a single institution. Materials and Methods: We retrospectively reviewed the records of patients (n = 27) who underwent resection of DSS between 2000 and 2017. Patients with major concomitant intracardiac anomalies were excluded. Indications for surgery were mean left ventricular outflow tract (LVOT), Doppler gradient >30 mmHg, and/or progressive aortic insufficiency. Results: The mean age at diagnosis was 3.77 ± 3.49 years (range, 0.25–13 years) and the mean age at surgery was 6.36 ± 3.69 years (range, 1–13 years). All patients underwent resection of subaortic membrane. The mean LVOT Doppler gradient decreased from 40.52 ± 11.41 mmHg preoperatively to 8.48 ± 5.06 mmHg postoperatively (P Conclusions: Resection of subaortic membrane in children is associated with low mortality. Higher LVOT gradient, younger age at initial repair, and shorter valve-to-membrane distance were found to be associated with adverse outcome. Recurrence and reoperation rates are high, and progression of aortic insufficiency following subaortic membrane resection is common. Therefore, these patients warrant close follow-up into adult life.

Abernethy malformation: Our experience from a tertiary cardiac care center and review of literature

Abstract: Abernethy malformation, also called as congenital extrahepatic portosystemic venous shunt, is a rare anomaly involving the portal venous system. Although rare, it is increasingly being reported and is important to diagnose given the adverse clinical consequences in untreated patients. It has myriad of clinical presentations, from being completely asymptomatic to causing hepatic carcinoma, hepatic encephalopathy, severe pulmonary hypertension, and diffuse pulmonary arteriovenous malformation. We describe our experience with five cases in a tertiary pediatric cardiac care center with Abernethy malformation, with review of literature and also discuss possible therapeutic implications.

Exercise-induced syncope and Brugada syndrome.

Abstract: Brugada syndrome (BrS) is a hereditary condition that is characterized by ST elevation, ventricular tachycardia or fibrillation, and sudden cardiac death in otherwise healthy patients. Life-threatening arrhythmias generally occur, while at rest, with fever or during vagotonic states. Exercise is generally not considered a trigger for ventricular arrhythmias or syncope in patients with BrS. We describe a patient who presented with exercise-induced syncope, ventricular tachycardia during an exercise test, and was found to be both genotypically and phenotypically positive for BrS. This case highlights a potentially important role of exercise testing in diagnosing and risk stratifying certain patients with BrS.

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

Abstract: 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.

Atrioventricular septal defect and tetralogy of Fallot - A single tertiary center experience: A retrospective review.

Abstract: Background: Complete atrioventricular septal defect (CAVSD) in association with tetralogy of Fallot is a rare and complex disease that makes its repair more difficult than repair of either lesion alone. We reviewed retrospectively our experience in managing this lesion. Patients and Methods: Between February 2006 and May 2017, 16 patients who underwent repair of CAVSD/tetralogy of Fallot (TOF) were reviewed retrospectively. Fifteen patients had trisomy 21. Five patients underwent primary repair while eleven patients went for staged repair in the form of right ventricular outflow tract (RVOT) stenting (n = 9) or systemic to pulmonary (S-P) surgical shunt (n = 2). RVOT stenting has replaced surgical shunt since 2012 in our center. Early presentation with cyanosis was the main determinant factor for staged versus primary repair. Results: The median age at first palliation was 46 days (range 15–99 days). The median age at total repair for both groups was 6 months (range 3–18 months); the median age for the palliated patients was 6.5 months (range 5–18 months) while the median age for primary repaired patients was 5 months (range 3–11 months). The median weight at final repair was 6.9 kg (3.7–8.2 kg). The pulmonary valve was preserved in five patients (31%), four of them had no prior palliation. Chylothorax occurred in 50% of the patients. One late mortality occurred after final repair due to sepsis. Conclusion: CAVSD/TOF can be repaired with low mortality and morbidity. The use of RVOT stent has replaced the surgical (S-P) shunt with acceptable results in our center.

Complete transposition of great arteries associated with total anomalous pulmonary venous connection: An unusual cause for early left ventricular myocardial mass regression

Abstract: A 24-day-old apparently asymptomatic neonate was found to have complete transposition of great arteries with small patent ductus arteriosus and restrictive patent foramen ovale. The neonate was found to have relatively high saturations (saturations = 88%) despite inadequate mixing communications. Echo findings were suggestive of significant dilatation of right atrium and right ventricle and left ventricular (LV) mass regression. Further echo interrogation revealed coexisting total anomalous pulmonary venous connection (TAPVC) as the cause of relatively high saturations and early LV mass regression. The patient was planned for follow-up and underwent successful Senning repair at the age of 8 months. Hemodynamics and echo findings of this association of TGA with TAPVC have been described in this case report.

“Idiopathic” pulmonary arterial hypertension in early infancy: Excluding NFU1 deficiency

Abstract: NFU1 deficiency is a rare metabolic disorder affecting iron-sulfur cluster synthesis, an essential pathway for lipoic acid-dependent enzymatic activities and mitochondrial respiratory chain complexes. It is a little-known cause of pulmonary arterial hypertension (PAH), while PAH is a prominent feature of the disease. We herein report on a female infant diagnosed as having idiopathic PAH since 1 month of age, who did not respond to bosentan plus sildenafil. NFU1 deficiency was only suggested and confirmed at 10 months of age when she demonstrated neurological deterioration along with high glycine levels in body fluids. Unexplained PAH in early infancy should prompt clinicians to perform amino acid chromatography searching for high glycine levels. Early recognition will avoid further invasive procedures and enable appropriate genetic counseling to be offered. No effective treatment is currently able to prevent the fatal course of this metabolic condition.

Retrograde transcatheter closure of anterior mitral valve leaflet perforation.

Abstract: Transcatheter closure of mitral valve leaflet perforation is a very rarely performed and a difficult procedure for repairing the defect. Herein, we are the first to report on both the safety and feasibility of percutaneous retrograde transcatheter closure of anterior mitral valve leaflet perforation with an AMPLATZER™ Duct Occluder II (6 mm × 6 mm, ADO II; Abbott Vascular, IL, USA) device in a 19-year-old patient with a severe mitral valve regurgitation following cardiac surgery.

Short-term clinical and echocardiographic outcomes after use of polytetrafluoroethylene bicuspid pulmonary valve during the repair of tetralogy of Fallot.

Abstract: Background: Application of transannular patch (TAP) during the repair of tetralogy of Fallot (TOF) leads to the development of pulmonary regurgitation (PR). This PR is known to cause right ventricular (RV) volume overload and dysfunction which in turn leads to increase in both morbidity and mortality both in immediate and long-term periods. Here, we sought to analyze the effects of polytetrafluoroethylene (PTFE) pulmonary bicuspid valve on the early outcome of patients with TOF repair where TAP is needed. Subjects and Methods: This is a retroprospective, observational study where PTFE bicuspid pulmonary valve was incorporated in all consecutive patients undergoing repair of TOF involving the application of TAP. Postoperative inhospital course was assessed, and patients were followed till 6 months for the status of PR and peak RV outflow tract (RVOT) gradient. Results: 38 patients were enrolled in the study. The mean age of participants was 53.66 ± 78.67 months. Extubation in operating room was done in 31 (81.58%) patients. Duration of ventilator support was 10.48 ± 24.22 h, and duration of hospital stay was 7.95 ± 3.08 days. These data were compared with three recent studies where only TAP was applied. Extubation in operating room, mechanical ventilation time, and hospital stay were significantly improved in the present study. Out of 37 live patients, four (10.81%) patients had no PR, 28 (75%) had mild, 1 (2.7) had moderate, and 4 (10.81%) had severe PR. Average RVOT peak gradient was 18.8 ± 8.1 mmHg. Conclusion: Incorporation of PTFE bicuspid pulmonary valve during the repair of TOF appears to facilitate early extubation and thus reduces ventilator-associated comorbidities. Study needs further long-term follow-up to look for behavior of PTFE bicuspid valve.

Isolated left common carotid artery in an infant with pulmonary atresia and intact ventricular septum

Abstract: Isolation of the left common carotid artery (LCCA) is a very rare congenital aortic arch anomaly. We present this finding in a female infant with pulmonary atresia and intact ventricular septum, with a clinical diagnosis of CHARGE syndrome. Cardiac catheterization revealed an anomalous origin of the LCCA from the pulmonary trunk, with retrograde filling of the pulmonary trunk seen during left subclavian artery injection. The LCCA was ligated during central shunt placement.

Early predictors of cardiac dysfunction in Egyptian children with chronic kidney disease

Abstract: Background: Cardiovascular morbidity (CVM) is the main etiology of mortality in children and adolescents with chronic kidney disease (CKD). CKD associated cardiovascular mortality is more common in children with diastolic cardiac dysfunction which was considered as an early indicator for death, while increased left ventricular mass (LVM) is a strong independent risk factor for these patients. Vitamin D deficiency was previously studied as one of the risk factors for CVM. Aim: The aim of the work was to investigate the relationship between biomarkers of mineral bone disorder including serum 25(OH) Vitamin D3 (25-OH D3), phosphorus and calcium × phosphorus (Ca×Po4) product with diastolic cardiac function and LVM in children and adolescents with CKD. Subjects and Methods: This was a cross-sectional observational study. Participants were classified into two groups: Group I including 86 pediatric patients with CKD (stages 4 or 5) and Group II including 40 healthy controls. Group I was subdivided into IA included children with diastolic dysfunction and IB included cases without diastolic dysfunction. 25-OH D3 level was measured by enhanced chemiluminescence method and intact parathyroid hormone (iPTH) by electrochemiluminescence method. Parameters for diastolic function and LVM were assessed by Doppler echocardiography, tissue Doppler imaging, and M-mode echocardiography. Results: 25-OH D3 level was significantly lower in Group I when compared to Group II. Diastolic dysfunction was present in 48.8% of the studied patients and was significantly associated with increased serum phosphorus and calcium-phosphorus product but not with decreased level of 25-OH D3. There was a significant positive correlation between LVM and iPTH. Conclusions: Hyperphosphatemia and high Ca×Po4 product were considered of prognostic value as they predict early diastolic dysfunction and increased LVM in children with CKD.

Unroofed coronary sinus: An unusual interatrial communication and a rare childhood entity.

Abstract: Unroofed coronary sinus, an unusual form of interatrial communication, is a rare cardiac anomaly. It is not a true defect of the atrial septum. It is described as a partial (focal or fenestrated) or complete absence of the roof of the coronary sinus, resulting in a communication between the coronary sinus and left atrium. It is presumably the least common variety of defects associated with interatrial shunting. Such defects are often difficult to diagnose and may even be overlooked during surgery for complex congenital heart disease. In most cases, they are associated with a persistent left superior vena cava, pulmonary or tricuspid atresia, and hearts with isomeric right atrial appendages. We report a case of this unusual form of interatrial communication without any associated anomalies in a 7-year-old girl child who presented to us with exertional dyspnea.

Transcatheter occlusion of partial anomalous pulmonary venous connection with dual drainage to left atrium

Abstract: Transcatheter therapy for partial anomalous pulmonary venous connection with dual drainage is unique and rarely reported. We report a 69-year-old female with recurrent brain abscess and partial anomalous connection of the left upper pulmonary vein with dual drainage to the vertical vein (VV) and left atrium (LA). Transcatheter occlusion of the VV was done using an 18-mm St. Jude Amplatzer Vascular Plug II, thus redirecting the left-sided pulmonary venous drainage to LA. Careful evaluation of partial anomalous pulmonary venous drainage with cross-sectional imaging is essential to allow the delineation of dual connections, enabling a less invasive transcatheter treatment approach.

Early right ventricular function following trans-right atrial versus trans-right atrial, trans-right ventricular repair of Tetralogy of Fallot: Results of a prospective randomized study

Abstract: Objective: We compared the pre- and post-operative right ventricular (RV) function by tricuspid annular plane systolic excursion (TAPSE) between trans-right atrial (t-RA) versus t-RA/RV (RA/RV) approach for the repair of Tetralogy of Fallot (TOF). Patients and Methods: Fifty consecutive patients, 1–15 years of age, undergoing intracardiac repair of TOF between September 2015 and June 2016 were randomized into two groups based on the approach for repair as follows: t-RA or t-RA/RV approach. TAPSE was used for the assessment of pre- and post-operative RV function. Results: Age, body surface area, preoperative saturation, cardiopulmonary bypass and aortic cross-clamp times, inotropic score, postoperative intensive care unit, and hospital stay were similar in both the groups. However, t-RA/RV group had significant mediastinal drainage (169 ± 163 ml vs. 90.6 ± 58.7 ml, P 0.05) and so was the post-operative TAPSE at discharge (1.52 ± 0.30 vs. 1.43 ± 0.32, P > 0.05), at 1 month (1.6 ± 0.27 vs. 1.43 ± 0.032, P > 0.05) and at last follow-up (1.79 ± 0.15, median 1.8 vs. 1.72 ± 0.17, median 1.7 P > 0.05). Conclusion: Both t-RA and t-RA/RV approaches provide safe palliation for patients with TOF. A limited right ventriculotomy neither leads to deleterious effects on early RV function nor does it increase the incidence of arrhythmias at early follow-up. Larger studies with longer follow-up are needed to further address these issues.

Neutrophil-to-lymphocyte ratio used as prognostic factor marker for dilated cardiomyopathy in childhood and adolescence.

Abstract: Objective: The objective of this study is to evaluate the neutrophil-to-lymphocyte ratio (NLR) and platelet–lymphocyte ratio, from the hemograms obtained from children and adolescents with dilated cardiomyopathy (DCM), and to correlate them with the levels of B-type natriuretic peptide (BNP) and with the clinical evolution of these patients in the long term. Materials and Methods: Follow-up of 57 patients with DCM was made retrospectively, with hemogram and BNP level determination being performed after optimized therapy for heart failure. We compared the findings of the patients' examinations that progressed with stability in relation to the occurrence of transplant listing, cardiac transplantation, or evolution to death. Results: The average age was 48 months, and the follow-up was 64 months. The average of the levels of neutrophils was greater in poor evolution group (7026 vs. 3903; P = 0.011) as well as the average of NLR (5.5 vs. 1.9; P = 0.034). The averages of hemoglobin, total leukocytes, lymphocytes, and platelets were similar in the groups. The area under the receiver operating characteristic curve for NLR in relation to the poor evolution was of 72.9%, being the best cutoff point of NLR ≥5.2 (sensitivity: 93.8% and specificity: 87.8%). Kaplan–Meier curves demonstrate that patients with NLR ≤5.2 (P = 0.001) and BNP Conclusions: NLR (≥5.2) and lymphopenia (≤1000 lymphocyte/μL) were associated with a poor prognosis and a higher chance of evolution to death or cardiac transplant, similar to the findings for BNP.

Systemic arterial aneurysm complicated by thrombosis in an infant with resistant Kawasaki disease.

Abstract: Kawasaki disease (KD) is a systemic vasculitis commonly associated with coronary artery aneurysms. Small-sized and medium-sized systemic arterial aneurysms have also been described, particularly in infants and patients with resistant KD. This case illustrates the presentation of a systemic arterial aneurysm complicated by arterial thrombosis and successful interventional management in a young infant.

Virtual three-dimensional model for preoperative planning in a complex case of a double outlet right ventricle.

Abstract: A 3-year-old child with a complex variant of double outlet right ventricle achieved a successful biventricular repair after detailed delineation of the intracardiac anatomy on multimodality imaging. A virtual three-dimensional (3D) model based on computed tomography was used successfully in the absence of an actual 3D-printed model. This case report seeks to highlight and hence increase the utilization of the virtual 3D model in resource-limited settings.

N-terminal-pro-b-type natriuretic peptide levels and cardiac hemosiderosis in adolescent β-thalassemia major patients

Abstract: Background: Iron-induced cardiomyopathy remains the leading cause of mortality in patients with β-thalassemia major. Iron overload cardiomyopathy, which may be reversible through iron chelation, is characterized by early diastolic dysfunction. Amino-terminal pro-brain natriuretic peptide (NT-proBNP) is a sensitive biomarker of diastolic dysfunction. Aim: The aim of the study is to evaluate the diagnostic value of NT-proBNP as a surrogate marker of iron overload examined with magnetic resonance imaging T2-star (MRI T2*). Methods: Sixty-eight β-thalassemia major patients (10–18 years) with no signs of heart failure underwent NT-proBNP measurement before routine transfusion. All participants prospectively underwent cardiac MRI T2* examination within 3 months (median 19 days). Patients were divided as cardiac hemosiderosis (cardiac MRI T2* 20 ms). Results: Of 68 patients, the male-to-female ratio was 1:1.1 and the median age was 14.1 years (range: 10–17.8 years). NT-proBNP levels were not different between hemosiderosis and nonhemosiderosis patients (P = 0.233). Further receiver operating characteristic analysis resulted in no significant correlation of NT-proBNP and MRI T2* (area under the curve 0.393, P = 0.233). Conclusion: Measurement of NT-proBNP levels cannot be used for early detection of cardiac iron overload in adolescent with β-thalassemia major.