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.

Aim : The aim of this study was to evaluate presenting symptoms, clinical features, and laboratory tests for the diagnosis of Kawasaki disease (KD) in children and their short-term follow-up at a tertiary care hospital of North India from April 2017 to March 2020. Materials and Methods : A total of 31 children (23 boys and 8 girls) up to 10 years of age were included in this study. The diagnosis of KD was made as per the American Heart Association 2017 guidelines. Clinical features, laboratory parameters, and coronary involvement were compared between the complete and incomplete KD groups. Results : The incidence of complete versus incomplete KD was 19 (61.2%) versus 12 (38.7%) children, respectively. Change in extremities and oral mucosal changes were more encountered in the complete KD group as compared to the incomplete KD group (100% vs. 58.3%, P = 0.004, and 78.9% vs. 33.3%, P = 0.002, respectively). Coronary artery aneurysm was seen in 54% of the patients on echocardiography which was greater in the incomplete KD group (83.3%) as compared to the complete KD group (36.8%). The median time from the onset of symptoms to intravenous immunoglobulin infusion was <10 days in 84.2% of the patients with complete KD versus 41.7% with incomplete KD which was statistically significant. Fifty percent of the children with coronary ectasia and small aneurysm had normal coronaries at follow-up of 6 months. Conclusion : KD is probably underdiagnosed in most developing countries, like that of ours, and requires a high index of suspicion.

Background : Hypertension (HTN) is a major health problem affecting different populations including adolescents worldwide, and it is strongly associated with obesity. Objectives : This study examined the predictive capacity of anthropometric proxies of body fat and determined the threshold values that would identify HTN among Nigerian adolescents. Setting and Design : A cross-sectional study with a total of 2228 in-school adolescents aged 12–16 years. Materials and Methods : Participants were evaluated for physical characteristics including five anthropometric indices of body fat and blood pressure. Receiver operating characteristics curves were used for the analysis of sensitivity, specificity, area under curve (AUC) of the fat indices in detecting HTN. Results : All body fat indicators with the exception of waist-to-height ratio in boys, had significant (P < 0.0005) AUC with total fat mass (TFM) in girls and waist circumference (WC) in boys as the best fat indicators for predicting systolic HTN in adolescents. The TFM cut-point for girls was 8.0 kg and the WC cut-point for boys was 66.3 cm. Both TFM and WC demonstrated a stronger association with systolic HTN than other fat indicators in both genders. The likelihood of a girl developing HTN is 1.1 (95% confidence interval [CI] =1.05–1.20) times with a unit increase in TFM, while boys with unhealthy WC had 3.2 (95% CI = 1.83–5.67) times odd of developing HTN compared to their healthy peers. Conclusions : This study showed that TFM and WC are useful tools for detecting HTN in Nigerian adolescent girls and boys, respectively. The fat indicators used in this study generally showed low predictive capacity.

Introduction : Transmission of Group A Streptococcus from asymptomatic children to their surrounding carries a risk of acute rheumatic fever in susceptible people. Aim and Objectives : We aimed to investigate the prevalence and predictors of GAS carrier state and evaluate the antibiotic sensitivity pattern of GAS in Jakarta, Indonesia. Material and Methods : We enrolled 201 asymptomatic schoolchildren (6–12 years) using stratified random sampling from a primary school in Jakarta. None of the children had a history of rheumatic fever or rheumatic heart disease. All participants underwent physical examination, and laboratory tests include complete blood count, erythrocyte sedimentation rate, C-reactive protein, antistreptolysin O titer, and throat swab culture. Results : The prevalence of GAS carrier was 13.9% (95% confidence interval: 9.2%–18.6%) in our study. On multivariate analysis, tonsillar enlargement was found to be the only predicting factor of GAS carrier (P = 0.03). GAS was sensitive to penicillin G, erythromycin, vancomycin, clindamycin, chloramphenicol, azithromycin, and tetracycline in 100%, 89%, 86%, 75%, 68%, 68%, and 32% of patients, respectively. Conclusion : The GAS carrier state is common among school-age children affecting approximately 13.9% children. Tonsillar enlargement is a significant finding predictive of GAS carrier state. All isolates are still sensitive to penicillin and mostly sensitive to erythromycin but are increasingly resistant to tetracycline.

Background : Beta-thalassemia major is the most common chronic hemolytic anemia among children and adolescents across the world. Several studies have demonstrated that thalassemic patients who have preserved left ventricle systolic function could still have subtle systolic dysfunction. Among patients with beta-thalassemia, early detection of transfusion-induced myocardial iron loading and its intervention with aggressive chelation therapy may delay or reverse heart failure. Two-dimensional speckle-tracking echocardiography (2D-STE) is a novel tool that may detect early myocardial dysfunction in these patients. Objective : The aim of this study was to investigate whether longitudinal strain based on speckle tracking can detect subtle right or left ventricular dysfunction. Patients and Methods : Fifty thalassemic patients with preserved left ventricular ejection fraction (>55%), mean age of 14.75 ± 4.73 years, and thirty age-matched healthy control subjects have been included in the study. Conventional echo Doppler, tissue Doppler echocardiography, and 2D-STE were performed in all patients and control subjects. Results : The right ventricular and left ventricular longitudinal strains were significantly lower in patients than in controls (21.67 ± 5.59 vs. 25.32 ± 2.29, P = 0.001 for right ventricular and 21.29 ± 3.49 vs. 24.90 ± 0.97, P = 0.001 for left ventricular). Conclusions : The 2D-STE can detect early ventricular (left and right) systolic dysfunction in thalassemic patients in the presence of normal systolic function by conventional methods. It may be suggested that the assessment of global longitudinal strain (GLS) can be used as a useful and less expensive tool for screening myocardial iron overload, especially in countries with a limited magnetic resonance imaging (MRI) availability for logistic and economic reasons. Hence, we can refer positive cases with GLS to a higher center to do MRI and start intensive iron chelation treatment.

Background and Objective : The long QT syndrome (LQTS) is a repolarization defect of heart involving potassium linked channels and it usually manifests clinically as seizures, syncope, or sudden cardiac death syndrome in children secondary to its characteristic ventricular tachy-arrhythmia like torsades de pointes. The reason behind epilepsy or seizures like activity in this disease is the sequelae of prolonged cerebral hypoperfusion secondary to the cardiac dysrhythmia. The aim of study is to look for clinical spectrum and risk factors associated with LQTS among children presenting with epilepsy, which can predict the early diagnosis of LQTS. Materials and Methods: For this observational study, 422 patients having epilepsy presenting for the first time in a 3-year period were enrolled. Demographical profile, LQTS measures, and various factors under observation were recorded. Results : Among the 422 enrolled children (M: F 1.8:1) with age ranging from 4 to 87 months (median 23 months), 8 (1.9%) children who presented with epilepsy had LQTS. Among those, mean QTc on electrocardiogram was 454 ± 31 msec and mean Schwartz score >3. Half of the patients with LQTS had deafness (P = 0.002) and 37.5% had a positive family history (P = 0.0045). Nearly a third (37.5%) presented with syncope and 87.5% patients with LQTS had no postictal drowsiness or sleep (P ≤ 0.004). Conclusions : LQTS is underestimated in children presenting with epilepsy and LQTS should be considered as an alternate diagnosis in children with recurrent seizures or syncopal attacks. The brief period of seizures with no postictal drowsiness, syncope, and strong family history are the features which may help in segregating LQTS from epilepsy.

Uniatrial but biventricular atrioventricular connection is a rare congenital cardiac abnormality where the left atria-ventricular connection is absent and the right atrio-ventricular connection straddles the crest of the muscular ventricular septum. This anomaly has been referred to as double outlet right atrium and the atrio-ventricular valve as a common atrioventricular valve in the past.In the absence of a primary atrial septal defect, the atrio-ventricular junction is not a common junction and the valve cannot hence be described as a common trio-ventricular valve. We demonstrate this paradox by an illustrative case and contrast this with two other cases where a common atrio-ventricular valve was predominantly committed to the right atrium.

Background and Objectives : Infective endocarditis (IE) involving the native pulmonary valve (PV) is extremely rare, with no data in Indian literature. The objective of this communication is to describe the clinical and diagnostic characteristics, underlying risk factors, microbiological features, and management of PVIE. Methods : This is a retrospective analysis of 8 cases of PVIE managed in a tertiary care center from 1992 to 2020. Results : PVIE was observed in 8 patients with underlying congenital cardiac malformation (Group A, 6 Patients) and in patients with central venous catheter (Group B, 2 patients). All the patients had prolonged febrile illness accompanied by right heart failure 4 (50%), septic pulmonary emboli 2 (25%), and pulmonary regurgitation 3 (37.5%). Trans-thoracic echocardiography demonstrated the vegetations, whereas computed tomography of chest diagnosed pulmonary emboli in 2 (25%), and pulmonary artery aneurysm in 1 (12.5%) patient. The early mortality was extremely high (5, 62.5%). Delayed diagnosis, fulminant septicemia, and multi-organ failure resulted in unfavorable outcomes. Conclusions : IE of the native PV is a rare and potentially lethal illness. Diagnosis should be considered in any febrile patient with an underlying congenital defect, central venous line, bacteremia, and comorbidities. Multi-modality imaging should be utilized to enhance the diagnostic yield and detect complications promptly.

Eating disorders are common. Between 1% and 2% of adolescent females and 0.5% of males suffer from anorexia nervosa, bulimia nervosa, and binge eating disorder. Although suicide represents nearly half of the mortality in patients with eating disorders, a majority of the remainder is cardiac arrest, likely secondary to cardiovascular complications of eating disorders such as bradycardia, hypotension, QT interval changes, structural heart disease, and pericardial effusion. Bradycardia is suspected to be secondary to increased vagal tone and is a common finding in patients admitted with disordered eating. Similarly, hypotension and orthostatic abnormalities are common complications due to atrophy of peripheral muscles. Descriptive studies report prolongation of the corrected QT interval (QTc) in these patients relative to controls, albeit within the normal reference range. Structural heart disease is also common, with left ventricular mass reported as lower than predicted in several studies compared to healthy controls. Pericardial effusion is also commonly described, although it is possible that this is underestimated, as not all patients with eating disorders undergo echocardiograms. Further, refeeding syndrome as a result of treatment of eating disorders carries its own cardiac risks. Cardiac complications of malnutrition are common but reversible with appropriate management and recovery. It is imperative that providers are aware of the epidemiology of these complications, as it is only with a high clinical suspicion that proper evaluation including a thorough history and physical examination, electrocardiogram, and when necessary echocardiogram can be performed.

Necrotizing enterocolitis (NEC) remains a prominent surgical emergency among infant population, associated with a significant mortality, as well as various subsequent morbidities. Congenital heart disease (CHD) has an increased associated incidence with NEC in infant population. Recent research has provided insight into the pathophysiology of NEC in patients with CHD and how this differs from those without CHD. The deviation from normal circulatory physiology has a suggested association in the pathophysiology of NEC in CHD, which may have implications for the risk factors of NEC in infants with CHD, the effect on outcomes of NEC, and whether alternative approaches to management may need to be considered in comparison to classical NEC. This review aims to highlight studies that provide insight and awareness into the relationship between NEC and CHD, in order that clinicians may direct themselves more clearly toward optimal management for infants in this category.

A 32-year-old male with symptomatic polycythemia was investigated by hematologist for myeloproliferative malignancies and underwent five therapeutic phlebotomies. The identification of hypoxia on pulse oximetry later led to pulmonology evaluation that showed normal lung function tests as well as normal lung parenchyma and airways on advanced imaging. The absence of murmurs and significant precordial findings, normal chest X-ray, and unremarkable findings in electrocardiogram apart from first-degree heart block delayed the cardiac referral. Cardiac imaging showed a significant right to left shunt through a large oval fossa defect, mild hypoplasia of the right ventricle, normal right ventricular function and mild fibrosis within the myocardium. Hemodynamic assessment with test balloon occlusion led finally to a complete closure of the defect, which normalized the hypoxia. A step-by-step clinical approach finally leading to the management is presented in this clinicopathology correlation and hemodynamic rounds.

Left ventricular noncompaction (LVNC) is a rare phenotype of dilated cardiomyopathy. We report a child with primary systemic carnitine deficiency having associated LVNC.

The incidence of unusual coronary patterns including single coronary artery is high in Taussig-Bing anomaly (TBA). The relocation of a single coronary artery from a nonfacing sinus can be technically challenging with implications on early and late outcomes. Many innovative techniques for coronary transfer have been described and no coronary pattern precludes arterial switch operation in the current era. We describe a technique of coronary transfer using autologous pericardial tube extension with good early outcome.

We hereby report rare occurrence of irreversible complete heart block in a child with tricuspid valve infective endocarditis. The tricuspid valve vegetation also caused complete closure of perimembranous ventricular septal defect, which was later discovered during surgery.

Rarely performed nowadays, the Björk procedure is an alternative to the Fontan palliation for biventricular repair in patients with tricuspid atresia. The right atrium to right ventricle conduit failure leads to serious long-term complications. The management of such patients at high surgical risk remains challenging. We report a successful reoperation in a young adult 25 years after the modified Björk intervention, who presented with severely narrowed and calcified valved conduit.

Pulmonary arteriovenous malformation (PAVM) is an abnormal communication between the pulmonary artery and the pulmonary vein. PAVMs are usually congenital in origin; however, they may be acquired. Three-dimensional rotational angiography (3DRA) is a technique used increasingly for imaging in congenital heart disease but to our knowledge has never been used in imaging and planning device closure of PAVM. We describe the use of 3DRA technique for imaging and planning device closure of PAVMs and discuss the advantages of this modality. 3DRA is an excellent tool for imaging of various vascular anomalies. It provides high-quality accurate images through a quick and safe procedure, it is also very useful in planning interventional procedures in PAVM, as it clearly delineates all feeders to PAVM and gives an exact view for analysis and intervention.

In the normal lung, the only communications between the systemic and pulmonary arterial systems are the connections between the bronchial and pulmonary arteries that occur at the respiratory bronchioles, where pulmonary and bronchial capillaries freely anastomose. Rarely, anomalous connections can occur between normal or aberrant systemic arteries and pulmonary vessels. We performed a comprehensive literature review of all available manuscripts on PubMed and Google Scholar that included a case report or case series with diagnosis of systemic artery to pulmonary venous fistulas who underwent percutaneous treatment. Furthermore, we report three cases of children diagnosed and treated in our Pediatric Cardiology Center.

Ascending aorta thrombosis (AAT) in a neonate is a rare and lethal event. To date, AAT has been reported in around 25 patients, out of whom only eight patients had concomitant arch involvement. We report a case of one such patient with ascending aorta and arch thrombosis and present a brief review of the available literature.

Left Ventricular Non Compaction (LVNC) is considered a unique cardiomyopathy according to the American Heart Association guidelines. The genetic ethology of LVNC in children is not completely understood although upto 41% of LVNC are thought to be genetic. We report a family with LVNC due to a novel mutation in the MYH 7 gene.

Pulmonary sequestrations are nonfunctioning pulmonary parenchyma supplied by a systemic artery. We describe an 18-month-old baby girl with no significant past medical history who was found to have a large isolated collateral from the aorta entirely perfusing the functional left lower lobe. Cardiac catheterization confirmed the major aortopulmonary collateral artery supplying the left lower lobe with normal drainage into the left atrium. At a multidisciplinary meeting, decision was made to preserve the functional lobe. During surgery, the collateral artery was dissected and its course within the pleural space appreciated, the main left pulmonary artery was isolated. Intraoperatively surgeons anastomosed an 8 mm ringed conduit from the left pulmonary artery, anteriorly to the proximal aspect of the collateral. The patient tolerated the surgery well. Postoperative echocardiogram showed pulmonary flow to the lower lobe, and she was discharged home on postoperative day 2 without complications.

Anomalies of systemic venous connections are extremely rare. We describe the case of an asymptomatic 29-year-old woman who was found to have systemic desaturation in the peripartum period and referred to us for suspected cyanotic heart disease. She was diagnosed to have hemianomalous systemic venous connection of the inferior vena cava (IVC) into the left atrium (LA). Transesophageal echocardiogram with contrast diagnosed anomalous connection of the IVC to the LA, further confirmed by computed tomography and conventional angiography. The patient underwent successful surgical correction with an uneventful postoperative course.

An 11-month-old girl was detected to have muscular ventricular septal defect with hyperkinetic pulmonary hypertension (PH) was urgently operated upon. On follow-up, her PH worsened, resulting in right ventricular dysfunction and was later detected to have absent portal vein.

We report a newborn with truncus arteriosus and a very unusual variant of “crossed” pulmonary arteries. The left pulmonary artery arose anterior and slightly to the right side of the common trunk and the right pulmonary artery arose from the posterior and left side of the common trunk. Computed tomographic images and a diagram are provided.

Congenital Heart Diseases occur in close to 90% of children with Trisomy 18. A ventricular septal defect along with abnormalities of more than one cardiac valve is considered to be an imaging hallmark of Trisomy 18. We present echocardiographic images of an infant with Trisomy 18 who had a large ventricular septal defect and abnormalities of all cardiac valves.

Midaortic syndrome (MAS) is characterized by narrowing of the descending aorta between the distal aortic arch and the aortic bifurcation. We present the case of a 4-year-old male presenting with a murmur and diagnosed with MAS. He was treated with a thoracoabdominal bypass graft.