Objective: To investigate patient-related factors, echocardiographic, and anatomic variables associated with immediate and long-term clinical outcomes after extracardiac Fontan procedure at our institution. Materials and Methods: Retrospective review of preoperative cardiac catheterizations and echocardiograms as well as medical records of all children with hypoplastic left heart syndrome (HLHS) who underwent Fontan between June 2002 and December 2018. Results: Seventy-seven patients with HLHS were included (age 4 years [1.5–11.7]). Seventy patients (91%) received a nonfenestrated Fontan and 57 patients (74%) underwent Fontan without cardiopulmonary bypass (CPB). Presence of a Fontan fenestration (P = 0.69) and use of CPB (P = 0.79) did not differ between those with <2 weeks compared to those with =2 weeks of chest tube drainage. There were no differences in either pre- or intra-operative hemodynamics between patients who weighed <15 kg compared to those who weighed ≥15 kg at time of surgery; incidence of death, transplant, and transplant listing were similar between weight groups. Inferior vena cava (IVC) diameter z-score did not differ among patients with and without chylous chest tube drainage (P = 0.78), with and without development of protein losing enteropathy (P = 0.23), or death/heart transplant/transplant listing compared to survivors without transplant (P = 0.26) Conclusion: In HLHS patients undergoing Fontan, preoperative weight and IVC diameter appeared to have no influence on immediate postoperative outcomes. Performing the Fontan off CPB and with a fenestration also conferred no added clinical benefit. These observations should be considered when deciding optimal timing for Fontan completion.

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Background: Pediatric heart transplantation is a now a well-established and standard treatment option for end stage heart failure for various conditions in children. Due to logistic issues, it is not an option for in most pediatric cardiac centres in the third world. Aim: We sought to describe our early experience in the current era in India. Methods: This is a short term retrospective chart review of pediatric patients who underwent heart transplantation at our centre. Mean/Median with standard deviation /range was used to present data. Results: Twenty patients underwent orthotopic heart transplant between January 2016 and June 2019. The median age at transplant was 12.4years (range 3.3 to 17.3 years). The median weight was 23.2kg (range 10-80kg). The mean donor/recipient weight ratio was 1.62± 0.84. The mean ICU stay was 12.1days. The mean follow up post transplant was 2.03± 0.97years (range 10 days-3.57years). The 1 month and the 1 year survival was 100%. Biopsies were positive for significant rejection in 7 patients (35%). At the time of last follow-up, 3 patients (15%) had expired. The major post transplant morbidities were mechanical circulatory support (n=3), hypertension with seizure complex (n=3), post transplant lympho-proliferative disorder (n=1), pseudocyst of pancreas (n=1), coronary allograft vasculopathy (n=3) and systemic hypertension (n=7). All surviving patients (n=17) were asymptomatic at last follow up. Conclusion: The results suggest acceptable short term outcomes in Indian pediatric patients can be achieved after heart transplantation in the current era. Significant rejection episodes and coronary allograft vasculopathy need careful follow up.

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Background: Pulmonary stenosis in patients with common arterial trunk protects the pulmonary vasculature. In our recently published prospective study of common arterial trunk, some patients with sinusal origin of the pulmonary arterial segment had pulmonary stenosis induced by systolic excursion of a truncal valvar leaflet. We aimed to determine the detailed morphologic characteristics of this unusual finding. Methods and Results: All 70 patients underwent echocardiography and computed tomographic angiography as per predefined study protocol. In selected cases, we also performed cardiac catheterization. Among 27 patients with aortic dominance, we found sinusal origin of the pulmonary arterial segment. In 5 of these patients, pulmonary stenosis was induced by systolic excursion of a truncal valvar leaflet. In all these patients, the truncal valve was trisinusate, albeit with asymmetric sinuses. The pulmonary arterial segment arose from the largest left sinus with its relatively large leaflet obstructing the pulmonary orifice during systole. In the remaining 22 patients, without pulmonary stenosis but with sinusal origin of pulmonary arterial segment, the truncal valve was quadrisinusate in 7, bisinusate in 2, and trisinusate in 13. None of the patients with quadrisinusate and bisinusate truncal valves had pulmonary stenosis. Among the 13 patients with trisinusate valves, the sinuses of the truncal valve were symmetrical in 6, while in 7, the pulmonary orifice originated from a smaller asymmetric sinus. Conclusion: Pulmonary stenosis, produced by a relatively large leaflet of an asymmetric truncal sinus, may prevent early development of pulmonary vascular disease. Timely recognition of this unusual mechanism of pulmonary stenosis is important for optimal management.

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Objectives: This study assesses the competency of pediatricians in interpreting electrocardiograms (ECGs). Methods: A cross-sectional study involving 125 pediatricians comprised of 71 general pediatricians, 15 pediatric cardiologists, and 39 other subspecialists recruited from all public hospitals and two specialty centers. Participants completed a questionnaire that included 10 ECGs and questions regarding backgrounds, attitudes, and practices. The ECGs were graded to obtain a knowledge score out of 30 points. Mann–Whitney U test and Kruskal-Wallis test with post hoc analysis and Bonferroni adjustment were used to compare groups. Results: The mean knowledge score ranged from 47.7% to 69.7% among various pediatric specialties (P = 0.006). Age, increasing years of experience, confidence level, number of cardiology referrals, and perceived importance of having good ECG interpretation skills were significantly related to the knowledge score (P = 0.05). Accuracy was highest in identifying normal ECGs (76.8%), supraventricular tachycardia (64.8%), along with long QT interval (58.4%), and was lowest for right bundle branch block (RBBB) (10.4%), 2:1 atrioventricular conduction (10.4%), and atrial tachycardia (AT) (4.8%). Accuracy among pediatric cardiologists was highest for long QT interval (100%), normal ECG (80%), as well as Wolff-Parkinson-White syndrome (80%), and lowest for RBBB (13.3%) and AT (0%). Most pediatricians believe that ECGs are “useful” (78.4%) and that having good interpretation skill is “important” (80.6%). Conclusions: Pediatricians recognize the importance of ECGs. However, their skill and level of accuracy at interpretation is suboptimal, including cardiologists, and may affect patient care. Thus, efforts should be made to improve ECG understanding to provide better service to patients.

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There is limited information about coronavirus disease 2019 (COVID-19) in the pediatric population. Preliminary data suggest a not insignificant prevalence of cardiac involvement. Here, we report our early experience with COVID-19 in the pediatric population. These patients display exceptionally high levels of acute-phase reactants. The clinical syndrome in these patients is somewhat similar to Kawasaki disease with or without myocardial involvement. In some cases, the presentation mimics typical myocarditis. Severe myocardial involvement is associated with transient electrocardiographic and echocardiographic abnormalities. These findings may be due to the cardiotropic nature of the virus or may be the result of an immunologic response to the infection.

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Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid-term outcomes of surgical correction of patients with APVS. Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients. Results: The overall 10-year survival was 82.1%. There was 81.1% overall freedom from re-intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re-intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10-year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications. Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems.

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We herein report a case of a 6-year-old boy with hypertension and complete heart block with a unique outcome. The patient was treated with an epicardial pacemaker followed by complete and sustained resolution of his hypertension with no further need for the previously prescribed antihypertensive medication.

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A 3-year-old male presents to the emergency department with chief complaints of fever and vomiting. He had a positive rapid streptococcus throat test with cervical lymphadenopathy. The patient was started on antibiotics. On examination, there was diffuse erythematous macular rash on the chest. Laboratory tests revealed elevated white cell count and C-reactive protein. Electrocardiogram was notable for prolonged PR interval indicating 1^st degree atrioventricular block. Echocardiogram revealed ectasia of the right coronary artery (RCA). A presumptive diagnosis of Kawasaki disease was made and the patient was started on high-dose aspirin and intravenous immunoglobulins. Cardiac computed tomography angiography (CTA) showed an aneurysm of the proximal RCA measuring up to 7.4 mm. The RCA immediately proximal to the aneurysm measured 3 mm in diameter. The Z score was 13.4. Oblique coronal image from cardiac CTA and volume rendered images demonstrated an aneurysm of the proximal RCA. The patient improved with treatment.

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Flecainide is a class IC antiarrhythmic indicated for ventricular and supraventricular arrhythmias in pediatric patients without structural heart disease. Flecainide has a narrow therapeutic window and proarrhythmic effect even in therapeutic doses and could lead to a life-threatening intoxication. Dosage errors, accidental intakes, and drug or food interactions, especially with dairy products, can be the cause of the intoxication. We report three consecutive cases of flecainide intoxication in children with supraventricular tachycardia (SVT) in our hospital from 2017 to 2019. Two cases had complete and spontaneous normalization of electrocardiogram (ECG) after flecainide removal. However, admission to the intensive care was required due to a sustained ventricular tachycardia in one case. Flecainide intoxication can be a life-threatening complication in patients with SVT. We believe all children should have close monitoring with serial ECG and plasma levels of flecainide during the 48–72 h after initiation of treatment, and consider hospitalization for patients <1 year of age.

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Coronary allograft vasculopathy fails to give a warning anginal pain due to denervation and often presents with acute coronary syndrome, ventricular dysfunction, or sudden cardiac death. Early diagnosis in a pediatric patient is difficult as it involves invasive coronary angiography or advanced imaging such as intravascular ultrasound or optical coherence tomography. A 12-year-old boy developed acute coronary syndrome, elevated troponins, and right bundle branch block, 5 years after cardiac transplantation and was treated with culprit-vessel angioplasty with a drug-eluting stent. Advanced imaging showed the involvement of nonculprit vessels too. In a detailed literature search, we failed to identify a similar clinical presentation and management in the subcontinent, hence our interest in publishing this report for educational value. Issues in diagnosis, management, prognosis, and prevention are discussed.

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Kawasaki disease (KD) is a systemic inflammatory condition primarily affecting young children. We present an adolescent male with two episodes of complete KD between the age of 2 and 14 years. At age 14, he presented with findings suggestive of a retropharyngeal abscess. This was later determined to be a recurrence of KD, diagnosed after the development of coronary artery aneurysms. Our case reinforces the role of maintaining a high index of suspicion for KD, both in patients with prior KD episodes and in those with persistent fever who do not fulfill the diagnostic criteria for typical KD. This is particularly important for patients presenting with atypical symptoms not commonly associated with KD, such as inflammation of the retropharyngeal and parapharyngeal spaces.

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Extraintestinal manifestations are common complications of inflammatory bowel disease (IBD), whereas the recurrent pericarditis during remission of Crohn's disease is rarely reported. Chest pain developed in a 13-year-old adolescent male who had a history of Crohn's colitis since 9 years of age and was in remission for 4 years after treatment with infliximab, adalimumab, and vedolizumab. Physicians should be aware of the pericardial involvement in patients with a history of IBD. The literature on pericardial involvement in Crohn's disease is reviewed with emphasis on the management of recurrent pericardial effusion in the pediatric age group.

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Holt–Oram syndrome (HOS) (OMIM#142900) is a rare condition with upper extremity malformations as well as structural and conduction cardiac anomalies. There are sparse reports in the literature documenting malignancy in association with HOS. We report a pediatric patient clinically diagnosed with HOS (missing thumbs bilaterally, atrial septal defect, ventricular septal defect, and first-degree heart block), who also developed B precursor acute lymphoblastic leukemia. During induction of chemotherapy with steroids, she developed profound bradycardia without clinical symptoms. The bradycardia resolved without intervention, but this case highlights the challenges of managing chemotherapy side effects in a patient with congenital heart disease. A literature review pertinent to the associated findings in the case is also presented.

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Fontan surgery streamlines the systemic venous return through the pulmonary circulation before filling the systemic ventricle in univentricular hearts. The venous congestion leads to effusions, lowers cardiac output, and affects organ perfusion. Fenestrations in the Fontan circuit improve forward flow through the ventricles, lower venous pressures, and reduce perioperative morbidity. When large fenestrations cause profound hypoxia and effort intolerance, there are no current techniques to reduce their size. Atrial flow regulators with a predetermined orifice were used off-label in three borderline patients with large undesirable fenestrations following extracardiac conduit Fontan surgeries. This resulted in improved oxygenation without marked elevation of venous pressures, while retaining the patency of the decompressive fenestration.

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Diffuse pulmonary arteriovenous malformations or pulmonary arterial hypertension (PAH) may result from congenital portosystemic venous shunts. Hemangioma as a physical sign of congenital portosystemic shunts (like Abernethy syndrome) has not been described. I report two children (one with severe cyanosis from pulmonary arteriovenous malformations and the other with severe PAH) with cutaneous hemangioma and Abernethy syndrome. Hemangioma may be a clinical pointer to portosystemic shunts even in the absence of obvious liver disease.

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Embolization of the patent ductus arteriosus (PDA) device is a known adverse event of percutaneous PDA closure, which can lead to complications. Embolization can occur into the pulmonary artery or into the aorta. Device embolization can be moderate adverse event (when retrieved percutaneously) or major adverse event (when retrieved surgically). We are describing a hybrid approach for aortic embolization of PDA device when the percutaneous retrieval fails, where device retrieval and PDA ligation can be done through thoracotomy incision, thus decreasing the complications.

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Cardiac tumors in neonates and infancy are one among the many known congenital cardiac diseases. Although fetal cardiac tumors are rare, there is increased detection because of expertise in echocadiographic examination. Rhabdomyomas are the most common cardiac tumors among infants and children. Here, we describe twin neonates who had multiple cardiac tumors. This kind of presentation appears to be a very rare situation.

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Mycotic pseudoaneurysm of the aorta is a rare and lethal complication of pediatric congenital heart surgery. We report the lethal consequences of recurrent mycotic pseudoaneurysm in an 18-month-old baby, early after subaortic membrane resection. We managed to repair the pseudoaneurysm successfully by replacing the infected ascending aorta using bovine jugular vein graft, but unfortunately, the patient developed new pseudoaneurysm at the site of anastomosis which led to his death. Although prompt diagnosis and surgical management can save the patient life, uncontrolled infection can lead to the recurrence of the problem and lethal results.

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Background: Acute rheumatic fever (ARF) is an important cause of valvular heart disease in children. Endothelial dysfunction plays an important role in the pathogenesis of valvular heart diseases. The role of endothelial dysfunction in valvular heart diseases due to ARF is not exactly known. In ARF, autoimmune injury, inflammation, oxidative stress, and impairment of nitric oxide in valvular endothelium may be the causes of endothelial dysfunction. The purpose of this study is to evaluate endothelial dysfunction and arterial stiffness in children with ARF. Materials and Methods: Thirty-six patients diagnosed with ARF (the mean age was 11.80 ± 2.82 years) and 36 volunteered individuals with similar age, sex, and body mass index were included in the study. The study groups were compared in terms of M-mode echocardiography parameters, carotid arterial strain (CAS), beta-stiffness index (βSI), and flow-mediated dilation (FMD). Results: In patients with ARF, there was a decrease in FMD% (10.36 ± 7.26 and 12.76 ± 4.59; P < 0.001) compared to the control group. In addition, CAS (0.16 ± 0.06 and 0.18 ± 0.08; P = 0.44) and βSI (3.65 ± 1.61 and 3.57 ± 2.38; P = 0.24) were similar in the patient and the control groups. Furthermore, no correlation was detected between decreased FMD value and mitral regurgitation (r = -0.07; P = 0.66), aortic regurgitation (r = -0.04; P = 0.78), CAS (r = -0.08; P = 0.61), and βSI (r = -0.20; P = 0.22). Conclusion: In our study, a decrease in FMD value, which is a marker of endothelial dysfunction, was found in children with rheumatic carditis.

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