Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital cardiac malformation, in which the morphologic right ventricle (RV) supports the systemic circulation. Systemic RV hypertrophy is usually secondary to pressure overload, while intrinsic myopathic hypertrophy due to sarcomeric gene mutation is exceedingly uncommon. We report a 37-year-old male who presented with exertional dyspnea and chest discomfort. Echocardiography and cardiac magnetic resonance (CMR) imaging revealed atrioventricular and ventriculoarterial discordance consistent with CCTGA, accompanied by asymmetric hypertrophy of the systemic RV and interventricular septum without significant outflow obstruction. Late gadolinium enhancement demonstrated patchy myocardial fibrosis. Genetic testing identified a heterozygous truncating variant in the TTN gene (exon 46, c. 14414del, p.Thr4805 LysfsTer24), confirming familial hypertrophic cardiomyopathy (HCM). The present case highlights an extremely rare coexistence of CCTGA and TTN-related HCM of the systemic RV. Recognition of this overlap requires high clinical suspicion, multimodal imaging, and molecular testing, which together enable accurate diagnosis, family counseling, and long-term management of this unique phenotype.

Fetal cardiac rhabdomyoma is the most common prenatal cardiac tumor and is frequently associated with tuberous sclerosis complex (TSC). Everolimus, a mammalian target of rapamycin inhibitor, is an established postnatal therapy for TSC-related tumors, but its prenatal use remains rare. A 27-year-old primigravida was referred at 21 weeks' gestation for evaluation of a left ventricular mass. Given the rapid, severe progression of tumor size, maternal oral everolimus therapy was initiated at 28 weeks, with dose titration to achieve drug levels of 3-8 ng/ml. At 32 weeks, the tumor size regressed significantly. Neonatal serum everolimus on day 1 was 5.2 ng/mL, confirming transplacental transfer. Therapy was resumed postnatally, and at 5 months, the tumor had completely resolved, with normal neurodevelopment and no seizures. While short-term safety and efficacy of prenatal everolimus therapy are promising, further studies are needed to establish standardized dosing, safety profiles, and long-term developmental outcomes.

This case series demonstrates the feasibility and potential safety of IPIG as a novel fetal immunomodulatory therapy. Although outcomes were encouraging, efficacy cannot be established without larger comparative studies.

BACKGROUND:The KONAR-MF™ occluder, with its flexible medium-profile design, has broadened the feasibility of transcatheter closure of muscular ventricular septal defects, particularly in infants. OBJECTIVE:To assess feasibility, safety, techniques, and outcomes of muscular ventricular septal defect closure using the KONAR-MF™ occluder in a multicentre paediatric cohort. METHODS:A retrospective review was conducted at three tertiary paediatric cardiac centres (2018-2024). Patient demographics, ventricular septal defect characteristics, procedural approaches, and follow-up outcomes were analysed. Device implantation was performed via retrograde, antegrade, transseptal, or hybrid approaches under fluoroscopic and echocardiographic guidance. RESULTS:Fifty patients (54 devices) were included (median age: 48 months [interquartile range 12-96]; weight: 12 kg [interquartile range 7.5-23]), including 14 infants (9 < 7 kg). Indications were failure to thrive (46%), heart failure (28%), recurrent infections (12%), and postoperative residual ventricular septal defect (14%). The mean ventricular septal defect size was 5.8 ± 2 mm. Median fluoroscopy time was 18 minutes (range: 3-71). Residual shunts were present in 18% immediately, reducing to 9% at one week and resolving by three months. Mild, transient tricuspid regurgitation occurred in 14%. Over a median 9-month follow-up (range 1-60), no cases of heart block or haemolysis occurred. One embolisation required surgical retrieval. Pulmonary artery pressure decreased significantly (37 ± 13.4 to 19 ± 3.8 mmHg, p < 0.001). CONCLUSIONS:Transcatheter closure of muscular ventricular septal defects with the KONAR-MF™ occluder is safe, effective, and versatile across paediatric age groups, including infants and postoperative cases. High success rates, minimal complications, and favourable short- to mid-term outcomes support its use in routine practice.

Neonatal heart failure has varied causes, namely left-sided obstructive lesions such as aortic stenosis, hypoplastic left heart syndrome, and coarctation of the aorta, as well as conditions such as Ebstein's anomaly, supraventricular tachycardia, atrioventricular regurgitation, and large posttricuspid shunts. However, neonatal thyrotoxicosis is a rare but potentially fatal cause of heart failure if left untreated. We present two cases of neonatal thyroid storm manifesting as heart failure and cardiogenic shock, emphasizing the diagnostic challenges, treatment strategies, and transient nature of the disease, which is mediated by maternal thyroid-stimulating hormone receptor antibodies. A literature review is also provided to highlight the clinical approach to this rare but critical condition.

Transcatheter closure of perimembranous ventricular septal defect (PmVSD) is an established procedure. However, the occurrence of complete heart block limits its scope. The newer KONAR-MF (TM) occluder has specific design characteristics that may improve the safety of PmVSD closure. The objective of the study was to describe the efficacy and mid-term follow-up of transcatheter closure of PmVSD using KONAR-MF (TM). The study was conducted prospectively in 3 Indian centers (January 2018-December 2022). PmVSD closure was done by both antegrade and retrograde methods, and patients were followed up at 1, 3, 6, 12 months, and annually after that. 121 out of 123 patients were included with the following characteristics: median age 4.4 (0.18-40) years; weight 15 (2.1-88) kg; mean Qp/Qs ratio 1.87 +/- 0.52 and pulmonary artery mean pressure: 22 +/- 6.9 mmHg. The procedure was successful in all but 3; the device was removed due to significant residual shunt (n = 2) and new development of aortic regurgitation (AR) (>= mild) in 1. The median defect size was 5.2 (2.5-12) mm. Device sizes from 6/4 to 14/12 were deployed (median fluoroscopy time 13.3 min; range 3.6-47.8). Shunt occlusion rates were 90%-Immediate, 95%-pre-discharge, and 97%-1 month, with no instances of complete heart block after the procedure and during follow-up. Six had new onset AR (mild: 2, trivial 4), and one had increased tricuspid regurgitation. All patients were well during follow-up (median: 20 months; range: 6-46). The new KONAR-MF (TM) occluder appears to be a promising and safe alternative for the closure of the PmVSD; further long-term follow is merited.

ABSTRACT Background: Transcatheter patent ductus arteriosus (PDA) closure with unusual morphology associated with multiple constrictions or unusual circumstances such as interruption of inferior vena cava (IVC) is still challenging. This study evaluates the use of KONAR-MF™ (Lifetech Scientific Co Ltd., Shenzhen) for transcatheter closure of PDA in such situations. Materials and Methods and Results: Between January 2021 and October 2021, 24 patients from three different tertiary care centers underwent PDA device closure using the KONAR-MF™ device and are included in the study. Patient demographics, echocardiographic assessment, procedural details including the approach, device details, complications, and follow-up data were recorded. The median age and weight of the cohort were 2 years (0.5–41) and 10 kg (5–98), respectively. The indications for using KONAR-MF™ were (a) unusual morphology of PDA in 14, long tubular PDA in 8, and long tubular with multiple constrictions in 6 patients and (b) unusual circumstances such as interrupted IVC in 6 and inability to cross PDA antegradely in 4 due to dilated main pulmonary artery. The procedure was successful in all the patients and no procedure-related complications were noted in this cohort. Median follow-up of 9 months (range: 5–18) showed no residual shunt and only one patient had flow acceleration across the left pulmonary artery (peak Doppler gradient 16 mmHg). Conclusions: KONAR-MF™ device can be used safely and effectively for transcatheter closure of PDA with unusual anatomy or in unusual circumstances.

Background: The ductus venosus (DV) is a pivotal component of fetal circulation. Absent DV (ADV) is associated with structural defects, portal vein (PV) anomalies, and chromosomal anomalies. This observational study aims to investigate the impact of ADV on fetal circulation and postnatal outcomes. Materials and Methods: This observational study was conducted from August 2016 to January 2020 at a fetal and pediatric cardiac center. The DV was evaluated as part of routine fetal echocardiography. Cases of ADV were identified. Blood flow and exit points of the umbilical vein were studied. Cardiothoracic ratio, hydrops, and PV were assessed during the initial and follow-up scans. The postnatal evaluation included an ultrasound abdomen and computed tomography with triple-phase imaging to assess portosystemic shunts (PSSs). Serum ammonia levels were monitored. Results: Twelve patients with ADV were identified. The median maternal age and median gestational age were 27.5 years and 22 weeks, respectively. Four patients had intrahepatic drainage, while eight had extrahepatic drainage. All patients (100%) exhibited cardiomegaly, but none developed hydrops. Four patients had persistent PSS postnatally. All four patients with PSS had asymptomatic hyperammonemia. Two patients underwent transcatheter closure of PSS. The intrahepatic variant showed good PV anatomy with no evidence of PSS. Conclusions: DV evaluation should be performed during fetal echocardiography. ADV can lead to cardiomegaly and dilation of the right atrium and ventricle. PSS can be a potential sequela of the extrahepatic variant of ADV.

To assess the feasibility and outcome of stent strut dilation after arterial duct stenting with associated branch pulmonary artery (BPA) stenosis. Stenting of arterial duct in infants with duct-dependent pulmonary circulation is technically challenging. The presence of BPA stenosis is a relative contraindication for stent implantation. Infants with duct-dependent pulmonary circulation and associated BPA stenosis were assessed either by transthoracic echocardiogram alone or additional computerized tomography angiogram when required. Following ductal stenting, the stent struts of the stenosed BPA were crossed with an additional 0.014″ coronary guide wire and dilated using coronary balloons (2.0 or 2.5 mm in diameter). Seventeen (12 male) patients were considered for the procedure. The median age and weight were 27 days (range 2–94) and 2.6 kg (range 2.2–5), respectively. Fourteen patients (82.4

The prevalence of coronavirus disease-2019 (COVID-19) is about 2% in children. Varied manifestations including pneumonia, myocarditis, and multisystem inflammatory syndrome, namely is commonly seen in children. Aortic pseudoaneurysm due to COVID-19 is rare and carries a poor prognosis. We came across a 5-year-girl who had presented with heart failure due to a large ascending aortic pseudoaneurysm associated with COVID-19 during the first wave of the pandemic. The patient underwent successful surgical repair.

Background: Congenital portosystemic shunts (CPSS) are rare and present variably with hepatic encephalopathy, pulmonary arteriovenous malformations (PAVMs), and pulmonary hypertension (PH). Objective: The objective of the study was to see the feasibility of transcatheter closure of CPSS and their outcome. Materials and Methods: We analyzed the data of 24 patients of CPSS who underwent transcatheter closure from five institutions (March 2013 to April 2019). Baseline evaluation included echocardiography with bubble contrast study, ultrasound examination of the abdomen, computed tomography angiogram, and cardiac catheterization with test balloon occlusion of the CPSS. The evaluation showed cyanosis due to PAVM in 12, PH in 8, and respiratory distress in 2. Two had both cyanosis and PH. Criteria for eligibility for complete catheter closure of CPSS included demonstration of intrahepatic portal vein (PV) radicals together with a PV pressure of ≤18 mmHg on occlusion. Results: The median age and weight were 8 years (0.5–21) and 19.5 kg (4.2–73), respectively. Transcatheter closure was performed in 21 patients (22 procedures) using a variety of occlusive devices and stent-graft exclusion was done in one patient. Closure was not done in 3 in view of high portal venous pressures and hypoplastic PVs. During the follow-up (median: 42 months and range: 61 days–4.8 years), saturation normalized in 14 patients with PAVM. PH declined in all eight patients who underwent the procedure. Respiratory distress improved in two patients. Conclusions: Early and short-term follow-up results of catheter closure of CPSS appear promising. However, further, follow-up is needed to demonstrate long-term effectiveness.

We report a case of dilated cardiomyopathy because of chronic mercury poisoning. A 12-year-old girl was repeatedly admitted with severe congestive heart failure. She received indigenous medication for vitiligo for several months. A transthoracic echocardiogram showed severe ventricular dysfunction (left ventricle fractional shortening: 14%). She had a stormy course in the hospital, complicated by ventricular arrhythmias and multiorgan dysfunction. Careful interrogation and analysis of local medication and patient serum showed high levels of mercury. She was treated with British anti-Lewisite. She improved over the period of time, and her left ventricle fractional shortening improved to 22%.

Percutaneous transcatheter closure of fossa ovalis atrial septal defect (FOASD) is an established procedure. When femoral venous approach is unfeasible due to interrupted inferior vena cava (IVC) or occluded femoral veins, other interventional methods through transjugular, transhepatic approach, or surgical closure can be performed. We report two cases of transhepatic device closure of FOASD associated with IVC interruption successfully performed without any complications.

Earlier studies that investigated the relation of atrioventricular (AV) conduction system to perimembranous ventricular septal defect (pmVSD) were based on cardiopathological specimens. To study the relationship of conduction system to pmVSD using 3-dimensional electroanatomic mapping system (EAMS) in patients undergoing device closure. Fifteen consecutive cases of pmVSD from January 2014 to July 2017 (age > 2 years and weight > 8 kg) were included in the study. The course of conduction system and its relationship with the pmVSD was mapped before and after device closure, with the use of EAMS. Median age and weight of the cohort was 10 years (range 4–21 years) and 25 kg (range 13–55) respectively. Device implantation was successful in all patients except 1. The course and relation of the conduction system were posteroinferior to the pmVSD in all cases (100%), and away from the defect in 67% (10/15). In patient with baseline RBBB, the right-sided conduction system was in close proximity to the pmVSD. Two patients had part of left-sided conduction system in close proximity to pmVSD or device edges. Two patients developed RBBB following device deployment, which reverted to normal on follow up. No patient developed high grade AV block during the median follow-up of 34 months (range 24–62). This experimental study has shown the feasibility of 3D EAM of conduction system during device closure of pmVSD. This novel concept can be utilized to understand the anatomy of conduction system in other congenital heart diseases.

Background : Vascular rings (VRs) present with varied symptoms and may result in significant morbidity before an accurate diagnosis is made. Prenatal diagnosis may be useful to plan surgery after birth. Objectives : The purpose of the study was to see the feasibility of accurate diagnosis of VR during antenatal ultrasound examination and describe their outcome. Methods : This is a retrospective observational study between January 2014 and December 2019. Vascular rings were diagnosed on the basis of three vessel tracheal view and neck vessels arrangements on fetal echocardiogram. Postnatal evaluation by transthoracic echocardiography and computerized tomography angiogram was performed. Surgical repair was done as per standard indications. Results : A total of 35 cases of fetal VRs (median gestational age: 24 weeks [range: 19–35]) were diagnosed during the study period. There were four dichorionic diamniotic twin gestation pregnancies. The right aortic arch (RAA) with anomalous left subclavian artery (ALSA) was suspected in 31 fetuses, double aortic arch (DAA) in 3, and circumflex aorta in 1. Twenty-six (74%) patients had successful deliveries. One patient had a spontaneous miscarriage, 2 underwent termination, and 6 were lost to follow-up. Postnatal assessment showed RAA with ALSA in 18, DAA in 5, circumflex aorta in 2, and no abnormality in 1. Twenty-two (86%) were operated (RAA with ALSA: 17, DAA: 4, and circumflex aorta: 1) and four were waiting for surgery. Two patients died due to prematurity-related complications. All survivors are symptom free during follow-up (median: 2.24; range: 0.2–5.6 years). Conclusions : Fetal echocardiography enables prenatal diagnosis and planning of postnatal repair of VRs.

We report a successful transcatheter exclusion of supra-celiac saccular aneurysm using duct occluder. Stent graft or covered stent was not considered in view of calcified irregular aorta with mild luminal narrowing. Duct occluder was positioned in reverse direction using bioptome. Follow-up assessment by CT angiogram showed complete exclusion of the aortic aneurysm.

Background : COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID-19 on pediatric cardiac care in India. Aims : The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design : This is a retrospective, multicentric, observational study. Methods : We collected monthly data on the number and characteristics of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries and major hospital statistics, over a period of 5 months (April to August 2020), which coincided with the first wave of COVID-19 pandemic in India and compared it with data from the corresponding months in 2019. Results : The outpatient visits across the 24 participating pediatric cardiac centers decreased by 74.5% in 2020 (n = 13,878) as compared to the corresponding period in 2019 (n = 54,213). The reduction in the number of hospitalizations, cardiac surgeries, and catheterization procedures was 66.8%, 73.0%, and 74.3%, respectively. The reduction in hospitalization was relatively less pronounced among neonates as compared to infants/children (47.6% vs. 70.1% reduction) and for emergency surgeries as compared to elective indications (27.8% vs. 79.2%). The overall in-hospital mortality was higher in 2020 (8.1%) as compared to 2019 (4.8%), with a higher postoperative mortality (9.1% vs. 4.3%). Conclusions : The current COVID-19 pandemic significantly impacted the delivery of pediatric cardiac care across India with two-third reduction in hospitalizations and cardiac surgeries. In an already resource-constrained environment, the impact of such a massive reduction in the number of surgeries could be significant over the coming years. These findings may prove useful in formulating strategy to manage subsequent waves of ongoing COVID-19 pandemic.

Embryologically, the common arterial trunk (CAT) is due to the failure of septation by the major outflow cushions. This invariably leads to the presence of an associated large juxta-arterial ventricular septal defect. Overriding of the ventricular septum leads to its biventricular origin in more than 2/3rd of cases. Uncommonly, CAT may be associated with an intact ventricular septum (IVS) and univentricular origin. We describe an infant with CAT, intact IVS, hypoplastic right ventricle, and Ebstein's anomaly of the tricuspid valve who presented to us and palliated successfully. The embryological mechanisms are discussed in this report.

Shock is a state of circulatory dysfunction and its diagnosis is complex in neonates. Hemodynamic assessment using echocardiography has potential to guide better management regimes in neonates with shock. Objective of this study is to analyze changes in the echocardiographic parameters in preterm neonates with shock at presentation and after resolution. In this prospective pragmatic Cohort study, eligible neonates with shock were monitored for changes in echocardiographic parameters at onset of shock and after resolution of shock. Paired data analysis was done for observed changes in the parameters. Based on initial clinical parameters and echocardiographic parameters, infants were assigned into different types of shock. Data of 37 infants were analyzed for baseline clinical and echocardiographic parameters, and data of 31 infants were analyzed for the changes in the observed parameters after shock resolution. Statistically significant changes were observed in inferior vena cava collapsibility index (ICI), left ventricular end diastolic volume (LVEDV), isovolemic ventricular relaxation time (IVRT), left and right ventricular stroke volume, and ejection fraction (EF). There was no agreement between clinical and echocardiographic definitions of shock. Conclusion: We noticed shock has overlapping pathophysiologic features. Our study highlights the importance of baseline documentation of echocardiographic parameters of all infants who are at risk of shock and repeat echocardiography at onset of shock to observe the changes in ICI, LVEDV, IVRT, stroke volume, and EF. This would guide pathophysiological management of shock in neonates.