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Mechanical thrombectomy has been described as the prompt procedure for emergent large vessel occlusion strokes resulting from cardioembolic events, arterial lesion-to-artery embolism, or from in situ atherosclerosis. This is the first reported case of an elderly woman who presented with bilateral paresis, progressive drowsiness, and dysarthria due to bilateral middle cerebral arterial occlusion, originating from the aortic valve, after transcatheter aortic valve implantation, leading to stroke. Despite intravenous thrombolysis being begun for this patient, it proved ineffective and she underwent mechanical thrombectomy, which led to complete revascularization of bilateral middle cerebral arteries following which she completely recovered without any deficits.

Rheumatic heart disease (RHD) continues to affect developing countries with low income due to the lack of resources and effective diagnostic techniques. Understanding the genetic basis common to both the diseases and that of progression from its prequel disease state, Acute Rheumatic Fever (ARF), would aid in developing predictive biomarkers and improving patient care. To gain system-wide molecular insights into possible causes for progression, in this pilot study, we collected blood transcriptomes from ARF (5) and RHD (5) patients. Using an integrated transcriptome and network analysis approach, we identified a subnetwork comprising the most significantly differentially expressed genes and most perturbed pathways in RHD compared to ARF. For example, the chemokine signaling pathway was seen to be upregulated, while tryptophan metabolism was found to be downregulated in RHD. The subnetworks of variation between the two conditions provide unbiased molecular-level insights into the host processes that may be linked with the progression of ARF to RHD, which has the potential to inform future diagnostics and therapeutic strategies. We also found a significantly raised neutrophil/lymphocyte ratio in both ARF and RHD cohorts. Activated neutrophils and inhibited Natural Killer cell gene signatures reflected the drivers of the inflammatory process typical to both disease conditions.

Behcet’s disease is a multisystemic vasculitis. It can affect the pulmonary artery in 2% to 5% cases. We discuss a case of a young male diagnosed with Behcet's disease on immunosuppressive therapy who presented with bilateral pulmonary artery aneurysms which were closed with covered stent and other devices.

Justification: A number of guidelines are available for management of congenital heart diseases 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 congenital heart diseases, as often these patients present late in the course of the disease and may have co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National Consensus Meeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of Medical Sciences, New Delhi. The meeting was supported by Children's HeartLink, a non-governmental organization based in Minnesota, USA. Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heart diseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases. Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenital heart diseases, 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 congenital heart diseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebstein anomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise. [ABSTRACT FROM AUTHOR]

The images depict a rare case of Scimitar syndrome involving the left lower pulmonary vein., Competing Interests: There are no conflicts of interest., (Copyright: © 2024 Annals of Pediatric Cardiology.)

Background: Budd - Chiari syndrome (BCS) due to hepatic venous outflow obstruction is a rare cause of liver disease with dismal outcome, often amenable to catheter intervention., Materials and Methods: This retrospective single-center study analyzed the clinical profile and medium-term outcome of interventional treatment with balloon angioplasty ± stenting in all pediatric BCS over a 10-year period. Clinical, laboratory, imaging, and interventional data were retrieved. Transhepatic (TH) access was utilized in the recent 3 years., Results: We included a total of 27 patients. Acute and subacute BCS comprised 93% of subjects. Ascites was the most common symptom. COVID-19 infection and Takayasu arteritis were two novel etiologies in our study. There was isolated hepatic vein (HV) narrowing in 11 (41%), isolated inferior vena cava obstruction in 4, and combined occlusion in 12 (44%). Intervention was successful in 22 (82%) patients. Stenting was required in 14 (64%) patients and the rest underwent balloon angioplasty. The immediate outcome was better with stenting than balloon (91% vs. 64%). Transhepatic access in 6 patients allowed HV cannulation in all and achieved patency in five patients. Two patients from the balloon group (25%) and 9 from the stent group (64%) are alive with patent veins at a median follow-up of 60 months, indicating a high attrition rate., Conclusion: Catheter interventions restored physiological blood flow in pediatric BCS. TH route improved cannulation of occluded HV compared to other accesses. Immediate and medium-term outcomes were better after stenting with lower rates of reinterventions than balloon angioplasty. Life-long surveillance is required as mortality is high on follow-up., Competing Interests: There are no conflicts of interest., (Copyright: © 2023 Annals of Pediatric Cardiology.)

Background: Data on congenital systemic arteriovenous fistulas are largely based on individual case reports. A true systemic arteriovenous fistula needs to be differentiated from other vascular malformations like capillary or venous hemangiomas, which are far more common., Objectives: We sought to identify the varied symptoms, diagnostic challenges, describe interventional treatment options, and postulate an embryological basis for this uncommonly described entity., Methods: This is a descriptive study of a cohort of systemic arteriovenous fistulas seen in the department of pediatric cardiology at a tertiary cardiac institute from 2010 to 2020, with prospective medium-term follow-up. A total of seven cases were identified. The diagnosis was confirmed by computed tomographic imaging, magnetic resonance angiography, or conventional angiography., Results: All were successfully closed using duct occluders or embolization coils with no recurrence in six cases over a median duration of follow-up of 48 months (interquartile range: 16; 36-52 months). Four of the seven cases underwent follow-up imaging using echocardiography or ultrasound., Conclusion: The incidence of congenital systemic arteriovenous fistulas is low and accounted for 0.009% of pediatric outpatients seen over 10 years at our institute. The spectrum of clinical presentation varies from an innocuous swelling or a pulsating mass to frank heart failure. Strong clinical suspicion and advanced imaging modalities have helped identify some hitherto undescribed connections. Large malformations with multiple communications may persist or recur despite transcatheter closure., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Annals of Pediatric Cardiology.)

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., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Annals of Pediatric Cardiology.)

Background: Outcome data of children with heart disease who acquired COVID-19 infection are limited., Aims: We sought to analyze outcome data and identify risk factors associated with mortality in children with heart disease and grown-ups with congenital heart disease (GUCH) who had a laboratory-confirmed COVID-19 infection., Settings and Design: This is a retrospective, multicentric, observational study., Materials and Methods: The study included children with heart disease and GUCH population, who presented with either symptomatic or asymptomatic COVID-19 infection to any of the participating centers. COVID-19-negative patients admitted to these centers constituted the control group., Results: From 24 pediatric cardiac centers across India, we included 94 patients with a median age of 12.5 (interquartile range 3-96) months and 49 (52.1%) patients were males. Majority (83 patients, 88.3%) were children. One-third of the patients ( n = 31, 33.0%) had acyanotic congenital heart disease, and 41.5% ( n = 39) were cyanotic, with > 80% of the patients being unoperated. Only 30 (31.9%) patients were symptomatic for COVID-19 infection, while the rest were incidentally detected positive on screening. A total of 13 patients died (case fatality rate: 13.8%). The in-hospital mortality rate among hospitalized patients was significantly higher among COVID-19-positive cases (13 of 48; 27.1%) as compared to COVID-negative admissions (9.2%) during the study period ( P < 0.001). On multivariate analysis, the independent predictors of mortality among COVID-19-positive cases were severity of illness at admission (odds ratio [OR]: 535.7, 95% confidence interval [CI]: 6.9-41,605, P = 0.005) and lower socioeconomic class (OR: 29.5, 95% CI: 1.1-814.7, P = 0.046)., Conclusions: Children with heart disease are at a higher risk of death when they acquire COVID-19 infection. Systematic preventive measures and management strategies are needed for improving the outcomes., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Pediatric Cardiology.)

Background: Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI., Methods: Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed., Results: Seven patients aged 17-60 years (median 26 years) had stenosed RV-PA conduits implanted 5-17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50-137 mmHg) to 12.7 ± 6.4 mmHg (5-20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10-22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events., Conclusions: Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Pediatric Cardiology.)

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., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Annals of Pediatric Cardiology.)

Objective: We sought to evaluate the feasibility, technical aspects, and outcome of transcatheter perimembranous ventricular septal defect (pmVSD) closure using duct occluder devices with a single retention disc., Background: Use of duct occluder devices to close pmVSD seems a promising alternative therapy. However, limited data exist on this technique., Methods: From 2010 to 2016, 222 patients (female 47.7%) were identified from databases of five participating institutions in whom pmVSD closure was attempted using an Amplatzer Duct Occluder I or Lifetech duct occluder device., Results: Patients ranged in age from 0.7 to 52 years (median, 7.0 years) and in weight from 4.0 to 70 kg (median, 18.0 kg). The mean size of the VSD was 6.8 ± 2.2 mm. A large defect (> 6 mm) was present in 137 patients (61.7%). Device closure was successful in 218 patients (98.2%). The 10/8 mm device was used in most patients (n = 85, 38.3%), and the vascular approach was from the femoral vein in 169 patients (76.1%). There were 18 early complications in 17/218 patients (7.8%). Three patients (1.4%) developed complete heart block (transient n = 2; requiring permanent pacing n = 1). Median follow-up was 6 months (6 months-6 years). A mild residual shunt was seen in 10 patients at 6 months follow-up., Conclusions: The immediate results of transcatheter pmVSD closure using a duct occluder device with a single retention disc are promising. It is an effective technique with a lower rate of complications than for other currently available devices., (© 2018 Wiley Periodicals, Inc.)