Novel Peri-Operative Strategy for Sickle Cell Disease with Tetralogy of Fallot.

ABSTRACT: Peri-operative management of cyanotic congenital heart disease in a patient of sickle cell disease (SCD) can be challenging. We report a case of Tetralogy of Fallot and homozygous SCD with history of multiple blood transfusions and sickle cell crises who underwent intracardiac repair. Hemoglobin S level was reduced from 75% pre-operative to 21.8% postoperative with a combination of pre-operative blood transfusion, intraoperative exchange transfusion, and normothermic cardiopulmonary bypass (CPB). Pre-operative optimization and safe intraoperative conduct were essential to avoid sickling crises.

Aortic Uncrossing for Circumflex Aorta.

Circumflex aorta is described as a retroesophageal aortic arch, with opposite-sided descending aorta forming a true vascular ring with ligamentum arteriosum. We report two cases of right-sided circumflex aorta with varied clinical presentation. Computed tomography diagnosed this vascular ring anomaly. The patients were managed with the aortic uncrossing procedure as a primary surgical strategy. Preoperative diagnosis and thorough planning were essential for the successful outcome. The patients did not have any residual symptoms of tracheal and esophageal compression on follow up.

COVID-19 international experience in paediatric patients with congenital heart disease.

OBJECTIVE: As COVID-19 continues to affect the global population, it is crucial to study the impact of the disease in vulnerable populations. This study of a diverse, international cohort aims to provide timely, experiential data on the course of disease in paediatric patients with congenital heart disease (CHD). METHODS: Data were collected by capitalising on two pre-existing CHD registries, the International Quality Improvement Collaborative for Congenital Heart Disease: Improving Care in Low- and Middle-Income Countries and the Congenital Cardiac Catheterization Project on Outcomes. 35 participating sites reported data for all patients under 18 years of age with diagnosed CHD and known COVID-19 illness during 2020 identified at their institution. Patients were classified as low, moderate or high risk for moderate or severe COVID-19 illness based on patient anatomy, physiology and genetic syndrome using current published guidelines. Association of risk factors with hospitalisation and intensive care unit (ICU) level care were assessed. RESULTS: The study included 339 COVID-19 cases in paediatric patients with CHD from 35 sites worldwide. Of these cases, 84 patients (25%) required hospitalisation, and 40 (12%) required ICU care. Age <1 year, recent cardiac intervention, anatomical complexity, clinical cardiac status and overall risk were all significantly associated with need for hospitalisation and ICU admission. A multivariable model for ICU admission including clinical cardiac status and recent cardiac intervention produced a c-statistic of 0.86. CONCLUSIONS: These observational data suggest risk factors for hospitalisation related to COVID-19 in paediatric CHD include age, lower functional cardiac status and recent cardiac interventions. There is a need for further data to identify factors relevant to the care of patients with CHD who contract COVID-19 illness.

Pediatric cardiac surgery following severe acute respiratory syndrome coronavirus-2 infection: Early experience and lessons learnt.

Background: We evaluated our early experience of cardiac procedures in children with congenital heart defects (CHD) after asymptomatic severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, to understand whether recent reverse transcription-polymerase chain reaction (RT-PCR) positivity had a negative impact on their postprocedural recovery and outcomes. Materials and Methods: In this retrospective observational study, all patients with CHD who underwent cardiac surgery or transcatheter intervention at our institution between March 2020 and June 2021 who were detected to have asymptomatic SARS-CoV-2 infection on routine RT-PCR were included. Details of the cardiac procedure and postprocedural recovery were reviewed and compared with RT-PCR-negative patients who concurrently underwent similar cardiac surgeries or interventions at our center. Results: Thirteen patients underwent cardiac surgery after recent SARS-CoV-2 positivity after a mean interval of 25.4 ± 12.9 days. One patient expired with multiorgan dysfunction and systemic inflammatory response with elevated D-dimer, serum Ferritin, C-reactive protein, and significant ground-glass opacities on chest radiograph. Another patient developed spontaneous thrombosis of the infrarenal abdominal aorta, bilateral iliac arteries, and bilateral femoral veins, requiring low-molecular weight heparin postoperatively. This patient's postoperative recovery was also prolonged due to lung changes delaying extubation. All other patients had uneventful postprocedural recovery with intensive care unit and hospital stays comparable to non-SARS-CoV-2-infected patients. Conclusions: From our early experience, we can surmise that an interval of 2-3 weeks after asymptomatic SARS-CoV-2 infection is adequate to undertake elective or semi-elective pediatric cardiac surgeries. For patients requiring emergent cardiac surgery prior to this interval, there is potentially increased risk of inflammatory and/or thrombotic complications.

Hybrid stenting of the arterial duct with carotid cutdown and flip technique: immediate and early results.

Stenting of the arterial duct (PDA) has become a standard palliation for ductal-dependent pulmonary circulation. Carotid arterial access provides a direct route for stenting vertical ducts. We evaluated our early results of hybrid ductal stenting via surgical carotid cutdown. METHODS AND RESULTS: In this retrospective single centre cohort study, hybrid PDA stenting was attempted in 11 patients with "flip technique", between January 2020 and February 2021, and was successful in 10. Median age was 29 days (interquartile range 17.5-87) and mean weight 3.37 ± 1.23 kg. Mean fluoroscopy time was 13.58 ± 5.35 minutes, mean procedure time was 48.50 ± 22.5 minutes, and mean radiation dose was 1719.5 ± 1217.6 mGycm2. Mean time for cutdown was 9.9 ± 2.4 minutes and for haemostasis and suturing was 25.3 ± 11.0 minutes. Median duration of ventilation post-stenting was 26 hours (interquartile range 21-43.75). The median ICU stay post-procedure was 5 days (interquartile range 4-7.25) and mean hospital stay was 12 ± 6.3 days. On early follow-up, carotid patency was confirmed in all patients with colour Doppler, with no intravascular thrombi, narrowing, haematomas, or aneurysms noted. There were no complications secondary to vascular access. There was one early mortality, 27 days post-stenting, which was unrelated to the procedure. CONCLUSION: This study adds to the limited literature on ductal stenting with carotid access and the flip technique. In our early experience, the hybrid carotid approach is an attractive alternative to percutaneous carotid puncture and has simplified a complex and challenging intervention, with good outcomes.

Use of cribriform amplatzer septal occluder in the pediatric population: Feasibility, safety, and technical considerations.

PURPOSE: Fenestrated atrial septal defects (F-ASDs) in the pediatric population pose a challenge for transcatheter device closure since multiple devices are not preferred in small hearts. Oversizing the Amplatzer Septal Occluder (ASO) to cover the surrounding fenestrations usually distorts the central waist as well as the disc profile and often defeats the purpose. This is a retrospective observational study with an aim to assess the feasibility and safety of cribriform ASO in closing F-ASDs in small children. METHODS: Sixteen children with F-ASD who underwent device closure with cribriform ASO were included in the study. The fenestrated septal length (FSL) and the total septal length (TSL) were measured on transesophageal echocardiogram. A device size which was 1.5-2 times the FSL but smaller than the TSL was selected. The defect was closed with a device passed through a relatively centrally placed smaller fenestration. RESULTS: The median age of the cohort was 5 years (2.5-10.5). Majority (14/16) required 25 or 30 mm cribriform ASO. Aneurysmal interatrial septum was seen in most of our patients (11/15). All the patients had successful device implantation. Complete closure of the defect was seen in 11 patients while 5 patients had insignificant residual shunt at a median follow-up of 40 months (1-60 months). There were no other complications. CONCLUSIONS: Cribriform ASO can be used safely and effectively in closing F-ASDs in children. Deployment of the device through a small central hole allows covering maximum fenestrations and gives more stability to the device. Residual shunts, although not infrequent, are insignificant.

Multisystem Inflammatory Syndrome in Children With COVID-19 in Mumbai, India.

OBJECTIVE: We describe the presentation, treatment and outcome of children with multisystem inflammatory syndrome with COVID-19 (MIS-C) in Mumbai metropolitan area in India. METHOD: This is an observational study conducted at four tertiary hospitals in Mumbai. Parameters including demographics, symptomatology, laboratory markers, medications and outcome were obtained from patient hospital records and analyzed in patients treated for MIS-C (as per WHO criteria) from 1 May, 2020 to 15 July, 2020. RESULTS: 23 patients (11 males) with median (range) age of 7.2 (0.8-14) years were included. COVID-19 RT-PCR or antibody was positive in 39.1% and 30.4%, respectively; 34.8% had a positive contact. 65% patients presented in shock; these children had a higher age (P=0.05), and significantly higher incidence of myocarditis with elevated troponin, NT pro BNP and left ventri-cular dysfunction, along with significant neutrophilia and lympho-penia, as compared to those without shock. Coronary artery dilation was seen in 26% patients overall. Steroids were used most commonly for treatment (96%), usually along with intra-venous immunoglobulin (IVIg) (65%). Outcome was good with only one death. CONCLUSION: Initial data on MIS-C from India is presented. Further studies and longer surveillance of patients with MIS-C are required to improve our diagnostic, treatment and surveillance criteria.

Transcatheter closure of elongated and pulmonary hypertensive patent arterial duct in infants using Amplatzer vascular plug II.

OBJECTIVE: To evaluate the feasibility, efficacy, and safety of Amplatzer vascular plug II in large and elongated ducts in infants. INTRODUCTION: Patent arterial duct device closure is technically challenging in infants with large and elongated ducts because Amplatzer duct occluder and Amplatzer duct occluder II have high chances of causing aortic coarctation and left pulmonary artery stenosis, respectively. The Amplatzer vascular plug II being soft with no retention discs on either sides helps in mitigating these problems. METHOD: This is a prospective, observational study involving infants with clinical, echocardiographic and angiographic evidence of large left to right shunt. All the children underwent duct closure using Amplatzer vascular plug II. RESULTS: Eighteen infants qualified for the study. Mean age and weight were 8.63 ± 3.84 months and 6.3 ± 1.7 kg, respectively. The angiographic mean duct diameter at the pulmonary artery end was 4.66 ± 0.92 mm, and the mean duct length was 9.4 ± 2.48 mm. The size of Amplatzer vascular plug II used varied from 6 mm to 10 mm. Technical success was achieved in 16/18 cases. One patient had device embolisation, and in the other, the device was found to be unstable. The ratio of Amplatzer vascular plug II size to the duct diameter was 1.65 ± 0.27, while the ratio of ductal length to device length was 1.48 ± 0.46 in those with successful outcome. CONCLUSIONS: Amplatzer vascular plug II is a safe and effective option in appropriately selected infants with elongated ducts. Diameter and length of Amplatzer vascular plug II vis-a-vis those of the ductus are important determinants of the successful outcome.

Embolisation of ceramic-coated PDA devices into the descending thoracic aorta: probable mechanisms and retrieval strategies.

We describe two cases of spontaneous embolisation and successful retrieval of ceramic-coated patent arterial duct devices. In both, the device embolised to the descending aorta in the absence of pulmonary hypertension and despite optimum placement. We have discussed possible mechanisms for embolisation in these patients and suggested alternative methods for device retrieval. Based on this limited experience, we conclude that for tubular ducts, ceramic-coated devices should be oversized to form a tighter waist or alternate devices may be considered.

Bacillus Clausii Septicemia in a Pediatric Patient After Treatment With Probiotics.

Isolated cases of septicemia associated with probiotics have been reported. We describe Bacillus clausii septicemia in a 4-month-old male infant with congenital heart disease following treatment for diarrhea with a probiotic containing B. clausii spores. The septicemia proved refractory to treatment with antibiotics active against the pathogen and progressed to multidrug-resistant sepsis with multiorgan failure.

Native aortic coarctation in neonates and infants: Immediate and midterm outcomes with balloon angioplasty and surgery.

BACKGROUND: Native aortic coarctation in young infants can be treated surgically or with balloon angioplasty, although the latter approach remains controversial. We sought to compare midterm outcomes of balloon and surgical coarctoplasty at our center and to review the current practices in literature. METHODS: The study design was a retrospective record review. Patients with coarctation aged <1 year who underwent balloon or surgical coarctoplasty at our center (January 2010-January 2016) with >6-month post-procedure follow-up were studied. Patients with significant arch hypoplasia or interruption, long-segment coarctation, and associated intracardiac lesions were excluded from the study. Clinical, echocardiographic, and procedural details were recorded at initial presentation and follow-up. Need for reintervention was noted. RESULTS: Out of a total of 75 patients, 28 underwent balloon (Group A) and 47 underwent surgical coarctoplasty (Group B). There were two deaths in Group B (mortality 4.2%) and none in Group A. In neonates, 63.6% of Group A and 17.4% of Group B patients required reintervention (P = 0.007). Beyond the neonatal age, 29.4% of Group A and 41.6% of Group B patients required reintervention (not statistically significant). On univariate analysis, residual coarctation on predischarge echocardiogram was the only significant predictor of reintervention (P = 0.04). On Kaplan-Meier analysis, with neonatal presentation, freedom from reintervention in Group B was significantly higher than Group A (P = 0.028). CONCLUSION: In neonates with native aortic coarctation, surgery unequivocally yields better immediate and midterm results and is the preferred treatment modality. In older infants, balloon coarctoplasty has good early and midterm outcomes with acceptable reintervention rates and should be considered a viable alternative to surgery, especially in critical patients with high surgical risk.

Isolated Subclavian Artery: A Rare Entity Revisited.

Isolation of the subclavian artery is a rare aortic arch anomaly with fewer than 100 cases reported in the literature. Left subclavian artery isolation is seen in 0.8% of right aortic arches, and right subclavian artery isolation is seen four times less frequently. Other intracardiac or arch malformations are usually associated, among which tetralogy of Fallot is commonly described. We describe the presentation, preoperative imaging, surgical strategies, and outcomes of four patients with this rare condition and review the published literature. The embryological basis and interesting pathophysiology are also discussed. Surgical division and reimplantation of the isolated subclavian artery onto the aortic arch or common carotid artery is the treatment of choice, with good immediate and midterm outcomes.

Rare associations of tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection.

We describe the cases of two patients with tetralogy of Fallot, aged 4 years and 8 months, who were incidentally detected to have concomitant anomalous left coronary artery from pulmonary artery and total anomalous pulmonary venous connection, respectively, on preoperative imaging. They underwent surgical correction with good mid-term outcomes. In this study, we discuss the embryological basis, physiological effects, and review the literature of these two unusual associations. Awareness of these rare associations will avoid missed diagnoses and consequent surgical surprises.

Partial anomalous left pulmonary artery: report of two cases and review of literature.

We describe two cases of anomalous origin of the left lower-lobe pulmonary artery from the right pulmonary artery. The primary diagnosis was mitral atresia, hypoplastic left ventricle, aortic arch hypoplasia in the first child, and tetralogy of Fallot in the second. In both cases, the pulmonary trunk gave rise to a left pulmonary artery in the normal position. In addition, a second branch of the left pulmonary artery arose from the right pulmonary artery, and passed posterior and inferior to the left main or upper-lobe bronchus to supply the left lower lobe. In this review, we compare our findings with previously reported examples of this extremely rare cardiac malformation, and discuss possible embryological explanations for the lesion.

Functional capacity following univentricular repair--midterm outcome.

OBJECTIVE: Previous studies have seldom compared functional capacity in children following Fontan procedure alongside those with Glenn operation as destination therapy. We hypothesized that Fontan circulation enables better midterm submaximal exercise capacity as compared to Glenn physiology and evaluated this using the 6-minute walk test. DESIGN AND PATIENTS: Fifty-seven children aged 5-18 years with Glenn (44) or Fontan (13) operations were evaluated with standard 6-minute walk protocols. RESULTS: Baseline SpO(2) was significantly lower in Glenn patients younger than 10 years compared to Fontan counterparts and similar in the two groups in older children. Postexercise SpO(2) fell significantly in Glenn patients compared to the Fontan group. There was no statistically significant difference in baseline, postexercise, or postrecovery heart rates (HRs), or 6-minute walk distances in the two groups. Multiple regression analysis revealed lower resting HR, higher resting SpO(2) , and younger age at latest operation to be significant determinants of longer 6-minute walk distance. Multiple regression analysis also established that younger age at operation, higher resting SpO(2) , Fontan operation, lower resting HR, and lower postexercise HR were significant determinants of higher postexercise SpO(2) . Younger age at operation and exercise, lower resting HR and postexercise HR, higher resting SpO(2) and postexercise SpO(2) , and dominant ventricular morphology being left ventricular or indeterminate/mixed had significant association with better 6-minute work on multiple regression analysis. Lower resting HR had linear association with longer 6-minute walk distances in the Glenn patients. CONCLUSIONS: Compared to Glenn physiology, Fontan operation did not have better submaximal exercise capacity assessed by walk distance or work on multiple regression analysis. Lower resting HR, higher resting SpO(2) , and younger age at operation were factors uniformly associated with better submaximal exercise capacity.

Device occlusion of pseudoaneurysm of ascending aorta.

Pseudoaneurysm of ascending aorta is an infrequent but well-recognized and potentially fatal complication after cardiac surgeries. The complication can develop early, delayed or late, and the presentation is also varied. We are presenting here two cases of pseudoaneurysm of ascending aorta following cardiac surgery that were successfully managed by the transcatheter method. The first one occurred following coronary artery bypass surgery and the second one occurred following double-valve replacement surgery. The aortic openings of these aneurysms were occluded with 12 mm and 10 mm atrial septal occluders, respectively, with a good outcome. An immediate postprocedure angiogram showed no residual flow into the sac. Six months of follow-up of both cases also showed excellent results.

Analysis of genotype and haplotype effects of ABCB1 (MDR1) polymorphisms in the risk of medically refractory epilepsy in an Indian population.

The transmembrane P-glycoprotein that functions as a drug-efflux transporter coded by ATP-binding cassette, subfamily B, member 1/Multidrug Resistance 1 (ABCB1/MDR1) gene is considered relevant to drug absorption and elimination, with access to the central nervous system. Effects of three ABCB1 single nucleotide polymorphisms (SNPs) in genotypic and haplotypic combination have been evaluated in a south Indian population for risk of pediatric medically refractory epilepsy. The study included age and sex matched medically refractory (N=113) cases and drug responsive epilepsy patients (N=129) as controls, belonging to the same ethnic population recruited from a tertiary referral centre, of Karnataka, Southern India. The genotype frequencies of SNPs c.1236C>T, c.2677G>T/A, and c.3435C>T were determined from genomic DNA of the cases and controls by PCR- RFLP and confirmatory DNA sequencing. 256 normal population samples of the same ethnicity were genotyped for the three loci to check for population stratification. Results indicate that there was no statistically significant difference between allele and genotype frequencies of refractory and drug responsive epilepsy patients. The predicted haplotype frequencies of the three polymorphisms did not show significant difference between cases and controls. The results confirm earlier observations on absence of association of ABCB1 polymorphisms with medically refractory epilepsy.

Subdural effusion complicating neonatal meningitis: successful treatment with acetazolamide.

Postmeningitis subdural effusion is rare in neonates when compared to infants and children. For treatment, various modalities are described. Serial subdural punctures and surgical drain placement are advised for cases having a mass effect on imaging. We report a neonate with symptomatic postmeningitis subdural effusion, who failed to respond to serial subdural punctures, but subsequently managed successfully with acetazolamide. He had no recurrence further. His development was normal at 18 months of age.

Transcatheter device closure of ruptured sinus of Valsalva: Immediate results and short term follow up.

This is a retrospective, observational study comprising of eight patients with isolated rupture of the sinus of Valsalva (RSOV) who underwent transcatheter device closure. The mean age of presentation was 32.8 +/- 10.0 years. New York Heart Association (NYHA) class at the time of presentation was II (six patients) and III (two patients). The RSOVs were all closed using a patent ductus arteriosus device. The mean procedural time was 42.3 +/- 5.4 minutes, while the fluoroscopic time was 24.5 +/- 6.9 minutes. All had complete closure of the shunt. The average hospital stay was 2.9 +/- 1.1 days. There were no major complications. The patients were followed up for a mean of 11.3 +/- 4.1 months. At the time of the last follow up all the patients were in NYHA class I. We conclude that in the short term, transcatheter closure of isolated RSOV is a viable alternative to surgical repair.