Incremental value of fetal spatiotemporal image correlation echocardiography in the diagnosis of tetralogy of Fallot with disconnected pulmonary arteries with ductus arteriosus supplying the left pulmonary artery.

Disconnected branch pulmonary arteries are sparsely reported cases in prenatal diagnosis literature. We report a case of tetralogy of Fallot with disconnected branch pulmonary arteries, the left pulmonary artery (LPA) arising from an indirect ductus arteriosus (DA) from the base of the innominate artery in a right aortic arch, diagnosed by fetal echocardiography with 3D/4D spatiotemporal image correlation (STIC) imaging. Prenatal diagnosis led to early neonatal intervention to maintain blood flow to the LPA by stenting of the DA. Fetal echocardiographic evaluation (Voluson E10 systems, GE Healthcare, Zipf) with acquisition of images and volumes in the right ventricular outflow tract and three-vessel trachea view with rendering of 3D/4D STIC volume datasets to display images in high-definition color format. Prenatal evaluation was initially done at 17-week gestation in a 28-year-old pregnant female which showed tetralogy of Fallot (TOF). Subsequent evaluation at 34 weeks with 3D/4D STIC datasets showed a small main pulmonary artery (MPA) continuing into an adequately sized right pulmonary artery. The LPA was very small (Z-score -2.63), with no visible connection to MPA. Rendering of the 3D/4D STIC datasets revealed disconnected pulmonary arteries with the vertical DA from the base of the innominate artery in a right aortic arch, continuing as the LPA. Findings were confirmed on postnatal high-resolution CT pulmonary angiography and cardiac catheterization with subsequent stenting of the ductus. This report highlights the incremental benefit of advanced 3D/4D STIC rendering in accurate prenatal diagnosis of a rare anomaly of TOF with disconnected pulmonary arteries, leading to early neonatal intervention to preserve the blood supply to the left lung.

Transcatheter closure of congenital portosystemic shunts - A multicenter experience.

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.

COVID-19 related multisystem inflammatory syndrome in children (MIS-C): a hospital-based prospective cohort study from Kerala, India.

Objectives: To study (1) epidemiological factors, clinical profile and outcomes of COVID-19 related multisystem inflammatory syndrome in children (MIS-C), (2) clinical profile across age groups, (3) medium-term outcomes and (4) parameters associated with disease severity. Design: Hospital-based prospective cohort study. Setting: Two tertiary care centres in Kerala, India. Participants: Diagnosed patients of MIS-C using the case definition of Centres for Disease Control and Prevention. Statistical analysis: Pearson χ2 test or Fisher's exact test was used to compare the categorical variables and independent sample t-test or Mann-Whitney test was used to compare the continuous variables between the subgroups categorised by the requirement of mechanical ventilation. Bonferroni's correction was used for multiple comparisons. Results: We report 41 patients with MIS-C, mean age was 6.2 (4.0) years, and 33 (80%) were previously healthy. Echocardiogram was abnormal in 23 (56%), and coronary abnormalities were noted in 15 (37%) patients. Immunomodulatory therapy was administered to 39 (95%), steroids and IVIg both were used in 35 (85%) and only steroids in 3 (7%) patients. Intensive care was required in 36 (88%), mechanical ventilation in 8 (20%), inotropic support in 21 (51%), and 2 (5%) patients died. Mechanical ventilation requirement in MIS-C was associated with hyperferritinaemia (p=0.001). Thirty-seven patients completed 3 months follow-up by April 2021, of whom 6 (16%) patients had some residual echocardiographic changes. Conclusions: Patients with MIS-C in our cohort had varied clinical manifestations ranging from fever with mild gastrointestinal and mucocutaneous involvement to fatal multiorgan dysfunction. Immediate and medium-term outcomes remain largely excellent except for the echocardiographic sequelae in a few patients which are also showing a resolving trend. Hyperferritinaemia was associated with the requirement of mechanical ventilation.

Impact of COVID-19 pandemic on pediatric cardiac services in India.

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.

Outcomes of Venus P-valve for dysfunctional right ventricular outflow tracts from Indian Venus P-valve database.

BACKGROUND: Balloon-expandable pulmonary valves are usually not suitable for dilated native outflow tracts. METHODS: Indian Venus P-valve registry was retrospectively analyzed for efficacy, complications, and midterm outcomes. Straight valve was used in prestented conduits in patients with right ventricular pressure above two-thirds systemic pressure and/or right ventricular dysfunction. Flared valve 1-4 mm larger than balloon waist was used in native outflow in symptomatic patients, large ventricular volumes, and ventricular dysfunction. OBJECTIVES: A self-expanding porcine pericardial Venus P-valve is available in straight and flared designs.. RESULTS: Twenty-nine patients were included. Straight valve was successful in all seven conduits, reducing gradients significantly, including one patient with left pulmonary artery (LPA) stent. Flared valve was successfully implanted in 20 out of 22 native outflow tracts. Sharp edges of the older design contributed to two failures. Complications included two migrations with one needing surgery, endocarditis in one, insignificant wire-frame fractures in three, and groin vascular complication in one patient. There were no deaths or valve-related reinterventions at a mean follow-up of 47.8 ± 24.5 months (1-85 months). Modifications of technique succeeded in three patients with narrow LPA. There was significant improvement in symptoms, right ventricular volume, and pulmonary regurgitant fraction. CONCLUSION: Straight and flared Venus P-valves are safe and effective in appropriate outflow tracts. Straight valve is an alternative to balloon-expandable valves in stenosed conduits. Flared valve is suitable for large outflows up to 34 mm, including patients with LPA stenosis. Recent design modifications may correct previous technical failures. Studies should focus on durability and late complications.

Conversion of prior univentricular repairs to septated circulation: Case selection, challenges, and outcomes.

OBJECTIVES: Complex congenital heart defects that present earlier in life are sometimes channelled in the single ventricle pathway, because of anatomical or logistic challenges involved in biventricular correction. Given the long-term functional and survival advantage, and with the surgeons' improved understanding of the cardiac anatomy, we have consciously explored the feasibility of a biventricular repair in these patients when they presented later for Fontan completion. We present a single institution's 10-year experience in achieving biventricular septation of prior univentricular repairs, the technical and physiological challenges and the surgical outcomes. METHODS: Between June 2010 and December 2019, 246 patients were channelized in the single ventricle pathway, of which 32 patients were identified as potential biventricular candidates at the time of evaluation for Fontan palliation, considering their anatomic feasibility. The surgical technique involves routing of the left ventricle to the aorta across the ventricular septal defect, ensuring an adequate sized right ventricular cavity, establishing right ventricle-pulmonary artery continuity and taking down the Glenn shunt with rerouting of the superior vena cava to the right atrium. This is a retrospective study where we reviewed the unique physiological and surgical characteristics of this subset of patients and analysed their surgical outcomes and complications. RESULTS: Biventricular conversion was achieved in all cases except in 3 patients, who had the Glenn shunt retained leading to a one and a half ventricle repair. The average age of the patients was 4.9 years of whom 18 were male. The average cardiopulmonary bypass time was 371 min with an average cross clamp time of 162 min. There was one mortality in a patient with corrected transposition of great arteries (c-TGA) with extensive arterio-venous malformations (AVMs). At a median follow-up of 60 months, all patients remained symptom free except two with NYHA II symptoms, one being treated for branch pulmonary artery stenosis with balloon dilatation and the other with multiple AVMs who needed coil closure. One patient with branch pulmonary artery (PA) stenosis required balloon dilatation and stent placement. CONCLUSION: The possibility of achieving the surgical goal in this unique subset of patients evolves with the progressive experience of the congenital heart surgeon. Case selection is a crucial aspect in achieving the desired outcome, and this 'borderline' substrate is often recognized at the time of evaluation for the Fontan completion. A comprehensive preoperative imaging and planning helps in achieving the surgical septation and reconnection to achieve the desired physiological circulation. Though technically challenging, the surgery has excellent short- and mid-term outcomes as evidenced by our 10-year experience.

Impact of transport on arrival status and outcomes in newborns with heart disease: a low-middle-income country perspective.

OBJECTIVES: We sought to systematically study determinants of "clinical status at arrival after transport" of neonates with CHD and its impact on clinical outcomes in a low- and middle-income country environment. METHODS AND RESULTS: Consecutive neonates with CHD (n = 138) transported (median distance 138 km; 5-425 km) to a paediatric cardiac programme in Southern India were studied prospectively. Among 138 neonatal transports, 134 were in ambulances. Four neonates were transported by family in private vehicles; 60% with duct-dependent circulation (n = 57) were transported without prostaglandin E1. Clinical status at arrival after transport was assessed using California modification of TRIPS Score (Ca-TRIPS), evidence of end-organ injury and metabolic insult.Upon arrival, 42% had end-organ injury, 24% had metabolic insult and 36% had Ca-TRIPS Score >25. Prior to surgery or catheter intervention, prolonged ICU stay (>48 hours), prolonged ventilation (>48 hours), blood stream sepsis, and death occurred in 48, 15, 19, and 3.6%, respectively. Ca-TRIPS Score >25 was significantly associated with mortality (p = 0.005), sepsis (p = 0.035), and prolonged ventilation (p < 0.001); end-organ injury with prolonged ICU stay (p = 0.031) and ventilation (p = 0.045); metabolic insult with mortality (p = 0.012) and sepsis (p = 0.015).Fifteen babies needed only medical management, 10 received comfort care (due to severe end-organ injury in 3), 107 underwent cardiac surgery (n = 83) or catheter intervention (n = 24), with a mortality of 6.5%. Clinical status at arrival after transport did not impact post-procedure outcomes. CONCLUSION: Neonates with CHD often arrive in suboptimal status after transport in low- and middle-income countries resulting in adverse clinical outcomes. Robust transport systems need to be integrated in plans to develop newborn heart surgery in low- and middle-income countries.

Comparison of Fontan Surgical Options for Patients with Apicocaval Juxtaposition.

Apicocaval juxtaposition (ACJ) is a rare form of viscerocardiac malpositions in association with single-ventricle congenital heart defects. The Fontan surgery is the common palliation, and possible surgical options include ipsilateral, contralateral, and intra-atrial conduits. Concerns include lower hemodynamic performances or risks of conduit compression by the cardiac mass. This study investigates the hemodynamics and clinical outcomes of ACJ patients and potential surgical improvements. Ten consecutive ACJ patients were included, along with a reference cohort of ten non-ACJ patients. Magnetic resonance images were acquired at 6 ± 0.6 year follow-up for anatomical analysis and hemodynamic assessments using computational fluid dynamics. Metrics of interest are deformation index (DI), indexed power loss (iPL), and hepatic flow distribution (HFDoff). A "virtual" surgery was performed to explore potential hemodynamic improvements using a straightened conduit. DI for ACJ patients fell within the DI range of non-ACJ patients. Contralateral conduits had insignificantly higher iPL (0.070 [0.032,0.137]) than ipsilateral conduits (0.041 [0.013,0.095]) and non-ACJ conduits (0.034 [0.011,0.061]). HFDoff was similar for the ipsilateral (21 [12,35]), contralateral (26 [7,41]), and non-ACJ Fontan conduits (17 [0,48]). Virtual surgery demonstrated that a straightened conduit reduced HFDoff and iPL for the contralateral and ipsilateral conduits, potentially leading to improved clinical outcomes. In this limited sample, the hemodynamic performance of ACJ patients was not significantly different from their non-ACJ counterparts. The use of a straightened conduit option could potentially improve patient outcomes. Additionally, the fear of significant compression of conduits for ACJ patients was unsupported.

Complete Sternal Cleft Repair.

Sternal cleft (SC) is a rare congenital malformation which can be partial or complete. We report a case of complete SC in a 9-month-old child. Our technique involves a combination of reinforcement with the deep cervical fascial extension, followed by the anterior perichondrial flaps, bridged with the rib graft, incorporating surplus resected cartilaginous xiphoid process, and covered with the bilateral pectoralis major muscle flap for the chest wall reconstruction with 3D printing assisting preoperative planning. The size of the defect in relation to the age of presentation was a deciding factor in the adoption of this alternative surgical technique.

Common inflammatory markers after cardiac surgery in infants and their relation to blood stream sepsis.

BACKGROUND: Limited information exists on trends of common inflammatory markers after infant heart surgery and their role in identifying post-operative sepsis. METHODS: 275 consecutive infants undergoing cardiac surgery (231 with and 44 without Cardiopulmonary Bypass) were studied prospectively. Daily trends (0-4 day post-operative) of leucocyte counts, platelet counts and C-reactive protein were recorded. Association of these trends with early post-operative bloodstream sepsis, Cardiopulmonary Bypass and surgical outcomes were studied. OBSERVATIONS: Trends of these inflammatory markers were noted. While off-Cardiopulmonary Bypass Surgery, and sepsis were associated with a statisticaly insignificant rise in total leucocyte count peaking on first post-operative day, Cardiopulmonary Bypass exposure was associated with significant decline (p = 0.002), more pronounced with Cardiopulmonary Bypass-exposure exceeding 150 min. Percentage of neutrophils showed a rise (maximum on first post-operative day) but no significant association with sepsis or Cardiopulmonary Bypass.Platelet counts significantly declined after surgery, with nadir on 2nd POD (p < 0.001), the drop being more marked in patients operated on Cardiopulmonary Bypass (p < 0.005). Counts were significantly lower in patients exposed to >150 min Cardiopulmonary Bypass compared to those with shorter Cardiopulmonary Bypass. Septic patients had significantly lower platelet counts than uninfected patients, decline >2 SD from mean pre-operative level strongly associated with sepsis (p < 0.001).C-Reactive Protein levels rose markedly after surgery, peaking on 2nd POD; levels were significantly higher if operated on Cardiopulmonary Bypass. Cardiopulmonary Bypass >150 min was associated with lower mean C-Reactive Protein on first post-operative day, but significantly higher values on third and fourth post-operative days, as compared to Cardiopulmonary Bypass <150 min. Comparison of infected versus non-infected patients showed significantly higher mean C-Reactive Protein in the former group. CONCLUSION: While leucocyte count, platelet count and C-Reactive Protein emerged as useful markers of post-operative inflammatory response and reaction to Cardiopulmonary Bypass, they proved unsatisfactory predictors of early post-operative sepsis.

Percutaneous ultrasound guided thrombin injection for axillary artery pseudoaneurysm following stenting of the arterial duct in two infants: Case report with review of literature.

We report pseudoaneurysm of the axillary artery following stenting of the arterial duct in two infants. They were both successfully managed by ultrasound guided thrombin injection with preservation of native arterial flow. We also review the relevant literature.

Does the clinical phenotype of mucolipidosis-IIIγ differ from its αß counterpart?: supporting facts in a cohort of 18 patients.

Mucolipidosis-IIIγ (ML-IIIγ) is a recessively inherited slowly progressive skeletal dysplasia caused by mutations in GNPTG. We report the genetic and clinical findings in the largest cohort with ML-IIIγ so far: 18 affected individuals from 12 families including 12 patients from India, five from Turkey, and one from the USA. With consanguinity confirmed in eight of 12 families, molecular characterization showed that all affected patients had homozygous pathogenic GNPTG genotypes, underscoring the rarity of the disorder. Unlike ML-IIIαß, which present with a broader spectrum of severity, the ML-III γ phenotype is milder, with onset in early school age, but nonetheless thus far considered phenotypically not differentiable from ML-IIIαß. Evaluation of this cohort has yielded phenotypic findings including hypertrophy of the forearms and restricted supination as clues for ML-IIIγ, facilitating an earlier correct choice of genotype screening. Early identification of this disorder may help in offering a timely intervention for the relief of carpal tunnel syndrome, monitoring and surgery for cardiac valve involvement, and evaluation of the need for joint replacement. As this condition may be confused with rheumatoid arthritis, confirmation of diagnosis will prevent inappropriate use of immunosuppressants and disease-modifying agents.

Medium-term results of percutaneous pulmonary valve implantation using the Venus P-valve: international experience.

AIMS: The aim of this study was to assess the international procedural and short-term to midterm experience with the new percutaneous Venus P-valve. METHODS AND RESULTS: Retrospective data of patient characteristics, clinical and imaging follow-up of Venus P-valve implantation outside China were collected. Thirty-eight patients underwent attempted Venus P-valve implantation between October 2013 and April 2017. Thirty-seven valves were successfully implanted during 38 procedures. There was one unsuccessful attempt and there were two valve migrations, one of which required surgical repositioning. The mean follow-up was 25 months with no short-term or midterm valve failure or deterioration in performance. Frame fractures occurred in 27% of patients. The cohort demonstrated a statistically significant reduction in pulmonary regurgitation fraction and indexed right ventricular diastolic volumes at six and 12 months. CONCLUSIONS: Implantation of the Venus P-valve has provided satisfactory short-term to midterm results with high success and low complication rates in an inherently challenging patient substrate.

Platelet parameters in children with chromosome 22q11 deletion and conotruncal heart defects.

BACKGROUND AND OBJECTIVES: The 22q11 deletion syndrome is associated with a wide spectrum of phenotypic features, hence clinical diagnosis is difficult. Individuals with this syndrome are found to have a risk of developing transfusion associated graft-versus-host reaction, if they are given nonirradiated blood. Our aim was to elucidate whether chromosome 22q11 deletion in children with syndromic conotruncal heart defects is associated with platelet abnormality. MATERIALS AND METHODS: The genetic analysis was performed by standard cytogenetic and Fluorescence in situ hybridization technique. The platelet parameters in 39 patients with chromosome 22q11 deletion were compared with 154 cases without deletion. RESULTS: In deletion versus no deletion group, the mean of mean platelet volume (MPV) was 10.5 ± 2.5 vs 7.6 ± 1.5 fL, platelet count was 225 ± 80.7 and 339 ± 127.3 × 10 9 /L and frequency of high MPV was 49% vs 7% (P < .0001). The MPV was associated with a sensitivity of 90.9% and a specificity of 79.6% at a cutoff value of 8.32 fL, (area under the ROC curve 91%). A nonsignificant negative correlation was found between MPV and platelet count (r = -0.152; P = .361) in children with deletion. CONCLUSION: A cutoff value of 8.32 fL for MPV can be an indicator of high risk of chromosome 22q11 deletion in individuals with syndromic conotruncal defects. Individuals with chromosome 22q11 deletion should be given irradiated blood especially during cardiac surgery. Further investigation should clarify the etiology behind variation in frequency of high MPV in different conotruncal lesions.

Pulmonary Hemorrhage Due to Unrecognized Bronchial Collateral After Arterial Switch Operation.

We report a neonate with transposition of great arteries and intact ventricular septum who had a massive pulmonary hemorrhage soon after an arterial switch operation. An emergency cardiac catheterization revealed a large bronchial collateral artery from the descending aorta feeding the right lung. The hemorrhage was controlled by coil embolization of the collateral, and the patient recovered after prolonged intensive care.