OBJECTIVES:Intraventricular tunneling is a pivotal step in biventricular or 1.5 ventricle repair of complex congenital heart disease, conventionally reliant on surgeon's intraoperative inspection and judgement. We evaluated novel assistive processes and translational tools for aiding intraventricular tunneling during complex surgical repairs using 3-dimensional virtual reality and 3-dimensional printing technologies. METHODS:Computed tomography data of prospective complex congenital heart disease cases considered for biventricular/1.5 ventricle repair with intraventricular tunneling were converted to "digital twins" and analyzed in 3-dimensional virtual reality using medical modeling software. Digital dissection, annotation, and measurement tools helped identify critical anatomic elements: interventricular septum to great vessel orientation, optimal pathway least likely to obstruct, dynamic changes, conduit requirement, and so forth. Virtual baffle design tool simulated baffles with morphometrics. Novel workflows helped convert virtual baffles to physical templates: 3-dimensionally printed in flexible resin, digitally unfolded and printed in rigid resin, and on plain paper. Templates were compared and used as surgical guides. Adherence to simulation-based plans and surgical baffle placement were assessed. RESULTS:Twenty cases (mean age 8.61 ± 7.61 years) included double outlet right ventricles (n = 12; 2 with superior-inferior ventricles, 2 with common atrioventricular canal), double outlet both ventricles (1), corrected transposition (5), and D-transposition (2) variants. Situs abnormalities (5/20), dextrocardia (7/20), and additional defects added to the complexity. Operative findings were as predicted, and repair was achieved in 100% (2 ventricle repair in 14 patients and 1.5 ventricle repair in 6 patients). Morphometrics of virtual, 3-dimensional printed, and paper-printed baffles were identical and successfully used as surgical guides; deviation from predicted geometry was less than 10%. All baffles were single patch, placed as per digital plan, with no residuals. Mean cardiopulmonary bypass time was 322 ± 109 minutes, and crossclamp time was 159 ± 54 minutes. Median hospital stay was 10 days (interquartile range, 8-16) with no operative mortality. No reinterventions or residuals were observed at 20 months median follow-up. CONCLUSIONS:Early-stage exploration of translational impact of 3-dimensional virtual reality-based baffle planning is encouraging. Workflow for creating paper-printable baffle templates shows great promise.

In this case report, we describe a rare congenital anomaly of a right aortic arch with a left innominate artery originating from the pulmonary artery through a left ductus arteriosus, highlighting the use of 3D Virtual Reality (VR) modeling for preoperative planning and the critical role of intraoperative monitoring, using Near-Infrared Spectroscopy (NIRS), to assess tissue oxygenation and guide surgical management. We also briefly discuss the embryological basis of this rare arch anomaly.

Background Neonatal aortic arch obstruction is typically related to ductal insertion and usually improves with prostaglandin E1 (PGE1). However, functional constriction caused by ectopic ductal tissue at atypical arch locations is rare and may mimic fixed coarctation. Case Summary A one-day-old neonate with suspected coarctation was found to have a right aortic arch with transverse arch hypoplasia, an aberrant left subclavian artery arising from a Kommerell diverticulum, and a large patent ductus arteriosus. Withdrawal of PGE1 led to progressive distal arch constriction with left ventricular dysfunction, which reversed after reinstitution of PGE1. Further imaging clarified the anatomy, and surgical excision of the transverse arch segment demonstrated ductal tissue on histopathology. Discussion This case illustrates a rare mechanism of prostaglandin-responsive arch obstruction caused by ectopic ductal tissue, highlighting the importance of dynamic prostaglandin testing and multimodality imaging in differentiating functional from fixed neonatal arch obstruction. Novelty We report, for the first time, a neonate with PGE1-responsive coarctation of the transverse aortic arch resulting from ectopic ductal tissue, anatomically separate from the native ductus arteriosus, confirmed on histopathology.

This case report demonstrates the feasibility of creating immersive 3-dimensional (3D) visualizations (3D virtual reality and 3D printing) from fetal echocardiographic volume data sets for both a normal heart and a heart with transposition of great arteries. Immersive 3D technologies could emerge as powerful tools for understanding fetal cardiac anatomy for clinical decision making as well as training and research.

This case report demonstrates the feasibility of creating immersive 3D visualizations ( 3D Virtual reality and 3D printing) from fetal echocardiographic volume datasets for both normal heart and a heart with transposition of great arteries. Immersive 3D technologies could emerge as powerful tools in future for understanding fetal cardiac anatomy for clinical decision making as well as training and research. ### Competing Interest Statement The authors have declared no competing interest. ### Funding Statement This study did not receive any funding ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: This study used electronic health records data ( fetal echocardiography datasets) obtained from human foetuses which were recorded after prior consent from the pregnant woman as per the Pre-conceptional and prenatal diagnostic techniques act of India. The institutional ethics committee of Amrita Institute of Medical Sciences waived the ethical approval for this work since the proper protocol for conduct of fetal echocardiography as per the PcPNT act of India was followed for acquisition of ultrasound datasets and the study involved only use of electronic patient data. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced in the present study are available upon reasonable request to the authors

Background Diagnosing, managing complex CHD demands excellent morphological understanding. Individual differences in visuospatial skills, training and experience can impact spatial interpretation of volumetric cardiac imaging. Immersive 3D visualisation may help overcome these challenges, but evidence of clinical benefit is lacking. This study explores variability in visuospatial abilities and interpretation of conventionally viewed volumetric cardiac imaging data among members of a pediatric cardiac unit, and impact of using immersive 3D formats (3D-prints, Virtual Reality) on spatial understanding of complex CHD morphology. Methods Prospective cohort study involving 9 heterogenous members of an advanced pediatric cardiac program [3 consultant cardiologists, 2 cardiothoracic surgeons, 1 cardiac radiologist, 3 cardiology trainees]. Participants’ visuospatial abilities were quantified using a validated test (Revised PSVT:R). Understanding of spatial relationships between anatomical structures was assessed using structured questionnaires for 17 unique anonymised volumetric cardiac scans (15 CT, 2 MRI) of complex CHD visualised in three formats 1). conventional DICOM (CDICOM); 2). 3D prints (3DP); 3). Virtual Reality (VR). Accuracy, time taken, perceived level of difficulty, and confidence in interpretation were assessed and compared. Results Spatial abilities varied widely (median 8, IQR 6-30), independent of expertise/experience. Limitations in conventional reading were significantly overcome with immersive 3D. Mean accuracy score of 60.48% ±17.13% with CDICOM increased to 83.93% ± 7.84% with 3DP, and 90.81% ± 5.03% with VR (p<0.001). 3DP and VR permitted significantly faster interpretation (p<0.001), with significantly better ease and confidence. While immersive 3D visualisation led to significantly improved spatial understanding for all, it also minimised differences between participants with widely variable skill and experience levels. Conclusion This study provides evidence that immersive 3D visualisation can enhance spatial understanding of complex CHD morphology, overcoming challenges in spatial ability, experience, expertise. These technologies may be suitably leveraged as effective clinical and teaching tools in congenital cardiology. ### Competing Interest Statement The authors have declared no competing interest. ### Funding Statement This study did not receive any external funding.Partially funded by Institutional research grant of Amrita Institute of Medical Sciences ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: Ethics Committee of Amrita Institute of Medical Sciences, Kochi, India gave ethical approval for this work I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes All data produced in the present study are available upon reasonable request to the authors * CHD : Congenital heart disease CT : computed tomography DICOM : Digital Imaging and Communication in Medicine MRI : magnetic resonance imaging PSVT:R : Perdue Spatial Visualisation Test - Test of Rotations

Background Infantile hypertrophic cardiomyopathy (HCM) is a heterogeneous disorder. Apart from registries in high-income nations, there is a shortage of data on the aetiological basis of infantile HCM in low- and middle-income nations. This study attempts to characterise the phenotypic expression, genetic architecture and short-term clinical outcomes of infantile HCM from a South Asian tertiary referral centre.Methods This study includes all infants from the Amrita HCM cohort between January 2011 and July 2021. Clinical history, ECG, echocardiographic data, and genetic analyses were evaluated.Results 34 patients with infantile HCM were diagnosed at a median age of 3.7 months (IQR 1-6 months). Underlying aetiologies were RASopathy (n=13; 38%), non-syndromic (n=12; 35%) and inborn errors of metabolism (n=9; 27%). Genetic analysis was done in 20 patients (59%) with a yield of 90%. Clinical presentation included failure to thrive (n=29; 85%), dyspnoea on exertion (n=23; 68%) and clinical heart failure (n=24; 71%). Echo showed concentric left ventricular hypertrophy in 22 patients (65%), obstructive HCM in 11 patients (32%) and left ventricular systolic dysfunction in 6 patients (18%). The mortality rate was 10.0 deaths per 100 patient years over a median follow-up period of 3.1 years. The main risk markers for mortality were the age at diagnosis, gender and concentric Left ventricular hypertrophy.Conclusions This cohort demonstrates the morphological, functional and genetical heterogeneity of infantile HCM, enunciating the need for integration of cardiology, metabolic and genetic services to achieve optimum outcomes in these patients.

A 15dayold term male neonate presented with respiratory distress, tachycardia, cyanosis (SpO2 85%), periorbital puffiness and pedal oedema (figure 1A). Echocardiography showed suprasystemic pulmonary artery pressures and a large (2.7×3 cm) mass compressing the right heart, producing cardiac tamponade (figure 1B; online supplemental video 1). Saline contrast echocardiography (figure 1C; online supplemental video 2) does not show any communication with the circulation. Contrast cardiac CT angiogram showed a large lobulated pericardial cyst encircling and compressing the right heart without any communication with the circulation (figure 1D). 3D CT reconstruction showed mass effect on the right heart structures (figure 1E). Echocardiographyguided fine needle aspiration of the cyst was performed with surgical backup. A 30 mL of pus was aspirated, and the culture showed methicillinresistant Staphylococcus aureus growth. The workup for primary immunodeficiency was negative. After aspiration, the cardiac haemodynamics were normalised, and the neonate recovered with antibiotics. At 3month followup, the infant is doing well, and the repeat echocardiography was normal. Pericardial cysts are relatively a frequent incidental finding at autopsy or on chest Xray in adults. It usually originates as a unilocular thinwalled cyst from the persistent blind ending recesses of the parietal pericardium. Although lifethreatening respiratory distress due to pericardial cyst requiring surgical excision in newborn has been reported, to our knowledge, infected pericardial cyst recovering with fine needle aspiration has not been previously reported. 2 Although CT gave good images, contrast echo itself confirmed absent communication of mass with the circulation, hence percutaneous aspiration was considered as a reasonable option.

Cutting-edge 3-dimensional technologies like 3-dimensional printing and extended reality visualization provide novel, immersive ways to understand and interact with volumetric medical imaging data for preprocedural planning. We present a case that illustrates the utility of these techniques in a patient requiring a complex transcatheter intervention.

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.

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.

HomeCirculation: Cardiovascular ImagingVol. 15, No. 11Prenatal Diagnosis of a Large Left Coronary Artery to Right Ventricle Fistula With 3-Dimensional/4-Dimensional Spatiotemporal Image Correlation Rendering Followed by Successful Neonatal Transcatheter Closure Free AccessCase ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessCase ReportPDF/EPUBPrenatal Diagnosis of a Large Left Coronary Artery to Right Ventricle Fistula With 3-Dimensional/4-Dimensional Spatiotemporal Image Correlation Rendering Followed by Successful Neonatal Transcatheter Closure Balu Vaidyanathan, Roma Verma, Mahesh Kappanayil and Raman Krishna Kumar Balu VaidyanathanBalu Vaidyanathan Correspondence to: Balu Vaidyanathan, DM, Department of Pediatric Cardiology, Head, Fetal Cardiology division, Amrita Institute of Medical Sciences, Kochi, Kerala, India. Email E-mail Address: [email protected] https://orcid.org/0000-0001-6636-8308 The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. , Roma VermaRoma Verma The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. , Mahesh KappanayilMahesh Kappanayil https://orcid.org/0000-0002-1331-6720 The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. and Raman Krishna KumarRaman Krishna Kumar The Fetal Cardiology Division, Department of Pediatric Cardiology, Amrita Institute of Medical Sciences, Amrita University, Kochi, Kerala, India. Originally published26 Aug 2022https://doi.org/10.1161/CIRCIMAGING.122.014247Circulation: Cardiovascular Imaging. 2022;15Other version(s) of this articleYou are viewing the most recent version of this article. Previous versions: August 26, 2022: Ahead of Print Isolated congenital coronary artery fistulas are rare congenital anomalies in which there is an abnormal communication between a coronary artery and one of the cardiac chambers or great vessels.1 These occur with a prevalence of 1:50 000 live births and accounts for 0.2% to 0.4% of all congenital heart defects. Prenatal diagnosis of isolated congenital coronary artery fistulae is relatively rare and is restricted to short series or case reports.2,3 We report a case of isolated congenital coronary artery fistulas from the left coronary artery to the right ventricle in which prenatal imaging using advanced 3-dimensional (3D)/4-dimensional (4D) spatiotemporal image correlation (STIC) rendering was used to delineate the precise anatomy, thereby aiding planned peripartum care and expedited catheter closure of the fistula immediately after birth with excellent outcomes.A 30-year-old second gravida was referred for fetal echocardiography to our center in view of suspicion of a channel with continuous flow from aorta to the left ventricle. Fetal echocardiography evaluation (Voluson E10 systems, GE healthcare, Zipf) at 24 weeks showed a to and fro flow in a channel arising from the aorta in the direction of the left ventricle without evidence of cardiomegaly. On reassessment at 27 weeks and again at 29 weeks (Figure 1; Video S1), the left coronary artery was found to be significantly dilated (Figure 1A) with a turbulent high velocity color jet coursing toward the right ventricular apex (Figure 1B). There was cardiomegaly at 29 weeks and the 3-vessel tracheal view showed evidence of significant flow reversal into the aortic arch (Figure 1C) suggesting a hemodynamically significant shunt. Spectral Doppler showed a characteristic to and fro pattern with forward flow into the aorta in systole and retrograde flow into the fistula in diastole (Figure 1D). We did a 3D/4D STIC rendering of the anatomy using volume datasets acquired and this clearly delineated the anatomy of the fistula as arising from the left coronary artery and coursing downward toward its entry point in the right ventricular (RV) apex (Figure 2A; Video S2). The fetus was carefully followed up during the rest of the prenatal period for evidence of hydrops or heart failure. All fetal Dopplers remained within normal range till term and there were no associated anomalies.Download figureDownload PowerPointFigure 1. Prenatal imaging of the coronary fistula from left coronary artery to right ventricle. A, Short-axis view of the outflow tracts showing the aorta (Ao) in the center with the right ventricle (RV) and its outflow tract. The left coronary artery (LCA) appeared dilated. The fistula (arrows) seemed to track toward the right ventricular outflow tract. B, Short-axis view of the outflows profiling the entire course of the fistula. From the dilated LCA, the fistula coursed toward the right ventricle and joined the RV near its apex (arrow). C, The 3-vessel tracheal view showing a reverse flow (red color with arrows) toward the aorta suggesting significant run-off through the fistula, indicating its hemodynamic severity. D, Spectral Doppler across the fistula showing the typical to and fro flow pattern with systolic flows toward aorta and diastolic flow toward the RV. PA indicates pulmonary artery.Download figureDownload PowerPointFigure 2. Comparison of anatomy of the coronary fistula rendered using prenatal 3-dimensional (3D)/4-dimensional (4D) spatiotemporal image correlation (STIC) versus postnatal 256 slice computed tomography. A, 3D/4D STIC rendering of the coronary fistula anatomy using prenatal volume datasets. The dilated left coronary artery (LCA) can be seen arising from the aortic sinus (Ao). The fistula arises from the LCA and courses down as a very dilated channel toward the right ventricle (RV) where it can be seen entering (arrow). The proximal portion of the fistula was especially large with a constriction in the terminal portion. B, The fistula anatomy reconstructed using a 256 slice post-natal computed tomography study. The RV is superimposed using blue color. The origin, course and the distal end of the fistula joining the RV apex is seen and is very similar to the prenatal anatomy demonstrated using 3D/4D STIC rendering. RA indicates right atrium.At 38 weeks' gestation, a baby boy was delivered by normal vaginal delivery with birth weight of 3.32 kg. The baby had normal APGAR scores (Appearance, Pulse, Grimace, Activity, and Respiration) at birth. Cardiac evaluation showed oxygen saturation of 99% with evidence of a continuous murmur. Neonatal echocardiography confirmed the diagnosis of a large coronary fistula from left coronary artery to the RV apex with significant dilatation of the right heart structures with flow reversal in aortic arch. A computed tomography pulmonary angiography with 3D reconstruction was done on day 2 of life and confirmed the diagnosis of a large coronary artery fistula from left coronary artery to the RV apex. There was excellent correlation between the computed tomography anatomy (Figure 2B) and the prenatal 3D/4D STIC rendering (Figure 2).In view of the hemodynamically significant fistula, the baby was taken up for a fistula occlusion by cardiac catheterization on day 4 of life under general anesthesia. Angiogram confirmed a large fistula from left coronary artery into RV with an area of constriction in the vertical limb of the fistula before entering the RV (Figure 3A; Video S3). The fistula was occluded using a 5F right coronary guiding catheter from the venous side using a 6-8 Konar-MF muscular VSD occluder (Lifetech Scientific, Shenzen, China; Figure 3B). There was some persistent residual shunt immediately post-procedure which disappeared over the next 1 month. The baby was weaned off anti-failure medications on follow-up. At 3-months post-procedure, the baby was clinically well with a weight of 6 kg and no heart failure.Download figureDownload PowerPointFigure 3. Cardiac catheterization images of the coronary fistula before and after device closure. A, Angiogram taken in the left anterior-oblique (LAO) view. The catheter coursed through the femoral venous access into the right heart and the fistula was entered through its right ventricle (RV) entry point (bold arrow) and tracked through the fistula toward the left coronary artery (LCA). The aorta (Ao) also filled retrograde along with the native coronary branches (small arrows). B, The angiogram taken from the aortic root (Ao) after deployment of the Konar-MF device (arrow) in the vertical limb of the fistula. The native LCA is seen well.This case report illustrates the significant benefit of prenatal diagnosis in planning the neonatal management of a neonate with a hemodynamically significant coronary artery fistulas. Isolated congenital coronary artery fistulae are rarely reported in the prenatal literature.1–3 More commonly, these fistulas arise from the right coronary system than the left with the vast majority draining into the right sided cardiac chambers.1 Prenatal findings suggesting high volume shunting through the fistula include cardiomegaly, flow reversal in the aortic arch, presence of turbulent flow throughout the cardiac cycle and presence of hydrops fetalis.1,2 Prenatal identification of these high-risk features should prompt careful surveillance and planned peri-partum care in a pediatric cardiac facility with an intent for expedited intervention of the fistula in the early neonatal period, as illustrated by our case (Figure 1; Video S1). We used advanced 3D/4D fetal echocardiography with STIC rendering to delineate the precise anatomic details of the fistula in the prenatal period (Figure 2A; Video S2). We had previously reported the incremental benefits of 3D/4D STIC technique in predicting the postnatal surgical pathway in fetuses with double outlet right ventricle.4 There was excellent correlation between the anatomy of the coronary artery fistulas as delineated by prenatal 3D/4D STIC compared with postnatal 256 slice computed tomography based reconstruction (Figure 2). The precise delineation of the anatomy in the prenatal period along with the identification of features of high-volume shunting enabled us to precisely plan the neonatal cardiac care for this baby culminating in a successful trans-catheter closure of the fistula (Figure 3; Video S3).In conclusion, prenatal diagnosis and precise delineation of the anatomy is feasible for isolated congenital coronary artery fistulae using fetal echocardiography assisted by newer techniques like 3D/4D STIC rendering. This helps in planning the neonatal care for these critically sick infants ensuring excellent clinical outcomes.Article InformationSources of FundingNone.FootnotesSupplemental Material is available at https://www.ahajournals.org/doi/suppl/10.1161/CIRCIMAGING.122.014247.For Disclosures, see page 855.Correspondence to: Balu Vaidyanathan, DM, Department of Pediatric Cardiology, Head, Fetal Cardiology division, Amrita Institute of Medical Sciences, Kochi, Kerala, India. Email baluvaidyanathan@gmail.comReferences1. Walter A, Calite E, Herberg U, Breuer J, Berg C, Geipel A, Gembruch U. Prenatal detected Isolated congenital coronary artery fistula (ICCAF) characteristics and impact on the fetal hemodyanamic situation: systematic literature review.Interv Cardiol. 2021; 13:316–322. doi: 10.1002/ccr3.3779CrossrefGoogle Scholar2. Sharland GK, Konta L, Qureshi SA. Prenatal diagnosis of isolated coronary artery fistulas: progression and outcome in five cases.Cardiol Young. 2016; 26:915–920. doi: 10.1017/S1047951115001535CrossrefMedlineGoogle Scholar3. Nagiub M, Mahadin D, Gowda S, Aggarwal S. Prenatal diagnosis of coronary artery fistula: a case report and review of literature.AJP Rep. 2014; 4:e83–e86. doi: 10.1055/s-0034-1386636CrossrefMedlineGoogle Scholar4. Karmegaraj B, Kumar S, Srimurugan B, Sudhakar A, Simpson JM, Vaidyanathan B. 3D/4D spatiotemporal image correlation (STIC) fetal echocardiography provides incremental benefit over 2D fetal echocardiography in predicting postnatal surgical approach in double-outlet right ventricle.Ultrasound Obstet Gynecol. 2021; 57:423–430. doi: 10.1002/uog.21988CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Karapurkar S, Kappanayil M, Kumar R and Vaidyanathan B (2023) 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, Annals of Pediatric Cardiology, 10.4103/apc.apc_163_22, 16:2, (150-153), . November 2022Vol 15, Issue 11 Advertisement Article InformationMetrics © 2022 American Heart Association, Inc.https://doi.org/10.1161/CIRCIMAGING.122.014247PMID: 36017699 Originally publishedAugust 26, 2022 Keywordscatheter closurecoronary fistulaprenatal diagnosisPDF download Advertisement SubjectsUltrasound

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.

Background: After the peak of COVID 19 pandemic, a surge of cases with multi-system involvement and hyperinflammatory state was reported in children, at multiple tertiary care centers across the world. (1) This COVID-19 associated multisystem inflammatory syndrome in children and adolescents is also known as multisystem inflammatory syndrome in children (MIS-C). There is a paucity of literature of clinical profile of MIS-C patients from India. Objectives: To study the clinical manifestations and treatment outcomes of COVID-19 related multisystem inflammatory syndrome in children (MIS-C) and adolescents. Methods: It was a cohort study including cases defnied as per the case definitions of MIS-C by World Health Organisation (WHO) or Centre for Disease Control and Prevention(CDC). (2) The clinical parameters, laboratory parameters (including inflammatory markers, D dimer and NT ProBNP), electrocardiogram, 2D echocardiogram, course in hospital, and immediate outcomes were noted. Results: Twenty five patients (male-14) with a mean age of 6.22 ±4.25 years and a mean body mass index of 16.21 ±3.36 kg/m 2 were enrolled on the study. Average length of hospital stay was 8.12±5.76 days. Associated comorbidities were present in 4 of our patients (16%). Figure 1 shows the frequency of clinical features in our study patients. Table 1 shows the clinical, laboratory, imaging findings, disease course and outcomes. Figure 1. Frequency of clinical features in our MIS-C cohort patients Table 1. Clinical characteristics of MIS-C patients (n)=25 Clinical Phenotypes: Incomplete/ Atypical Kawasaki Disease (KD) 18 (72%) Macrophage activation syndrome (MAS) 11 (44%) Unexplained Shock 13 (52%) Fever with gastrointestinal symptoms 17 (68%) Fever with neurological symptoms 9 (36%) Fever with respiratory symptoms 5 (20%) COVID chronology: MISC with acute COVID 19 with MAS 1 (4%) MISC with acute COVID 19 with incomplete/atypical KD 1 (4%) COVID 19 tests: COVID 19 RTPCR positive 2 (8%) COVID 19 RTPCR negative 23 (92%) COVID 19 IgG positive 20 (80%) COVID 19 IgM positive 6 (24%) COVID 19 IgG and IgM both positive 2 (8%) COVID 19 IgG and IgM not done 4 (16%) Other laboratory investigations (Peak/ trough values): Anaemia 20 (80%) Leukopenia 2 (8%) Thrombocytopenia 6 (24%) Pancytopenia 2 (8%) Positive CRP 23 (92%) High Procalcitonin 9 (36%) High ferritin 10 (40%) High D- dimer 17 (68%) High NT ProBNP 23 (92%) Hypoalbuminemia 14 (56%) Sterile pyuria 7 (28%) Proteinuria 4 (16%) Cardiac assessment: Abnormal Electrocardiogram (ECG) 5 (20%) Abnormal Echocardiography 15 (60%) Coronary dilation/prominence/non-tapering 9 (36%) Coronary aneurysm 1 (4%) LAD Z score (Mean±SD) 2.12 ± 1.11 Decreased Ejection fraction (EF) 5 (20%) Systolic Dysfunction 5 (20%) Pericardial effusion 9 (36%) Global/septal hypokinesia 4 (16%) Mitral Valve regurgitation 3 (12%) Complete heart block 1 (4%) Twenty patients (80%) had severe illness requiring intensive care. Fourteen patients (56%) required inotropic support, 8 patients (32%) required supplemental oxygen, 5 patients (20%) required mechanical ventilation and 2 patients (8%) expired. A total of 23 patients (92%) received pulse steroids followed by oral/iv steroids, 22 patients (88%) received IVIG (2g/kg) and 3 patients (12%) required anticoagulation. Conclusion: Our MIS-C cohort had varied clinical manifestations ranging from the mild cutaneous and gastrointestinal symptoms to fatal multiorgan dysfunctions. In contrast to western cohorts, our study cohort had higher number of patients from younger age group and lower BMI. References: [1]Ahmed M, Advani S, Moreira A, Zoretic S, Martinez J, Chorath K et al. Multisystem inflammatory syndrome in children: A systematic review. EClinicalMedicine. 2020;26:100527. [2]Jiang L, Tang K, Levin M, Irfan O, Morris S, Wilson K et al. COVID-19 and multisystem inflammatory syndrome in children and adolescents. The Lancet Infectious Diseases. 2020;20(11):e276-e288. Disclosure of Interests: None declared

A 24-day-old female neonate was admitted to the paediatric cardiac intensive care unit with features of cardiogenic shock. She was born at term with a birthweight of 3·75 kg to a mother with a history of RT-PCR-positive, mild COVID-19 at 31 weeks gestation, which was managed with symptomatic and supportive measures. There were no fetal complications during the illness. The mother was RT-PCR-negative at delivery. Following an uncomplicated vaginal delivery, the neonate required supportive care for 5 days in a neonatal intensive care unit for mild tachypnoea and was subsequently discharged home with normal clinical, radiological, and laboratory parameters; RT-PCR for SARS-CoV-2 was not done. She stayed well at home until day 22 of life when she required admission to a local hospital with heart failure and systemic hypoperfusion. There was no preceding history of fever, rash, loose stools, abdominal distention, lethargy, or seizures. Following inadequate response to treatment, she was referred to our department (the Department of Pediatric Cardiology at the Amrita Institute of Medical Sciences and Research Centre, Kochi, India), with suspicion of a critical congenital heart disease. At presentation (day 0) she was afebrile, drowsy, tachycardic (heart rate: 160 beats per minute), hypotensive (mean blood pressure: 25 mm Hg), and tachypnoeic (respiratory rate: 55 breaths per minute), with chest retractions, oxygen saturation of 97% on supplemental oxygen, cool peripheries, and delayed capillary refill. The precordium was hyperdynamic, with no cardiac murmurs; the chest was clear. Hepatomegaly was present. Neither neurological or musculoskeletal abnormalities nor dysmorphisms were noted. Erythema was noted at pressure points—over the occiput and at bilateral gluteal regions; no other skin or mucosal lesions were present. The patient had metabolic acidosis with an arterial pH of 7·173 and lactate concentration of 22 mmol/L. The electrocardiogram showed sinus rhythm and the chest x-ray showed cardiomegaly, with normal lung fields. The point-of-care echocardiogram showed severe biventricular dysfunction with a left ventricular ejection fraction of 10% and global hypokinesia. Coronary arteries were normal in origin and luminal dimensions, but appeared prominent and hyperechoic. Major congenital heart disease, including outflow tract obstructions, was ruled out. Features of primary cardiomyopathies or storage disorders were absent. Initial management included mechanical ventilation, inotropic support (intravenous infusions of epinephrine [0·04 μg/kg per min] and milrinone [0·5 μg/kg per min]), diuretic (intravenous infusion of frusemide [2·0 mg/kg per day]), antimicrobial cover (intravenous infusion of parenteral cefoperazone plus sulbactam [50·0 mg/kg every 8 h]), and appropriate fluid and electrolyte management. Cardiac markers were substantially elevated, with an N-terminal-pro-B-type natriuretic peptide concentration of 157 000 pg/mL, a creatine kinase myocardial band concentration of 14·7 ng/mL, and a troponin T concentration of 1·23 ng/mL (figure 1). The aspartate aminotransferase concentration was 2240 IU/L and the alanine aminotransferase concentration 866 IU/L, whereas the blood urea concentration was 75 mg/dL and the serum creatinine concentration 0·9 mg/dL. Inflammatory markers were also markedly elevated: the serum ferritin concentration was 56 400 ng/mL (reference range 12–327 ng/mL) and the serum lactate dehydrogenase concentration 6280 U/L (0–451 U/L). The C-reactive protein concentration was 6·5 mg/L and the D-Dimer concentration 20 μg/mL. There was mild leucocytosis: total leucocyte count was 16 400 cells per microlitre (75% neutrophils) and platelet count was 185 000 cells per microlitre. Serum electrolytes, thyroid hormones, and vitamin D concentrations were within normal ranges. Two blood cultures—at admission, and 5 days later—were sterile. Qualitative antibody assay (VIDAS, Biomerieux, Marcy-l'Étoile, France) detected IgG antibodies against SARS-CoV-2 spike protein in both maternal and neonatal serum samples, with a titre of 20·11 in the maternal serum sample and a titre of 10·00 in the neonatal serum sample; no IgM antibodies were detected. Nasopharyngeal swabs and rectal swab were negative for SARS-CoV-2 on RT-PCR. Cerebrospinal fluid was not tested. Qualitative RT-PCR panel of the nasal swab for common respiratory viruses detected human rhinovirus; none of the other viruses on the panel were detected. This clinical presentation with severe acute myocardial injury, multiorgan dysfunction, elevated inflammatory markers, temporal association with prenatal exposure to COVID-19, and laboratory evidence of IgG antibodies to SARS-CoV-2, in the absence of other plausible explanations, led us to consider the possibility of a hyperinflammatory response to prenatal exposure to COVID-19. Immunomodulatory therapy was instituted on day 1 of hospitalisation, with a single dose of intravenous immunoglobulin (2 g/kg; slow infusion) as well as an injection of methylprednisolone once daily for 3 days (5 mg/kg; slow infusion). Continuous heparin infusion was added in a dose of 10 U/kg per h and titrated for optimal anticoagulation. Calcium (calcium gluconate intravenous infusion of 4 mg/kg per day), vitamin C (50 mg once daily, orally), vitamin D (400 IU once daily, orally), and zinc (10 mg once daily, orally) were supplemented. No antiviral agents were prescribed. Clinical stability was attained within the next 48 h. Laboratory markers of inflammation and end-organ functions showed improvements starting 24 h after the initiation of treatment. Serial echocardiograms showed progressive improvement in ventricular systolic function. The dose of methylprednisolone was halved to 2·5 mg/kg per day on days 4 and 5. Following an unsuccessful attempt at extubation on day 5, mechanical ventilation and inotropic support were electively continued for the next 5 days. Oral prednisolone (2 mg/kg per day) was added after stopping methylprednisolone on day 6. The patient was successfully extubated to non-invasive ventilation on day 11 of admission and to oxygen by nasal cannula on day 14. Inotropes were tapered off by day 13, and intravenous diuretic and heparin were stopped by day 15. Enalapril (initiated at 0·25 mg and gradually stepped up to 0·75 mg twice daily, orally), frusemide (1 mg/kg twice daily, orally) and aspirin (3 mg/kg once daily, orally) were added. Figure 1 shows the trends of the laboratory markers of inflammation, myocardial injury, and hepatic and renal functions during the clinical course. The trends for leucocyte counts, platelet counts, and C-reactive protein concentrations are depicted in the appendix (p 1). Erythema over the scalp (figure 2A) spontaneously resolved. Gluteal skin lesions progressed into symmetrical, well demarcated purple patches by day 2 of admission (figure 2B). Superficial layers subsequently underwent necrosis, scab formation, and sloughing, leaving behind sharply marginated ulcers that finally healed with epithelialisation (figures 2B–2H). These ulcers were conservatively managed with topical antibiotic (mupirocin ointment [2·15%, twice daily]), zinc oxide ointment (15%, twice daily), and posture care. Neurological status remained normal throughout the clinical course. In view of the modest fall in platelet counts, the elevated D-Dimer concentration, and the bruise-like gluteal skin lesion, point-of-care cranial ultrasound was done on day 3 of hospitalisation, which excluded intracranial bleed; no other neuroimaging was done. Patient was successfully transitioned out of the intensive care unit to the wards on day 20 of admission and discharged home on day 29. The echocardiogram at discharge showed a left ventricular ejection fraction of 58%, with mild dyskinesia of the apical-septal regions; coronaries appeared less hyperechoic than at presentation. Discharge medications included oral enalapril, aspirin, tapering doses of tablet prednisolone, and oral supplements (calcium, vitamin D, vitamin C, and zinc). Acute COVID-19 is significantly less common and less severe in children, with few reports of neonates being affected.1De Rose DU Piersigilli F Ronchetti MP et al.Novel coronavirus disease (COVID-19) in newborns and infants: what we know so far.Ital J Pediatr. 2020; 46: 56Crossref PubMed Scopus (109) Google Scholar, 2Cook J Harman K Zoica B Verma A D'Silva P Gupta A Horizontal transmission of severe acute respiratory syndrome coronavirus 2 to a premature infant: multiple organ injury and association with markers of inflammation.Lancet Child Adolesc Health. 2020; 4: 548-551Summary Full Text Full Text PDF PubMed Scopus (25) Google Scholar This optimistic outlook has been altered by reports of multisystem inflammatory syndrome in children (MIS-C; also known as paediatric multisystem inflammatory syndrome temporally associated with SARS-CoV-2 [PIMS-TS]), a rare but potentially fatal hyperinflammatory response that can occur in children 2–6 weeks after SARS-CoV-2 exposure, with a median age of occurrence between 7 and 9 years.3Radia T Williams N Agrawal P et al.Multi-system inflammatory syndrome in children & adolescents (MIS-C): a systematic review of clinical features and presentation.Paediatr Respir Rev. 2020; (published online Aug 11.)DOI:10.1016/j.prrv.2020.08.001PubMed Google Scholar, 4Alsaied T Tremoulet AH Burns JC et al.Review of cardiac involvement in multisystem inflammatory syndrome in children.Circulation. 2021; 143: 78-88Crossref PubMed Scopus (209) Google Scholar, 5Jain S Sen S Lakshmivenkateshiah S et al.Multisystem inflammatory syndrome in children with COVID-19 in Mumbai, India.Indian Pediatr. 2020; 57: 1015-1019Crossref PubMed Scopus (75) Google Scholar Immunomodulatory therapy—particularly intravenous immunoglobulin, corticosteroids, and interleukin-1-receptor antagonist (anakinra)—have been successfully used for treatment. Molecular mechanisms underlying MIS-C are not fully understood. Consiglio and colleagues6Consiglio CR Cotugno N Sardh F et al.The immunology of multisystem inflammatory syndrome in children with COVID-19.Cell. 2020; 183: 968-981.e7Summary Full Text Full Text PDF PubMed Scopus (626) Google Scholar showed that the pathophysiology of MIS-C is distinct from the cytokine storm of severe acute COVID-19 as well as from the inflammatory response of Kawasaki disease, in addition to finding evidence of autoantibody-mediated pathology. There is debate on the possibility and effect of vertical transmission of SARS-CoV-2 infection to the fetus.7Karimi-Zarchi M Neamatzadeh H Dastgheib SA et al.Vertical transmission of coronavirus disease 19 (COVID-19) from infected pregnant mothers to neonates: a review.Fetal Pediatr Pathol. 2020; 39: 246-250Crossref PubMed Scopus (293) Google Scholar, 8Egloff C Vauloup-Fellous C Picone O Mandelbrot L Roques P Evidence and possible mechanisms of rare maternal-fetal transmission of SARS-CoV-2.J Clin Virol. 2020; 128104447Crossref PubMed Scopus (91) Google Scholar, 9Diriba K Awulachew E Getu E The effect of coronavirus infection (SARS-CoV-2, MERS-CoV, and SARS-CoV) during pregnancy and the possibility of vertical maternal-fetal transmission: a systematic review and meta-analysis.Eur J Med Res. 2020; 25: 39Crossref PubMed Scopus (153) Google Scholar, 10Dong L Tian J He S et al.Possible vertical transmission of SARS-CoV-2 from an infected mother to her newborn.JAMA. 2020; 323: 1846-1848PubMed Google Scholar, 11Zeng H Xu C Fan J et al.Antibodies in infants born to mothers with COVID-19 pneumonia.JAMA. 2020; 323: 1848-1849PubMed Google Scholar A systematic review and meta-analysis found no conclusive evidence for in-utero transmission among 1316 pregnant women across 39 studies.9Diriba K Awulachew E Getu E The effect of coronavirus infection (SARS-CoV-2, MERS-CoV, and SARS-CoV) during pregnancy and the possibility of vertical maternal-fetal transmission: a systematic review and meta-analysis.Eur J Med Res. 2020; 25: 39Crossref PubMed Scopus (153) Google Scholar Isolated case reports from China have supported the possibility of vertical transmission.10Dong L Tian J He S et al.Possible vertical transmission of SARS-CoV-2 from an infected mother to her newborn.JAMA. 2020; 323: 1846-1848PubMed Google Scholar, 11Zeng H Xu C Fan J et al.Antibodies in infants born to mothers with COVID-19 pneumonia.JAMA. 2020; 323: 1848-1849PubMed Google Scholar Vivanti and colleagues12Vivanti AJ Vauloup-Fellous C Prevot S et al.Transplacental transmission of SARS-CoV-2 infection.Nat Commun. 2020; 113572Crossref PubMed Scopus (19) Google Scholar provided virological and pathological evidence for late-gestational maternal-fetal transmission of SARS-CoV-2 in one case. In a study on transplacental transfer of SARS-CoV-2 antibodies involving a cohort of 1471 mother–newborn dyads, Flannery and colleagues13Flannery DD Gouma S Dhudasia MB et al.Assessment of maternal and neonatal cord blood SARS-CoV-2 antibodies and placental transfer ratios.JAMA Pediatr. 2021; (published online Jan 29.)https://doi.org/10.1001/jamapediatrics.2021.0038Crossref Scopus (184) Google Scholar showed efficient transplacental transfer of IgG antibodies in 72 (87%) of 83 seropositive mothers. There is little information regarding the protection offered by maternal antibodies, as well as the possibility of hyperinflammatory responses in fetuses and neonates following antenatal exposure to SARS-CoV-2. One previous report indicated that potential MIS-C was suspected in a neonate presenting with persistent pulmonary hypertension, elevated inflammatory markers, and anti-SARS-CoV-2 IgG antibodies.14Khaund Borkotoky R Banerjee Barua P Paul SP Heaton PA COVID-19-related potential multisystem inflammatory syndrome in childhood in a neonate presenting as persistent pulmonary hypertension of the newborn.Pediatr Infect Dis J. 2021; (published online Jan 12.)DOI:10.1097/INF.0000000000003054Crossref PubMed Scopus (32) Google Scholar To the best of our knowledge, this Case Report is the first description of severe hyperinflammatory syndrome in a neonate presenting catastrophically with myocarditis, shock, and multiorgan dysfunction following proven prenatal exposure to COVID-19. The diagnosis is supported by presence of anti-SARS-CoV-2 IgG antibodies and the excellent response to immunomodulatory therapy. Detection of human rhinovirus in nasal-swab RT-PCR is likely to be incidental because it has not been reported to be associated with such presentation in neonates. Additional clinical clues in our case were the coronary findings (appendix p 2) and skin manifestations. Coronary vasculitis has been described in association with MIS-C, although neither specific nor diagnostic. The morphology and evolution of the skin lesions over the buttocks might indicate acro-ischaemia analogous to so-called COVID toes seen in older children in association with the coagulopathy and microangiopathy of severe COVID-19.15Calvão J Relvas M Pinho A Brinca A Cardoso JC Acro-ischaemia and COVID-19 infection: clinical and histopathological features.J Eur Acad Dermatol Venereol. 2020; 34: e653-e754Crossref PubMed Scopus (20) Google Scholar, 16Fernandez-Nieto D Jimenez-Cauhe J Suarez-Valle A et al.Characterization of acute acral skin lesions in nonhospitalized patients: a case series of 132 patients during the COVID-19 outbreak.J Am Acad Dermatol. 2020; 83: e61-e63Summary Full Text Full Text PDF PubMed Scopus (170) Google Scholar Young and colleagues17Young S Narang J Kumar S et al.Large sacral/buttocks ulcerations in the setting of coagulopathy: a case series establishing the skin as a target organ of significant damage and potential morbidity in patients with severe COVID-19.Int Wound J. 2020; 17: 2033-2037Crossref PubMed Scopus (14) Google Scholar have described three adult patients presenting with ulcerations on the buttocks in the setting of COVID-19 coagulopathy. MIS-C can occur in a neonate following in-utero exposure to SARS-CoV-2, resulting in multiple organ injury. This case highlights the need to better understand the effect of COVID-19 on the maternal–fetal dyad. It might have important implications for health-care professionals looking after peripartum women and their neonates, and also for the postnatal counselling and care of infants born with antenatal exposure to COVID-19. With pregnant women, globally, becoming more vulnerable to COVID-19 exposure because of relaxation of restrictions and setting in of pandemic fatigue, protecting the maternal–fetal dyad from SARS-CoV-2 through appropriate vaccination strategies and other measures might become an important public health need. Contributors MK and SB conceptualised and prepared the Case Report. SA, SM, SPL, SG, and JPJ contributed to the literature search, data collection, and data interpretation. SJ contributed to the literature search and manuscript preparation. AK helped with data collection, data interpretation, and microbiological investigations. MK, SB, and RKK had full access to all the data and verified it, and had final responsibility for the decision to submit for publication. AG and RKK supervised manuscript preparation and critically reviewed and edited the final manuscript. Declaration of interests We declare no competing interests. Acknowledgments Parent of the patient provided informed written consent for publication of clinical information and photographs. This Case Report was approved by the Department of Pediatric Cardiology of the Amrita Institute of Medical Sciences and Research Centre, Kochi, India, with a waiver of informed ethical approval. Download .pdf (.31 MB) Help with pdf files Supplementary appendix

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.