Characteristics, Cardiac Involvement, and Outcomes of Multisystem Inflammatory Syndrome of Childhood Associated with severe acute respiratory syndrome coronavirus 2 Infection.

We report on the presentation and course of 33 children with multisystem inflammatory syndrome in children and confirmed severe acute respiratory syndrome coronavirus 2 infection. Hemodynamic instability and cardiac dysfunction were prominent findings, with most patients exhibiting rapid resolution following anti-inflammatory therapy.

Physiologic diagnosis of congenital heart disease in cyanotic neonates.

PURPOSE OF REVIEW: We aim to improve diagnosis of congenital heart disease (CHD) with cyanosis by physiology for general practitioners to reduce time to appropriate treatment. RECENT FINDINGS: New implementation of the critical congenital heart disease (CCHD) pulse oximetry screen has improved rate of diagnosis of CHD in recent years. However, many infants with cyanotic heart lesions often decompensate before screening in the newborn nursery, or have lesions that are not amenable to pulse oximetry screening and that present later in the emergency room. Recent literature has shown preoperative acidosis because of delayed diagnosis of cyanotic CHD worsens outcomes postoperatively. Wide availability of prostaglandin therapy and catheter procedures help to preoperatively stabilize critical cardiac patients. With a firm grasp of the underlying physiology of neonatal cyanotic CHD, practitioners can appropriately implement these therapies more judiciously. This early recognition will subsequently improve overall outcomes. SUMMARY: Physiologic diagnosis of CHD with cyanosis by general practitioners will allow initiation of appropriate management more quickly and effectively. This may avoid progressive clinical decompensation and acidosis until cardiology consultation and potential intervention are available.

Comparison of Patients Undergoing Surgical Versus Transcatheter Pulmonary Valve Replacement: Criteria for Referral and Mid-Term Outcome.

Pulmonary regurgitation and/or stenosis (PS) is challenging in patients with congenital heart defects. Our aim was to identify if criteria for referral were different between surgical (SPVR) and transcatheter pulmonary valve replacement (TPVR) populations, and to further assess if any baseline differences influence the resultant ventricular remodeling at medium-term follow-up. Retrospective chart review of patients post-SPVR or TPVR at our center from 2013 to 2015 was conducted. Volumetric data from cardiac magnetic resonance (CMR), 1 year before and 1 year after PVR was obtained. PS was defined as peak-peak gradient ≥35 mmHg by catheterization or peak gradient ≥50 mmHg by echocardiography. Thirty patients underwent PVR: 15 SPVR and 15 TPVR (1 hybrid). The indications for SPVR referral were: 2+ CMR parameters in 80% of patients; decreased left ventricular ejection fraction and hemodynamic findings and/or abnormal exercise stress test in 20%. The indications for TPVR referral were predominantly symptoms ± hemodynamic findings in 66% of patients, 2+ CMR findings in 44% of patients. At referral, SPVR group had significantly larger right ventricular (RV) volumes than TPVR group. Biventricular function was not significantly different. Post-PVR, both groups had significantly decreased RV volumes and increased LV diastolic volumes. The SPVR group improved LV cardiac output and biventricular function whereas TPVR group had no significant improvement. The patients in the SPVR group were mostly referred based on CMR volumetric criteria, whereas the patients in the TPVR group were mostly referred due to exercise intolerance with only occasional abnormalities on CMR. 1 year after PVR, both groups had near-normal biventricular volumes and function irrespective of characteristics at referral.

Left main coronary artery stenting after cardiac arrest in an infant with William's syndrome.

A 6-month-old female with William's syndrome and biventricular outflow obstruction had a cardiac arrest due to myocardial ischemia soon after induction of anesthesia during cardiac catheterization. The patient was supported with extra corporeal membrane oxygenator (ECMO) and successful stenting of the left main coronary artery was performed as a rescue measure. This intervention allowed successful weaning from ECMO and subsequent discharge from the hospital. Coronary intervention in infants may be successfully performed in dire situations.

Six Month Follow-up of Patients With Multi-System Inflammatory Syndrome in Children.

BACKGROUND AND OBJECTIVES: Myocardial dysfunction and coronary abnormalities are prominent features of multisystem inflammatory syndrome in children (MIS-C). In this study we aim to evaluate the early and midterm outcomes of MIS-C. METHODS: This is a longitudinal 6-month cohort study of all children admitted and treated for MIS-C from April 17 to June 20, 2020. Patients were followed ∼2 weeks, 8 weeks, and 6 months postadmission, with those with coronary aneurysms evaluated more frequently. RESULTS: Acutely, 31 (62%) patients required intensive care with vasoactive support, 26 (52%) had left ventricular (LV) systolic dysfunction, 16 (32%) had LV diastolic dysfunction, 8 (16%) had coronary aneurysms (z score ≥2.5), and 4 (8%) had coronary dilation (z score <2.5). A total of 48 patients (96%) received immunomodulatory treatment. At 2 weeks, there was persistent mild LV systolic dysfunction in 1 patient, coronary aneurysms in 2, and dilated coronary artery in 1. By 8 weeks through 6 months, all patients returned to functional baseline with normal LV systolic function and resolution of coronary abnormalities. Cardiac MRI performed during recovery in select patients revealed no myocardial edema or fibrosis. Some patients demonstrated persistent diastolic dysfunction at 2 weeks (5, 11%), 8 weeks (4, 9%), and 6 months (1, 4%). CONCLUSIONS: Children with MIS-C treated with immunomodulators have favorable early outcomes with no mortality, normalization of LV systolic function, recovery of coronary abnormalities, and no inflammation or scarring on cardiac MRI. Persistence of diastolic dysfunction is of uncertain significance and indicates need for larger studies to improve understanding of MIS-C. These findings may help guide clinical management, outpatient monitoring, and considerations for sports clearance.

Resource Allocation and Decision Making for Pediatric and Congenital Cardiac Catheterization During the Novel Coronavirus SARS-CoV-2 (COVID-19) Pandemic: A U.S. Multi-Institutional Perspective.

BACKGROUND: The novel coronavirus (COVID-19) pandemic has placed severe stress on healthcare systems around the world. There is limited information on current practices in pediatric cardiac catheterization laboratories in the United States (US). OBJECTIVES: To describe current practice patterns and make recommendations regarding potential resource allocation for congenital cardiac catheterization during the COVID-19 pandemic. METHODS: A web-based survey was distributed regarding case candidacy and catheterization laboratory preparedness. Centers were categorized based on the current degree of disease burden in that community (as of April 1, 2020). Data and consensus opinion were utilized to develop recommendations. RESULTS: Respondents belonged to 56 unique US centers, with 27 (48.2%) located in counties with a high number of COVID-19 cases. All centers have canceled elective procedures. There was relative uniformity (>88% agreement) among centers as to which procedures were considered elective. To date, only three centers have performed a catheterization on a confirmed COVID-19 positive patient. Centers located in areas with a higher number of COVID-9 cases have been more involved in a simulation of donning and doffing personal protective equipment (PPE) than low-prevalence centers (46.7% vs 10.3%, respectively; P<.001). Currently, only a small fraction of operators has been reassigned to provide clinical services outside their scope of practice. CONCLUSIONS: At this stage in the COVID-19 pandemic, pediatric/congenital catheterization laboratories have dramatically reduced case volumes. This document serves to define current patterns and provides guidance and recommendations on the preservation and repurposing of resources to help pediatric cardiac programs develop strategies for patient care during this unprecedented crisis.

Promoting Pulmonary Arterial Growth via Right Ventricle-to-Pulmonary Artery Connection in Children With Pulmonary Atresia, Ventricular Septal Defect, and Hypoplastic Pulmonary Arteries.

BACKGROUND: Complete repair of pulmonary atresia (PA) ventricular septal defect (VSD) with hypoplastic or absent native pulmonary arteries, often with major aortopulmonary collateral arteries (MAPCAs), involves construction of an adequate sized pulmonary arterial tree. We report our results with a previously described staged strategy using initial right ventricle (RV)-to-reconstructed pulmonary arterial tree (RV-PA) connection to promote pulmonary arterial growth and facilitate later ventricular septation. METHODS: We retrospectively reviewed data for all patients (N = 10) with initial echocardiographic diagnosis of PA-VSD and hypoplastic pulmonary arteries operated in our center from October 2008 to August 2016. Pulmonary arterial vessel size measured on preoperative and postoperative angiography was used to calculate Nakata index. RESULTS: Seven patients had PA-VSD, three had virtual PA-VSD, and seven had MAPCAs. All underwent creation of RV-PA connection at a median age of 7.5 days and weight 3.6 kg. Eight patients had RV-PA conduits, two had a transannular patches, and seven had major pulmonary artery reconstruction simultaneously. There were no deaths or serious morbidity; one conduit required revision prior to complete repair. Complete repair with ventricular septation and RV pressure less than half systemic was achieved in all patients at a median age of 239 days. Nakata index in neonatal period was 54 mm2/m2 (range 15-144 mm2/m2) and at time of septation 184 mm2/m2 (range 56-510 mm2/m2; P = .004). Growth rates of right and left branch pulmonary arteries were similar. The 10 patients underwent 28 catheterizations with 13 interventions in 8 patients prior to full repair. CONCLUSION: Early palliative RV-PA connection promotes pulmonary arterial growth and facilitates eventual full repair with VSD closure with low RV pressure and operative risk.

Partial loading of the left ventricle during mechanical assist device support is associated with improved myocardial function, blood flow and metabolism and increased exercise capacity.

BACKGROUND: Myocardial recovery has been observed after placement of left ventricular assist devices in some patients awaiting cardiac transplantation. Left ventricular assist devices provide profound volume and pressure unloading while restoring systemic blood flow. However, the optimal degree of left ventricular unloading during left ventricular assist device support is unknown. The purpose of this study was to assess the effect of the degree of left ventricular decompression, during left ventricular assist device support, on myocardial function and exercise capacity. METHODS: Twenty patients with a left ventricular assist device performed cardiopulmonary exercise testing with simultaneous echocardiographic and hemodynamic measurements during full, and then partial, device support. Eleven patients underwent positron emission tomographic scanning for measurement of myocardial blood flow and oxygen consumption. RESULTS: Patients were divided into two groups based on the degree of left ventricular decompression as assessed by echocardiographic measurements. Patients with partially decompressed ventricles (n = 13, LVEDD = 4.8 +/- 1.0 cm) as compared to those with fully decompressed ventricles (n = 10, LVEDD = 3.0 +/- 0.3 cm) demonstrated significant improvements in: peak oxygen consumption (16.8 +/- 4.3 versus 12.8 +/- 3.0 ml/kg.min), native left ventricular index during exercise (2.5 +/- 1.4 versus 0.8 +/- 0.8 liters/min.m(2)); ability to exercise with the device weaned (10 of 13 versus 1 of 10 patients); resting myocardial blood flow (0.55 +/- 0.11 versus 0.21 +/- 0.13 ml/g.min); and myocardial oxygen consumption (0.04 +/- 0.01 versus 0.02 +/- 0.001 min-1) (all p < 0.05). CONCLUSIONS: These results suggest that partial loading of the left ventricle during left ventricular assist device support may be beneficial. Further study of optimal ventricular decompression during device support is needed, as this may be important in improving myocardial recovery.