Coronary Abnormalities Research Articles

Abstract 13796: Diffuse Regional Strain Abnormalities Despite Normal Ejection Fraction Suggestive of Occult Myocardial Dysfunction in Multisystem Inflammatory Syndrome in Children

Background: Multisystem Inflammatory Syndrome in Children (MIS-C) patients frequently present with significant hypotension and ventricular dysfunction. While they appear to recover quickly after treatment of MIS-C, long-term sequelae of the myocardium are unknown. We aimed to evaluate ventricular function longitudinally using TTE and CMR. Methods: Retrospective review was performed of all patients with MIS-C who underwent CMR from September 2020 to May 2021. Measures of ventricular size and function during admission, 2-week, and 6-week follow up by TTE as well as global and regional longitudinal strain, as measured by Strain ENCoded imaging (SENC) by CMR were analyzed. The magnitude of strain is expressed as an absolute value, with >17% considered normal. Results: Nineteen patients (13.9±1.9 years, 63% males) with MIS-C underwent CMR. Troponin leak was seen in 16/19 (84%), 15/19 (79%) had ventricular dysfunction, 3/19 (16%) had coronary abnormality, and 1/19 (5%) required ECMO. All patients had normalized function at 2- and 6-week visits (mean LVEF 62±6.3% on TTE at 6-weeks). Group 1 (n=2) had CMR during hospital admission (5, 11 days), Group 2 (n=13) during early outpatient follow-up (median 34 days, range 15-59 days), and Group 3 (n=5) during later outpatient follow-up (median 98 days, range 72-231 days). All patients had normal LVEF on CMR. LVGLS by SENC was significantly impaired throughout follow up. The mean LVGLS of Group 1, Group 2, and Group 3 were 15±0.8%, 15.4±2.4%, and 16.6±1.6%, respectively. The mean number LV segments (from the AHA modified 16 segment model) with abnormal LS in each group were 10±2, 9.2±3.2, and 8.6±2, respectively. Conclusions: There were significant global and regional strain abnormalities despite normalized LVEF on TTE and CMR. This represents occult myocardial dysfunction after MIS-C. It is unclear if these findings will persist. Longitudinal studies are needed to monitor for progression or resolution of these findings.

Abstract 13024: Excessive Slow Flow and Thick Boundary Layers Cause Non ST Segment Elevation in Patients With Patent Coronary Arteries

Introduction: Many patients arrived to emergency room with atypical chest pain, borderline ST segment elevation, troponin level significantly elevated however the coronary angiogram was normal. Why? Hypothesis: Based on damages by flow disturbances in pipes, our research is built on the hypothesis that flow abnormalities disrupt delivery of oxygenated blood to the myocardium. Our strategy is to use today’s practice of fluid mechanics to unlock the mechanism of non-ST Segment elevation myocardial infarction (non-NSTEMI) and patent coronary arteries. What coronary flow abnormalities were the culprit? Methods: Patients with non-STEMI were enrolled if the coronary arteries were patent. In our new dynamic angiographic protocol, contrast was injected until the index coronary artery was completely opacified. Once the manual injection stopped, the blood moved in, displacing the contrast. The movements and directions of the blood flow in “white” color on top of a “black contrast” background could be clearly analyzed. The main focuses were the types of flows and their duration, direction and length. Results: 12 patients with non-STEMI and patent coronary arteries were enrolled. 100% patients had severe slow flow with prolonged arterial phase (>1.8secs) compared with historical control (0.72 secs) (p<0.05). 83% patients had retention flow with thick stagnant boundary layers at the proximal and mid segment of right coronary artery. The retrograde flow was short (0.18secs). Two patients showed excessive contraction of left ventricle. 4 patients revealed history of intense emotional stress however no sign of adrenergic excess (elevated blood pressure, heart rate or extreme peripheral vasoconstriction). All patients recovered and remained asymptomatic after one year follow-up. Conclusions: In non-STEMI and patent coronary arteries, severe slow flow and prolonged retention of thick boundary layers were the culprits. Besides the ischemia caused by delayed delivery of new blood to myocardium, the prolonged retention of thick boundary layers could prevent the intima from absorbing new oxygenated blood and provoked local ischemia. These new discoveries open new management and preventive options for patients with non-STEMI and patent coronary arteries.

The coronary arteries in adults after arterial switch: a systematic review

Abstract Background Coronary artery status in adults longterm after the arterial switch operation (ASO) is unclear. As a consequence, current follow-up strategies for coronary assessment remain controversial. We conducted a systemic review to provide an overview of coronary complications during adulthood and to evaluate the value of coronary imaging in adults after ASO, in light of current guidelines. Material and method Studies describing coronary complications or coronary imaging after ASO in adults were considered eligible for review and analysis. Articles were screened for the inclusion of adult ASO patients and data on coronary complications and findings of coronary imaging were collected. In cohort studies with both adults (≥18 years) and non-adults (<18 years) only outcomes in identifiable adults were analyzed. Results A total of 993 adults were followed with a median follow-up of 2.0 years after reaching adulthood. Myocardial ischemia was suspected in 16/192 patients (6.8%). The number of coronary interventions was 4 (0.4%) and coronary death was reported in 4 (0.4%) patients. The following coronary abnormalities were found by routine coronary computer tomography CT (cCT): stenosis (4%), acute angle (40%), kinking (24%) and interaterial course (11%). No coronary events were reported during pregnancy (n=45). Conclusion The reported number of coronary interventions (0.4%) and of coronary death (0.4%) during a median follow-up of 2 years in 993 ASO adults is low. Coronary abnormalities including acute angle, kinking and interarterial course were commonly found by cCT. The 2020 European Society of Cardiology (ESC) guidelines state that routine screening for coronary pathologies is questionable. However, based on current findings and in line with the 2018 American ACC/AHA guidelines we suggest a baseline assessment of the coronary arteries in all adult ASO patients. Thereafter, an individualized coronary follow-up strategy, based upon coronary findings, is advisable. Funding Acknowledgement Type of funding sources: Foundation. Main funding source(s): Hartstichting Freedom from coronary complications

Sinus bradycardia as the initial manifestation of multisystem inflammatory syndrome in children

BackgroundWhile cardiovascular complications, including arrhythmias are now a recognized manifestation of Multisystem inflammatory syndrome in children (MIS-C), there are no reports of primary bradycardia preceding the clinical presentation. We sought to describe a case series of sinus bradycardia as an initial manifestation of MIS-C.MethodsWe included a series of 10 consecutive patients with confirmed COVID-19 who met WHO and CDC criteria for MIS-C, who developed sinus bradycardia with a heart rate measured in the awake state that was below the normal range for age for children, as an initial manifestation of the disease, in a prospective observational multicenter study. Patients underwent clinical, laboratory evaluation, ECG, Holter, telemetry, echocardiogram, chest X Ray, and a chest CT scan.ResultsOf the 10 patients included, 6 were male, with a mean age of 6.52±5.35 years, range 4 months to 14 years. All cases were Hispanic. Bradycardia was transient and did not merit treatment. Coronary abnormalities were noted in 6 cases; 4 patients had mild coronary ectasia; 9 patients had pericardial effusion with no evidence of tamponade. All patients had a mild clinical course; none had shock, heart failure, the need for mechanical ventilation, or died. All blood markers (Troponin, BNP, Platelet count, C-reactive protein, D-dimer, Ferritin) returned to normal levels by discharge/follow-up with a favorable outcome including resolution of coronary dilatation in all but 2 in which aneurysm persisted.TreatmentAll patients received steroids and low-weight-molecular heparin 10 patients, 8 aspirin and 8 intravenous immunoglobulins.ConclusionSinus bradycardia may be the initial manifestation of MIS-C, usually transient and mild. Physicians should be aware of this presentation.Funding AcknowledgementType of funding sources: None. Kid, MIS-C. Bradycardia/Atrial Rhythm

Slow flow or prolonged arterial phase in coronary arteries is the cause of ischemia or sudden death in patients with dilated cardiomyopathy

Abstract Background In patients with dilated cardiomyopathy (DCM), the patients could be symptomatic or presented with recurrent fluid overload, syncope or sudden cardiac death (SCD) while the coronary arteries were patent. The aim of our study was to use the coronary flow abnormalities to stratify the high risk symptomatic versus the low risk asymptomatic patient with DCM. Methods Consecutive patients with DCM were enrolled. Twenty patients with normal ejection fraction (EF) without coronary artery disease served as control. The study patients were checked for symptoms (fluid overload, syncope, SCD) and re-admission. All patients underwent a new coronary angiographic technique with injection of contrast until all the coronary arteries were completely filled. As the injection of contrast stopped, the blood in white color moved in and the blood movement could be clearly observed. The angiogram was recorded from the entry of blood flow until all the contrast was cleared. During the review, the investigators downloaded, selected the angiogram from the electronic medical record, tapped on the Key Image and used the Up and Down arrow to move the images, frame-by-frame. Each frame represented a 0.06-second recording. The duration of the arterial phase was calculated starting the time when the blood entered the ostium of the index artery until all the contrast disappeared from the distal vasculature. At the same time, an AI program was trained to measure the length of the arterial phase by Machine learning, supervised and unsupervised Deep Learning and Convoluted Neural Networks. (Figure 1) The AI programs compared the time when the arteries were full with contrast until there was no contrast left in the distal vasculature. (Figure 2) Results One hundred patients with DCM were consecutively enrolled. Twenty patients served as control. In the control group with normal flow and EF, the duration of the arterial phase was 24–30 frames (1.44 to 2 seconds). In the study group, seventy patients had extremely prolonged arterial phase (average of 120 frames or >8 seconds (p<0.05). These patients were very symptomatic and had recurrent hospitalizations. Thirty patients had normal arterial phase of <2 seconds. These patients had shorter length of stay (<3 days), became asymptomatic after only 2 days of treatment and had rare readmission. (p<0.05) The AI programs confirmed the results of the arterial phases calculated manually by junior investigators. Conclusions In patients with DCM, the extreme prolonged arterial phase caused ischemia in the myocardium even there was no coronary artery disease. This ischemic burden triggered recurrent ventricular dysfunction, arrhythmia, syncope and SCD. The patients with normal arterial phase became asymptomatic after optimal medical treatment. With these results, more effective prevention and management could be achieved for high risk symptomatic patients with high mortality and readmissions. Funding Acknowledgement Type of funding sources: None. U Net architectureArterial Phase

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

ObjectivesTo 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...

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

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. 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. 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%). 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.

Myocardial bridge analysis by multidetector computed tomography and its association with coronary atherosclerosis

Abstract Background Myocardial Bridging an inborn coronary abnormality, is defined as a segment of a major epicardial coronary artery, the tunneled artery, that goes intramurally through the myocardium beneath the muscle bridge. Objective To evaluate noninvasively the presence and distribution of atherosclerotic plaques in relation to myocardial bridge coronary segments and to determine the prevalence of myocardial bridges and their location and morphology by using MDCT. Patients and Methods The study population consisted of 55 patients presented with chest pain, referred for MDCT coronary angiography and found to have myocardial bridge at during the period from April 2018 and August 2019. 23 patients was found having Myocardial Bridge and coronary artery atherosclerotic plaques included in Group (A), 29 persons found having Myocardial Bridge without CAD included in Group (B). All patients were subjected to detailed history taking, clinical evaluation, ECG analysis and MSCT coronary angiography. Results In the present study, patients with atherosclerotic plaque (group A) (n = 29), mean age was 52.41 ± 10.55 years ranged from 35.0 to 73.0 years, 41.4% were males, while in Myocardial bridge without atherosclerotic lesion group (group B) (n = 26), mean age was 51.65 ± 7.58 ranged from 38.0 to 65.0 years., 30.8% were males. Conclusion Our study showed that diabetes mellitus and dyslipidemia are a significant risk factor for developing atherosclerotic plaque in the segment proximal to myocardial bridge. Myocardial bridge were usually located over the Mid segment of the left anterior descending coronary artery

Low-intensity pulsed ultrasound therapy suppresses coronary adventitial inflammatory changes and hyperconstricting responses after coronary stent implantation in pigs in vivo.

We demonstrated that coronary adventitial inflammation plays important roles in the pathogenesis of drug-eluting stent (DES)-induced coronary hyperconstricting responses in pigs in vivo. However, no therapy is yet available to treat coronary adventitial inflammation. We thus developed the low-intensity pulsed ultrasound (LIPUS) therapy that ameliorates myocardial ischemia by enhancing angiogenesis. We aimed to examine whether our LIPUS therapy suppresses DES-induced coronary hyperconstricting responses in pigs in vivo, and if so, what mechanisms are involved. Sixteen normal male pigs were randomly assigned to the LIPUS or the sham therapy groups after DES implantation into the left anterior descending (LAD) coronary artery. In the LIPUS group, LIPUS (32 cycles, 193 mW/cm2) was applied to the heart at 3 different levels (segments proximal and distal to the stent edges and middle of the stent) for 20 min at each level for every other day for 2 weeks. The sham therapy group was treated in the same manner but without LIPUS. At 4 weeks after stent implantation, we performed coronary angiography, followed by immunohistological analysis. Coronary vasoconstricting responses to serotonin in LAD at DES edges were significantly suppressed in the LIPUS group compared with the sham group. Furthermore, lymph transport speed in vivo was significantly faster in the LIPUS group than in the sham group. Histological analysis at DES edges showed that inflammatory changes and Rho-kinase activity were significantly suppressed in the LIPUS group, associated with eNOS up-regulation and enhanced lymph-angiogenesis. These results suggest that our non-invasive LIPUS therapy is useful to treat coronary functional abnormalities caused by coronary adventitial inflammation, indicating its potential for the novel and safe therapeutic approach of coronary artery disease.

Pathophysiology and Diagnosis of Coronary Functional Abnormalities.

Approximately one-half of patients undergoing diagnostic coronary angiography for angina have no significant coronary atherosclerotic stenosis. This clinical condition has recently been described as ischaemia with non-obstructive coronary arteries (INOCA). Coronary functional abnormalities are central to the pathogenesis of INOCA, including epicardial coronary spasm and coronary microvascular dysfunction composed of a variable combination of increased vasoconstrictive reactivity and/or reduced vasodilator function. During the last decade – in INOCA patients in particular – evidence for the prognostic impact of coronary functional abnormalities has accumulated and various non-invasive and invasive diagnostic techniques have enabled the evaluation of coronary vasomotor function in a comprehensive manner. In this review, the authors briefly summarise the recent advances in the understanding of pathophysiology and diagnosis of epicardial coronary artery spasm and coronary microvascular dysfunction.

Non coronary applications of cardiac computed tomography: A review

Acquired heart diseases including valvular pathologies and conduction abnormalities, along with coronary artery disease make cardiovascular disease one of the major causes of mortality and morbidity worldwide. Advances in cardiac computed tomography (CCT) have led to markedly improved image quality for assessment of several coronary and noncoronary cardiac abnormalities. With the latest versions of CT scanners, image acquisition can be completed within a few seconds, in a single breath hold and with much less radiation exposure, thus making CT an even more attractive diagnostic tool with its high temporal and excellent spatial resolution. Additional advantages are its noninvasive nature, wide availability, fast image acquisition and ability to provide additional data about the cardiac structure, function, valvular motion, and presence or absence of valvular vegetation, mass or intracardiac thrombus. These factors can result in change in management in many valvular pathologies pre- and post-intervention, and in electrophysiological procedures. The goal of this article is to review applications of cardiac CT in non-coronary indications including valvular assessment, pulmonary vein isolation procedure, and left atrial appendage evaluation for its transcatheter occlusion.

Late myocardial infarct in pulmonary atresia and intact ventricular septum: A must-know complication!

Pulmonary atresia with intact ventricular septum is a rare congenital heart disease. Frequently associated coronary abnormalities impact on acute management but may also complicate long-term clinical course and worsen prognosis. Little is known about their clinical spectrum and management. An 8-year-old girl, born with pulmonary atresia and intact ventricular septum, who uneventfully achieved Fontan completion, presented with chest pain and high troponinemia. Electrocardiogram (ECG) showed significant acute repolarization anomalies ( Fig. 1 ). Cardiac magnetic resonance imaging (MRI) showed ischemic sequelae in the territory of the right coronary artery ( Fig. 2 ). Coronarography showed an abnormal diffuse collateral coronary network but no stenotic lesions of the main coronaries ( Fig. 3 ). Medical treatment was initiated with good recovery. Coronary abnormalities are frequently encountered in patients with atresia pulmonary with intact ventricular septum [1] . Abnormal sinusoids, fistulas but also diffuse modifications of the vessel walls are described who may significantly impact on neonatal management [2] , [3] . Our case report highlights the possibility of late coronary complications that invariably will impact on long-term prognosis. Close follow-up is warranted in all patients with pulmonary atresia and intact ventricular septum in order to detect ischemic events and treat them appropriately. The risk of ventricular arrhythmias and ventricular dysfunction may require the introduction of appropriate medical treatment or even more aggressive interventions such as implantable cardioverter-defibrillator or cardiac transplantation.

Kawasaki disease in children younger than 6months of age: characteristics of a Spanish cohort.

A retrospective study that compared children younger than 6months versus older children of a Spanish cohort of patients diagnosed with Kawasaki disease between 2011 and 2016 (Kawa-Race study). From the 598 patients recruited, 42 patients were younger than 6months (7%) and presented more frequently with an incomplete diagnosis of Kawasaki disease (52.4 vs 27.9%, p = 0.001). Cardiac abnormalities detected by echocardiography were more common in younger patients (52.4 vs 30%, p = 0.002). These younger patients presented with a higher proportion of coronary aneurysms as well (19 vs 8.6%, p < 0.001). Shock at diagnosis (9.5 vs 1.9%, p = 0.016) and admission to intensive care units (17.7 vs 4.1%, p = 0.003) were more frequent in patients younger than 6months. There were no statistically significant differences in relation to infections, non-response to IVIG, or mid- or long-term outcomes.Conclusion: Data of the Spanish cohort are consistent with other American and Asian studies, although Spanish children younger than 6months had a lower rate of non-response to IVIG and better clinical outcomes. A high index of suspicion should be considered for this population due to a higher risk of coronary abnormalities, presentation of shock, and admission to the intensive care unit. What is Known: •Children below 6 months of age with Kawasaki disease (KD) have different features compared to older. •Younger patients usually have an incomplete form of KD and coronary artery abnormalities. What is New: •Younger than 6 months with KD presented with shock and required admission to PICU more frequently compared to older. •Infections play a similar role in KD despite the age of the patients.

Surgical intervention and outcome for treatment of myocardial bridging refractory to medication.

Myocardial bridging (MB) is a common and usually benign inborn coronary abnormality that may lead to anginal symptoms, acute coronary syndrome, arrhythmias, and rarely sudden cardiac death. MB are most commonly localized in the middle segment of the left anterior descending coronary artery (LAD). The treatment of LAD-MB is still challenging. Our objective was to assess the short- and long-term results of surgical procedures in patients with LAD-MB who had chest pain refractory to medical therapy. Between March 2005 and January 2020, 26 patients (19 males and 7 females; mean 55.8 ± 12.4 years) with MB underwent surgery. All MB was located in the mid-segment of the LAD with a mean length of 4.2 ± 1.7 cm. Coronary angiography before surgery demonstrated LAD-MB with systolic compression more than or equal to 70% in all patients. Twenty-five patients underwent myotomy and one patient underwent coronary artery bypass grafting (CABG). All patients survived and recovered uneventfully. Neither hospital or late death nor major complications occurred. Follow-up time was 3-173 months (mean 55.7 months). Follow-up of coronary angiography or computed tomography scan performed in 16 patients demonstrated restoration of coronary blood flow and myocardial perfusion without significant residual compression of the artery. All patients were symptom-free and are currently in NYHA Class I. The symptomatic LAD-MB patients who are refractory to medication should actively undergo the surgical intervention such as myotomy and CABG to eliminate the clinical symptoms and achieve satisfactory results by follow-up findings. Myotomy is a preferred procedure because of its safety and satisfactory results.

Contrast Echocardiography for the Detection of a Giant Right Coronary Artery Aneurysm: A Useful Diagnostic Tool

Coronary artery aneurysm is an uncommon condition defined as abnormal dilatation of the coronary artery. We report the case of a man admitted to the Emergency Department for suspected intestinal obstruction. At abdominal/thoracic Computed Tomography (CT) it was reported a suspected pericardial cyst. Contrast echocardiography showed a clear pattern of blood flow inside the mass with a probable origin at a very limited region where it was also possible to sample an ECG-synchronized system-diastolic Pulse-Wave (PW)-Doppler pattern, typical for a coronary artery flow. Our suspicion of a giant coronary artery aneurysm was then confirmed by coronary CT and coronary angiography. The patient was transferred to a Cardiac Surgery center where he underwent surgical treatment for the aneurysm. Most coronary aneurysms are clinically silent and are often incidentally detected. Coronary angiography is the ‘‘gold standard’’ for diagnosis and evaluation of coronary artery aneurysms. Among non-invasive diagnostic techniques, Computed Tomography Coronary Angiography is the best method for coronary artery anatomy and coronary abnormalities. However, echocardiography with the use of a contrast agent, a cost-effective non-radiation nature, and a widespread use method, maybe a reliable method to detect and characterize the masses, allowing a differential diagnosis. Learning objective 1. Recognition and evaluation of cardiac masses require first-line imaging methods such as echocardiography. 2. The use of additional non-invasive (cardiac CT or MRI) and/or invasive imaging methods are often required. 3. Contrast echocardiography may be helpful, as second-line imaging methods, to better characterize the mass and approach the correct diagnosis.

Entrapped Coronary Arteries in Constrictive Pericarditis.

Pericardial constriction can be present without pericardial calcium and often without pericardial thickening. This epicardial coronary artery motion abnormality due to entrapment in a thickened, fibrotic pericardium, is characteristic of constrictive pericarditis, and differentiates this entity from other close differential diagnoses, such as restrictive and dilated cardiomyopathy.

Longitudinal Outcomes for Multisystem Inflammatory Syndrome in Children.

In spring 2020, a novel hyperinflammatory process associated with severe acute respiratory syndrome coronavirus 2 multisystem inflammatory syndrome in children (MIS-C) was described. The long-term impact remains unknown. We report longitudinal outcomes from a New York interdisciplinary follow-up program. All children <21 years of age, admitted to NewYork-Presbyterian with MIS-C in 2020, were included. Children were followed at 1 to 4 weeks, 1 to 4 months, and 4 to 9 months postdischarge. In total, 45 children were admitted with MIS-C. The median time to last follow-up was 5.8 months (interquartile range 1.3-6.7). Of those admitted, 76% required intensive care and 64% required vasopressors and/or inotropes. On admission, patients exhibited significant nonspecific inflammation, generalized lymphopenia, and thrombocytopenia. Soluble interleukin (IL) IL-2R, IL-6, IL-10, IL-17, IL-18, and C-X-C Motif Chemokine Ligand 9 were elevated. A total of 80% (n = 36) had at least mild and 44% (n = 20) had moderate-severe echocardiographic abnormalities including coronary abnormalities (9% had a z score of 2-2.5; 7% had a z score > 2.5). Whereas most inflammatory markers normalized by 1 to 4 weeks, 32% (n = 11 of 34) exhibited persistent lymphocytosis, with increased double-negative T cells in 96% of assessed patients (n = 23 of 24). By 1 to 4 weeks, only 18% (n = 7 of 39) had mild echocardiographic findings; all had normal coronaries. At 1 to 4 months, the proportion of double-negative T cells remained elevated in 92% (median 9%). At 4 to 9 months, only 1 child had persistent mild dysfunction. One had mild mitral and/or tricuspid regurgitation. Although the majority of children with MIS-C present critically ill, most inflammatory and cardiac manifestations in our cohort resolved rapidly.

Coronary Microvascular Dysfunction in Systemic Lupus Erythematosus.

BackgroundSystemic lupus erythematosus (SLE) is a systemic autoimmune inflammatory disorder associated with premature atherosclerosis and increased cardiovascular risk. Systemic inflammation is an emerging risk factor for coronary microvascular dysfunction (CMD). We aimed to test whether CMD, defined as abnormal myocardial flow reserve (MFR) by positron emission tomography‐computed tomography, would be independently associated with SLE after adjusting for nonobstructive atherosclerotic burden and common cardiovascular risk factors.Methods and ResultsConsecutive patients with SLE who underwent symptom‐prompted stress cardiac positron emission tomography‐computed tomography were included (n=42). Obstructive coronary artery disease and systolic dysfunction were excluded. MFR was quantified by positron emission tomography‐computed tomography, and CMD was defined as MFR <2. We frequency matched patients who did not have SLE and had symptom‐prompted positron emission tomography studies on age, sex, and key cardiovascular risk factors (n=69). The attenuation correction computed tomography scans were reviewed for qualitative assessment of coronary artery calcium. Patients with SLE had a more severe reduction in global MFR compared with controls and a higher prevalence of CMD, despite a similar degree of nonobstructive atherosclerotic burden (1.91±0.5 versus 2.4±0.7, respectively, P<0.0001; CMD, 57.1% versus 33.3%, respectively, P=0.017).ConclusionsWe demonstrated that patients with SLE with cardiac symptoms without obstructive coronary artery disease have a high prevalence of coronary vasomotor abnormalities. In comparison with symptomatic matched controls, patients with SLE have a more severe reduction in MFR that is not accounted for by common cardiovascular factors or atherosclerotic burden.

Evaluation of Coronary Circulation after Arterial Switch Operation.

ResumoFundamentoA avaliação da artéria coronária após a cirurgia de Jatene ainda é um desafio clínico.ObjetivoCorrelacionar alterações anatômicas identificadas por tomografia computadorizada cardíaca (TCC) com alterações fisiológicas detectadas na avaliação clínica para diagnosticar obstrução coronária no pós-operatório tardio de pacientes submetidos à cirurgia de Jatene.MétodosEste estudo incluiu 61 pacientes consecutivos com idade média de 9,4 anos que foram submetidos à cirurgia de Jatene. Os pacientes realizaram ecocardiografia, eletrocardiografia, teste cardiopulmonar do exercício, e tomografia computadorizada cardíaca para avaliação da capacidade funcional e anatomia da artéria coronária.ResultadosA tomografia computadorizada cardíaca revelou que somente 3,3% dos pacientes apresentaram estenose da artéria coronária. Esses pacientes eram assintomáticos, e não foram detectados sinais de isquemia miocárdicas pelos exames realizados.ConclusãoA incidência de anormalidades da artéria coronária é de 3,3% no seguimento tardio de nossa coorte de pacientes submetidos à cirurgia de Jatene. Não existe uma diretriz clara sobre o porquê, quando, e como esses pacientes deveriam ser rastreados, ou o que propor quando pacientes assintomáticos forem diagnosticados com obstrução coronária.

Clinical Profile and Short-Term Outcome of Children With SARS-CoV-2 Related Multisystem Inflammatory Syndrome (MIS-C) Treated With Pulse Methylprednisolone

ObjectiveTo study the clinical profile and outcome of children with MIS-C treated with methylprednisolone pulse therapy and/or intravenous immunoglobulin (IVIG).MethodThis prospective observational study included children satisfying CDC MIS-C criteria admitted from September to November, 2020. Primary outcome was persistence of fever beyond 36 hours after start of immunomodulation therapy. Secondary outcomes included duration of ICU stay, mortality, need for repeat immunomodulation, time to normalization of CRP and persistence of coronary abnormalities at 2 weeks.ResultsStudy population included 32 patients with MIS-C with median (IQR) age of 7.5 (5–9.5) years. The proportion of children with gastrointestinal symptoms was 27 (84%), cardiac was 29 (91%) and coronary artery dilatation was 11 (34%). Pulse methylprednisolone and intravenous immunoglobulin were used as first line therapy in 26 (81%), and 6 (19%) patients, respec-tively. Treatment failure was observed in 2/26 patients in methylprednisolone group and 2/6 patients in IVIG group. C-reactive protein levels less than 60mg/L by day 3 was seen in 17(74%) in methylprednisolone group and 2 (25%) in IVIG group (P=0.014). There was no mortality. At 2 weeks follow-up coronary artery dilatation persisted in 4 in methylprednisolone group and 1 in IVIG group.ConclusionIn patients with SARS-CoV-2 related MIS-C, methylprednisolone pulse therapy was associated with favorable short-term outcomes.