Coronary Artery Abnormalities Research Articles

면역 글로불린 저항성을 보인 5명의 가와사키병 환아에서의 혈장 교환술을 통한 치료

The efficacy of high-dose intravenous immunoglobulin (IVIG) treatment for acute-stage Kawasaki disease patients has become widely recognized. However, in the case of refractory Kawasaki disease, alternative treatment should be considered to prevent coronary artery abnormality (CAA). Here, we present several cases of Kawasaki disease in children treated with plasma exchange (PE). We retrospectively reviewed the medical records of 5 subjects who had been unresponsive to IVIG treatments and treated with PE consecutively. The fever subsided and the other clinical manifestations were improved after PE in all cases. In two cases, giant coronary artery aneurysms were detected on echocardiographic follow-up study. PE should be a method with promising efficacy and might be considered in refractory Kawasaki disease patients for whom other treatment options have failed.

Characteristics and outcomes of multisystem inflammatory syndrome in children: A multicenter, retrospective, observational cohort study in Mexico.

Multisystem inflammatory syndrome in children associated with coronavirus disease 2019 (MIS-C), a novel hyperinflammatory condition secondary to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, is associated with severe outcomes such as coronary artery aneurysm and death. This multicenter, retrospective, observational cohort study including eight centers in Mexico, aimed to describe the clinical characteristics and outcomes of patients with MIS-C. Patient data were evaluated using latent class analysis (LCA) to categorize patients into three phenotypes: toxic shock syndrome-like (TSSL)-MIS-C, Kawasaki disease-like (KDL)-MIS-C, and nonspecific MIS-C (NS-MIS-C). Risk factors for adverse outcomes were estimated using multilevel mixed-effects logistic regression. The study included 239 patients with MIS-C, including 61 (26%), 70 (29%), and 108 (45%) patients in the TSSL-MIS-C, KDL-MIS-C, and NS-MIS-C groups, respectively. Fifty-four percent of the patients were admitted to the intensive care unit, and 42%, 78%, and 41% received intravenous immunoglobulin, systemic glucocorticoids, and anticoagulants, respectively. Coronary artery dilatation and aneurysms were found in 5.7% and 13.2% of the patients in whom coronary artery diameter was measured, respectively. Any cause in-hospital mortality was 5.4%. Hospitalization after ten days of symptoms was associated with coronary artery abnormalities (odds ratio [OR] 1.6, 95% confidence interval [CI] 1.2-2.0). Age ≥10 years (OR: 5.6, 95% CI: 1.4-2.04), severe underlying condition (OR: 9.3, 95% CI: 2.8-31.0), platelet count <150,000 /mm3 (OR: 4.2, 95% CI: 1.2-14.7), international normalized ratio >1.2 (OR: 3.8, 95% CI: 1.05-13.9), and serum ferritin concentration >1,500 mg/dl at admission (OR: 52, 95% CI: 5.9-463) were risk factors for death. Mortality in patients with MIS-C was higher than reported in other series, probably because of a high rate of cases with serious underlying diseases.

Microvascular changes on nailfold capillaroscopy in acute stage of Kawasaki disease: a new diagnostic paradigm for an enigmatic condition.

Kawasaki disease (KD) is a medium vessel vasculitis with a predilection to involve coronary arteries. However, there is a paucity of literature on microvascular changes in patients with KD. Children diagnosed with KD based on American Heart Association guidelines 2017 were enrolled prospectively. Demographic details and echocardiographic changes in coronaries were recorded. Nailfold capillaries were assessed using Optilia Video capillaroscopy and data were analysed using Optilia Optiflix Capillaroscopy software at acute (prior to IVIG administration) and subacute/convalescent phase. We enrolled 32 children with KD (17 boys) with a median age of 3 years. Nailfold capillaroscopy (NFC) was performed in 32 patients in the acute phase (compared with 32 controls) and in 17 during the subacute/convalescent phase at a median follow-up of 15 (15-90) days after IVIG treatment. The following findings were seen in NFC in the acute phase of KD: reduced capillary density (n = 12, 38.6%), dilated capillaries (n = 3, 9.3%), ramifications (n = 3, 9.3%) and capillary haemorrhages (n = 2, 6.2%). Capillary density was reduced significantly in the acute phase of KD (38.6%) as compared with the subacute/convalescent phase (25.4%) (P-value <0.001) and controls (0%) (P-value = 0.03). We observed no correlation between coronary artery involvement and mean capillary density (P = 0.870). Results show that patients with KD have significant nailfold capillary changes in the acute phase. These findings may provide a new diagnostic paradigm for KD and a window to predict coronary artery abnormalities.

Novel Score to Predict Immunoglobulin Resistance in Kawasaki Disease.

To evaluate existing scoring systems and develop a new model to predict intravenous immunoglobulin (IVIG) resistance in patients with Kawasaki disease (KD). A retrospective cohort study performed between 2004 and 2017 identified 115 patients treated with IVIG for classic or incomplete KD. In our practice, IVIG resistance was defined as fever for > 24h and patients were divided into responders and non-responders. A univariate analysis was performed to identify independent predictors of IVIG resistance. The predictors were combined into a new scoring system and compared with existing scoring systems. Sixty-five patients had classic KD and 50 had incomplete KD. Among the 115 patients, 80 (69.6%) responded and the remaining 35 were resistant (30.4%) to IVIG. Of the 35 resistant patients, 16 patients had incomplete KD. Hispanic children comprised 43% of our sample population. Coronary artery abnormalities developed in 14 of the 35 IVIG-resistant patients (39%). Univariate analysis showed that IVIG-resistant patients were older and present with lower platelets, potassium, and creatinine (P < 0.05). Multivariate logistic regression analysis used platelets, potassium, body surface area (BSA), and creatinine to devise the Las Vegas Scoring System (LVSS), which demonstrated a sensitivity of 76.2% and a specificity of 68.6%. Compared to published data, we observed a higher rate of IVIG resistance and coronary artery abnormalities in our patient population. The LVSS (using platelets, potassium, BSA, and creatinine) showed higher specificity and comparable sensitivity to other scoring systems devised to predict IVIG resistance.

Kawasaki disease in children with Bacillus Calmette-Guérin scar reactivity: Focus on coronary outcomes

Background/Purpose: Reactivity at the Bacillus Calmette-Guérin (BCG) scar is a pathognomonic feature of Kawasaki disease (KD). However, its value in predicting KD outcomes has not been emphasized. This study explored the clinical significance of BCG scar redness with respect to coronary artery outcomes. MethodsThis retrospective study collected data on children with KD from 13 hospitals in Taiwan during 2019–2021. Children with KD were categorized into four groups based on the KD type and BCG scar reactivity. Risk factors of coronary artery abnormalities (CAA) were analyzed in all groups. ResultsBCG scar redness occurred in 49% of 388 children with KD. BCG scar redness was associated with younger age, early intravenous immunoglobulin (IVIG) treatment, hypoalbuminemia, and CAA at the first echocardiogram (p < 0.01). BCG scar redness (RR 0.56) and pyuria (RR 2.61) were independent predictors of any CAA within 1 month (p < 0.05). Moreover, pyuria (RR 5.85, p < 0.05) in children with complete KD plus BCG scar redness was associated with CAA at 2–3 months; first IVIG resistance (RR 15.2) and neutrophil levels ≥80% (RR 8.37) in children with complete KD plus BCG scar non-redness were associated with CAA at 2–3 months (p < 0.05). We failed to detect any significant risk factors of CAA at 2–3 months in children with incomplete KD. ConclusionBCG scar reactivity contributes to diverse clinical features in KD. It can be effectively applied to determine the risk factors of any CAA within 1 month and CAA at 2–3 months.

Six-month cardiac outcomes in children with multisystem inflammatory syndrome in children.

Multisystem inflammatory syndrome in children is a rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to assess the long-term sequelae, particularly cardiac, in a large, diverse population. We performed a retrospective cohort study of all children (aged 0-20 years, n = 304) admitted to a tertiary care centre with a diagnosis of multisystem inflammatory syndrome in children from March 1, 2020 to August 31, 2021 and had at least one follow-up visit through December 31, 2021. Data were collected at hospitalisation, 2 weeks, 6 weeks, 3 months, and 1 year after diagnosis, where applicable. Cardiovascular outcomes included left ventricular ejection fraction, presence or absence of pericardial effusion, coronary artery abnormalities, and abnormal electrocardiogram findings. Population was median age 9 years (IQR 5-12), 62.2% male, 61.8% African American (AA), and 15.8% Hispanic. Hospitalisation findings included abnormal echocardiogram 57.2%, mean worst recorded left ventricular ejection fraction 52.4% ± 12.4%, non-trivial pericardial effusion 13.4%, coronary artery abnormalities 10.6%, and abnormal ECG 19.6%. During follow-up, abnormal echocardiogram significantly decreased to 6.0% at 2 weeks and 4.7% at 6 weeks. Mean left ventricular ejection fraction significantly increased to 65.4% ± 5.6% at 2 weeks and stabilised. Pericardial effusion significantly decreased to 3.2% at 2 weeks and stabilised. Coronary artery abnormalities significantly decreased to 2.0% and abnormal electrocardiograms significantly decreased to 6.4% at 2 weeks and stabilised. Children with multisystem inflammatory syndrome in children have significant echocardiographic abnormalities during the acute presentation, but these findings typically improve within weeks. However, a small subset of patients may have persistent coronary abnormalities.

Prediction Models for Intravenous Immunoglobulin Resistance in Kawasaki Disease: A Meta-analysis.

Approximately 10% to 20% of patients with Kawasaki disease (KD) are refractory to initial intravenous immunoglobulin (IVIG) therapy. KD is mainly associated with coronary artery abnormalities. To identify and evaluate all developed prediction models for IVIG resistance in patients with KD and synthesize evidence from external validation studies that evaluated their predictive performances. PubMed Medline, Dialog Embase, the Cochrane Central Register of Controlled Trials, the World Health Organization International Clinical Trials Registry Platform, and ClinicalTrials.gov were searched from inception until October 5, 2021. All cohort studies that reported patients diagnosed with KD who underwent an initial IVIG of 2 g/kg were selected. Study and patient characteristics and model performance measures. Two authors independently extracted data from the studies. The Kobayashi, Egami, Sano, Formosa, and Harada scores were the only prediction models with 3 or more external validation of the161 model analyses in 48 studies. The summary C-statistics were 0.65 (95% confidence interval [CI]: 0.57-0.73), 0.63 (95% CI: 0.55-0.71), 0.58 (95% CI: 0.55-0.60), 0.50 (95% CI: 0.36-0.63), and 0.63 (95% CI: 0.44-0.78) for the Kobayashi, Egami, Sano, Formosa, and Harada models, respectively. All 5 models showed low positive predictive values (0.14-0.39) and high negative predictive values (0.85-0.92). Potential differences in the characteristics of the target population among studies and lack of assessment of calibrations. None of the 5 prediction models with external validation accurately distinguished between patients with and without IVIG resistance.

Cardiac Involvement in Patients with Multisystem Inflammatory Syndrome in Children (MIS-C) in Poland.

Multisystem inflammatory syndrome in children (MIS-C) is an immune-mediated complication of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Cardiovascular system is commonly involved. Acute heart failure (AHF) is the most severe complication of MIS-C, leading to cardiogenic shock. The aim of the study was to characterise the course of MIS-C with a focus on cardiovascular involvement, based on echocardiographic (echo) evaluation, in 498 children (median age 8.3 years, 63% boys) hospitalised in 50 cities in Poland. Among them, 456 (91.5%) had cardiovascular system involvement: 190 (48.2%) of patients had (most commonly atrioventricular) valvular insufficiency, 155 (41.0%) had contractility abnormalities and 132 (35.6%) had decreased left ventricular ejection fraction (LVEF < 55%). Most of these abnormalities improved within a few days. Analysis of the results obtained from two echo descriptions (a median of 5 days apart) revealed a >10% increase in LVEF even in children with primarily normal LVEF. Lower levels of lymphocytes, platelets and sodium and higher levels of inflammatory markers on admission were significantly more common among older children with contractility dysfunction, while younger children developed coronary artery abnormality (CAA) more often. The incidence of ventricular dysfunction might be underestimated. The majority of children with AHF improved significantly within a few days. CAAs were relatively rare. Children with impaired contractility as well as other cardiac abnormalities differed significantly from children without such conditions. Due to the exploratory nature of this study, these findings should be confirmed in further studies.

The importance of color doppler echocardiography and advanced imaging in evaluation of pediatric cardiac symptoms

Congenital coronary artery anomalies (CCAs) are rare and can be very challenging to diagnose. We present 4 cases of abnormal coronary arteries origins, a child with a left main coronary artery (LMCA) from the right coronary artery (RCA) and 3 others with an RCA from the LMCA.

Multisystem inflammatory syndrome in children: A dysregulated autoimmune disorder following COVID-19.

Multisystem inflammatory syndrome in children (MIS-C) is a dysregulated autoimmune-mediated illness in genetically susceptible patients following COVID-19 with an interval of 2-6 weeks. The median age of patients with MIS-C is 6-11 years. Most common manifestations are involvement of gastrointestinal tract, cardiovascular system, hematological system, and mucocutaneous system. Respiratory tract, neurological system, musculoskeletal system, and kidney are less frequently affected. Mucocutaneous manifestations and coronary artery abnormalities characteristic for Kawasaki disease (KD) may be observed in a significant proportion of MIS-C patients that may make the differential diagnosis be difficult for some patients, especially in the post-pandemic era. The mortality rate is 1-3%. Management and prognosis of MIS-C are similar to that of KD. MIS-C and KD may share a common pathogenic process. Based on the observation of MIS-C-like illness in uninfected neonates, i.e. multisystem inflammatory syndrome in neonates, both MIS-C and KD may be a consequence of dysregulated, over-exaggerated humoral immune responses triggered by a specific infectious agent.

Clinical profile and outcome of cardiac manifestations in patients presenting with multisystem inflammatory syndrome in children associated with SARS-CoV-2 infection.

Multisystem inflammatory syndrome in children (MIS-C) can cause significant morbidity and mortality in children. This study was conducted to assess the pattern and outcome of cardiac abnormalities in MIS-C. This retrospective study was conducted in children with MIS-C between 1 month and 18 years. We enrolled 53 children with a mean age of 7.78 ± 4.62 years. Overall, 35.8% of children with MIS-C had cardiac manifestations in the form of coronary artery abnormalities (CAAs) or left ventricular (LV) dysfunction. Younger age (P 0.009) and high C-reactive protein at admission (P = 0.001) were significant predictors of cardiac involvement. CAAs were seen in 11.3% of children. On follow-up, 67% and 83% of children showed regression of CAA at 1 and 6 months, respectively. 24.5% of patients had presented with LV dysfunction. LV ejection fraction improved significantly at 1 month (P = 0.002) and 6 months (P = 0.001). Cardiac outcomes in MIS-C were favorable with timely identification and treatment.

Absent Right Coronary Artery in an Active Young Female

Absent Right Coronary Artery in an Active Young Female

Clinical Profile, Outcome, and Short-Term Follow-up of Pediatric Multisystem Inflammatory Syndrome Cases in a Tertiary Care Center of Western India

Abstract Background: There are several reports of the pediatric multisystem inflammatory syndrome associated with COVID-19 in children globally. The current Indian data on PIMS-TS associated with COVID-19 are scarce. Aims and Objectives: To describe demographic, clinical and laboratory findings, management, and follow - up of cases of PIMS-TS. Materials and Method: We analyzed children presenting to our hospital from July 2020 to January 2021, with PIMS-TS. Cases were categorized into two subsets: Group 1 (MIS-C with shock) and Group 2 (MIS-C without shock). Coronary Z scores of &gt;2.5 were considered as dilated. We followed all the patients at 15 days, 3 months, 6 months, and 12 months. Results: Total 11 patients with diagnosis of MIS-C were treated during this study period. Mean age of presentation was 6.5 years. All patients in Group 1 needed inotropic support and treated with steroids and intravenous immunoglobulin. Three patients required administration of tocilizumab. All these were girls &lt;6 years with neutrophil to lymphocyte ratio (NLR) &gt;3.5, had dilated cardiac chambers with significantly decreased ventricular ejection fractions and dilated coronary arteries. Mean length of hospital stay was around 10 days. In Group 1, gastrointestinal and cardiovascular symptoms were prominent. While in group 2, prominent symptoms were gastrointestinal and mucocutaneous. Conclusion: Most of the children in this study had coronary artery abnormalities, low incidence of RT-PCR positivity and presence of SARS–CoV-2 antibodies. Use of tocilizumab to control the inflammatory response is likely to be beneficial in steroid resistant cases. Coronaries and cardiac function reverts to normal in a month without any residual effects.

COVID-19-related cardiomyopathy: Can dual-energy computed tomography be a diagnostic tool?

No study on dual energy computed tomography (DECT) has been found in the literature to evaluate possibly fatal cardiac/myocardial problems in corona virus disease 2019 (COVID-19) patients. Myocardial perfusion deficits can be found in COVID-19 patients even without any significant coronary artery occlusion, and these deficits can be shown via DECT with a perfect interrater agreement. To assess lung perfusion alterations in COVID-19 patients. To our knowledge, no study using DECT has been performed to evaluate possibly fatal cardiac/ myocardial problems in COVID-19 patients. The purpose of this study is to evaluate the role of DECT in the detection of COVID-19-related cardiac diseases. Two blinded independent examiners evaluated CT images using the 17-segment model according to the American Heart Association's classification of the segmentation of the left ventricular myocardium. Additionally, intraluminal diseases and abnormalities in the main coronary arteries and branches were investigated. Following segment-by-segment analysis, perfusion deficiencies identified on the iodine map pictures on DECT were identified. The study enrolled a total of 87 patients. Forty-two of these individuals were classified as COVID-19 positive, and 45 were classified as controls. Perfusion deficits were identified in 66.6% (n = 30) of the cases. All control patients had a normal iodine distribution map. Perfusion deficits were found on DECT iodine map images with subepicardial (n = 12, 40%), intramyocardial (n = 8, 26.6%), or transmural (n = 10, 33.3%) anatomical locations within the left ventricular wall. There was no subendocardial involvement in any of the patients. Myocardial perfusion deficits can be found in COVID-19 patients even without any significant coronary artery occlusion. These deficits can be shown via DECT with a perfect interrater agreement. Additionally, the presence of perfusion deficit is positively correlated with D-dimer levels.

Prevalence and Prognostic Significance of Malnutrition in Patients with Abnormal Glycemic Status and Coronary Artery Disease: A Multicenter Cohort Study in China.

This study sought to investigate the prevalence and prognostic significance of malnutrition in patients with an abnormal glycemic status and coronary artery disease (CAD). This secondary analysis of a multicenter prospective cohort included 5710 CAD patients with prediabetes and 9328 with diabetes. Four objective tools were applied to assess the nutritional status of the study population. The primary endpoint was all-cause death. The association of malnutrition with clinical outcomes was examined using Cox proportional hazards regression. The proportion of malnutrition varied from 8% to 57% across the assessment tools. Diabetic patients were more likely to be malnourished than prediabetic patients. During a median follow-up of 2.1 years, 456 all-cause deaths occurred. The adjusted hazard ratios and 95% confidence interval for all-cause deaths of moderate-severe malnutrition defined by different tools ranged from 1.59 (1.03, 2.46) to 2.08 (0.92, 4.73) in prediabetic patients and 1.51 (1.00, 2.34) to 2.41 (1.78, 3.27) in diabetic patients. In conclusion, malnutrition is not rare in CAD patients with abnormal glycemic status. Moderate-severe malnutrition strongly predicted all-cause death regardless of the assessment tool. Assessing the nutritional status for all CAD patients with prediabetes and diabetes to identify individuals at high risk of all-cause death may help the risk assessment and prognosis improvement.

Describing our experience with the effects of multisystem inflammatory syndrome in children with COVID-19 on the cardiovascular system.

Cardiac involvement with multisystem inflammatory syndrome in children can include coronary artery abnormalities, ventricular dysfunction, conduction abnormalities, arrhythmias, pericarditis, and myocarditis. We report the cardiac findings in 34 patients with multisystem inflammatory syndrome in children admitted to a single institution. We looked at patient age, sex, brain natriuretic peptide levels, troponin levels, ejection fraction, presence of pericardial effusion, valvular changes, need for inotropic agents, and electrocardiogram findings. Our data showed that elevated brain natriuretic peptide did not predict troponin elevation and vice versa. Additionally, troponin rise was not a reliable marker for decreased left ventricular ejection fraction. All changes tracked were proven to be transient and resolved after initiating steroids, Intravenous immune globulin (IVIG), and occasionally anakinra.

Left Main Coronary Artery Ostial Stenosis in a Pediatric Patient With Sudden Cardiac Arrest

Pediatric resuscitated sudden cardiac arrest may result from diverse conditions and, therefore, warrants a comprehensive work-up. Although rare, coronary artery abnormalities must be ruled out in these patients. We describe a case with congenital left main coronary artery ostial stenosis diagnosed using advanced imaging techniques. (Level of Difficulty: Advanced.)

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Cardiac MRI with late gadolinium enhancement shows cardiac involvement 3-6 months after severe acute COVID-19 similar to or worse than PIMS.

Coronavirus disease 2019 (COVID-19) in children is rarely severe. However, severe courses occur, especially in the presence of risk factors. A minority of children develop pediatric inflammatory multisystem syndrome (PIMS) with substantial morbidity. While the importance of cardiac involvement after PIMS is well established, its role after severe acute COVID-19 remains unclear. We aim to compare cardiac sequelae of children after severe acute COVID-19 using cardiac MRI and compare them with patients after PIMS. For this prospective cohort study, we recruited patients with acute COVID or PIMS in a single center. Clinical follow-up, lab work, ECG, and echocardiography were done within 2 days after disease onset and 3-6 months after discharge. At the last visit 3-6 months later, cardiac MRI (CMR) with late gadolinium enhancement (LGE) was performed to evaluate cardiac sequelae and compare both groups. Data were obtained from n = 14 patients with PIMS and n = 7 patients with severe acute COVID-19. At the start of the respective disease, left ventricular (LV) ejection fraction was reduced in seven patients with PIMS but none in the acute COVID-19 group. Transient mitral valve insufficiency was present in 38% of patients, of whom PIMS accounted for 7/8 cases. Eight patients (38%) with PIMS presented coronary artery abnormalities, with normalization in 7/8 patients. A significant decrease in LV mass index 3-6 months after disease onset was observed in both groups. MRI follow-up revealed non-ischemic myocardial pattern of LGE in 12/21 patients- in all (6/6) after severe acute COVID-19 and in less than half (6/14) after PIMS. Normal body weight-adjusted stroke volumes and end-diastolic volumes were found in 20/21 patients. We show that children suffering from severe acute COVID-19 have a similar, or worse, cardiac risk profile as patients with PIMS. Both patient groups should therefore receive close pediatric cardiac follow-up examinations. Cardiac MRI is the technique of choice, as most patients presented with delayed LGE as a sign of persistent cardiac injury despite normalization of laboratory and echocardiographic findings.

Clinical profile and outcome of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 infection: a single-center observational study from South India

BackgroundMultisystem inflammatory syndrome in children (MIS-C) is a post-infectious sequelae of acute COVID-19 infection affecting children. This study was done over a period of 12 months from December 2020 to November 2021 to describe the clinical presentation, laboratory abnormalities, and outcome of children with MIS-C.MethodsSeventy-eight children below 12 years of age who satisfied the WHO diagnostic criteria for MIS-C were included in the study. Clinical parameters were recorded at admission. Relevant laboratory investigations, radiological studies, and outcome were documented.ResultsThe most commonly affected age group was 6–12 years with a female predominance. COVID RTPCR was negative in all patients. Most cases presented 2–6 weeks after the onset of acute COVID-19 infection. Lethargy, poor feeding, vomiting, abdominal pain, loose stools, cough, and cold are common symptoms of MIS-C syndrome in children and the common signs were rash, conjunctival congestion, hypotension, tachycardia, tachypnea, and hypoxemia. Gastrointestinal system was the commonly affected followed by the hepatic, renal, and cardiovascular systems. Coronary artery abnormalities were seen in 20% of cases. IVIg was the mainstay of therapy used in 95% of patients. Mortality was 1.3%. Cases responded well to IVIg and steroids.ConclusionOverall, the short-term outcome was favorable with low mortality in our study cohort. One-fifth of children had coronary artery abnormalities during acute phase underscoring the need for long-term follow-up.