Kawasaki disease (KD) is an inflammatory vasculitis and is the most common cause of acquired childhood heart disease in developed countries. Current treatment with intravenous immunoglobulin (IVIG) is often ineffective in patients with delayed or refractory disease. We present a case of combination anticytokine therapy in an infant with delayed and refractory KD. Case Presentation. A 3-month-old infant presented with refractory KD with giant aneurysms after a delayed diagnosis of one month. Use of combination anticytokine therapy led to resolution of giant aneurysms over approximately 6 months.Our case is unique in effective use of anticytokine therapy in very delayed disease with giant aneurysms. Additionally, we review other cases for a broader perspective. Prospective study of anticytokine therapy for patients with giant aneurysms may be warranted.
Background/Aim: We previously reported that late gadolinium enhancement (LGE) on cardiac MRI (CMR) was as high as 82% in pediatric patients with COVID-19 vaccine-associated myocarditis (C-VAM) despite mild clinical symptoms and normal left ventricular function. As LGE can be a harbinger for future adverse events including arrhythmias, heart failure or sudden cardiac death, we sought to identify predictors for LGE in C-VAM, specifically assessing troponin as a screening marker for C-VAM patients at risk for myocardial scarring who could then be referred for a confirmatory CMR with LGE. Methods: In this longitudinal multicenter retrospective observational study across 38 U.S. member institutions of the M yocarditis A fter C OVID V accination (MACiV) study network, 333 patients with C-VAM based on CDC criteria were included from April 2021 to November 2022. Data collected included demographics, laboratory values, clinical and cardiac imaging characteristics and outcomes. Using logistic regression, troponin levels at presentation were assessed as a log transformed continuous variable and categorized into tertiles. Results: The C-VAM patients were predominantly white (67%) adolescent males (91%, 15.7± 2.8 years). There were 216/333 (65%) patients who had both a reported troponin value and had a CMR. On univariate analysis, elevated troponin increased the probability of having LGE (OR=1.29, 95% CI: 1.06, 1.58, p=0.012). Even after controlling for age, race, sex, number of vaccine doses and left ventricular ejection fraction (OR=1.32, 95% CI: 1.06, 1.65, p=0.013). Patients >15 years compared to those ≤15 years of age were 2.94 (95% CI: 1.28, 6.75, p=0.011) times more likely to have LGE at presentation. Patients with troponin levels in the highest tertile compared to lowest tertile were 2.66 times (95% CI: 1.04, 6.83, p=0.042) more likely to have LGE along with a greater involvement > 4 AHA myocardial segments with LGE (p=0.004) Conclusions: Higher troponin values are associated with presence of late gadolinium enhancement on cardiac MRI in patients with COVID-19 vaccine-associated myocarditis. Troponin levels at presentation may facilitate risk stratification and function as a screening tool to identify those C-VAM patients with the greatest likelihood of myocardial scarring, who may benefit from undergoing CMR for tissue characterization.
Choroidal metastases from gynaecological primary are extremely rare. There is no documented case in the literature of choroid metastasis in a patient with primary peritoneal carcinoma (PPC).We describe the first case of a 54-year-old woman with a history of borderline mucinous tumour who presented 17 months later with PPC and 21 months after with recurrent disease metastatic to the eye, and review pertinent literature.High index of suspicion is warranted when patients with history of primary peritoneal carcinoma present with visual complaints in order to treat and/or relieve symptomatology from metastatic eye disease.
Radiation exposure related to medical procedures carries known medical risk. Electrophysiology (EP) and catheter ablation procedures are traditionally performed under fluoroscopic guidance. Three-dimensional (3D) electroanatomical navigation systems decrease or eliminate fluoroscopy use in EP procedures.The aim of this study was to assess the efficacy and outcome of a minimal or no fluoroscopic electroanatomical mapping approach for catheter ablations for supraventricular tachycardia (SVT) in the pediatric population.Patients were identified through our EP database. A retrospective chart review was performed at a single institution.Sixty-three pediatric patients underwent catheter ablations with a minimal fluoroscopic and 3D electroanatomical mapping using CARTO-3 system (Biosense Webster, Diamond Bar, CA, USA) between October 2012 and March 2015. We selected 20 age-matched patients who underwent ablations for SVT by the same operator prior to October 2012 with fluoroscopy use as our control group. The mean age in the study and control group was 13.9 years and 13.7 years, respectively. Mean procedure time was 208.7 minutes and 217.2 minutes in the study and control group (P = NS). Thirty-four (54%) in the study group had no fluoroscopy use. Mean fluoroscopy time was 4.1 minutes versus 35.4 minutes between the study and the control group (P < 0.001). Radiation dose was 6.7 mGy versus 209.3 mGy between the study and the control group (P < 0.001). Acute procedural success was achieved in 95% and 90% of patients in the study and control groups, respectively. On follow-up, the recurrence rate was 5.3% in the study group and 5.6% in the control group.Catheter ablation for SVT in children can be successfully performed in patients with normal cardiac anatomy using minimal or no fluoroscopy with favorable outcomes.
Background: The sequelae of COVID-19 vaccine associated myocarditis (C-VAM) are incompletely understood. We sought to characterize the clinical course, myocardial injury, and outcomes in C-VAM.Methods: In this retrospective observational cohort multicenter study across 38 hospitals in the U.S., we compared the clinical and cardiac imaging characteristics in 333 C-VAM patients from April 2021 to November 2022 with those in 100 multisystem inflammatory syndrome in children (MIS-C) patients and explored the risk factors for myocardial injury in C-VAM.Findings: The C-VAM patients were predominantly white (67%) adolescent males (91%, 15·7±2·8 years) with a mild initial clinical course and a lower prevalence of left ventricular dysfunction, in comparison to MIS-C patients (17% vs 68%, p<0·0001). Conversely, acute cardiomyocyte injury as evidenced by myocardial late gadolinium enhancement (LGE) on cardiac magnetic resonance imaging was higher in C-VAM compared to MIS-C (82% vs.16%, p<0·001). The odds of having LGE in C-VAM were 2·74 (95% CI: 1·28, 5·83, p=0·009) times higher for older adolescents (>15 years) compared to younger patients, 3·28 (95% CI: 0·99, 10·6, p=0·052) times higher for males compared to females, and 7·18 (95% CI: 1·05, 49·09, p=0·045) times higher with the first dose and 4·5 times higher (95% CI: 1·23, 16·44, p=0·023) with the second dose compared to the third dose of the mRNA vaccine. There were no cardiac deaths or need for heart transplantation in C-VAM at a median follow-up duration of 178 days (IQR 114-285 days) and LGE decreased in prevalence and severity but remained present in 60% of the patients at follow up.Interpretation: COVID-19 vaccine-associated myocarditis is characterized with a milder initial clinical presentation but higher prevalence of myocardial injury, in contrast to MIS-C. Mid-term clinical outcomes are reassuring but the persistence of LGE warrants continued surveillance in C-VAM. Funding: This study was funded by the U.S Food and Drug Administration, FDA-75F40122C00148. Declaration of Interest: SSJ, LGW, SA, JMS, HCW, JCM, JHS, RSB, VM, XJ, OHF, BF, SB, RCA, SAM, NM, JAS, SS, DV, AKVH, MJC, JK, SH, CM, MDC, MS, LN, JYA, SCU, PR, JKP, JGM, JAV, MPD, MB, PB, PE, KM, KG, MLD, KAA, AK, SBB, ALD, PKM, JS, ALC, JDR, ZP, AC, YS, LG, MA, MJ, JDR, NN, EDD report no competing interests. ASH: Site PI for the CAMP study - NHLBI funded, Site PI for MUSIC – NIH funded, Site PI for PREVAIL, supported by a sub-agreement from the Johns Hopkins University with funds provided by Grant No. R61HD105591 from the Eunice Kennedy Shriver National Institute of Child Health & Human Development and the Office of the Director, National Institute of Health (OD). Scientific advisory board member of OP2 DRUGS ("OP2"), states that no work has been done. ABY: Institution received funds for conducting phase 3 clinical trials for Pfizer mRNA COVID-19 vaccine (C4591007 and C4591048). LEH: Patent US11457889B2, issued: Oct 4, 2022, Patent US2023/0016283A1 Published: Jan 19, 2023. FH: Payment for Expert Testimony in pending court case as an expert witness to discuss the risk of C-VAM. DT and SS: Grant or contract from New England Research Institute for participation in Pediatric Heart Network CAMP Study. MJC: Subject matter expert for CDC CISA program, Consulting fees Longerone Inc.Ethical Approval: nstitutional research ethics boards approved the study at every participating site and waived the requirements for informed written consent.
Infective endocarditis is extremely rare in children with structurally normal hearts. The most common etiological agents are staphylococcal and streptococcal species. Nutritionally variant streptococci also classified as Abiotrophia species are a group of fastidious organisms that account for only 5% to 6% of all cases of culture-negative infective endocarditis. Only seven cases of Abiotrophia infective endocarditis have been previously reported in children with no underlying structural heart disease. We report two cases of Abiotrophia infective endocarditis in children without any predisposing factors. Both patients presented with nonspecific symptoms leading to delay in diagnosis. While bacteriological clearance was achieved in both cases, both had a complicated course including development of brain mycotic aneurysms, splenic infarction, renal failure, and irreversible damage to the mitral valve. Both patients required surgical removal of the native mitral valve and replacement. We also present review of seven cases with similar diagnosis published previously in literature and highlight important differences. Our cases highlight special challenges in management of Abiotrophia endocarditis in pediatric patients. As the organism may not be isolated in routine culture media, may present with atypical clinical symptoms and may have a complicated course even without antibiotic failure, a high index of suspicion should be maintained in children with subacute symptoms even with no underlying structural cardiac disease.
