Cardiac evaluation of patients with juvenile dermatomyositis.

BACKGROUND: The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM. METHODS: The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement. RESULTS: In the present study, although baseline cardiologic evaluations including electrocardiography and echocardiography were within normal limits, we showed late gadolinium enhancement on cardiac MRI in 3 of 11 JDM patients. In the literature review, we identified 25 articles related to cardiac involvement in JDM. However, none of them, except one case report, included cardiac MRI of JDM patients. CONCLUSION: Cardiac abnormalities have been reported among the less frequent findings in patients with JDM. Cardiovascular complications during the long-term disease course are a leading cause of morbidity and mortality in these patients. Early detection of cardiac involvement by cardiac MRI in patients with JDM and aggressive treatment of them may improve the clinical course of these patients. IMPACT: The myocardium in patients with JDM may be involved by inflammation. Myocardial involvement may be evaluated by using contrast-enhanced cardiac MRI. This is the first study evaluating cardiac involvement by cardiac MRI in JDM patients. MRI may show early cardiac involvement in patients whose baseline cardiologic evaluations are within normal limits. Early detection of cardiac involvement by cardiac MRI may improve the long-term prognosis of patients with JDM.

Experience of left ventricular outflow tract arrhythmia ablation in paediatric patients using limited fluoroscopy and three-dimensional mapping technique.

OBJECTIVE: The left ventricular outflow tract is an important source of ventricular arrhythmias. Up to one-third of all idiopathic ventricular arrhythmias in patients with structurally normal hearts may arise from this region. We would like to share the results of our left ventricular outflow tract ablation using three-dimensional mapping and limited fluoroscopy. MATERIALS AND METHODS: This is a single-centre retrospective cohort study. Forty-six consecutive patients who underwent left ventricular outflow tract ablation procedures between January 2015 and June 2023 were included in the study. The EnSite Precision System (Abbott, St. Paul, MN, USA) was used to facilitate mapping and to reduce or eliminate the need for fluoroscopy. RESULTS: The study group comprised 29 males and 17 females, with a mean age of 13.4 ± 4.5 years. The most common location for arrhythmias was the left coronary cusp (n : 21). Other locations, in sequence, included the junction of the right and left coronary commissure (n : 10), right coronary cusp (n : 10), left ventricular outflow tract endocardium (n:4), aorto-mitral junction (n : 1), and great cardiac vein (n : 1). Nine of these patients had previously undergone unsuccessful right ventricular outflow tract ablation at another centre. Cryoablation was performed in three patients, irrigated radiofrequency ablation in three patients, and conventional radiofrequency ablation in the remaining patients. The acute success rate was 100%, and no recurrences were observed. The mean follow-up period was 49.6 ± 24.4 months. All patients were asymptomatic and were being followed without antiarrhythmic medication. CONCLUSION: Although left ventricular outflow tract ablations pose a risk for coronary artery and heart valve complications, they can be performed successfully and safely with the guidance of three-dimensional mapping.

Is strain echocardiography a more sensitive indicator of myocardial involvement in patients with multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2?

OBJECTIVE: The aim of the study was to evaluate the early myocardial dysfunction detected by strain echocardiography in children with multisystem inflammatory syndrome related to SARS-CoV-2 infection. METHODS: This cross-sectional study was conducted with 47 patients diagnosed with MIS-C and 32 healthy age- and gender-matched children. All patients underwent two-dimensional, colour, pulsed, and tissue Doppler, and 2D speckle tracking echocardiography examination at admission, 2 weeks, and 2 months after discharge. The MIS-C patient group was compared with the control group. Echocardiographic changes in MIS-C patients during follow-up were evaluated. RESULTS: Of 47 patients, 30 (63.8%) were male and 17 (36.2%) were female. The mean age at diagnosis was 9.1 ± 4.3 (1.25-17) years. At admission, 25 patients had abnormal findings on conventional echocardiography. Among them, eight patients had left ventricular systolic dysfunction. Ejection fraction and fractional shortening were significantly lower in the patient group at admission compared to controls (p = 0.013, p = 0.010, respectively). While the ejection fraction was <55% in eight patients, and global longitudinal strain was lower than -2SD in 29 patients at admission. Global longitudinal strain z-score <-2SD persisted in 13 patients at 2-month follow-up. Ejection fraction increased above 55% in 3.42 ± 0.53 days in 7 of 8 patients with left ventricular systolic dysfunction, ejection fraction was 51% at discharge in one patient, and left ventricular systolic dysfunction persisted at the 6-month of follow-up. CONCLUSION: These results confirmed that speckle tracking echocardiography is more likely to detect subclinical myocardial damage compared to conventional echocardiography. In addition, it is a valuable method for follow-up in this patient group.

CXCL10/IP10 as a Biomarker Linking Multisystem Inflammatory Syndrome and Left Ventricular Dysfunction in Children with SARS-CoV-2.

BACKGROUND: To investigate the diagnostic accuracy of CXCL10/IP10 for left ventricular (LV) dysfunction in multisystemic inflammatory syndrome (MIS-C). METHODS: This cross-sectional, longitudinal study included 36 patients with MIS-C. Patients were classified as follows: (1) patients presenting with Kawasaki-like features (group I = 11); (2) patients presenting with LV systolic dysfunction (group II = 9); and (3) other presentations (group III = 3). CXCL10/IP10 levels were measured upon admission and on days 3 and 7 of treatment. RESULTS: Twenty patients were male and 16 were female. The median age of patients at diagnosis was 7.5 (1.5-17) years. All patients had a fever lasting for a median of 4 (2-7) days. Ten patients had LV systolic dysfunction. The duration of hospitalization was longer in group II. Lymphocyte and platelet counts were lower, whereas NT-pro-BNP, troponin-I, D-dimer, and CXCL10/IP10 levels were higher in group II. Baseline levels of CXCL10/IP10 were weakly negatively correlated with ejection fraction (r = -0.387, p = 0.022). Receiver operator characteristic curve analysis yielded a cutoff value of CXCL10/IP10 to discriminate patients with LV dysfunction was 1839 pg/mL with sensitivity 88% and specificity 68% (Area under curve (AUC) = 0.827, 95% CI 0.682-0.972, p = 0.003). CONCLUSION: Having a good correlation with cardiac function, CXCL10/IP10 is a potential biomarker to predict LV dysfunction in MIS-C patients.

Risk Factors Associated with Ascending Aortic Aneurysms and Aortic Elasticity Parameters in Children with a Bicuspid Aortic Valve.

Aneurysms of the ascending aorta are frequently found in patients with a bicuspid aortic valve (BAV). This study assessed the risk factors of ascending aortic aneurysms and aortic elasticity in children with BAV. The study included 66 patients with no history of transcatheter intervention or surgical procedure who had been diagnosed with isolated BAV. Echocardiographic, blood pressure, and pulse measurements were obtained for all patients. The BAVs were classified as described by Sievers et al. (J Thorac Cardiovasc Surg 133:1226-1233, 2007), and aortic elasticity parameters were calculated using various formulas. The patients were divided into groups with and without cusp fusion, aortic stenosis (AS), aortic regurgitation (AR), or mixed lesions; the groups were then compared. The mean patient age was 10.43 ± 3.91 years; 15%he patients had no AS or AR, 33% had both AS and AR, 17% had AS alone, and 35% had AR alone. The most common type of BAV was type 5, and the ascending aorta z-scores were higher in children with mixed lesions and without a cusp fusion. Aortic distensibility (AD) was significantly higher, and the stiffness index was significantly lower, in patients with an ascending aorta z-score > 4. The ascending aortic z-scores were higher in the no-fusion and mixed lesion (AS + AR) groups, especially those originating from post-stenotic dilation due to AS. The AD was increased in patients with an ascending aorta z-score > 4. Patients should thus be monitored closely for dissection risk, and preventive medical treatment should be started early in those with AS without cusp fusion.

A "congenitally corrected" variant of Ebstein's anomaly.

Ebstein's anomaly is a rare CHD that is characterised by caudal displacement of the functional tricuspid annulus and a dysfunctional tricuspid valve owing to a failure of proper leaflet coaptation. We present a balanced variant of Ebstein's anomaly, in which the overgrowth of the septal leaflet had allowed proper coaptation of the tricuspid leaflets, thus preserving the valve function.