Search

SummaryAim We analysed 25 children with 21-hydroxylase deficiency who received glucocorticoid and/or mineralocorticoid treatment for at least 12 months to determine the effects of the disease and its treatment on vascular structures and ventricular function. Methods Twenty-five patients with 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) and 25 control subjects were enrolled into this observational, cross-sectional study. The patients were investigated in terms of fasting blood glucose and insulin; fasting serum lipid profile; serum 17-hydroxyprogesterone; dehydroepiandrosterone sulphate; androstenedione; and adrenocorticotropic hormone. M-mode tracings of the wall motion of major arteries were obtained to measure carotid intima-media thickness (cIMT), as well as elasticity and distensibility of the aorta and carotid artery. Conventional and relatively new tissue Doppler imaging techniques were employed to assess ventricular systolic and diastolic functions. Results The median age and weight of patients were 9·4 years (1·5–16·75) and 35·5 kg (7·5–76·3), respectively. The median duration of treatment was 52·2 months. Tissue Doppler imaging measurements revealed left ventricular diastolic impairment in the patient group compared to the controls. Carotid intima-media thickness, stiffness index, elastic modulus of the aorta and carotid artery were significantly higher; meanwhile, aortic distensibility and carotid distensibility were lower in the patient group, all of which indicates the presence of subclinical atherosclerosis. BMI was found to be an independent variable for cIMT (s: 0·5, P = 0·01) and aortic stiffness index (s: 0·52, P < 0·001). Conclusion Cardiovascular function and the elastic properties of major arteries are disturbed in children and adolescents with 21-hydroxylase-deficient CAH.

Summary Introduction In this study, we aimed to evaluate Red blood cell distribution width (RDW) in patients with acute rheumatic carditis during the acute phase and after anti-inflammatory therapy. Methods Pediatric patients diagnosed with acute rheumatic carditis (ARC) between 2006 and 2014 and age- and sex-matched controls were retrospectively analyzed. At the time of diagnosis and after 2 months of medical therapy, we reviewed the obtained demographic features; echocardiographic data; complete blood count reports, including RDW; acute phase reactants, including C-reactive protein; and erythrocyte sedimentation rate values. Results The number of the cases with ARC and age- and sex-matched controls were 100 and 110, respectively. The mean age of patients was 11.6 ± 2.5 years. WBC and platelet counts, RDW were found to be significantly higher in patient group compared with controls at the time of diagnosis, prior to the onset of treatment. RDW, platelet count, CRP, and ESR levels significantly decreased after an 8 weeks of medical treatment. RDW values after the medical treatment were still significantly higher compared with controls. RDW values were significantly higher in patients with multiple valvular involvement both prior to and after the treatment. Moreover, we found a significant and positive correlation between the RDW and the severity of mitral regurgitation in our patients (r: 0.46, P < 0.001). Conclusions High levels of RDW after initial medical treatment may indicate an ongoing subtle inflammatory process that leads to future stenotic valvular lesions. However, long-term follow-up studies are needed involving adulthood period to support this hypothesis.

Background Radiofrequency (RF) catheter ablation in the septal arrhythmia substrates has an increased risk of irreversible atrioventricular block. Despite its safety profile, several studies reported a lower acute success rate and a higher recurrence rate with cryoablation of septal accessory pathways (APs) when compared to RF ablation. The aim of this study was to assess the efficacy and safety of cryoablation of right septal APs using an electroanatomical mapping system guidance. Methods and Results A total of 43 consecutive patients (13.2 ± 5.5 years) underwent cryoablation for right septal APs. The EnSite system (St. Jude Medical, St. Paul, MN, USA) was used in all procedures. No fluoroscopy was used in 90% of patients (39/43). The mean fluoroscopy time in the remaining four patients was 3.7 ± 0.7 minutes. An electrophysiology catheter with 2-mm distal spacing was used to determine the precise AP location. The majority of the patients (20/43) had anteroseptal, and remaining of the patients had posteroseptal (15/43) and midseptal (8/43) APs. A 6-mm-tip catheter was used in 33 patients and 8-mm-tip catheter was used in nine patients (both catheters were used in one patient). The mean procedure duration and number of complete cryoablation lesions were 181.5 ± 60.6 minutes and 6.1 ± 3.1 minutes, respectively. Acute success was achieved in 40 of 43 patients (93%). No complications were noted. During a mean follow-up of 8.8 ± 4.8 months, five patients (12.5%) experienced recurrence. Conclusions Our data suggest that cryoablation of septal APs can be performed safely with comparable efficacy to the reported RF ablation results using a limited fluoroscopy approach.

Background There is increasing evidence of clustering of certain cardiac anomalies in some families. The frequency and echocardiographic features of such anomalies among the relatives of patients with bicuspid aortic valve (BAV) or other left-sided cardiovascular anomalies (LSCA) were evaluated. Methods The patients with BAV or any other LSCA and their relatives were enrolled in the study. They underwent an echocardiographic examination. The probands were assessed in three groups: BAV, BAV + coarctation of aorta (CoA), and other LSCA. Their relatives were also grouped and evaluated accordingly. The echocardiographic measurements were standardized by Z-scores. Results Eighty-six probands and 261 relatives were evaluated. The numbers of the patients in the BAV, BAV + CoA, and other LSCA group were 52, 14, and 20, respectively. Any LSCA was determined in 17 (6.5%) of the relatives. Thirteen (5%) had aortic dilatation and the remainder (1.5%) had BAV. Accordingly, BAV incidence among relatives of patients with BAV was found to be 1.9%. A second individual with an LSCA was observed in 12.8% of 86 families investigated. The frequencies of aortic stenosis, aortic regurgitation, aortic stenosis + aortic regurgitation, and aortic dilatation in the patients with BAV were found to be 37.9%, 53%, 25.8% and 48.5%, respectively. In contrast to previous reports, no enlargement was observed in the pulmonary arteries of BAV patients. Conclusions BAV and other LSCA are of clinical significance. Because the clustering of LSCA in some families is observed, we recommend echocardiographic screening of those relatives. If this is not possible, at least it should be achieved for BAV patients.