Endomyocardial substrate of ventricular arrhythmias in patients with autoimmune rheumatic diseases.

INTRODUCTION: Delayed enhancement-magnetic resonance imaging (DE-MRI) has demonstrated that nonischemic cardiomyopathy is mainly characterized by intramural or epicardial fibrosis whereas global endomyocardial fibrosis suggests cardiac involvement in autoimmune rheumatic diseases or amyloidosis. Conduction disorders and sudden cardiac death are important manifestations of autoimmune rheumatic diseases with cardiac involvement but the substrates of ventricular arrhythmias in autoimmune rheumatic diseases have not been fully elucidated. METHODS AND RESULTS: 20 patients with autoimmune rheumatic diseases presenting with ventricular tachycardia (VT) (n = 11) or frequent ventricular extrasystoles (n = 9) underwent DE-MRI and/or endocardial electroanatomical mapping of the left ventricle (LV). Ten patients with autoimmune rheumatic diseases underwent VT ablation. Global endomyocardial fibrosis without myocardial thickening and unrelated to coronary territories was detected by DE-MRI or electroanatomical voltage mapping in 9 of 20 patients with autoimmune rheumatic diseases. In the other patients with autoimmune rheumatic diseases, limited regions of predominantly epicardial (n = 4) and intramyocardial (n = 5) fibrosis or only minimal fibrosis (n = 2) were found using DE-MRI. Endocardial low-amplitude diastolic potentials and pre-systolic Purkinje or fascicular potentials, mostly within fibrotic areas, were identified as the targets of successful VT ablation in 7 of 10 patients with autoimmune rheumatic diseases. CONCLUSION: Global endomyocardial fibrosis can be a tool to diagnose severe cardiac involvement in autoimmune rheumatic diseases and may serve as the substrate of ventricular arrhythmias in a substantial part of patients.

Magnetic resonance imaging abnormalities in the basal interventricular septum of patients with left ventricular outflow tract arrhythmias.

INTRODUCTION: Concealed structural abnormalities were detected by delayed enhancement - magnetic resonance imaging (DE-MRI) in patients with apparently idiopathic tachycardia of left ventricular (LV) origin. Basal septal fibrosis was evaluated as a potential arrhythmia substrate in patients with left ventricular outflow tract (LVOT) arrhythmias. METHODS AND RESULTS: A total of 22 patients with LVOT arrhythmias, including frequent monomorphic premature ventricular complexes (PVCs) in 15 patients and ventricular tachycardia (VT) in 7 patients, underwent catheter ablation and DE-MRI. A total of 19 patients with frequent PVCs and 17 patients with idiopathic VT of other origin served as a control group. Basal septal intramural fibrosis as thin strip-shaped intramyocardial DE or as marked intramyocardial DE involving >25% of wall thickness was detected more frequently in patients with LVOT arrhythmias (41% and 32%) than in patients with non LVOT arrhythmias (14% and 3%). After successful ablation, 4/16 patients with basal septal intramural fibrosis and LVOT PVCs (n = 3) or LVOT VT (n = 1) compared with no patient without basal septal fibrosis experienced episodes of sustained VT with similar or different QRS morphology resulting in ICD therapy in three patients. Follow-up DE-MRI after PVC ablation (17 ± 7 months) revealed an increase in LV ejection fraction from 49 ± 5% to 56 ± 5% (n = 9) but the amount of septal DE remained unchanged. CONCLUSIONS: Basal septal intramural fibrosis may serve as the arrhythmia substrate in a substantial part of patients with premature ventricular complexes (PVCs) and VT originating from the LVOT and identifies patients with continued risk for VT recurrence after initially successful ablation of LVOT arrhythmias.

Botulinum toxin type A and B for the reduction of hypersalivation in children with neurological disorders: a focus on effectiveness and therapy adherence.

Botulinum toxin (BoNT) is an established treatment option to reduce hypersalivation in children with chronic neurological disorders. Objective of this study was (1) to discriminate differences in efficacy and safety of repeated interventions using BoNT with a focus on different preparations used and (2) to look for effectiveness and treatment adherence from a qualitative research perspective in this single-center cohort study. We prospectively assessed goal attainment scaling, drooling severity and frequency score and the number of towels/day before, and 4 to 8 weeks after intervention. A parent questionnaire assessed therapy-related effects on quality of life retrospectively. A total of 19 out of 34 patients received repeated injections of BoNT (106 total). Mean dose: 95 units onabotulinumtoxinA (Botox®), 2383 units rimabotulinumtoxinB (Neuro-/Myobloc®). Outcome parameters showed a distinct reduction in all treatment groups with a higher efficacy of riB. The child's need for care was reduced in 79% and social interaction improved in 84%. Main reason for discontinuation was "not enough effect" and "formation of antibodies." riB showed to be more effective in reducing hypersalivation, but antibody-formation seems to be clinically relevant. Despite clinical efficacy treatment adherence is influenced by personal and environmental factors of parents and caretakers balancing the short-term clinical benefit versus the burden of intervention.