REduction of THRomboembolic EVents during Ablation using the laserballoon: The RETHREVA registry.

INTRODUCTION: Cerebral events (CEs), including silent (SCEs), are a known complication of left atrial catheter ablation (LACA) in patients with atrial fibrillation. The aim of this prospective registry was to gain more information about CEs during laserballoon LACA and to reduce the risk of their occurrence. METHODS AND RESULTS: We enrolled 74 patients (age 61 ± 11 years; 74% male; CHA2 DS2 -VASc 1.9 ± 1.4). Cerebral MRI (1.5 Tesla) was performed to detect CEs. ASPItest identified aspirin-resistant patients (ARPs). All bleeding complications were recorded. Due to an initial high CE rate, we evolved our clinical procedure step-by-step arriving at an optimized protocol: -Group 1: heparin after single transseptal puncture (TP), activated clotting time (ACT) > 300 seconds (CE: 64.3%). -Group 2: heparin after double TP, ACT > 300 seconds; 2a without (CE: 45.5%, RRR: -29.2%) and 2b with additional intravenous aspirin (CE: 36.4%, RRR: -43.4%; excluding ARP: 30%, RRR: -53.3%). -Group 3: heparin before double TP, ACT > 350 seconds; 3a without (CE: 54.5%, RRR: -15.2%) and 3b with aspirin (CE: 18.5%, RRR: -71.2%; excluding ARP: 8.7%, RRR: -86.5%). Larger LA diameter > 44 mm (OR: 1.149, P  =  0.005) and no aspirin use (OR: 4.308, P  =  0.008) were CE risk factors in multivariate logistic regression. In those patients receiving aspirin, aspirin resistance (OR: 22.4, P  =  0.011) was an exceptionally strong risk factor. CONCLUSION: These data support the use of intravenous aspirin including monitoring of aspirin resistance in addition to ACT-guided heparin. An optimized protocol of heparin before TP, double TP, and intravenous aspirin in non-ARP resulted in a significantly lowered CE incidence and severity.

Clinical and morphological phenotype of the filamin myopathy: a study of 31 German patients.

Mutations in the filamin C gene (FLNC) cause a myofibrillar myopathy (MFM), morphologically characterized by focal myofibrillar destruction and abnormal accumulation of several proteins within skeletal muscle fibres. We studied 31 patients from four German families to evaluate the phenotype of filaminopathy. All patients harboured the same p.W2710X mutation in FLNC. Haplotype analysis suggested a founder mutation in these German filaminopathy families. The mean age at onset of clinical symptoms was 44 +/- 6 years (range, 24-57 years). Slowly progressive muscle weakness was mostly pronounced proximally, initially affecting the lower extremities and involving the upper extremities in the course of disease progression, similar to the distribution of weakness seen in limb-girdle muscular dystrophies (LGMD). Patients frequently developed respiratory muscle weakness. About one-third of the patients showed cardiac abnormalities comprising conduction blocks, tachycardia, diastolic dysfunction and left ventricular hypertrophy indicating a cardiac involvement in filaminopathy. Serum creatine kinase levels varied from normal up to 10-fold of the upper limit. Magnetic resonance imaging studies showed a rather homogenous pattern of muscle involvement in the lower extremities differing from that in other types of MFM. Myopathological features included perturbation of myofibrillar alignment, accumulation of granulofilamentous material similar to that seen in primary desminopathies and abnormal intracellular protein deposits typical of MFM. Decreased activities of oxidative enzymes and fibre hypertrophy seem to be early features, whereas dystrophic changes were present in advanced stages of filaminopathy. Rimmed vacuoles were detected in only a few cases. The intracellular aggregates were composed of a variety of proteins including filamin C, desmin, myotilin, Xin, dystrophin and sarcoglycans. Therapy is so far limited to symptomatic treatment. The German filaminopathy cohort, the largest group of patients studied so far, shares phenotypic features with LGMD and presents with characteristic histopathological findings of MFM.

Intra-operative cooled-tip radiofrequency linear atrial ablation to treat permanent atrial fibrillation.

AIMS: To demonstrate the safety and efficacy of saline irrigated cooled-tip atrial linear endocardial radiofrequency ablation (SICTRA) concomitant to open-heart surgical procedures in the treatment of permanent atrial fibrillation (AF). METHODS AND RESULTS: Two hundred and twenty-two patients presenting with permanent AF and the need for cardiac surgery were included. In addition to the cardio-surgical procedure [mitral valve (MV) surgery (n = 94), aortic valve replacement (n = 29), bypass surgery (n = 76 including 24 patients with additional MV surgery), and combined procedures (n = 23)] concomitant SICTRA was performed. In 116 patients, the ablation pattern was restricted to the left atrium alone. During the mean follow-up of 29 months, 174 patients (78%) converted to sinusrhythm (SR). In patients with SICTRA restricted to the left atrium conversion rates were not different compared to a biatrial approach (83 vs. 74%, P = 0.47). Thirty-days mortality was found to be 4% (9/222). Post-mortem evaluation revealed 23% of all lesions to be histologically non-transmural. In the overall group, only 4% of patients developed sustained secondary regular atrial arrhythmia. CONCLUSIONS: SICTRA safely and effectively restores stable SR in 78% of patients with permanent AF undergoing open-heart surgery. Rhythm outcome is not influenced by treatment of the right atrium. Sustained regular atrial arrhythmia with the need for invasive treatment strategies occurs in 4% although intra-operative ablation lesions are often non-transmural.