Search results for "Heart Block"
showing 10 items of 19 documents
High prevalence of arrhythmic and myocardial complications in patients with cardiac glycogenosis due to PRKAG2 mutations
2016
International audience; AIMS: Mutations in PRKAG2, the gene encoding for the γ2 subunit of 5'-AMP-activated protein kinase (AMPK), are responsible for an autosomal dominant glycogenosis with a cardiac presentation, associating hypertrophic cardiomyopathy (HCM), ventricular pre-excitation (VPE), and progressive heart block. The aim of this study was to perform a retrospective time-to-event study of the clinical manifestations associated with PRKAG2 mutations.METHODS AND RESULTS: A cohort of 34 patients from 9 families was recruited between 2001 and 2010. DNA were sequenced on all exons and flanking sequences of the PRKAG2 gene using Sanger sequencing. Overall, four families carried the recur…
Wall motion characteristic of the right pulmonary artery in the suprasternal echocardiogram
1980
This study describes the motion pattern of the right pulmonary artery (RPA) as it can be assessed from the suprasternal echocardiogram. The motion characteristic of the RPA is dependent on hemodynamic factors within the lumen of the RPA and those within the left atrium and the aortic arch. During atrial contraction the superior wall of the left atrium separates from the inferior wall of the RPA (IWRPA) and produces an "a" dip in the wall motion of the IWRPA. During isovolumic contraction the RPA is shifted upward (IC point). The incisura in the pulmonary artery pressure curve reflecting pulmonic valve closure can be seen by a sudden decrease in the diameter of the RPA (PC point). In conditi…
Cardiac output in single-lead VDD pacing versus rate-matched VVIR pacing.
1995
The importance of atrioventricular synchronous pacing compared with single-chamber rate-responsive pacing is still under discussion, especially for low-intensity workload representing daily life activities. We evaluated hemodynamics in single-lead VDD pacing versus VVIR pacing in 11 patients (8 men and 3 women, aged 58.6 +/- 13.8 years) with normal left ventricular function and a previously implanted single-lead VDDR pacemaker. A low-intensity steady-state treadmill test at 1 to 2.5 mph with a gradient of 2% to 4% was performed. Cardiac output was determined using a standard carbon dioxide rebreathing technique. Initially, the VDD mode was programmed, and after 5 minutes of exercise, cardia…
A series of West European patients with severe cardiac and skeletal myopathy associated with a de novo R406W mutation in desmin.
2003
Desminopathy is a familial or sporadic cardiac and skeletal muscular dystrophy associated with mutations in desmin. We have previously characterized a de novo desmin R406W mutation in a patient of European origin with early onset muscle weakness in the lower extremities and atrioventricular conduction block requiring a permanent pacemaker. The disease relentlessly progressed resulting in severe incapacity within 5 years after onset. We have now identified three other patients with early onset rapidly progressive cardiac and skeletal myopathy caused by this same desmin R406W mutation. The mutation was present in each studied patient, but not in their parents or other unaffected family member…
Implantation Strategy of the Atrial Dipole Impacts Atrial Sensing Performance of Single Lead VDD Pacemakers
2002
WIEGAND, U.K.H., et al.: Implantation Strategy of the Atrial Dipole Impacts Atrial Sensing Perfor-mance of Single Lead VDD Pacemakers. Intermittent atrial undersensing is observed in a considerable percentage of patients with single lead VDD pacemakers. Analyzing the 2-year data of the Saphir Multicenter Follow-Up Study, the authors investigated predictors for the occurrence of undersensing. The study included 194 patients with high degree AV block who received a VDD pacemaker system with an identical sensing amplifier. Placement strategy of the atrial dipole was left to the discretion of the implanting physician. At the final position, atrial potential amplitudes were measured during deep …
Complete congenital heart block in autoimmune hepatitis (SLA-positive).
1994
Complete congenital heart block is a serious complication of neonatal lupus erythematosus which most often occurs in children of mothers suffering from connective tissue disease. We report the occurrence of complete congenital heart block associated with autoimmune hepatitis (SLA-positive). A 32-year-old woman was treated for more than 10 years for autoimmune hepatitis (SLA-/ANA-positive) and remained in clinical remission under immunosuppressive therapy. She showed an MHC-haplotype typical for autoimmune hepatitis (A1, B8, DR3). After a normal first pregnancy, an emergency caesarean section was performed in the 32nd week of her second pregnancy because of fetal bradycardia. The child died …
Isomeric arrangement of the left atrial appendages and visceral heterotaxy: two atypical cases.
2000
AbstractWe present two cases in which there was isomeric arrangement oi the left atrial appendages in association with multiple spleens and complete heart block. In both of these, the venous connections were normal. In one case the arrangement of the bronchial tree and the lungs was normal, the liver was left-sided, and the stomach was right-sided. In the other case, there was left isomeric broncho-pulmonary morphology, and both liver and stomach were right-sided. These unusual combinations show the need for full description of the morphology of other organs, and the venoatrial connections, in cases with isomeric arrangement of the atrial appendages. They also demonstrate that connection of…
“Three-level shock” in ablation-refractory paroxysmal atrial fibrillation
1987
His-bundle ablation is a very effective method to control drug-refractory supraventricular arrhythmias. We present a 69-year-old woman with paroxysmal atrial fibrillation, in whom several attempts of unipolar and bipolar His-bundle ablation were ineffective. Ultimately a stable 2/1 atrioventricular block was induced when three successive shocks of 400 W were applied. The first shock was given in that position with the largest His-potential deflection. The other two shocks were applied at catheter positions more distal and more proximal from the first one, respectively. We suppose that the initial lack of success was due to an atypical atrioventricular junction anatomy.
Pacemaker therapy in premature children with high degree AV block.
1998
The smallest pacemaker pulse generator and a steroid-eluting bipolar epicardial lead were implanted in two premature children with symptomatic AV block. Stable capture threshold and high amplitude evoked response electrogram resulted in normal function of the pacemaker Autocapture algorithm, which adjusts output 0.3 V above the measured capture threshold. Autocapture had previously been used only with endocardial leads. Longer-term observation is required.
Genome-wide association meta-analysis of PR interval identifies 47 novel loci associated with atrial and atrioventricular electrical activity
2018
ABSTRACTElectrocardiographic PR interval measures atrial and atrioventricular depolarization and conduction, and abnormal PR interval is a risk factor for atrial fibrillation and heart block. We performed a genome-wide association study in over 92,000 individuals of European descent and identified 44 loci associated with PR interval (34 novel). Examination of the 44 loci revealed known and novel biological processes involved in cardiac atrial electrical activity, and genes in these loci were highly over-represented in several cardiac disease processes. Nearly half of the 61 independent index variants in the 44 loci were associated with atrial or blood transcript expression levels, or were i…