1/49. Electrophysiologic characteristics of accessory atrioventricular connections in an inherited form of wolff-parkinson-white syndrome.INTRODUCTION: A familial form of wolff-parkinson-white syndrome (WPW) occurs in association with hypertrophic cardiomyopathy and intraventricular conduction abnormalities. This syndrome, demonstrating autosomal dominant inheritance and segregating with a high degree of penetrance but variable expressivity, has been genetically linked to chromosome 7q3. The purpose of this study is to detail the electrophysiologic characteristics of accessory atrioventricular connections (AC) in four members of a kindred with this syndrome. methods AND RESULTS: We clinically evaluated 32 members of a single kindred and identified 20 individuals with ventricular preexcitation, abnormal intraventricular conduction including complete AV block and/or ventricular hypertrophy. genetic linkage analysis mapped the disease gene in this kindred to the chromosome 7q3 locus (maximum logarithm of the odds score = 6.88, theta = 0); recombination events in affected individuals reduced the genetic interval from 7 centimorgans (cM) to 5 cM. Electrophysiologic study of four individuals with preexcitation, identified seven AC (1 right sided, 3 septal, and 3 left sided). All four individuals had inducible orthodromic tachycardia; while three had multiple AC. Bidirectional conduction was demonstrated in 6 of 7 AC. Successful ablation was accomplished in 5 of 7 AC. CONCLUSION: The electrophysiologic characteristics and location of AC in family members having this complex cardiac phenotype are similar to those seen in individuals with isolated WPW. Identification of WPW in more than one family member should prompt clinical evaluation of relatives for additional findings of ventricular hypertrophy or conduction abnormalities.- - - - - - - - - - ranking = 1keywords = ablation (Clic here for more details about this article) |
2/49. pericardium-covered stent for septal myocardial ablation in hypertrophic obstructive cardiomyopathy.This report describes a patient with severe hypertrophic obstructive cardiomyopathy in new york Heart association functional class III. Complete reduction of left ventricular outflow tract gradient was achieved by the selective occlusion of three target septal arteries with a pericardium-covered stent. The patient's in-hospital course was uneventful and has improved to functional class I.- - - - - - - - - - ranking = 4keywords = ablation (Clic here for more details about this article) |
3/49. Intraprocedural myocardial contrast echocardiography as a routine procedure in percutaneous transluminal septal myocardial ablation: detection of threatening myocardial necrosis distant from the septal target area.Percutaneous transluminal septal myocardial ablation (PTSMA) has been introduced as an alternative to surgery for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). Visualization of the ablation area prior to induction of the chemical necrosis is possible by intraprocedural myocardial contrast echocardiography (MCE). We report on two patients in whom MCE showed opacification of the medial papillary muscle or the left ventricular posterolateral free wall. In both patients the correct ablation area could be identified by MCE after a change of the target vessel, thus avoiding potentially fatal complications due to induction of a necrosis of myocardium distant from the septal target area.- - - - - - - - - - ranking = 7keywords = ablation (Clic here for more details about this article) |
4/49. Coexistent atrioventricular and nodoventricular pathways in a patient with hypertrophic cardiomyopathy.A 17-year-old girl with concentric hypertrophic cardiomyopathy presented with a wide complex tachycardia and underwent electrophysiological study. She was found to have an antidromic tachycardia utilizing a decremental atrioventricular fiber as the anterograde limb with retrograde conduction occurring through the septum. Ablation of a right free-wall pathway rendered tachycardia noninducible, yet ventricular preexcitation remained. After ablation there was evidence of a second nodoventricular connection. We believe this to be the first report of coexistent "Mahaim" fibers; one a decremental atrioventricular connection and the second nodoventricular.- - - - - - - - - - ranking = 1keywords = ablation (Clic here for more details about this article) |
5/49. ventricular fibrillation induced by rapid atrial rates in patients with hypertrophic cardiomyopathy.AIMS: To describe the mechanisms of induction of ventricular fibrillation (VF) by rapid atrial rates in patients with hypertrophic cardiomyopathy (HCM). methods: Electrophysiological studies, management and follow-up in three patients with HCM with VF induced by atrial pacing. RESULTS: In one patient, spontaneous sinus tachycardia triggered VF. In another patient, VF occurred after verapamil infusion during rapid atrial fibrillation, and in the remaining patient there was no clinical VF. In all three patients, short runs of atrial pacing (cycle length 272-380 ms) induced VF, and QRS widening preceded fibrillation in all patients. Marked ventricular electrogram fragmentation was documented in one patient during atrial pacing and in another patient during late ventricular extra-stimuli. hypotension was associated with sinus tachycardia in one patient. The two patients developing clinical VF underwent atrioventricular (AV) junctional ablation; a ventricular defibrillator was implanted in one, and a mode-switching dual-chamber pacemaker in the other. No arrhythmic events occurred during 34- and 35-month follow-up, respectively. In the other patient, postatrial fibrillation pauses caused syncope, and he is asymptomatic 52 months after implantation of a dual-chamber pacemaker. CONCLUSIONS: Rapid atrial rates can trigger VF in some patients with HCM, probably through a combination of electrophysiological and ischaemic mechanisms. AV junctional ablation may prevent VF in selected cases.- - - - - - - - - - ranking = 2keywords = ablation (Clic here for more details about this article) |
6/49. Right ventricular involvement in hypertrophic cardiomyopathy: a case report and literature review.Although hypertrophic cardiomyopathy (HCM) is classically considered a disease of the left ventricle, right ventricular (RV) abnormalities have also been reported. However, involvement of the right ventricle in HCM has not been extensively characterized. The literature regarding prevalence, genetics, patterns of involvement, histologic findings, symptoms, diagnosis, and treatment of RV abnormalities in HCM is reviewed. To highlight the salient points, a case is presented of apical HCM with significant RV involvement, with an RV outflow tract gradient and near obliteration of the RV cavity, in the absence of a left intraventricular gradient. Right ventricular involvement in HCM appears to be as heterogeneous as that of the left ventricle. The spectrum extends from mild concentric hypertrophy to more unusual severe, obstructive disease. While in some cases the extent of RV involvement correlates with left ventricular (LV) involvement, predominant RV disease can be seen as well. While the genetics of RV involvement have not been well characterized, histologic findings appear to be similar to those in the left ventricle, suggesting similar pathogenesis. Significant RV involvement may result in RV outflow obstruction and/or reduced RV diastolic filling, with potentially increased incidence of severe dyspnea, supraventricular arrhythmias, and pulmonary thromboembolism. The optimal treatment for patients with significant RV disease is unknown. Medical and surgical therapies have been attempted with variable success; experience with newer techniques such as percutaneous catheter ablation has not been reported. Further characterization of RV involvement in HCM is necessary to elucidate more clearly the clinical features and optimal treatments of this manifestation of HCM.- - - - - - - - - - ranking = 1keywords = ablation (Clic here for more details about this article) |
7/49. Myectomy for hypertrophic obstructive cardiomyopathy after failed alcohol septal ablation: clinicopathological correlations.Hypertrophic cardiomyopathy - a genetically transmitted cardiac disorder - has diverse clinical, pathological and molecular manifestations. echocardiography is the most reliable tool for clinical diagnosis of hypertrophic cardiomyopathy. Reduction of the intraventricular pressure gradient and improvement of symptoms are major objectives of all therapeutic strategies. The recently introduced concept of catheter-based interventional treatment involves selective coronary perforator branch injection of 96% ethanol to reduce septal thickness, left ventricular outflow obstruction, left ventricular filling pressure and symptoms. The long term morphological features after medical ablation are presented for the first time and compared with both the echocardiographic findings and the findings reported in the English-language literature. The findings show that injection of ethanol into the perforator branch is associated with a fairly localized area of myocardial scarring.- - - - - - - - - - ranking = 5keywords = ablation (Clic here for more details about this article) |
8/49. ethanol septal ablation for hypertrophic obstructive cardiomyopathy in a very old patient.BACKGROUND: A 90-year-old woman who had hypertrophic obstructive cardiomyopathy presented with drug-refractory heart failure. METHOD: She successfully underwent transcoronary ethanol septal ablation under sedation. OUTCOME: There was instantaneous improvement in left ventricular outflow tract gradient 6 months after discharge, and the symptoms of heart failure have resolved.- - - - - - - - - - ranking = 5keywords = ablation (Clic here for more details about this article) |
9/49. Usefulness of selective myocardial contrast echocardiography in percutaneous transluminal septal myocardial ablation: a case report.A 67-year-old woman with hypertrophic obstructive cardiomyopathy that was refractory to medical treatment underwent percutaneous transluminal septal myocardial ablation (PTSMA). The septal branch supplying the myocardium involved in the left ventricular outflow tract (LVOT) obstruction was identified by selective myocardial contrast echocardiography (MCE). MCE for the third and largest septal branch opacified the right side of the mid-septal myocardium and MCE for the second septal branch opacified the right side of the basal portion of the septal myocardium. Finally, contrast agent was injected into the first, small branch, which opacificied the myocardium protruding into the LVOT. Subsequently, septal myocardial ablation for this vessel with intracoronary alcohol was performed, followed by a reduction of the LVOT gradient and successful, dramatic improvement in the patient's clinical condition. Selective MCE was very useful to identify the appropriate septal branch for PTSMA and enabled maximal effect of this treatment with minimal myocardial damage.- - - - - - - - - - ranking = 6keywords = ablation (Clic here for more details about this article) |
10/49. Percutaneous septal ablation for hypertrophic cardiomyopathy and mid-ventricular obstruction.AIMS: Percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion is a new treatment option in symptomatic patients with hypertrophic cardiomyopathy and subaortic, SAM-associated obstruction. We report on a patient with mid-ventricular obstruction and echocardiographic-guided reduction of septal hypertrophy. methods AND RESULTS: A 52-year-old woman with NYHA class III and recurrent exercise-induced syncope suffered from hypertrophic cardiomyopathy with mid-ventricular obstruction. She had a systolic gradient of 71 mmHg at rest and 153 mmHg post-extrasystole, and diastolic inflow gradient of 20 mmHg. Echo-guided percutaneous transluminal septal myocardial ablation with occlusion of the fourth septal branch resulted in acute reduction and final elimination of systolic, as well as diastolic resting and provocable gradients. Complications were not seen. At 3 months' follow-up the patient was asymptomatic and without further syncopes. CONCLUSIONS: Echocardiographic-guided percutaneous transluminal septal myocardial ablation is able to reduce gradients in hypertrophic cardiomyopathy and mid-ventricular obstruction with consecutive improvement of symptoms.- - - - - - - - - - ranking = 7keywords = ablation (Clic here for more details about this article) |
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