Right atrial hemangioma in modern cardiac imaging.
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Biomedical subjects
Publications and source records attributed to L Faber.
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Resynchronization of segmental left ventricular mechanics as well as re-coordination of both atrio-ventricular and inter-ventricular contraction are potential mechanisms responsible for the clinical benefit observed in patients with advanced congestive heart failure treated by cardiac resynchronization therapy (CRT). Initially electrical conduction problems, in the majority of cases a left bundle branch block (LBBB), were considered the target for CRT. However, growing experience with CRT in different patient populations including those with milder degrees of conduction disturbance, and improved cardiac imaging utilizing the tissue Doppler approach, have shown the complexity of CRT and the usefulness of sophisticated echocardiographic imaging techniques for therapeutic decision making and optimization of CRT device settings.
INTRODUCTION: In late 1997, the German Cardiac Society set up a multicenter registry to evaluate the acute and mid-term course of all patients (pts.) treated with septal ablation for symptomatic hypertrophic obstructive cardiomyopathy (HOCM). An analysis of the acute results has already been published. We now report on the mid-term course (3-6 months) of 242 pts. registered through September 1999. RESULTS: Follow-up was 92% complete (n=222). During follow-up (mean: 4.9+/-2.3 months), an additional 3 pts. died (in-hospital mortality: 3 pts.). A satisfactory clinical effect was reported by 195 pts. (88%); 27 pts. (12%) remained in NYHA classes III and IV. Overall symptomatic improvement (NYHA class: from 2.8+/-0.7 to 1.7+/-0.7) paralleled the outflow gradient (LVOTG) reduction which was further accentuated as compared with the acute result (Doppler measurement at rest: from 57+/-31 to 25+/-25 mmHg to 20+/-21 mmHg; with provocation: from 107+/-53 to 49+/-40, to 44+/-40 mmHg, p<0.001, resp.). Left atrial (LA) diameter (from 46+/-8 to 44+/-7 mm) and septal thickness (from 20+/-5 to 15+/-5 mm; p<0.001, resp.) were also reduced. Comparing the methods for target vessel selection (i.e., with contrast echo monitoring vs pressurefluoroscopy guidance), at followup clinical improvement and hemodynamic measurements were comparable. CONCLUSION: Clinical success can be achieved by septal ablation, both with the echocontrast guided and gradient-fluoroscopy guided method, in 88% of highly symptomatic HOCM pts. At mid-term follow-up, symptoms, left atrial size and septal thickness are reduced, and outflow gradients are further improved as compared to the acute result.
BACKGROUND AND INTRODUCTION: Damage to the AV conduction system is a frequent complication of percutaneous septal ablation (PTSMA) that needs early and reliable identification of those patients (pts.) at risk for complete heart block (CHB) and subsequent pacemaker implantation. METHODS AND RESULTS: In the first 39 pts. who underwent PTSMA in 1996, AV conduction recovery needed up to 11 days. One pt. suffered from unexpected CHB after 9 days. Seven pts. who needed a DDD pacemaker (DDD-PM) were compared to those without conduction disturbances. A score was established which identified all DDD-PM candidates retrospectively if they presented with >12 score points. In the following 137 consecutive pts. treated in 1997 and 1998, this score was applied prospectively, and again correctly identified all candidates for a DDD-PM. In addition, a low risk group was identified with <8 score points. From 1999 on, the score was applied in routine clinical decision-making in 120 consecutive pts. with respect to DDD-PM implantation. All low risk pts. (<8 points) remained free from bradycardias, while 2/54 pts. (4%) of the intermediate risk group, and 20/23 pts. (87%) of the high risk group had to undergo DDD-PM implantation. Pts. with a first-degree AV block or those with a right bundle branch block at baseline had no excess risk, while 50% of the pts. with a left bundle branch block (LBBB) needed a DDD-PM. CONCLUSIONS: Based on pre-interventional data and careful monitoring of the first 48 hours after PTSMA, identification of pts. at risk for CHB and subsequent DDD-PM implantation seems to be possible. Pts. with a score <8 seem to be at low, those with >12 points at high risk. In the remaining cases watchful waiting with prolonged monitoring may allow AV conduction to recover, thus, reducing the number of unnecessary DDD-PM implantations. In cases with LBBB at baseline, however, implantation of a DDD-PM should be considered first-line therapy.
BACKGROUND AND INTRODUCTION: Cardiac resynchronization therapy (CRT) is a promising non-pharmacological treatment option for patients (pts) with severe severe heart failure (CHF), systolic left ventricular (LV) dysfunction, and ventricular conduction abnormalities (VCA). Pt selection for CRT, however, is still controversial. Tissue Doppler echocardiography (TDE) can be used to analyze regional wall motion with high temporal resolution. PATIENTS AND METHODS: In 33 CHF pts with VCA (QRS width > or =140 ms) and 20 normal probands, left and right ventricular (RV) filling and emptying were analyzed by flow and tissue Doppler to assess regional (anterior, lateral, inferior, and septal) asynchrony within the LV as well as asynchrony between the RVand LV. All time measurements were corrected for a heart rate of 60 bpm. Results Maximum interventricular and segmental intraventricular delay was 30 ms in the normals. LV asynchrony, defined as a regional delay of > or =40 ms, was found in 29/33 (88%) of the CHF pts, in 4 cases there was synchronous LV contraction despite VCA. In the pts with LV asynchrony, 22 (67%) showed the maximum delay in the lateral wall, 7 (21%) in the septum. Inter- and intra-ventricular asynchrony correlated weakly. CONCLUSIONS: In many CHF pts with VCA, there is a delay both between the two ventricles, and among different LV regions. Predominantly but not exclusively, the LV lateral wall shows the maximum intra-LV delay. Some CHF pts, however, seem to have a synchronous LV contraction despite VCA. TDE thus adds important information for pt selection with respect to CRT.
BACKGROUND: Percutaneous transluminal septal myocardial ablation by alcohol-induced septal branch occlusion was introduced as a new treatment option in symptomatic patients with hypertrophic obstructive cardiomyopathy. Echocardiographic monitoring of the procedure resulted in improvement of acute results. In this study we analyzed the follow-up after echocardiographic guided septal ablation. METHODS: 100 consecutive symptomatic patients (50 men; age 52.7 +/- 15.7 years; NYHA class 2.8 +/- 0.6) underwent percutaneous septal ablation with echocardiographic monitoring. All patients had clinical and non-invasive 3-months and 1 year follow-up. RESULTS: Acute reduction of the left ventricular outflow tract gradient was achieved in 99 patients from 76 +/- 37 to 19 +/- 21 mm Hg at rest, from 104 +/- 34 to 43 +/- 31 mm Hg during Valsalva maneuver, and from 146 +/- 45 to 59 +/- 42 mm Hg post extrasystole (p < 0.0001, each). Mean CK rise was 570 +/- 236 U/l. One patient died at day 2 due to fulminant pulmonary embolism following deep venous thrombosis, and 8 patients required a permanent DDD-pacemaker due to postinterventional complete heart block. During follow-up no further patient died. All living 99 patients showed clinical improvement to NYHA-class 1.4 +/- 0.6 after 3 months and 1.5 +/- 0.6 after 1 year (p < 0.0001, each). Non-invasive follow-up studies observed ongoing outflow tract gradient reduction, decrease of septal and left ventricular posterior wall thickness, and improvement of exercise capacity and time. SUMMARY: Percutaneous septal ablation is an effective treatment of symptomatic patients with hypertrophic obstructive cardiomyopathy. During one-year follow-up remodeling after induced therapeutic septal infarction resulted in improvement of acute gradient reduction with ongoing symptomatic and objective improvement and without significant complications and side-effects.
UNLABELLED: High prevalence of autoantibodies against glutamic acid decarboxylase (GAD-Ab) in stiff man syndrome (SMS) not only helps diagnosis, but also suggests immune mediated impairment of GABAergic functions. However, the presence of GAD-Ab has also been reported in other neurological syndromes. Therefore the prevalence of GAD-Ab was investigated in SMS, progressive encephalomyelitis with rigidity and myoclonus (PERM), and in other neurological diseases (OND). Serum antibodies against the GAD isoforms, GAD65 and GAD67, were investigated with radioimmunoassays in 13 patients with SMS, nine with PERM, 279 consecutive patients with OND, and in 100 normal controls. RESULTS: Prevalence of GAD65Ab was around 80% in patients with SMS/PERM compared with 5% in patients with OND and 1% in normal controls. Prevalence of GAD67Ab was 60% in SMS/PERM, 2% in patients with OND, and 1% in normal controls. Raised GAD-Ab clustered in an OND subgroup with sporadic progressive ataxia, but not in OND subgroups with recognised neuroimmunological diseases. In conclusion, increased GAD-Ab is neither a non-specific epiphenomenon of neuronal damage nor a common feature of recognised neuroimmunological disorders. In neurological diseases, GAD-Ab may be a pathogenetic agent or a marker for an ongoing autoimmune process, or both.
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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.
BACKGROUND: Percutaneous septal ablation has evolved as an alternative to surgery for reducing symptoms and outflow gradients in patients with hypertrophic obstructive cardiomyopathy. Intraprocedural echo-cardiographic imaging can improve clinical and hemodynamic results. Growing experience with this method has additionally shown that threatening necrosis of the myocardium distant from the septal target region can be detected. METHODS AND RESULTS: Percutaneous septal ablation was performed in 162 patients (80 women, 82 men; aged 54.1 +/- 15.5 years); 131 of whom were targeted by intraprocedural myocardial contrast echocardiography. In 11 patients (7%), an atypical target vessel or a perfusion area distant from the expected septal target region was detected, leading to a target vessel change. Permanent pacing was necessary in 14 patients (9%). Three patients (2%) died. After 3 months, the mean New York Heart Association functional class was reduced in the returning 159 patients from 2.8 +/- 0.5 to 1.3 +/- 1.0 (P <.0001) along with a gradient reduction from 77 +/- 35 to 12 +/- 22 mm Hg at rest, and from 147 +/- 43 to 44 +/- 45 mm Hg with provocation (P < .0001 each). The main reason for unsatisfactory gradient reduction was suboptimal scar placement in the patients treated before the introduction of intraprocedural myocardial contrast echocardiography. CONCLUSIONS: Percutaneous septal ablation is an effective nonsurgical technique for reducing symptoms and outflow gradients in hypertrophic obstructive cardio-myopathy. Echocardiographic guidance adds substantially to safety and efficacy of the procedure and should therefore be considered routinely.
OBJECTIVE: To determine the long term outcome in patients treated with percutaneous transluminal septal myocardial ablation (PTSMA) for hypertrophic obstructive cardiomyopathy (HOCM). DESIGN AND SETTING: Observational, single centre study. PATIENTS: 25 patients (13 women, 12 men, mean (SD) age 54.7 (15.0) years) with drug treatment resistant New York Heart Association (NYHA) class 2.8 (0. 6) symptoms attributed to a high left ventricular outflow gradient (LVOTG) and a coronary artery anatomy suitable for intervention. INTERVENTION: PTSMA by injection of 4.1 (2.6) ml of alcohol (96%) into 1.4 (0.6) septal perforator arteries to ablate the hypertrophied interventricular septum. OUTCOME MEASURES: During in-hospital follow up, enzyme rise, the frequency of atrioventricular conduction lesions requiring permanent DDD pacing, and in-hospital mortality were assessed. Long term follow up (30 (4) months, range 24-36 months) included symptoms, echocardiographic measurements of left atrial and left ventricular dimensions and function, and LVOTG. RESULTS: Mean postinterventional creatine kinase rise was 780 (436) U/l. During PTSMA 13 patents developed total heart block, permanent pacing being necessary in five of them. One 86 year old patient died from ventricular fibrillation associated with intensive treatment (beta mimetic and theophylline) for coexistent severe obstructive airway disease. After three months, three patients underwent re-PTSMA because of a dissatisfactory primary result, leading to LVOTG elimination in all of them. During long term follow up, LVOTG showed sustained reduction (3 (6) mm Hg at rest and 12 (19) mm Hg with provocation) associated with stable symptomatic improvement (NYHA class 1.2 (1.0)) and without significant global left ventricular dilatation. CONCLUSIONS: PTSMA is an effective non-surgical technique for reduction of symptoms and LVOTG in HOCM. Prospective, long term observations of larger populations are necessary in order to determine the definitive significance of the procedure.
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.
BACKGROUND: Percutaneous transluminal septal myocardial ablation (PTSMA) by alcohol-induced occlusion of septal branches with resulting reduction of LV outflow-tract gradient (LVOTG) is a new treatment option in symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). METHODS: In 1996 and 1997 we treated 114 symptomatic patients (56 female; age 53.3 +/- 15.6 years; 5 patients with prior myectomy and 5 with DDD pacer; most in NYHA class III. Five patients underwent re-PTSMA after failed first treatment. In the first 30 patients 1 to 3 septal branches were occluded by injection of 3.4 +/- 1.6 ml absolute alcohol via the central lumen after balloon occlusion of the proximal part of the septal branch. In the remaining patients myocardial contrast echocardiography was available, so that only one branch needed to be occluded. RESULTS: LVOTG reduction was achieved in 107 (94%) patients: at rest from 73.8 +/- 36.5 to 18.6 +/- 19.7 mmHg (p < 0.00001). Maximal CK rise was 647 +/- 330 U/L. Two (1.8%) patients died during hospital stay. Due to permanent trifascicular block 11 (9.6%) patients required a permanent pacemaker. At 3 months follow-up in 87 patients we observed no cardiac complications, a further LVOTG reduction in 61 % patients, an ongoing symptomatic improvement (NYHA I or II; p < 0.0001 vs. pre PTSMA), and significant reduction of the left posterior wall thickness. CONCLUSIONS: PTSMA of HOCM results in significant reduction of LVOTG. Careful monitoring during hospital stay is necessary because of the potential risks of the induced therapeutic infarction. Mid-term follow-up showed ongoing symptomatic improvement without cardiac complications. Remodeling after circumscribed septal infarction results in further LVOTG reduction in over 50% of the patients.
BACKGROUND: Percutaneous transluminal septal myocardial ablation (PTSMA) has been introduced as an alternative procedure for reducing the left ventricular outflow tract gradient (LVOTG) in hypertrophic obstructive cardiomyopathy. We report on the acute and mid-term results in 91 symptomatic patients with respect to intraprocedural myocardial contrast echocardiography (MCE). METHODS AND RESULTS: PTSMA was intended for 46 women and 45 men (54.1+/-15.5 years). In 2 patients, the intervention could not be completed. In the first 30 patients the target vessel was determined by probatory balloon occlusion alone and in the remainder by additional intraprocedural MCE. Resting LVOTG was reduced from 73.8+/-35.4 to 16.6+/-18.1 and nostextrasystolic LVOTG from 149.3+/-42.5 to 61. 9+/-43.0 mm Hg (P<0.0001 each). In 10 (11%) patients, permanent DDD pacemaker implantation was necessary. Two (2%) patients died, 1 from ventricular fibrillation associated with treatment for chronic obstructive pulmonary disease after 9 days and 1 from fulminant pulmonary embolism after 2 days. After 3 months, mean New York Heart Association class was reduced from 2.8+/-0.6 to 1.1+/-1.0 (P<0.0001). The LVOTG remained reduced to 14.6+/-25.5 mm Hg at rest and 49. 1+/-48.7 mm Hg (P<0.0001 each). Four patients underwent successful repeat PTSMA. Determination of the target vessel by MCE was associated with a higher rate of acute (92% vs 70%; P<0.01) and mid-term (94% vs 64%; P<0.01) success. CONCLUSIONS: PTSMA is a promising nonsurgical technique for reduction of symptoms and LVOTG in hypertrophic obstructive cardiomyopathy. MCE has been shown to be a useful addition to probatory balloon occlusion for target vessel selection. Prospective, long-term observations of larger populations and a comparison with the established forms of therapy are necessary to determine the definitive significance of PTSMA.
CASE REPORT: A 26-year-old pregnant woman (18th week of pregnancy) was admitted to a hospital with right heart failure and pulmonary congestion. After establishing the diagnosis of mitral stenosis, a first stabilization could be achieved by medical therapy with digitalis, diuretics, and beta-blockers. Readmission was necessary in the 23rd week. After failure of medical treatment the patient was transferred to our center. We decided to perform an emergency mitral valvulotomy with the Inoue balloon. Taking care of maximal radiation protection for mother and fetus doubling of the mitral valve opening are (from 0.6 cm2 to 1.3 cm2) could be achieved. The pleural effusions and tricuspid regurgitation disappeared. The patient was symptom-free and could be delivered from a male infant on schedule.
Percutaneous transluminal coronary angioplasty (PTCA) is an established therapy for coronary artery disease (CAD), whereas percutaneous transluminal septal myocardial ablation (PTSMA) is becoming increasingly significant in the therapy of symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). We report the first ever simultaneous treatment, in a 62-yr-old patient, of significant HOCM and a 75% LAD stenosis from which the septal branch to be occluded stemmed. Using a double wire technique, first the septal branch was occluded through a fractional injection of 4 ml absolute alcohol, thus ablating the hypertrophied septal myocardium with reduction of the left ventricular outflow tract (LVOT) gradient at rest from 80 to 9 mmHg. Following this, the LAD stenosis was dilated and stented. Complications, in particular a trifascicular block or ventricular dysrhythmia, did not occur during the hospital stay. To conclude, combined PTSMA and PTCA may be considered as a therapeutic alternative to a combined surgical intervention in individual cases of symptomatic HOCM and CAD, provided that the potential complications are taken into account.
BACKGROUND: In hypertrophic obstructive cardiomyopathy (HOCM) therapy, surgical myectomy and DDD pacemaker implantation are considered to be established extensions to medical treatment. As an alternative procedure for reducing the left ventricular outflow tract gradient (LVOTG), percutaneous transluminal septal myocardial ablation (PTSMA) by alcohol-induced septal branch occlusion has been introduced. We report on the acute results and the short-term clinical course following 66 PTSMA interventions in symptomatic patients (pts.) with HOCM. METHODS: In pts. who were symptomatic despite adequate drug therapy (31 women, 35 men; mean age 52.9 +/- 15.0 years, range: 16-86) 66 PTSMA interventions were performed (4 pts. with a re-intervention). Septal branches were occluded by injection of 3.5 +/- 1.8 (1.5-11.0) ml ethanol (96%). In the first 30 pts. the target vessel was determined by probatory balloon occlusion (PBO) alone, in the following 36 by additional myocardial contrast echocardiography (MCE). In-hospital follow-up of LVOTG and clinical course were determined. RESULTS: The invasively determined LVOTG could be reduced by > 50% or eliminated in 54 interventions (82%) with a mean reduction from 71.2 +/- 34.4 (4-174) to 18.0 +/- 21.5 (0-105) mmHg at rest and from 145.7 < or = 42.3 (68-257) to 63.7 +/- 49.3 (0-185) mmHg post extrasystole (p < 0.0001). All pts. experienced angina pectoris within the first 24 hours. The creatine kinase peak was 690 +/- 364 (201-1810) U/l after 11.0 +/- 5.4 (4-24) hours. 45 pts. (68%) developed trifascicular block, requiring temporary, or in 9 cases (14%) permanent, (DDD) pacemaker implantation. Two pts. (3%) died 9 and 2 days after successful intervention, due to uncontrollable ventricular fibrillation associated with betasympathomimetic and theophylline treatment for chronic obstructive pulmonary disease in one case, and fulminant pulmonary embolism in the other. The remaining pts. were discharged after 11.1 +/- 4.6 (5-24) days following an uncomplicated hospital course. The introduction of MCE was associated with a higher percentage of short-term success (92% vs. 70%, p < 0.015). CONCLUSIONS: PTSMA in HOCM is a promising non-surgical technique for septal myocardial reduction with a consecutive reduction of the LVOTG. MCE has shown to be a useful addition to PBO for selection of the target vessel. Possible complications are trifascicular blocks requiring permanent pacemaker implantation and tachycardiac rhythm disturbances. Prospective, long-term observations of larger populations and a comparison with the established forms of therapy are necessary in order to determine the definitive significance of PTSMA.
OBJECTIVES: We report the acute results and midterm clinical course after percutaneous transluminal septal myocardial ablation (PTSMA) in symptomatic patients with hypertrophic obstructive cardiomyopathy (HOCM). BACKGROUND: In the treatment of HOCM, surgical myectomy and DDD pacemaker therapy are considered the standard procedural extensions to drug therapy with negatively inotropic drugs. As an alternative nonsurgical procedure for reducing the left ventricular outflow tract (LVOT) gradient, PTSMA by alcohol-induced septal branch occlusion was introduced. However, clinical follow-up has not been sufficiently described. METHODS: In 25 patients (13 women, 12 men; mean [+/- SD] age 54.7 +/- 15.0 years) who were symptomatic despite sufficient drug therapy, 1.4 +/- 0.6 septal branches were occluded with an injection of 4.1 +/- 2.6 ml of alcohol (96%) to ablate the hypertrophied interventricular septum. After 3-months, follow-up results of LVOT gradients and clinical course were determined. RESULTS: The invasively determined LVOT gradients could be reduced in 22 patients (88%), with a mean reduction from 61.8 +/- 29.8 mm Hg (range 4 to 152) to 19.4 +/- 20.8 mm Hg (range 0 to 74) at rest (p < 0.0001) and from 141.4 +/- 45.3 mm Hg (range 76 to 240) to 61.1 +/- 40.1 mm Hg (range 0 to 135) after extrasystole. All patients had angina pectoris for 24 h. The maximal creatine kinase increase was 780 +/- 436 U/liter (range 305 to 1,810) after 11.1 +/- 6.0 h (range 4 to 24). Thirteen patients (52%) developed a trifascicular block for 5 min to 8 days requiring temporary (n = 8 [32%]) or permanent (DDD) pacemaker implantation (n = 5 [20%]). An 86-year old woman died 8 days after successful intervention of uncontrollable ventricular fibrillation in conjunction with beta-sympathomimetics in chronically obstructive pulmonary disease. The remaining patients were discharged after 11.3 +/- 5.4 days (range 5 to 24), after an uncomplicated hospital course. Clinical and echocardiographic follow-up was achieved in all 24 surviving patients after 3 months. No cardiac complications occurred. Twenty-one patients (88%) showed clinical improvement, with a New York Heart Association functional class of 1.4 +/- 1.1. A further reduction in LVOT gradient was shown in 14 patients (58%). CONCLUSIONS: PTSMA of HOCM is a promising nonsurgical technique for septal myocardial reduction, with a consecutive reduction in LVOT gradient. Possible complications are trifascicular blocks, requiring permanent pacemaker implantation, and tachycardiac rhythm disturbances. Clinical long-term observations of larger patient series and a comparison with conventional forms of therapy are necessary to determine the conclusive therapeutic significance.