[Clinical value of dual source computerized tomography in primary diagnosis of coronary heart disease]. / Klinische Wertigkeit der Dual-Source-Computertomographie (DSCT) in der Primärdiagnostik der koronaren Herzkrankheit.

UNLABELLED: Dual-source-CT-technology (DSCT) improves temporal resolution of cardiac computed tomography to 83 ms per heart-phase. In this study, the clinical performance of this new method is evaluated. MATERIALS AND METHODS: In fifty patients (33 male, 17 female; age 50 +/- 13 years) with suspected coronary heart disease, CT angiography (slice thickness 0.75 mm, contrast-agent 60-80 ml iomeprol) was performed with a Somatom Definition scanner. Based on the coronary 15-segment-model of the AHA, scores for image quality and lumen reduction were established to enable the observer, to give recommendations for further therapy. RESULTS: Out of 750 possible AHA-segments, 655 were depicted (87.3%). 591 segments (90.2%) were assessed without any limitation of quality, 49 (7.5%) segments showed moderate, and 15 (2.3%) segments severe limitation in image quality. 508 (77.6%) segments were without pathological findings, 92 (14.0%) segments had minimal atherosclerotic lesions, 42 (6.4%) segments suffered from stenoses with lumen reduction less than 70%, and 13 (2.0%) showed significant stenoses of more than 70%. In 31 patients (62%), coronary heart disease was ruled out by CT angiography without any need for further non-invasive or invasive diagnostics. 8 patients (16%) underwent stress-testing for ischemia. In 11 (22%) patients coronary angiography was recommended, and DSCT findings were confirmed in 10 cases. Only one LCx stenosis was overestimated in DSCT. CONCLUSION: Contrast-enhanced DSCT is a powerful tool in diagnosis of coronary heart disease. 98% of coronary segments could be assessed in diagnostic quality, and at least 90% of haemodynamically significant coronary stenoses were detected.

Effect of transcoronary ablation of septal hypertrophy on clinical outcome in hypertrophic obstructive cardiomyopathy associated with atrial fibrillation.

BACKGROUND: Relatively few reports on the clinical impact of atrial fibrillation (AF) in hypertrophic obstructive cardiomyopathy (HOCM) are available. The aims of our study are to report the effect of transcoronary ablation of septal hypertrophy (TASH) on clinical outcome in HOCM associated with AF and to evaluate the influence of AF on symptoms and quality of life in HOCM. PATIENT AND METHODS: In 80 consecutive patients (38 f, mean age 56 +/- 17 years) with severely symptomatic HOCM referred for interventional treatment, we analyzed the prevalence of AF based on 240 Holter ECG recordings and patients' history, retrospectively. Symptoms, quality of life, number of hospital admissions and hemodynamic performance were obtained in all patients before and after TASH. Mortality was additionally investigated by letter and telephone contact. RESULTS: The overall prevalence of AF was 29%. Paroxysmal AF was detected in 17 pts (21.3%), persistent AF in 5 pts (6.3%). Only 1 pt (1.3%) suffered from permanent AF. Symptoms due to AF were present in 52.6% of the AF patients. Quality of life score was markedly improved after TASH (15.9 +/- 3.8 vs. 20.7 +/- 3.8, p < 0.001) with no difference between sinus rhythm and atrial fibrillation. However, hospital admissions were more frequent in the AF group (0.85 +/- 1.84 vs. 0.28 +/- 0.81, p = 0.03) in 32 +/- 13 months. AF patients suffered more often from syncope before TASH (30 +/- 70% vs. 10 +/- 30%, p = 0.008). Two patients with sinus rhythm at baseline died after 32 +/- 13 months from cardiovascular cause. CONCLUSIONS: Atrial fibrillation is the major cardiac arrhythmia in severe HOCM. The majority of AF patients demonstrate AF specific symptoms. The paroxysmal type of atrial fibrillation dominates by far. Both patients with and without atrial fibrillation showed similar quality of life with marked improvement after TASH.

Catheter-based septal ablation for symptomatic hypertrophic obstructive cardiomyopathy: follow-up results of the TASH-registry of the German Cardiac Society.

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.

Transcoronary ablation of septal hypertrophy for hypertrophic obstructive cardiomyopathy: feasibility, clinical benefit, and short term results in elderly patients.

OBJECTIVE: To evaluate symptomatic and haemodynamic results of transcoronary ablation of septal hypertrophy for hypertrophic obstructive cardiomyopathy in elderly patients. SETTING: Tertiary referral centre for patients with hypertrophic obstructive cardiomyopathy. DESIGN: Retrospective study of two groups of consecutive patients divided at a median age (59 years). PATIENTS: Transcoronary ablation of septal hypertrophy was compared for 80 patients (group 1) < 60 years of age and 77 patients (group 2) > or = 60 years of age. At baseline both groups were similar concerning the proportion of familial hypertrophic cardiomyopathy, concomitant moderate hypertension, prior syncope, left ventricular outflow obstruction, left ventricular end diastolic pressure, and left ventricular ejection fraction. Patients in group 2 had a lower interventricular septal thickness and more severe disease as measured by New York Heart Association (NYHA) functional class, exercise capacity, pulmonary artery mean pressure at workload, and cardiac index at peak exercise. RESULTS: Median follow up was seven months after transcoronary ablation of septal hypertrophy. Both groups had a significant and similar improvement in basal and provokable obstruction, septal thickness, NYHA functional class, exercise tolerance, peak oxygen consumption, and pulmonary artery mean pressure at workload. Significant differences, compared with the younger group, were a higher proportion of persistent total atrioventricular block (5% v 17%, p = 0.015) and a slight decrease in left ventricular ejection fraction (3 (12) v -6 (11)%, p = 0.001) in the elderly, despite a trend to a lower induced peak creatine kinase activity (596 (339) v 491 (331) U/l, p = 0.051). CONCLUSIONS: Short term results with transcoronary ablation of septal hypertrophy suggest that independent of a patient's age similar treatment strategies are justified in hypertrophic obstructive cardiomyopathy.

Catheter-based therapy for hypertrophic obstructive cardiomyopathy. First in-hospital outcome analysis of the German TASH Registry.

BACKGROUND: Registry results of the new catheter-based method in the treatment for HOCM are missing so far. In 1997, the Transcoronary Ablation of Septal Hypertrophy Registry (TASH Registry) was established by the German Cardiac Society (GCS) as a multicenter, national registry of patients with HOCM undergoing the new catheter interventional therapy. This is the report of the in-hospital outcome of patients who underwent the procedure during the first two years of data collection in the registry. METHODS AND RESULTS: Information was based on three standard forms for each patient, with a total of 86 variables. Information was collected on an "intention to treat" basis. The TASH Registry includes the establishment of a data base in the data collecting center. Ten centers participated. Enrollment forms were received for 264 patients out of 279 patients registered up to January 2000. There was a history of medical treatment of 3.6+/-3.9 years. The vast majority of patients (91%) were treated in three centers. The Vasalva maneuver and the exercise Doppler echocardiography were used for noninvasive stress testing. Exercise Doppler echocardiography induced a significantly higher augmentation of the baseline gradient (70.1% vs 133.4%; p<0.01). The echo-contrast guided technique was used for the intervention in 50.8% and the pressure angiography guided technique in 49.2%. On the average 2.8+/-1.3 ml of alcohol were injected. Before the procedure, the gradient measured by catheterization was 60.4+/-38.6 mmHg at baseline and 142.7+/-46.2 mmHg following the extrasystolic beat. At the end of the session it was reduced significantly by 75% and 67%. The peak phosphocreatine kinase activity was 482.5+/-246.4 U/L. Major complications occurred in 15.6% including a mortality rate of 1.2% and a permanent pacemaker implantation rate because of total heart block in 9.6%. There was an early in-hospital improvement of dyspnoe corresponding to a significant decrease of NYHA functional class from 2.8+/-0.7 to 1.8+/-0.6 (p<0.001). Similar hemodynamic and clinical benefit was found in patients with and without resting gradient at baseline. CONCLUSION: This analysis for the first time gives a comprehensive overview of clinical characteristics, technique, procedural data, in-hospital outcome and complications in a large number of patients with HOCM who were treated by the new catheter-based method and prospectively enrolled in a registry. The results contribute considerably to critical evaluation and validation of the new technique. This analysis supports the catheter-based method to constitute a new therapeutic option for very symptomatic patients, to be effective both in patients with and without intraventricular pressure gradient at rest and to be an alternative to surgical treatment, as has been stated recently.

[Simultaneous hypertrophic obstructive cardiomyopathy and long QT syndrome: a potentially malignant association]. / Gleichzeitiges Vorliegen einer hypertrophisch-obstruktiven Kardiomyopathie sowie eines Long-QT-Syndroms: eine potentiell maligne Assoziation.

Hypertrophic obstructive cardiomyopathy (HOCM) and long-QT syndrome (LQTS) are cardiac diseases with known genetic disorders. They are inherited in an autosomal-dominant way. From a clinical point of view, both diseases share a disturbed repolarization and the risk of sudden cardiac death. In 1998, during the annual meeting of the German Cardiac Society, we presented the case report of two patients (mother and daughter) with the combination of both diseases, being the first scientific communication on this subject. Both patients experienced sudden cardiac death due to ventricular fibrillation more than 10 years after the first diagnosis of the diseases. Resuscitation resulted in an apallic syndrome in both. The mother died from complications during the course of the apallic syndrome. In contrast to actual therapeutic strategies and data on prophylactic ICD-implantation available today, the daughter received no defibrillator. Sudden death in the presented cases may be due to either disease or their possible malignant association. Thus, the combination of both diseases forms the argument for early prophylactic ICD-implantation in these rare cases. Moleculargenetic studies are needed in large families to elucidate the potential of a common etiology.

Transcoronary ablation of septal hypertrophy (TASH): a new treatment option for hypertrophic obstructive cardiomyopathy.

In 1991, our group started to develop a catheter interventional therapy for hypertrophic obstructive cardiomyopathy (HOCM). The new concept was proposed in 1994. It is based on the conventional PTCA technique with the aim of inducing an artificial myocardial infarction by instillation of 96% ethanol into the most proximally situated septal branch of the left anterior descending coronary artery. This leads to a subaortic contraction disorder with subsequent decrease of the intraventricular pressure gradient, shrinkage of the hypertrophied septal bulge and widening of the outflow tract ("therapeutic remodeling"). The subaortic defect is small and well demarcated as assessed by left ventricular angiography, transesophageal echocardiography and 18 F-glucose positron emission tomography. The term transcoronary ablation of septum hypertrophy (TASH) was suggested. Our patient cohort that now comprises 215 therapeutic procedures in 187 patients underwent a large variety of prospective studies (maximum follow-up 4.5 years) including invasive controls at regular intervals, investigation of hemodynamics at rest and at exercise, transesophageal and transthoracic echocardiography, Doppler echocardiography during bicycle exercise, electrophysiologic testing, Holter monitoring and measurement of myocardial metabolism and perfusion, assessment of microembolic events by transcranial Doppler sonography and histological examinations. This article gives an overview and reports our increasing experience in applying TASH. The following post-TASH findings were obtained: significant hemodynamic and clinical improvement at rest and at exercise, decrease of septum thickness, increase of outflow tract area and decrease of induced ventricular tachycardia. There were well-demarcated, histologically atypical subaortic myocardial defects, no microembolic events, abnormal early peak of infarct related enzymes, and no change of baroreflex sensitivity. Pre-/post-TASH evaluations of the patients should be based in particular on clinical symptoms correlated to the intraventricular gradient measured by bicycle exercise Doppler echocardiography and to outflow tract area as assessed by transesophageal echocardiography. Since 1994, as a roughly estimate, worldwide 1000 patients in 20 countries have been treated. According to published articles, abstract presentations and workshops, TASH consistently leads to a pronounced clinical and hemodynamic benefit for patients with HOCM. TASH has become an established technique. At least in centers with a high level of expertise, it is no longer experimental but a routinely performed alternative to surgical treatment for HOCM, i.e., the previous gold standard of therapy. Of course, patient outcome needs further careful clinical and prognostic evaluation. With respect to complications, TASH appears to be superior to surgery (transaortic septal myectomy) for HOCM. Like surgical treatment, TASH is currently indicated in critically ill patients with typical HOCM (subaortic form), who exhibit with drug refractory symptoms, including patients, who preferred DDD pacemaker therapy as a first therapeutic step but in whom this produced no subsequent clinical benefit.

Changes in the left ventricular outflow tract after transcoronary ablation of septal hypertrophy (TASH) for hypertrophic obstructive cardiomyopathy as assessed by transoesophageal echocardiography and by measuring myocardial glucose utilization and perfusion.

AIMS AND METHODS: Transcoronary ablation of septal hypertrophy (TASH) leads to marked clinical and haemodynamic improvement in patients with hypertrophic obstructive cardiomyopathy. In order to obtain more detailed information about changes in the outflow tract after TASH, transoesophageal echocardiography and a repeat invasive investigation were conducted before as well as 2 weeks and 6 months after TASH (n=62). In a subset of patients (n=11), metabolism and perfusion of the myocardium ((18)F-FDG-PET and(99m)Tc-MIBI-SPET) were investigated. RESULTS: After TASH there was a typical regional subaortic contraction disorder. It was quantified by a significant decrease in the fractional shortening of the left ventricular end-diastolic diameter, which declined from an average of 40.6% to 18.0%. The end-diastolic diameter increased from an average of 39.1 to 40.6 mm. There was also a significant reduction in septal thickness, which continued for up to 6 months after TASH, from an average of 20.0 mm to 11.1 mm in the region of ablation and from 23. 2 to 21.7 mm outside this region. The decrease in the gradient post TASH corresponded with a concomitant significant increase in the outflow tract area from a mean value of 1.04 cm(2)before the process to a value of 3.0 cm(2)after. In contrast to coronary heart disease, these changes were accompanied by non-diffuse, well demarcated subaortic-septal necrosis verified by(18)F-FDG-PET and(99m)Tc-MIBI-SPET. On average the TASH induced necrotic area comprised 6.6% of the left ventricle and correlated significantly with echocardiographic changes in the outflow tract. CONCLUSIONS: Alterations post TASH indicated that this catheter interventional treatment for hypertrophic obstructive cardiomyopathy affects the specific region of obstruction. The changes reflect a 'therapeutic remodelling' of the outflow tract of the left ventricle. They were demonstrable over the entire 6 months investigation period and obviously constituted the basis of post TASH clinical and haemodynamic improvement. Progressive alterations post TASH (post TASH reduction of subaortic septal thickness and an increase in the end-diastolic diameter) need special consideration during long-term follow up.

Acute and long-term results after transcoronary ablation of septal hypertrophy (TASH). Catheter interventional treatment for hypertrophic obstructive cardiomyopathy.

AIMS: To evaluate acute and long-term symptomatic, haemodynamic (at rest and during exercise) and electrophysiological results of transcoronary ablation of septal hypertrophy (TASH), a catheter interventional treatment for hypertrophic obstructive cardiomyopathy. METHODS AND RESULTS: Sixty-two transcoronary ablations of septal hypertrophy were performed by injection of 4.6+/-2.6 ml 96% ethanol into septal branches in 50 patients with hypertrophic obstructive cardiomyopathy and severe symptoms. Serial left and right heart catheterization, transoesophageal echocardiography and electrophysiological investigations were repeated 2 weeks and 7+/-1 months (n=37) after intervention. Transcoronary ablation of septal hypertrophy led to a reduction in septal thickness, sustained elimination of the outflow obstruction (51+/-41 vs 6+/-10 mmHg at rest, P<0.001; 134+/-48 vs 28+/-32 mmHg, P<0.001, post-extrasystolic), a decrease in left ventricular filling pressures at rest and during exercise and a pronounced clinical improvement. There was no evidence for the creation of an arrhythmogenic substrate as assessed by serial programmed electrical stimulation in 39 patients. However, permanent high-grade atrioventricular block occurred in 17% of the patients. There were two early, but no late deaths during a mean follow-up time of 10. 6+/-5.6 months. CONCLUSION: Transcoronary ablation of septal hypertrophy is a promising new treatment for hypertrophic obstructive cardiomyopathy in patients with severe symptoms. It should now be compared with alternative treatment strategies in prospective randomized studies.