[Acute coronary syndrome: peri-interventional antithrombotic therapy]. / Akutes Koronarsyndrom. Peri-interventionelle antithrombotische Therapie.

In patients with acute coronary syndrome (ACS), the periinterventional antithrombotic treatment has become increasingly important for the choice of reperfusion strategy and as an adjunct pharmacological treatment prior, during and after percutaneous coronary interventions (PCI). In NSTE-ACS and early invasive strategy (<48 h), treatment with ASA, clopidogrel and heparin - unfractionated heparin (UFH) preferred - should be initiated as soon as possible. Direct thrombin inhibitors are an alternative to heparin, particularly in the setting of increased risk of bleeding and heparin-induced thrombocytopenia. In highrisk patients, an so-called upstream therapy with glycoprotein IIb/IIIa inhibitors (tirofiban, eptifibatide) is recommended as an adjunct to PCI. In STEMI, primary PCI is the reperfusion therapy of choice and should be supported by early adjunct treatment with ASA, clopidogrel, UFH and glycoprotein IIb/IIIa inhibitors (abciximab, eptifibatide). "Facilitated" PCI with thrombolytics is not recommended because of increased mortality and complication rates.

Arrhythmogenic right ventricular dysplasia/cardiomyopathy: need for an international registry. Study Group on Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation.

Arrhythmogenic right ventricular (RV) dysplasia/cardiomyopathy (ARVD/C) is a heart muscle disease characterized by peculiar RV involvement and electrical instability that precipitates ventricular arrhythmias and sudden death. The purpose of the present consensus report of the Study Group on ARVD/C of the Working Groups on Myocardial and Pericardial Disease and Arrhythmias of the European Society of Cardiology and of the Scientific Council on Cardiomyopathies of the World Heart Federation is to review the considerable progress in our understanding of the etiopathogenesis, morbid anatomy, and clinical presentation of ARVD/C since it first was described in 1977. The present article focuses on important but still unanswered issues, mostly regarding risk stratification, clinical outcome, and management of affected patients. Because ARVD/C is relatively uncommon and any one center may have experience with only a few patients, an international registry is being established to accumulate information and enhance the numbers of patients that can be analyzed and thus answer pending questions. The registry also will facilitate pathological, molecular, and genetics research on the causes and pathogenesis of the ARVD/C. Furthermore, availability of an international database will enhance awareness of this largely unrecognized condition among the medical community. Physicians are encouraged to enroll patients in the International Registry of ARVD/C.

Abnormalities of cardiac sympathetic innervation in arrhythmogenic right ventricular cardiomyopathy : quantitative assessment of presynaptic norepinephrine reuptake and postsynaptic beta-adrenergic receptor density with positron emission tomography.

BACKGROUND: The frequent provocation of ventricular tachycardia by stress or catecholamines and the efficacy of antiarrhythmic drugs with antiadrenergic properties suggest an involvement of the cardiac adrenergic system in arrhythmogenesis in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC). Previous studies demonstrated abnormalities of the presynaptic uptake-1 assessed by (123)I-MIBG-single-photon emission computed tomography. METHODS AND RESULTS: This study investigated neuronal reuptake of norepinephrine (uptake-1) and beta-adrenergic receptor density in 8 patients with ARVC and 29 age-matched control subjects. All subjects underwent positron emission tomography with the volume of distribution (V(d)) of [(11)C]hydroxyephedrine ((11)C-HED) used to assess presynaptic norepinephrine reuptake, the maximum binding capacity (B(max)) of [(11)C]CGP-12177 ((11)C-CGP-12177) to assess postsynaptic beta-adrenergic receptor density, and [(15)O]H(2)O for quantification of myocardial blood flow. Patients with ARVC demonstrated a highly significant global reduction in postsynaptic beta-adrenergic receptor density compared with that in control subjects (B(max) of (11)C-CGP-12177: 5.9+/-1.3 vs 10.2+/-2.9 pmol/g tissue, P<0.0007), whereas the presynaptic uptake-1 tended toward reduction only (V(d) of (11)C-HED: 59.1+/-25.2 vs 71.0+/-18.8 mL/g tissue, NS). There were no differences in myocardial blood flow between the groups, and plasma norepinephrine was within normal limits in patients and control subjects. CONCLUSIONS: The findings demonstrate a significant reduction of myocardial beta-adrenergic receptor density in patients with ARVC. This may result from a secondary downregulation after increased local synaptic norepinephrine levels caused by increased firing rates of the efferent neurons or as the result of impaired presynaptic catecholamine reuptake. These findings give new insights into the pathophysiology of arrhythmogenesis in ARVC, with potential impact on diagnostic evaluation and therapeutic management.

Efficacy and safety of d,l-sotalol in patients with ventricular tachycardia and in survivors of cardiac arrest.

OBJECTIVE: The aim of this study was to assess the antiarrhythmic efficacy and safety of d,l-sotalol in patients with ventricular tachycardia (VT) or ventricular fibrillation (VF) and in survivors of cardiac arrest and to identify the factors that are associated with arrhythmia suppression and therefore might be helpful in predicting drug efficacy. BACKGROUND: Despite increasing use of the class III antiarrhythmic agent d,l-sotalol, data on its short- and long-term efficacy in a large patient cohort are lacking. Information on its long-term tolerability and safety is limited. METHODS: A total of 396 patients with inducible sustained VT or VF (VT/VF) underwent programmed stimulation before and after receiving oral d,l-sotalol (240 to 640 mg/day). Patients in whom VT/VF was rendered either noninducible or more difficult to induce (more extrastimuli or faster drive cycle length needed for VT/VF induction) were discharged on a regimen of oral d,l-sotalol. RESULTS: d,l-Sotalol suppressed VT/VF in 151 patients (38.1%) and rendered the arrhythmia more difficult to induce in 76 patients (19.2%). The extent of drug-induced prolongation of right ventricular refractoriness and a shorter VT cycle length at baseline were independent predictors of immediate drug efficacy. Torsade de pointes developed in seven patients (1.8%). Two hundred ten patients (53%) continued to receive d,l-sotalol and were followed up for 34 +/- 18 months (mean +/- SD). The actuarial rates for the absence of arrhythmic recurrence (either VT/VF or sudden death) at 1 and 3 years were 89% and 77%, respectively. Actuarial rates for overall survival at 1 and 3 years were 94% and 86%, respectively. VT/VF suppression by d,l-sotalol was an independent discriminant variable that separated patients with and without arrhythmia recurrence. However, noninducibility of VT/VF did not predict freedom from sudden death. CONCLUSION: Oral d,l-sotalol is effective and safe in patients with VT/VF. However, sudden cardiac death develops in a significant proportion of patients, and programmed stimulation seems to be of limited value for its prediction.

Cardiac sympathetic innervation in patients with idiopathic right ventricular outflow tract tachycardia.

OBJECTIVES: This study investigated the neuronal reuptake of norepinephrine (uptake-1) and the beta-adrenoceptor density in patients with idiopathic right ventricular outflow tract tachycardia (RVO-VT). BACKGROUND: Clinical findings, such as the inducibility of ventricular tachycardia by stress or catecholamine infusion, and the therapeutic efficacy of antiarrhythmic drugs with antiadrenergic properties suggest abnormalities of cardiac sympathetic innervation in patients with idiopathic RVO-VT. METHODS: Eight patients with idiopathic RVO-VT and a total of 29 age-matched control subjects were investigated by positron emission tomography using [11C]hydroxyephedrine (HED) (volume of distribution of [11C]HED) to assess presynaptic norepinephrine reuptake; [11C]CGP 12177 (maximal binding capacity of [11C]CGP 12177) to measure postsynaptic beta-adrenoceptor density; and oxygen-15-labeled water for quantification of myocardial blood flow (MBF). RESULTS: Both myocardial catecholamine reuptake and beta-adrenoceptor density were significantly reduced in patients with idiopathic RVO-VT. The volume of distribution of [11C]HED in patients with RVO-VT was (mean +/- SD) 41.0 +/- 13.5 versus 71.0 +/- 18.8 ml/g in control subjects (p < 0.002). The maximal binding capacity of the beta-adrenoceptor antagonist [11C] CGP 12177 was 6.8 +/- 1.2 pmol/g in patients with RVO-VT versus 10.2 +/- 2.9 pmol/g in control subjects (p < 0.004). There were no significant differences in MBF at rest (0.98 +/- 0.14 vs. 0.97 +/- 0.24 ml/min per g, p = NS) between patients with RVO-VT and control subjects. CONCLUSIONS: The findings of the present study suggest that myocardial beta-adrenoceptor downregulation in patients with RVO-VT occurs subsequently to increased local synaptic catecholamine levels caused by impaired catecholamine reuptake.

[Right ventricular tachyarrhythmias--diagnostics and therapy]. / Rechtsventrikuläre Tachyarrhythmien--Diagnostik und Therapie.

Ventricular tachyarrhythmias originating from the right ventricle frequently occur in young, apparently healthy patients with rare underlying cardiac diseases. Among these are arrhythmogenic right ventricular cardiomyopathy (ARVC), idiopathic right ventricular outflow-tract tachycardia (RVOVT), and Brugada syndrome (BrS). All harbor the risk of sudden cardiac death, whereas they differ substantially as to diagnosis, therapy and prognosis. This is the reason why detailed investigations are an essential prerequisite for further efficient individualized management strategies which are mainly directed to prevent sudden cardiac death and to minimize the risk of arrhythmia recurrences in affected patients, respectively. Both antiarrhythmic drug therapy, catheter ablation, and the implantation of an automatic cardioverter defibrillator may, therefore, be a first-line therapeutic option in tailored treatment regimens. This review is a summary of the available literature on pathogenesis, diagnosis, treatment, and prognosis of such diseases associated with right ventricular tachyarrhythmias.

Cardiac 123I-MIBG uptake in idiopathic ventricular tachycardia and fibrillation.

UNLABELLED: Patients with idiopathic ventricular tachycardia or fibrillation have no additional structural or functional myocardial abnormalities. However, the inducibility of typical tachyarrhythmias by physical or mental stress or by catecholamine infusion suggests the involvement of the adrenergic system in the pathogenesis of these potentially life-threatening diseases. METHODS: 45 patients with idiopathic right ventricular outflow tract tachycardia (RVO-VT), 25 patients with idiopathic left ventricular tachycardia (ILVT), 15 patients with idiopathic ventricular fibrillation (IVF) and 10 age-matched control patients were investigated in this study. Diagnoses were made on the basis of detailed evaluation of the results of two-dimensional echocardiography, left and right ventricular angiography, coronary angiography and endomyocardial biopsy. Local presynaptic norepinephrine re-uptake was assessed using the norepinephrine analog 1231-metaiodobenzylguanidine (MIBG), SPECT and semiquantitative 33-segment bull's-eye analysis. RESULTS: Locally reduced 123I-MIBG uptake was found in 27 of 45 RVO-VT patients (60%), 5 of 15 ILVT patients (33%) and 17 of 25 IVF patients (68%). Unlike ILVT patients, RVO-VT and IVF patients had significantly reduced segmental 123I-MIBG uptake of the posterior wall compared with control patients. CONCLUSION: Patients with idiopathic tachycardia and fibrillation show abnormal 1231-MIBG uptake, which indicates presynaptic sympathetic dysfunction. RVO-VT and IVF patients exhibit significantly reduced 123I-MIBG uptake in the posterior left ventricular wall, whereas ILVT patients do not.

Response to sotalol predicts the response to amiodarone during serial drug testing in patients with sustained ventricular tachycardia and coronary artery disease.

UNLABELLED: It was analyzed whether the response to sotalol can predict the response to amiodarone as evaluated by programmed ventricular stimulation in 30 patients with coronary artery disease and documented recurrent sustained ventricular tachycardia (VT). Programmed ventricular stimulation was performed using 1 or 2 extrastimuli during sinus rhythm and 4 drive cycle lengths at 2 right ventricular sites. If no ventricular tachyarrhythmia was induced, a third extrastimulus was introduced during a paced cycle length of 500 ms. During the control study, VT (mean cycle length 305 +/- 63 ms) was induced in all patients, and the right ventricular effective refractory period (during S1-S1 = 500 ms) was 223 +/- 12 ms. After sotalol, sustained and nonsustained VT were inducible in 22 (73%) and 7 (23%) patients, respectively. One patient did not undergo stimulation on sotalol, because of side effects. After amiodarone, sustained and nonsustained VT were inducible in 23 (77%) and 7 (23%) patients, respectively. The mean cycle length of the induced VT was prolonged after both drugs by 17% (p < 0.001). The effective refractory period was prolonged by 15% (p < 0.001) after sotalol and by 13% (p < 0.001 compared with baseline study; p = NS between both drugs) after amiodarone. Thus, concordant results (effective or ineffective drug) between sotalol and amiodarone were found in 26 patients (87%). IN CONCLUSION: (1) The effects of sotalol and amiodarone on the cycle length of induced VT and on right ventricular effective refractory period were similar; and (2) inability to suppress VT by amiodarone can be predicted from the response to sotalol.(ABSTRACT TRUNCATED AT 250 WORDS)

Activation of the cardiac interleukin-6 system in advanced heart failure.

OBJECTIVES: The study objective was to assess the cardiac expression of interleukin-6 (IL6) and its receptor (IL6R) in advanced heart failure. BACKGROUND: While IL6 plasma levels are elevated and associated with an impaired prognosis in advanced heart failure, little is known about the intracardiac expression of the IL6 system. METHODS: Heart tissue was obtained from 20 patients (n=10, idiopathic dilated cardiomyopathy, age 44+/-15 years; n=10, ischemic cardiomyopathy, age 55+/-8 years) at the time of transplantation. Left and right ventricular tissue was subjected to in situ hybridization, Northern blot analysis, and RT-PCR. Signals were quantified by densitometric scanning and corrected for G3PDH-mRNA levels. Right ventricular biopsy specimens (n=11) of patients with arrhythmias and normal cardiac function served as controls. In addition, data were correlated with cardiac catheterization and echocardiography data obtained at transplant evaluation. RESULTS: Ventricular IL6 and IL6R transcripts were detected in all explant specimens examined. Expression of both mRNA species was higher than in controls (P=0.001). Left ventricular IL6 mRNA levels correlated positively with heart rate (r=0.77; P=0.009), pulmonary capillary wedge pressure (r=0.53; P=0.03), right atrial pressure (r=0.77; P=0.003), and inversely with left ventricular ejection fraction (r=-0.61; P=0.03). Right ventricular IL6 mRNA levels correlated inversely with cardiac index (r=-0.48; P=0.05). IL6R expression did not correlate with hemodynamic data. CONCLUSIONS: In advanced heart failure, cardiac IL6/IL6R mRNA expression is increased and may play a role in the pathophysiology of advanced heart failure.

Metallothionein: localization in human transplant endomyocardium, relation to cytokines and allograft function.

BACKGROUND: The aim of this study was to investigate the role of metallothionein in cardiac transplants in relation to cytokines and allograft function. Recent studies have revealed an association of allograft dysfunction with elevated proinflammatory cytokines independent of cellular rejection. In animal experiments, cytokines induced overexpression of metallothionein, a low-molecular-weight protein implicated in cellular stress response. METHODS: In 105 consecutive biopsies from 15 patients during the first 3 months after heart transplantation, metallothionein expression was investigated immunohistochemically. Its relation to serum interleukin-6, tumor necrosis factor-alpha, interleukin-2 (IL-2), soluble interleukin-2 receptor rejection, and echocardiographic parameters was determined. Forty-three biopsies of 12 patients with idiopathic ventricular tachycardia served as controls. RESULTS: Metallothionein expression was demonstrated in small vessels, cardiomyocytes, fibrocytes, and interstitial round cells. A positive relation between interleukin-6 levels and the number of metallothionein-positive small vessels (p < 0.028) was observed. Patients with lower serum IL-2 levels showed significantly higher numbers of metallothionein-positive small vessels (p < 0.043). Grafts with prolonged ischemic time (>150 minutes) showed a significantly higher myocardial metallothionein score (p < 0.021). Metallothionein expression was associated with lower fractional shortening, larger left ventricular end-systolic diameter, and lower mean arterial pressure but not with acute cellular rejection. CONCLUSIONS: Metallothionein expression is associated with elevated interleukin-6 and decreased interleukin-2 serum levels and left ventricular allograft dysfunction in the absence of rejection.

Left ventricular pseudoaneurysm in a patient with Dressler's syndrome after myocardial infarction.

Successful recanalisation of the left anterior descending coronary artery was performed in a 51 year old man who was admitted two weeks after acute anterior myocardial infarction. Fourteen days later, the patient developed Dressler's syndrome with cardiac tamponade, which was immediately punctured. Sternotomy was performed after two weeks because of progressive haemodynamic deterioration, and fibrinous masses were removed from the pericardium. The patient recovered but two weeks later echocardiography showed a perforation of the left ventricular free wall and formation of a pseudoaneurysm. Intensive monitoring showed significant enlargement of the pseudoaneurysm, which was subsequently resected. This case demonstrates that dangerous formation of a pseudoaneurysm can occur not only during the first days of acute myocardial infarction but also after weeks in patients suffering from non-infectious pericarditis caused by Dressler's syndrome. Although the incidence of Dressler's syndrome is declining, patients should be monitored carefully for several weeks, especially by echocardiography.

Minimally-invasive versus conventional aortic valve replacement--perioperative course and mid-term results.

OBJECTIVE: We performed a case-control-study to compare perioperative and mid-term results of minimally invasive with conventional aortic valve replacement. METHODS: Between 8/96 and 7/97, 113 patients underwent isolated aortic valve replacement (minimally invasive: 29, conventional: 84) in our Department. Diagnosis, ejection fraction, pressure gradient/regurgitation fraction, age, gender and body-mass-index were used as matching criteria for the case-control-study. For qualitative data correspondence was requested, for quantitative data deviations up to 10% were accepted. With these criteria 25 patients of the minimally invasive group were matched to 25 patients of conventional group. All patients were reexplored 1 year after aortic valve replacement. Statistical analysis was done by the Fisher's exact test for qualitative data and the Mann-Whitney test for quantitative data. RESULTS: We implanted 15 (20) bioprosthesis' and 10 (five) mechanical prosthesis' in the minimally invasive, respectively, conventional group. There were no statistically significant differences between both groups with respect to the perioperative course, only duration of surgery (mean 201.6 vs. 143.9 min, P < 0.01) and extracorporeal circulation (mean 116.1 vs. 71.3 min, P < 0.01) as well as aortic-cross-clamp-time (mean 77.9 vs. 46.9 min, P < 0.01) were significantly longer in the minimally invasive group. Postoperative complications occurred in one patient of the minimally invasive group (dissection of the right coronary artery) and four patients of the conventional group (third degree AV block, pneumothorax, grand mal convulsion, cardiopulmonary resuscitation). Two patients, one of each group, died during follow-up for unknown reasons. Follow-up revealed no significant differences with respect to clinical and echocardiographic data, but the shorter skin incision was cosmetically more accepted by patients of the minimally invasive group. Minor paravalvular leaks occurred in four patients of the minimally invasive and three patients of the conventional group as diagnosed by transthoracic echocardiography. CONCLUSIONS: Both surgical techniques may be performed with comparable perioperative and mid-term results, but the better cosmetic result in the minimally invasive group is paid by a longer duration of surgery.

[MRI study of left ventricular function in patients with coronary disease and myocardial dysfunction before and after coronary revascularization]. / MR-tomographische Untersuchung der linksventrikulären Funktion bei Patienten mit koronarer Herzerkrankung und myokardialer Dysfunktion vor und nach koronarchirurgischer Revaskularisation.

PURPOSE: To evaluate left ventricular (LV) myocardial function in ten patients with coronary artery disease (CAD) preoperatively and 6 months after coronary bypass grafting (CABG) by cardiac MRI. MATERIAL AND METHODS: Ten patients (mean 65.2 +/- 5.9 years) with angiographically proven CAD and an indication for elective CABG underwent prospective evaluation of global LV function and regional wall motion by Cine-MRI at rest using a multiphase FLASH-2D sequence following regions of interest (ROI)-defined diagnostics of regional myocardial wall motion by means of levocardiography. Within the ROIs a total of 613 LV myocardial segments were analyzed preceding and following surgical revascularization. Results were compared with the data of 10 healthy volunteers. RESULTS: Preoperatively, patients showed reduced stroke volume and ejection fraction compared with volunteers (p < 0.01). Enddiastolic wall thickness (EDWT) and systolic wall thickening (SWT) were significantly lower in the patients (p < 0.01). Based on preoperative levocardiography ROI-defined myocardial segments showed a significantly lower preoperative EDWT in areas with wall motion abnormalities (7.4 +/- 2.5 mm; p < 0.01) than in normal myocardium (9.2 +/- 2.1 mm). Ejection fraction (p < 0.05), endsystolic wall thickness, and SWT (p < 0.01) improved significantly after bypass surgery. On ROI-defined analysis myocardial segments with impaired preoperative wall motion (n = 243) showed a significant increase of EDWT, ESWT and SWT (p < 0.01). CONCLUSION: In patients with CAD, cardiac MRI enables the non-invasive determination of postinfarctional LV remodeling with an increased EDWT of myocardial segments with normal regional wall motion and of the improvement in global and regional myocardial function following coronary bypass surgery.

Radioligands for imaging myocardial alpha- and beta-adrenoceptors.

Alpha- and beta-adrenoceptors play an important role in the control of heart function. According to their molecular, biological, and pharmacological characteristics, they are subdivided into alpha(1)-, alpha(2)- and beta(1)-, beta(2)-, beta(3)-, beta(4)-adrenoceptors. In cardiac disease, there is often a selective downregulation of beta(1)-adrenoceptors associated with a relative increase in beta(2)- and alpha(1)-adrenoceptors. Functional imaging techniques like single-photon emission tomography (SPECT) and positron emission tomography (PET) provide the unique capability for non-invasive assessment of cardiac adrenoceptors. Radioligands with high specific binding to cardiac alpha- and beta-adrenoceptors suitable for radiolabelling are required for clinical studies. The non-selective beta-adrenoceptor antagonist [(11)C]CGP-12177 was used to quantify beta-adrenoceptor density using PET in patients with heart disease. New non-selective ligands (e. g. [(11)C]CGP-12388, [(18)F]CGP-12388, [(11)C]carazolol and [(18)F]fluorocarazolol) are currently evaluated; beta(1)-selective radioligands (e. g. [(11)C]CGP-26505, [(11)C]bisoprolol, [(11)C]HX-CH 44) and beta(2)-selective radioligands (e. g. [(11)C]formoterol, [(11)C]ICI-118551) were assessed in animals. None of them turned out as suitable for cardiac PET. Potential radioligands for imaging cardiac alpha(1)-adrenoceptors are based on prazosin. Whereas [(11)C]prazosin shows low specific binding to myocardium, its derivative [(11)C]GB67 looks more promising. The putative alpha(2)-adrenoceptor radioligand [(11)C]MK-912 shows high uptake in rodent myocardium but has not yet been evaluated in man. A number of radioligands were evaluated for assessing cardiac adrenoceptors using PET. New radioligands are needed to provide more insight into cardiac pathophysiology which may influence the therapeutic management of patients with cardiovascular disease.

Are there gender differences in patients with coronary artery disease presenting with spontaneous sustained ventricular tachycardia and ventricular fibrillation?

The incidence of coronary artery disease (CAD) is greater in men than in women. The aim of the study was to analyze whether any gender-related differences in patients with CAD and documented spontaneous sustained ventricular tachyarrhythmias exist, and which parameters influence the induction of sustained ventricular tachyarrhythmias. The data of 250 patients [43 women (17.2%) and 207 men (82.8%)] with spontaneous sustained ventricular tachycardia [n = 190 (76%)] and fibrillation [n = 60 (24%)] who underwent coronary and left ventricular angiography, electrophysiological study, and signal-averaging electrocardiogram (ECG) form the basis of this analysis. No gender-related differences could be observed in age, number of diseased coronary arteries, history, location and number of myocardial infarctions, presence of left ventricular aneurysm, ejection fraction, type of spontaneous or induced arrhythmias, right ventricular effective refractory period, and signal-averaged ECG parameters. Age, presence of previous myocardial infarction, and ejection fraction were significant predictors (p < 0.001) of inducibility of sustained ventricular tachyarrhythmias. Once CAD has begun, female and male patients present similar clinical and electrophysiologic characteristics. Thus, both genders should benefit similarly from diagnostic and therapeutic approaches if they are referred to the hospital or to invasive interventions at similar intervals in the course of their illness.

[Arrhythmogenic right ventricular cardiomyopathy. Etiology, diagnosis and therapy]. / Arrhythmogene rechtsventrikuläre Kardiomyopathie. Atiologie, Diagnostik und Therapie.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is characterized by regional atrophy of right ventricular myocardium and subsequent replacement by fatty and fibrous tissue. The disease manifests in young adulthood with a predominance of males. Hallmarks of ARVC are ventricular tachyarrhythmias of left bundle branch block pattern which frequently occur during exercise. However, sudden death may also be the first manifestation of the disease. Characteristic findings are repolarization abnormalities and QRS prolongation in the right precordial leads of the surface ECG and regional abnormalities of right ventricular structure and wall motion. Left ventricular involvement may occur in later stages of the disease but rarely leads to progressive biventricular heart failure. Therapeutic efforts are mainly directed to the treatment of ventricular tachyarrhythmias and the prevention of sudden death. A tailored treatment strategy including antiarrhythmic drug therapy, catheter ablation and implantation of cardioverter-defibrillators may be used to improve the long-term prognosis of patients with ARVC.

Arrhythmogenic right ventricular cardiomyopathy: an update on pathophysiology, genetics, diagnosis, and risk stratification.

Arrhythmogenic right ventricular cardiomyopathy (ARVC) is an inherited cardiomyopathy accounting for life-threatening ventricular tachyarrhythmias and sudden death in young individuals and athletes. Over the past years, mutations in desmosomal genes have been identified as disease-causative. However, genetic heterogeneity and variable phenotypic expression alongside with diverse disease progression still render the evaluation of its prognostic implication difficult. ARVC was initially entered into the canon of cardiomyopathies of the World Health Organization in 1995, and international efforts have resulted in the 2010 modified diagnostic criteria for ARVC. Despite all additional insights into pathophysiology, clinical management, and modern risk stratification, under-/misdiagnosing of ARVC remains a problem and hampers reliable statements on the incidence, prevalence, and natural course of the disease.This review provides a comprehensive overview of the current literature on the pathogenesis, diagnosis, treatment, and prognosis of ARVC and sheds some light on potential new developments in these areas.