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Alzheimer's disease (AD), the most common cause of dementia in the elderly, is a neurodegenerative disorder associated with neurovascular dysfunction and cognitive decline. While the deposition of amyloid β peptide (Aβ) and the formation of neurofibrillary tangles (NFTs) are the pathological hallmarks of AD-affected brains, the majority of cases exhibits a combination of comorbidities that ultimately lead to multi-organ failure. Of particular interest, it can be demonstrated that Aβ pathology is present in the hearts of patients with AD, while the formation of NFT in the auditory system can be detected much earlier than the onset of symptoms. Progressive hearing impairment may beget social isolation and accelerate cognitive decline and increase the risk of developing dementia. The current review discusses the concept of a brain-ear-heart axis by which Aβ and NFT inhibition could be achieved through targeted supplementation of neurotrophic factors to the cochlea and the brain. Such amyloid inhibition might also indirectly affect amyloid accumulation in the heart, thus reducing the risk of developing AD-associated amyloid cardiomyopathy and cardiovascular disease.

Purpose: Amyloidosis represents an increasingly recognized but still frequently missed cause of heart failure. In the light of many effective therapies for light chain (AL) amyloidosis and promising new treatment options for transthyretin (ATTR) amyloidosis, awareness among caregivers needs to be raised to screen for amyloidosis as an important and potentially treatable differential diagnosis. This review outlines the diversity of cardiac amyloidosis, its relation to heart failure, the diagnostic algorithm, and therapeutic considerations that should be applied depending on the underlying type of amyloidosis., Recent Findings: Non-biopsy diagnosis is feasible in ATTR amyloidosis in the absence of a monoclonal component resulting in higher detection rates of cardiac ATTR amyloidosis. Biomarker-guided staging systems have been updated to facilitate risk stratification according to currently available biomarkers independent of regional differences, but have not yet prospectively been tested. Novel therapies for hereditary and wild-type ATTR amyloidosis are increasingly available. The complex treatment options for AL amyloidosis are improving continuously, resulting in better survival and quality of life. Mortality in advanced cardiac amyloidosis remains high, underlining the importance of early diagnosis and treatment initiation. Cardiac amyloidosis is characterized by etiologic and clinical heterogeneity resulting in a frequently delayed diagnosis and an inappropriately high mortality risk. New treatment options for this hitherto partially untreatable condition have become and will become available, but raise challenges regarding their implementation. Referral to specialized centers providing access to extensive and targeted diagnostic investigations and treatment initiation may help to face these challenges.

Prognosis of patients with light-chain cardiac amyloidosis (AL-CA) is poor. Speckle tracking imaging (STI) derived longitudinal deformation parameters and Doppler-derived left ventricular (LV) Tei index are valuable predictors of outcome in patients with AL-CA. We estimated the prognostic utility of Tei index and deformation parameters in 58 comprehensively phenotyped patients with AL-CA after a median follow-up of 365 days (quartiles 121, 365 days). The primary end point was all-cause mortality. 19 (33%) patients died during follow-up. Tei index (0.89 ± 0.29 vs. 0.61 ± 0.16, p < 0.001) and E to global early diastolic strain rate ratio (E/GLSR dias ) were higher while global longitudinal systolic strain (GLS sys ) was lower in non-survivors than in survivors (all p < 0.05). Tei index, NYHA functional class, GLS sys and E/GLSR dias were independent predictors of all-cause mortality risk, and Tei index ≥0.9 (HR 7.01, 95% CI 2.43-20.21, p < 0.001) was the best predictor of poor outcome. Combining Tei index and GLS sys yielded the best results on predicting death within 1 year (100% with Tei index ≥0.9 and GLS sys ≤13%) or survival (95% with Tei index ≤0.9 and GLS sys ≥13%). We conclude that 1-year mortality risk in AL-CA patients can be reliably predicted using Tei index or deformation parameters, with combined analysis offering best performance.

Background: High-sensitivity troponin (hs-TNT), a biomarker of myocardial damage, might be useful for assessing fibrosis in Fabry cardiomyopathy. We performed a prospective analysis of hs-TNT as a biomarker for myocardial changes in Fabry patients and a retrospective longitudinal follow-up study to assess longitudinal hs-TNT changes relative to fibrosis and cardiomyopathy progression., Methods and Results: For the prospective analysis, hs-TNT from 75 consecutive patients with genetically confirmed Fabry disease was analyzed relative to typical Fabry-associated echocardiographic findings and total myocardial fibrosis as measured by late gadolinium enhancement (LE) on magnetic resonance imaging. Longitudinal data (3.9±2.0 years), including hs-TNT, LE, and echocardiographic findings from 58 Fabry patients, were retrospectively collected. Hs-TNT level positively correlated with LE (linear correlation coefficient, 0.72; odds ratio, 32.81 [95% CI, 3.56-302.59]; P=0.002); patients with elevated baseline hs-TNT (>14 ng/L) showed significantly increased LE (median: baseline, 1.9 [1.1-3.3] %; follow-up, 3.2 [2.3-4.9] %; P<0.001) and slightly elevated hs-TNT (baseline, 44.7 [30.1-65.3] ng/L; follow-up, 49.1 [27.6-69.5] ng/L; P=0.116) during follow-up. Left ventricular wall thickness and EF of patients with elevated hs-TNT were decreased during follow-up, indicating potential cardiomyopathy progression., Conclusions: hs-TNT is an accurate, easily accessible clinical blood biomarker for detecting replacement fibrosis in patients with Fabry disease and a qualified predictor of cardiomyopathy progression. Thus, hs-TNT could be helpful for staging and follow-up of Fabry patients., (© 2016 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Despite substantial advances in the imaging techniques and pathophysiological understanding over the last decades, identification of the underlying causes of left ventricular hypertrophy by means of echocardiographic examination remains a challenge in current clinical practice. The longitudinal strain bull's eye plot derived from 2D speckle tracking imaging offers an intuitive visual overview of the global and regional left ventricular myocardial function in a single diagram. The bull's eye mapping is clinically feasible and the plot patterns could provide clues to the etiology of cardiomyopathies. The present review summarizes the longitudinal strain, bull's eye plot features in patients with various cardiomyopathies and concentric left ventricular hypertrophy and the bull's eye plot features might serve as one of the cardiac workup steps on evaluating patients with left ventricular hypertrophy.

Background: In spite of several research studies help to describe the heart in Fabry disease (FD), the cardiomyopathy is not entirely understood. In addition, the impact of blood pressure and alterations in geometry have not been systematically evaluated., Methods: In 74 FD patients (mean age 36±12 years; 45 females) the extent of myocardial fibrosis and its progression were quantified using cardiac magnetic-resonance-imaging with late enhancement technique (LE). Results were compared to standard echocardiography complemented by 2D-speckle-tracking, 3D-sphericity-index (SI) and standardized blood pressure measurement. At baseline, no patient received enzyme replacement therapy (ERT). After 51±24 months, a follow-up examination was performed., Results: Systolic blood pressure (SBP) was higher in patients with vs. without LE: 123±17 mmHg vs. 115±13 mmHg; P = 0.04. A positive correlation was found between SI and the amount of LE-positive myocardium (r = 0.51; P<0.001) indicating an association of higher SI in more advanced stages of the cardiomyopathy. SI at baseline was positively associated with the increase of LE-positive myocardium during follow-up. The highest SBP (125±19 mmHg) and also the highest SI (0.32±0.05) was found in the subgroup with a rapidly increasing LE (ie, ≥0.2% per year; n = 16; P = 0.04). Multivariate logistic regression analysis including SI, SBP, EF, left ventricular volumes, wall thickness and NT-proBNP adjusted for age and sex showed SI as the most powerful parameter to detect rapid progression of LE (AUC = 0.785; P<0.05)., Conclusions: LV geometry as assessed by the sphericity index is altered in relation to the stage of the Fabry cardiomyopathy. Although patients with FD are not hypertensive, the SBP has a clear impact on the progression of the cardiomyopathy.

Relative apical sparing of longitudinal systolic strain (LSsys) with preserved LSsys at apical and significantly reduced LSsys at mid/basal segments is a typical echocardiographic feature in AL amyloidosis patients with cardiac involvement. The present study aims to evaluate the change of this typical feature over time by serial echocardiography and its impact on outcome in AL amyloidosis patients with cardiac involvement. Echocardiography was performed in 24 consecutive patients with biopsy-proven AL amyloidosis (mean age 64 ± 9 years; 50% male) at baseline and during a median of 257 (quartiles 103-651) days follow-up. Global and segmental LSsys were assessed by two-dimensional speckle-tracking-imaging in septal and lateral segments of the left ventricle (LV) from the apical 4-chamber view. Sixteen (67%) patients died during a median follow-up of 487 days (quartiles 223-872). LV global and segmental LSsys remained unchanged over time in survivors (all P > 0.05), while LV global, septal-apical and lateral-apical LSsys significantly decreased in non-survivors. A decrease in lateral-apical LSsys > 3.0% independently predicted a fivefold increased all-cause mortality risk after adjustment for age, gender, NYHA class, and treatment strategies. Further, baseline serum NT-proBNP, serum albumin decrease during follow-up, baseline septal apical-to-basal LSsys ratio and lateral-apical LSsys decrease during follow-up remained independently predictive of increased all-cause mortality risk. Serial monitoring of serological and echocardiographic parameters is valuable to predict outcome in AL amyloidosis patients with cardiac involvement. The best follow-up parameter to predict risk for imminent death is a decrease of longitudinal systolic strain at the lateral apical segment.

Fabry disease is induced by a mutation in the alpha-galactosidase A gene, causing a deficiency of the enzyme alpha-galactosidase A. (1) The enzyme defect leads to progressive intracellular accumulation of globotriaosylceramide in lysosomes of various tissues and organs, including heart, kidney and nerve system. Cardiac involvement is common and is presenting as concentric left ventricular hypertrophy. Myocardial replacement fibrosis is a typical feature of more advanced stages of Fabry cardiomyopathy, first limited to the mid-myocardial layers of the basal postero-lateral wall, then spreading to transmural fibrosis. Since 2001, enzyme replacement therapy is available. If therapy is started early, before myocardial fibrosis has developed, a long-term improvement of myocardial morphology, function and exercise capacity can be achieved. In end-stage cardiomyopathy enzyme replacement therapy might prevent further progression of the disease. This review provides an overview of Fabry disease, with a focus on cardiac involvement with its characteristic features, clinical presentation and possible treatment., (Copyright © 2014 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Objectives: Since diastolic abnormalities are typical findings of cardiac amyloidosis (CA), we hypothesized that speckle-tracking-imaging (STI) derived longitudinal early diastolic strain rate (LSRdias) could predict outcome in CA patients with preserved left ventricular ejection fraction (LVEF >50%)., Background: Diastolic abnormalities including altered early filling are typical findings and are related to outcome in CA patients. Reduced longitudinal systolic strain (LSsys) assessed by STI predicts increased mortality in CA patients. It remains unknown if LSRdias also related to outcome in these patients., Methods: Conventional echocardiography and STI were performed in 41 CA patients with preserved LVEF (25 male; mean age 65±9 years). Global and segmental LSsys and LSRdias were obtained in six LV segments from apical 4-chamber views., Results: Nineteen (46%) out of 41 CA patients died during a median of 16 months (quartiles 5-35 months) follow-up. Baseline mitral annular plane systolic excursion (MAPSE, 6 ± 2 vs. 8 ± 3 mm), global LSRdias and basal-septal LSRdias were significantly lower in non-survivors than in survivors (all p < 0.05). NYHA class, number of non-cardiac organs involved, MAPSE, mid-septal LSsys, global LSRdias, basal-septal LSRdias and E/LSRdias were the univariable predictors of all-cause death. Multivariable analysis showed that number of non-cardiac organs involved (hazard ratio [HR] = 1.96, 95% confidence interval [CI] 1.17-3.26, P = 0.010), global LSRdias (HR = 7.30, 95% CI 2.08-25.65, P = 0.002), and E/LSRdias (HR = 2.98, 95% CI 1.54-5.79, P = 0.001) remained independently predictive of increased mortality risk. The prognostic performance of global LSRdias was optimal at a cutoff value of 0.85 S-1 (sensitivity 68%, specificity 67%). Global LSRdias < 0.85 S-1 predicted a 4-fold increased mortality in CA patients with preserved LVEF., Conclusions: STI-derived early diastolic strain rate is a powerful independent predictor of survival in CA patients with preserved LVEF.

The aim of this study was to investigate the impact of myocardial fibrosis in Fabry disease. Seventy-three patients with genetically confirmed Fabry disease were followed for 4.8 ± 2.4 years. In accordance with current guidelines, 57 patients received enzyme replacement therapy (ERT) after study inclusion, whereas 16 did not. At baseline and latest possible follow-up, myocardial fibrosis was assessed noninvasively by cardiac magnetic resonance, and biomarkers of collagen metabolism were determined. Holter electrocardiography and clinical follow-up at yearly intervals were used to monitor malignant ventricular arrhythmias (MVAs; nonsustained and sustained ventricular tachycardia and sudden cardiac death). In total, 48 patients (66%) showed fibrosis assessed by late enhancement (LE) at baseline, and 4 patients developed new LE during follow-up, 2 of them despite ERT. The 2 patients receiving ERT (1.4 ± 1.9% vs 2.5 ± 2.6%, p <0.001) and the patients not receiving ERT (0.5 ± 0.8% vs 0.7 ± 1.0%, p = 0.035) showed a progression of LE during follow-up. None of the patients displayed reductions of LE during follow-up. Collagen biomarkers were elevated in patients with and without LE but did not correlate with LE amount. Thirteen LE-positive patients at the baseline examination had documented MVAs (including 5 sudden cardiac deaths), whereas none of the patients without LE had MVAs. The yearly increase in fibrosis was 0.9 ± 0.6% in patients with MVAs and 0.2 ± 0.3% in patients without MVAs (p <0.001). Logistic multivariate regression analysis revealed that the annual increase in fibrosis during follow-up was the only independent predictor of MVAs. In conclusion, myocardial fibrosis in Fabry disease is progressive, apparently not modified by ERT, and a crucial outcome determinant., (Copyright © 2014 The Authors. Published by Elsevier Inc. All rights reserved.)

Sarcoidosis is a multisystemic granulomatous disorder which affects the respiratory system in the majority of the cases. Cardiac manifestations are found in up to 10 % of the affected cohort and show a large heterogeneity based on the ethnic background. Cardiac sarcoidosis are not only found in patients with rhythmogenic heart disease such as atrial and ventricular fibrillation but also in all phenotypes of cardiomyopathies. The overall morbidity and mortality caused by cardiac sarcoidois in Germany is unclear and no large prospective international studies are published on this topic. This consensus paper on diagnostic and therapeutic algorithms in cardiac sarcoidosis is based on a current literature search and forms a expert opinion statement under the hospices of the "Deutsche Gesellschaft für Pneumologie" and "Deutsche Gesellschaft für Kardiologie". It is the rationale of this statement to offer algorithms to facilitate clinical decision-making based on the individual case., (© Georg Thieme Verlag KG Stuttgart · New York.)

Background: Differentiation of cardiac amyloidosis (CA) from other causes of concentric left ventricular hypertrophy remains a clinical challenge, especially in patients with preserved ejection fraction at the early disease stages., Methods and Results: Consecutive hypertrophic patients with CA, isolated arterial hypertension, Fabry disease, and Friedreich ataxia (n=25 per group) were investigated; 25 healthy volunteers served as a control group. Standard echocardiography was performed, and segmental longitudinal peak systolic strain (LSsys) in the septum was assessed by 2-dimensional speckle tracking imaging. Indices of left ventricular hypertrophy and ejection fraction were similar among all patient groups. Deceleration time of early filling was significantly lower in patients with CA (147±46 milliseconds) compared with those with isolated arterial hypertension, Fabry disease, or control subjects (all P<0.0125). Septal basal LSsys (-6±2%) was significantly lower in patients with CA compared with those with isolated arterial hypertension (-14±6%), Fabry disease (-12±5%), Friedreich ataxia (-16±2%), or control subjects (-17±3%; all P<0.001), whereas septal apical LSsys was similar among all patient groups and control subjects (all P>0.05). A data-driven cutoff value for the ratio of septal apical to basal LSsys ratio >2.1 differentiated CA from other causes of left ventricular hypertrophy (sensitivity, 88%; specificity, 85%; positive predictive value, 67%; negative predictive value, 96%). The prevalence of septal apical to basal LSsys ratio >2.1 plus deceleration time of early filling <200 milliseconds was 88% in CA but 0% in all other groups., Conclusions: A systolic septal longitudinal base-to-apex strain gradient (septal apical to basal LSsys ratio >2.1), combined with a shortened diastolic deceleration time of early filling (deceleration time of early filling <200 milliseconds), aids in differentiating CA from other causes of concentric left ventricular hypertrophy.

Friedreich's ataxia is a rare hereditary, predominantly neurologically defined multisystem disorder of mitochondrial function. Although the gene defect has been identified, the precise pathophysiology of the deficient mitochondrial protein, frataxin, is unknown. Besides the characteristic features of spinocerebellar ataxia the heart may also be affected, and patients may experience a hypertrophic cardiomyopathy eventually progressing toward heart failure and death. So far, research focused on the neurological aspects and little attention has been paid to better characterize and understand the cardiac involvement in Friedreich's ataxia. For that, a better understanding of longitudinal progression, cardiac complications and long-term cardiac outcome is warranted. In addition, the clinician should be familiar with the therapeutic option in Friedreich cardiomyopathy. This review discusses important clinical and diagnostic features of the cardiomyopathy in Friedreich's ataxia and potential therapeutic developments., (© 2013 International Society for Neurochemistry.)

Peripartum cardiomyopathy (PPCM) is a rare disease of the heart muscle that affects women with previously unknown heart diseases during pregnancy or in the first months after delivery. Cardinal symptoms are dyspnoea and fluid retention. However, tachycardia, cardiogenic thromboembolism and other clinical signs of cardiac dysfunction may also herald this uncommon cause of heart failure. The estimated incidence of PPCM shows large regional variations: in Europe and the United States it is between 1:2000 and 1:4000. The markedly higher incidence rates observed in Haitian or South African women (up to 1:300) suggest that genetic or environmental factors may play a pathogenetic role. However, the underlying aetiology of PPCM still is unclear. Besides genetic susceptibility an abnormal autoimmune response against cardiac tissue components, viral infections or an irregular activity of cathepsin D generating a potentially cardio-toxic splice variant of prolactin have been discussed. New therapeutic strategies as immune modulation or prolactin inhibition were therefore suggested, but are not yet established. Treatment strategies focus on the standard therapies for heart failure and its complications. During pregnancy heart failure therapy is limited to substances without fetotoxic effects. But even with optimal heart failure therapy the course of the disease exhibits large variations ranging from full recovery to deterioration of heart function and even early cardiac death. This review cumulates the current knowledge on PPCM and aims to raise awareness for this rare and potentially life-threatening disorder amongst all medical professionals involved in the care for pregnant women.

Background: This cross-sectional study provides a practical approach for the clinical assessment of Friedreich ataxia (FA) cardiomyopathy (FA-CM)., Methods and Results: A comprehensive cardiac assessment, including standard echocardiography, color Doppler myocardial imaging, cardiac magnetic resonance imaging, ECG, and exercise stress testing, was performed in 205 FA patients. To assess myocardial hypertrophy in FA-CM, the end-diastolic interventricular septal wall thickness (IVSTd) was found to be the best echocardiographic parameter compared with cardiac magnetic resonance imaging-determined left ventricular mass. With the use of this parameter, 4 groups of patients with FA-CM could be defined. Patients with normal values for IVSTd (31.7%) were classified as having no FA-CM. Patients with an IVSTd exceeding the predicted normal IVSTd were classified as having mild FA-CM (40%) if IVSTd exceeded the normal value by <18% or as having intermediate FA-CM (16.1%) if IVSTd exceeded the normal value by ≥18%. Patients with ejection fraction <50% were classified as having severe FA-CM (12.2%). In addition to increased myocardial mass, severe FA-CM was further characterized by dilatation of the left ventricle, reduced systolic strain rate of the posterior wall, and ECG abnormalities. Regional myocardial function correlated negatively with FA-CM groups. Younger patients had a tendency for more advanced FA-CM. Importantly, no clear correlation was found between FA-CM groups and neurological function., Conclusions: We provide and describe a readily applicable clinical grouping of the cardiomyopathy associated with FA based on echocardiographic IVSTd and ejection fraction data. Because no distinct interrelations between FA-CM and neurological status could be determined, regular follow-up of potential cardiac involvement in FA patients is essential in clinical practice.

Amyloidoses are a heterogeneous group of multisystem disorders, which are characterized by an extracellular deposition of amyloid fibrils. Typically affected are the heart, liver, kidneys, and nervous system. More than half of the patients die due to cardiac involvement. Clinical signs of cardiac amyloidosis are edema of the lower limbs, hepatomegaly, ascites and elevated jugular vein pressure, frequently in combination with dyspnea. There can also be chest pain, probably due to microvessel disease. Dysfunction of the autonomous nervous system or arrhythmias may cause low blood pressure, dizziness, or recurrent syncope. The AL amyloidosis caused by the deposition of immunoglobulin light chains is the most common form. It can be performed by monoclonal gammopathy. The desirable treatment therapy consists of high-dose melphalan therapy twice followed by autologous stem cell transplantation. Due to the high peritransplantation mortality, selection of appropriate patients is mandatory. The ATTR amyloidosis is an autosomal dominant disorder caused by the amyloidogenic form of transthyretin, a plasmaprotein that is synthesized in the liver. Therefore, liver transplantation is the only curative therapy. The symptomatic treatment of cardiac amyloidosis is based on the current guidelines for chronic heart failure according to the patient's New York Heart Association (NYHA) state. Further types of amyloidosis with possible cardiac involvement comprise the senile systemic amyloidosis caused by the wild-type transthyretin, secondary amyloidosis after chronic systemic inflammation, and the beta(2)-microglobulin amyloidosis after long-term dialysis treatment.

Background: The aim of this study was to estimate the independent and incremental prognostic value of the presence of stimulating autoantibodies directed against the human beta1-adrenergic receptor (anti-beta1-AR) in patients with chronic heart failure., Methods: One hundred five antibody-typed chronic heart failure patients with dilated cardiomyopathy (DCM, n = 65) or ischemic cardiomyopathy (ICM, n = 40) were prospectively followed for 10.7 +/- 2.5 years. Information on all-cause and cardiovascular mortality was collected throughout the observation period., Results: Stimulating anti-beta1-AR were prevalent in 26% (17/65) of patients with DCM and 13% (5/40) with ICM. All-cause mortality in antibody-positive patients was 65% in those with DCM and 80% in those with ICM, and in antibody-negative patients 44% and 49%, respectively. In univariate and multivariable Cox regression analysis (P < .05), presence of stimulating anti-beta1-AR was associated with increased all-cause and cardiovascular mortality risk in DCM but not in ICM. Information on antibody status improved the prognostic capacity in models containing already extensive information on clinical profile, Holter electrocardiography, and invasive hemodynamic measurements (area under the receiver operating characteristic curve, 0.91; 95% confidence interval, 0.85-0.97; P < .05 for increase in receiver operating characteristic area)., Conclusion: The presence of stimulating anti-beta1-AR autoantibodies independently predicts increased all-cause and cardiovascular mortality risk in DCM conferring incremental prognostic value in addition to established risk predictors. Our data indicate a clinical relevance of stimulating anti-beta1-AR in DCM and encourage further research into antibody-directed strategies as a therapeutic principle.

Aims: The aim of this clinical cross-sectional study was to investigate the cardiac interrelation of morphological and functional abnormalities in patients with Fabry disease., Methods and Results: Fifty-one patients (5-78 years) were compared with 25 controls (8-77 years). In all subjects, end-diastolic thickness of the left ventricle was measured by echocardiography and ultrasonic peak systolic strain rate (SR) was extracted to assess regional myocardial function. Magnetic resonance imaging was performed to assess late-enhancement for the detection of myocardial fibrosis in Fabry patients (n=39). In patients, women <20 years of age had no hypertrophy, no late-enhancement, and normal radial and longitudinal function (SR longitudinal=-1.7+/-0.5 s(-1); P=n.s. compared with controls). Ten women, >20 years of age, had no hypertrophy, no late-enhancement, normal radial and longitudinal function in the septal wall, but reduced longitudinal function in the lateral wall (SR=-1.4+/-0.5 s(-1)). All male patients without hypertrophy and no late-enhancement had normal radial function but reduced longitudinal function in both the septal and lateral walls (SR=-1.3+/-0.3 s(-1)). Patients with hypertrophy but without late-enhancement (n=13) had reduced radial and longitudinal function. Twelve patients displaying hypertrophy and late-enhancement had severely reduced radial and longitudinal function (SR=-1.1+/-0.5 s(-1)). Two of them with the worst impairment of regional function (SR=-0.8+/-0.6 s(-1)) died in the follow-up period., Conclusion: These results illustrate the variation of morphological changes and its functional consequences in Fabry cardiomyopathy.

Patients' health-related quality of life is increasingly being included as an additional endpoint when evaluating the treatment of chronic heart failure. Although generic self-report instruments measuring health-related quality of life are available, there is a lack of disease-specific instruments covering various dimensions of quality of life with high reliability, validity and sensitivity to chance. Thus, the aim of the present study was to evaluate the German version of a new heart failure-specific quality of life measure, the Kansas City Cardiomyopathy Questionnaire (KCCQ). The sample consisted of 233 consecutively recruited outpatients of a university department in Germany. Test-retest-reliability was high (intraclass correlation coefficient 0.93 for both the Functional State and the Clinical Summary total scores). Construct validity was demonstrated with strong correlations to respective subscales of the SF-36. Known groups validity was shown by both statistically and clinically significant differences between NYHA classes. The examination of sensitivity to change yielded promising results. The questionnaire was well accepted by the participating patients. The KCCQ proved to be a reliable and valid self-report instrument for measuring disease-specific quality of life in chronic heart failure.

The article discusses peripartum cardiomyopathy (PPCM), a disease of the heart muscle affecting women who had no known history of heart disease during pregnancy or in the first months after delivery. Symptoms include dyspnoea, fluid retention, tachycardia, cardiogenic and thromboembolism. Observations of incidence rates vary according to region. New therapeutic strategies as immune modulation or prolactin inhibition were suggested. Risk factors for PPCM, etiology, prognosis, and heart failure medication in pregnancy are discussed.

The study investigated the predictive value of activating, anti-&b.beta;1-AR antibodies and various haemodynamic (left heart catheterisation) and clinical baseline parameters including occurrence and severity of ventricular arrhythmias for the outcome of 104 dilated cardiomyopathies and ischemic cardiomyopathies patients, both by univariate and by multivariate Cox regression analysis. The results indicated that activating autoantibodies directed against cardiac &b.beta;1-AR are associated with a twofold increase in mortality in dilated, but not in ischemic cardiomyopathy.