[CME: Heart Failure- Overview, Clinical Manifestation, Diagnosis and Management]. / CME: Herzinsuffizienz ­ Überblick, Klinik, Diagnostik und Therapie.

CME: Heart Failure- Overview, Clinical Manifestation, Diagnosis and Management Abstract. Heart failure is one of the most common diseases in internal medicine with estimated more than 10 million patients in Europe. Another 10 million people suffer from myocardial insufficiency without symptoms. Along with injuries and pathologies of the musculoskeletal system, heart failure is one of the most common causes for hospitalization also in Switzerland. This article provides a brief overview of this common disease. Diagnostic tools as well as therapy options will be discussed.

High Incidence of Inappropriate Alarms in Patients with Wearable Cardioverter-Defibrillators: Findings from the Swiss WCD Registry.

BACKGROUND: The wearable cardioverter defibrillator (WCD) uses surface electrodes to detect arrhythmia before initiating a treatment sequence. However, it is also prone to inappropriate detection due to artefacts. OBJECTIVE: The aim of this study is to assess the alarm burden in patients and its impact on clinical outcomes. METHODS: Patients from the nationwide Swiss WCD Registry were included. Clinical characteristics and data were obtained from the WCDs. Arrhythmia recordings ≥30 s in length were analysed and categorized as VT/VF, atrial fibrillation (AF), supraventricular tachycardia (SVT) or artefact. RESULTS: A total of 10653 device alarms were documented in 324 of 456 patients (71.1%) over a mean WCD wear-time of 2.0 ± 1.6 months. Episode duration was 30 s or more in 2996 alarms (28.2%). One hundred and eleven (3.7%) were VT/VF episodes. The remaining recordings were inappropriate detections (2736 (91%) due to artefacts; 117 (3.7%) AF; 48 (1.6%) SVT). Two-hundred and seven patients (45%) had three or more alarms per month. Obesity was significantly associated with three or more alarms per month (p = 0.01, 27.7% vs. 15.9%). High alarm burden was not associated with a lower average daily wear time (20.8 h vs. 20.7 h, p = 0.785) or a decreased implantable cardioverter defibrillator implantation rate after stopping WCD use (48% vs. 47.3%, p = 0.156). CONCLUSIONS: In patients using WCDs, alarms emitted by the device and impending inappropriate shocks were frequent and most commonly caused by artefacts. A high alarm burden was associated with obesity but did not lead to a decreased adherence.

Marfan syndrome and related connective tissue disorders in the current era in Switzerland in 103 patients: medical and surgical management and impact of genetic testing.

INTRODUCTION: Marfan syndrome (MFS) and related connective tissue disorders (CTDs) are increasingly recognised. Genetic testing has greatly improved the diagnostic outcome/power over the last two decades. In this study we describe a multicentre cohort of adults with MFS and related CTDs, with a particular focus on results from genetic testing. METHODS: All patients with MFS and related CTDs were identified from the databases of five centres in the canton of Zurich. Echocardiographic and clinical findings including systemic Marfan score, use of medication and genetic results were retrospectively analysed. MFS was diagnosed using the revised Ghent criteria (including FBN1 genetic testing if available); other CTDs (Loeys-Dietz syndrome) were diagnosed by genetic testing only. RESULTS: A cohort of 103 patients were identified (62 index patients, 41 relatives of family members): 96 patients with MFS and 7 patients with other CTD, 54 males (52%), median age 23 years (range 1–75). The median systemic Marfan score was 5 (range 0–18). Only 40 patients (40/103, 39%) fulfilled criteria for systemic involvement (≥7 points). A history of aortic dissection was present in 14 out of 103 patients (14%). Echocardiographic data were available for all: aortic root enlargement (Z-score ≥2 in adults, Z-score ≥3 in children) was found in 49 patients (48%) and mitral valve prolapse in 64 (62%). Genetic testing had been performed in 80 patients (78%); FBN1 mutations were present in 69 patients (86%); other pathogenic mutations could be identified in seven patients (9%); no disease-causing mutation was found in four patients, three of them fulfilling the Ghent criteria of MFS. Of the mutation-positive patients, 33 had a systemic score of ≥7 and 43 had a systemic score of ≥5. Revised Ghent criteria were fulfilled in 70 patients: in 69 patients with FBN1 mutations and 1 patient with another CTD. Recommended treatment (beta-blocker, angiotensin receptor blocker) was taken by 63% of patients. CONCLUSIONS: In this cohort a high percentage of patients fulfilling the revised Ghent criteria for MFS underwent genetic testing, often leading to or confirming the diagnosis of MFS. Other CTDs could be discriminated best by genetic testing. With respect to the diagnosis of MFS and related CTDs, the usefulness of the systemic score is limited, showing the importance of genetic testing, which enabled definitive diagnosis in 95% of tested patients. Patient education on medical treatment still has to be improved. (Trial registration no: KEK-ZH-Nr. 2013-0241).

Use of the wearable cardioverter-defibrillator - the Swiss experience.

INTRODUCTION: Sudden cardiac death caused by malignant arrhythmia can be prevented by the use of defibrillators. Although the wearable cardioverter defibrillator (WCD) can prevent such an event, its role in clinical practice is ill defined. We investigated the use of the WCD in Switzerland with emphasis on prescription rate, therapy adherence and treatment rate. MATERIALS AND METHODS: The Swiss WCD Registry is a retrospective observational registry including patients using a WCD. Patients were included from the first WCD use in Switzerland until February 2018. Baseline characteristics and data on WCD usage were examined for the total study population, and separately for each hospital. RESULTS: From 1 December 2011 to 18 February 2018, a total of 456 patients (67.1% of all WCDs prescribed in Switzerland and 81.1% of all prescribed in the participating hospitals) were included in the registry. Up to 2017 there was a yearly increase in the number of prescribed WCDs to a maximum of 271 prescriptions per year. The mean age of patients was 57 years (± 14), 81 (17.8%) were female and mean left ventricular ejection fraction (EF) was 32% (± 13). The most common indications for WCD use were new-onset ischaemic cardiomyopathy (ICM) with EF ≤35% (206 patients, 45.2%), new-onset nonischaemic cardiomyopathy (NICM) with EF ≤35% (115 patients, 25.2%), unknown arrhythmic risk (83 patients, 18.2%), bridging to implantable cardioverter-defibrillator implantation or heart transplant (37 patients, 8.1%) and congenital/inherited heart disease (15 patients, 3.3%). Median wear duration was 58 days (interquartile range [IQR] 31–94) with a median average daily wear time of 22.6 hours (IQR 20–23.2). Seventeen appropriate therapies from the WCD were delivered in the whole population (treatment rate: 3.7%) to a total of 12 patients (2.6% of all patients). The most common underlying heart disease in patients with a treatment was ICM (13/17, 76.5%). There were no inappropriate treatments. CONCLUSION: The use of WCDs has increased in Switzerland over the years for a variety of indications. There is high therapy adherence to the WCD, and a treatment rate comparable to previously published registry data.  .

Left ventricular non-compaction: is it really isolated?

Isolated ventricular non-compaction (IVNC) is a genetically determined cardiac disease mainly involving the left ventricle. Since the first description and recognition of IVNC as a distinct cardiomyopathy, many cases have been described. However, the associated right-sided cardiac anomalies are unusual. Two cases of IVNC showing associated right-sided pathology on transthoracic echocardiography are presented.

Diffusion-weighted imaging in stroke attributable to patent foramen ovale: significance of concomitant atrial septum aneurysm.

BACKGROUND AND PURPOSE: Patent foramen ovale (PFO) is an established cause of stroke in young patients without other determined etiologies (ie, cryptogenic stroke). The additional presence of atrial septum aneurysm (ASA) possibly increases stroke risk, but it remains undetermined which factors best predict thromboembolism in patients with PFO. Diffusion-weighted imaging (DWI) may help to distinguish the characteristics of cerebral embolism associated with different features of the interatrial septum in PFO stroke. METHODS: In a stroke databank-based cohort study, DWI and transthoracic/transesophageal echocardiography findings were assessed in 48 consecutive patients with cryptogenic ischemic stroke associated with PFO. The number, size, and distribution of acute ischemic lesions on DWI were correlated with PFO size, degree of interatrial right-to-left shunt (RLS), and the presence of ASA. RESULTS: Patients with PFO plus ASA combined more often had multiple acute DWI lesions (16 of 30, 53%) than those with PFO alone (3 of 18, 17%; P=0.01). This association remained significant after correction for PFO size, degree of RLS, and vascular risk factors in a logistic-regression analysis (P=0.04). No significant associations between DWI lesion characteristics and PFO size or degree of RLS were found. CONCLUSIONS: The presence of concomitant ASA is independently associated with multiple cerebral ischemic lesions in PFO stroke, which may indicate an increased embolic risk.

Incremental cost-effectiveness of drug-eluting stents compared with a third-generation bare-metal stent in a real-world setting: randomised Basel Stent Kosten Effektivitäts Trial (BASKET).

BACKGROUND: No prospective trial-based data are available for incremental cost-effectiveness of drug-eluting stents (DES) compared with bare-metal stents (BMS) in unselected patients, as treated in everyday practice. METHODS: The Basel stent cost-effectiveness trial (BASKET) included 826 consecutive patients treated with angioplasty and stenting for 1281 de-novo lesions, irrespective of indication for angioplasty. Patients were randomised to one of two DES (Cypher, n=264; Taxus, n=281) or to a cobalt-chromium-based BMS (Vision, n=281) and followed up for 6 months for occurrence of major adverse cardiac events and costs. Analysis was by intention-to-treat. The primary endpoint was cost-effectiveness after 6 months, with effectiveness defined as reduction of major adverse cardiac events. FINDINGS: Cardiac death, myocardial infarction, or target vessel revascularisation occurred in 39 of 544 (7.2%) patients with DES and 34 of 280 (12.1%) with BMS (odds ratio 0.56, 95% CI 0.35-0.91; p=0.02), without significant differences between the two DES. Total costs at 6 months were higher with DES (mean 10,544, SD 6849) than with BMS (9639, 9067; p<0.0001); higher stent costs of DES were not compensated for by lower follow-up costs. Incremental cost-effectiveness ratio of DES compared with BMS to avoid one major event was 18,311, and costs per quality-adjusted life-year gained were more than 50 000. Subgroup analyses showed that DES were more cost-effective for elderly patients in specific high-risk groups. INTERPRETATION: In a real-world setting, use of DES in all patients is less cost effective than in studies with selected patients. Use of these stents could be restricted to patients in high-risk groups.

Right atrial pacing impairs cardiac function during resynchronization therapy: acute effects of DDD pacing compared to VDD pacing.

OBJECTIVES: We aimed to compare the hemodynamic effects of right-atrial-paced (DDD) and right-atrial-sensed (VDD) biventricular paced rhythm on cardiac resynchronization therapy (CRT). BACKGROUND: Cardiac resynchronization therapy improves hemodynamics in patients with severe heart failure and left ventricular (LV) dyssynchrony. However, the impact of active right atrial pacing on resynchronization therapy is unknown. METHODS: Seventeen CRT patients were studied 10 months (range: 1 to 46 months) after implantation. At baseline, the programmed atrioventricular delay was optimized by timing LV contraction properly at the end of atrial contraction. In both modes the acute hemodynamic effects were assessed by multiple Doppler echocardiographic parameters. RESULTS: Compared to DDD pacing, VDD pacing resulted in much better improvement of intraventricular dyssynchrony assessed by the septal-to-posterior wall motion delay (VDD 106 +/- 83 ms vs. DDD 145 +/- 95 ms; p = 0.001), whereas the interventricular mechanical delay (difference between onset of pulmonary and aortic outflow) did not differ (VDD 20 +/- 21 ms vs. DDD 18 +/- 17 ms; p = NS). Furthermore, VDD pacing significantly prolonged the rate-corrected LV filling period (VDD 458 +/- 123 ms vs. DDD 371 +/- 94 ms; p = 0.0001) and improved the myocardial performance index (VDD 0.60 +/- 0.18 vs. DDD 0.71 +/- 0.23; p < 0.01). CONCLUSIONS: Our findings suggest that avoidance of right atrial pacing results in a higher degree of LV resynchronization, in a substantial prolongation of the LV filling period, and in an improved myocardial performance. Thus, the VDD mode seems to be superior to the DDD mode in CRT patients.

Unsupervised image classification of medical ultrasound data by multiresolution elastic registration.

Thousands of medical images are saved in databases every day and the need for algorithms able to handle such data in an unsupervised manner is steadily increasing. The classification of ultrasound images is an outstandingly difficult task, due to the high noise level of these images. We present a detailed description of an algorithm based on multiscale elastic registration capable of unsupervised, landmark-free classification of cardiac ultrasound images into their respective views (apical four chamber, two chamber, parasternal long axis and short axis views). We validated the algorithm with 90 unselected, consecutive echocardiographic images recorded during daily clinical work. When the two visually very similar apical views (four chamber and two chamber) are combined into one class, we obtained a 93.0% correct classification (chi2 = 123.8, p < 0.0001, cross-validation 93.0%; chi2 = 131.1, p < 0.0001). Classification into the 4 classes reached a 90.0% correct classification (chi2 = 205.4, p < 0.0001, cross-validation 82.2%; chi2 = 165.9, p < 0.0001).

Cardiac resynchronization in severe heart failure and left bundle branch block: a single center experience.

OBJECTIVE: To assess the feasibility and longterm outcome of cardiac resynchronization therapy (CRT) in patients with impaired left ventricular function (LVEF <35%), left bundle branch block (QRS >120 ms) and dyspnoea NYHA 0 III at a single centre. METHODS AND RESULTS: Forty-seven patients were referred for implantation of a CRT device. In only 4 patients (9%) the device could not be implanted due to technical problems during the procedure. In the remaining 43 patients (65 +/- 10 years; 7 female) a CRT device was implanted. Follow-up time was 12 +/- 10 months. Twenty-one patients had dilated cardiomyopathy (DCM) and 22 patients had coronary artery disease (CAD). NYHA functional class improved from 3.0 +/- 1.4 to 2.5 +/- 0.7 (p <0.0001), accompanied by an improvement of LVEF [median 20% (range 15-25) vs 32% (range 20-40); p <0.0001]. A significant reduction of hospitalisation time for heart failure was found when the year before and the year after device implantation [18 days (range 5-27) vs 1 day (range 0-3); p <0.0001] were compared. Twelve (28%) patients, 9 with CAD, and 3 with DCM died. Two CAD patients and all patients with DCM who died had a combined CRT device with implantable cardioverter/defibrillator. CONCLUSION: In patients with severely impaired LVEF and wide QRS due to LBBB, CRT is feasible and safe. It improved dyspnoea and LVEF and reduced hospitalisation stays for heart failure during long-term follow-up.

Right ventricle best predicts the race performance in amateur ironman athletes.

PURPOSE: The ironman (IM) triathlon is a popular ultraendurance competition, consisting of 3.8 km of swimming, 180.2 km of cycling, and 42.2 km of running. The aim of this study was to investigate the predictors of IM race time, comparing echocardiographic findings, anthropometric measures, and training characteristics. METHODS: Amateur IM athletes (ATHL) participating in the Zurich IM race in 2010 were included. Participants were examined the day before the race by a comprehensive echocardiographic examination. Moreover, anthropometric measurements were obtained the same day. During the 3 months before the race, each IM-ATHL maintained a detailed training diary. Recorded data were related to total IM race time. RESULTS: Thirty-eight IM finishers (mean ± SD age = 38 ± 9 yr, 32 men [84%]) were evaluated. Total race time was 684 ± 89 min (mean ± SD). For right ventricular fractional area change (45% ± 7%, Spearman ρ = -0.33, P = 0.05), a weak correlation with race time was observed. Race performance exhibited stronger associations with percent body fat (15.2 ± 5.6%, ρ = 0.56, P = 0.001), speed in running training (11.7 ± 1.2 km · h(-1), ρ = -0.52, P = 0.002), and left ventricular myocardial mass index (98 ± 24 g · m(-2), ρ = -0.42, P = 0.009). The strongest association was found between race time and right ventricular end-diastolic area (22 ± 4 cm2, ρ = -0.64, P < 0.0001). In multivariate analysis, right ventricular end-diastolic area (ß = -16.7, 95% confidence interval = -27.3 to -6.1, P = 0.003) and percent body fat (ß = 6.8, 95% confidence interval = 1.1-12.6, P = 0.02) were independently predictive of IM race time. CONCLUSIONS: In amateur IM-ATHL, RV end-diastolic area and percent body fat were independently related to race performance. RV end-diastolic area was the strongest predictor of race time. The role of the RV in endurance exercise may thus be more important than previously thought and needs to be further studied.

Pharmacokinetics and pharmacodynamics of sildenafil in a patient treated with human immunodeficiency virus protease inhibitors.

We describe a 44-year-old male patient with human immunodeficiency virus (HIV) infection and pulmonary arterial hypertension who was treated with several protease inhibitors and with sildenafil. In order to guide treatment with sildenafil, the pharmacokinetics and dynamics of sildenafil were monitored at various time points. In comparison with healthy subjects, the maximal concentration in plasma (Cmax), area under the curve (AUC), and elimination half-life of sildenafil were approximately doubled in the patient. After increasing the sildenafil dose to ensure therapeutic drug levels over 24 hours, the pulmonary arterial hypertension and physical performance of the patient improved significantly. We conclude that the elimination of sildenafil is impaired in patients treated with protease inhibitors, but to a lesser extent than predicted from single-dose studies reported in the literature. Patients treated concomitantly with protease inhibitors and sildenafil need close monitoring of plasma levels, pharmacodynamics, and toxicity of sildenafil in order to be treated optimally.

Adenosine myocardial contrast echo in intermediate severity coronary stenoses: a prospective two-center study.

BACKGROUND: We sought to evaluate the role of adenosine myocardial contrast echocardiography (MCE) for the determination of functional relevance of coronary stenoses with intermediate angiographic severity and compared the results to single photon imaging (SPECT). We hypothezised that sole assessment of myocardial blood volume changes during adenosine on MCE would indicate functional stensosis relevance when accompanied by increased myocardial oxygen consumption (MVO2). METHODS: Fifty-seven patients with >or=1 coronary stenosis underwent adenosine MCE (ultraharmonic imaging) and exercise SPECT. On MCE, myocardial blood volume was assessed and constant or increased myocardial opacification during adenosine coupled with increased MVO2 was defined as normal and decreased opacification as abnormal. RESULTS: Rate-pressure product significantly increased during adenosine in all patients due to reflex tachycardia following mild hypotension, indicative of increased MVO2. Concordance between MCE and SPECT for the detection of reversible myocardial perfusion defects was 89% (kappa = 0.83). Comparison of regions between rest and during adenosine as opposed to comparison to remote regions of the same stage was important for accurate assessment because concordance betweenn MCE and SPECT was less on separate assessment at rest (73%, kappa = 0.40) compared to stress (91%, kappa = 0.81, P < 0.05) mainly due to territories scored normal on SPECT and abnormal on MCE. CONCLUSIONS: Assessment of myocardial blood volume changes during adenosine using MCE can be used for the determination of the functional relevance of coronary stenoses of intermediate angiographic severity if MVO2 is increased during adenosine.

Inferior sinus venosus defect associated with incomplete cor triatriatum dexter and patent foramen ovale.

Sinus venosus atrial septal defect (SVD) is a rare cardiac abnormality in adults. Particularly, the inferior type is difficult to depict by transthoracic echocardiography because of its infero-posterior location to the fossa ovalis. We describe the case of a 33-year-old woman whose chest X-ray taken during bronchopneumonia revealed a cardiomegaly. Further echocardiographic investigations showed an underlying inferior SVD, an incomplete cor triatriatum dexter and a large patent foramen ovale (PFO). The diagnosis was confirmed by cardiac magnetic resonance imaging (CMR) and during surgical repair.

Value of repeated cardiac magnetic resonance imaging in patients with suspected arrhythmogenic right ventricular cardiomyopathy.

AIM: Diagnosis of early stages of arrhythmogenic right ventricular cardiomyopathy (ARVC) with minimal structural abnormalities is challenging. The purpose of this study was to assess the value of repeated cardiac magnetic resonance imaging (CMR) in patients referred for right ventricular arrhythmias and clinical suspicion of ARVC. METHODS AND RESULTS: Prospective follow-up study of 18 patients (8 females) studied with CMR for suspected ARVC. Patients with implanted defibrillators (ICD) were excluded. Mean follow-up was 37 +/- 16 (12-59) months. Patients were assigned to 2 categories (ARVC likely or ARVC unlikely) according to a CMR-score based on right ventricular abnormalities. Clinical follow-up revealed no disease progression in 17 patients (94%). In 1 patient, an ICD was implanted because of disease progression. Of 9 patients with initial findings suggestive of ARVC, follow-up CMR remained positive in 3 and was diagnosed as normal in 6, mainly due to the inability to confirm the presence of fatty infiltrates at follow-up (5 of 6 patients). Initially, 9 patients had a normal CMR and 8 of those remained normal during follow-up. CONCLUSION: Repeated CMR after an average follow-up of 3 years was normal in 6 of 9 patients with clinical findings consistent with early stages of ARVC at the time of baseline CMR. Thus, CMR diagnosis of early stage ARVC is difficult and should be made with caution.

Mechanical complications after myocardial infarction reliably predicted using C-reactive protein levels and lymphocytopenia.

We assessed the accuracy of C-reactive protein (CRP) levels and lymphocyte counts to predict a mechanical complication (MC) after myocardial infarction (MI). Within 10 years, we identified 36 patients with 39 echocardiographically confirmed MC within 30 days of MI: ventricular septal defect (17 cases), papillary muscle rupture (10 cases), and left ventricular free wall rupture (12 cases). They were compared to 41 controls with an uncomplicated hospital course after MI. Peak CRP levels and minimum relative lymphocyte counts obtained within 96 h of the acute MI (AMI) and before diagnosis of the complication were compared with clinical parameters. Prior to the MC, peak CRP levels were significantly higher (p < 0.001) and relative lymphocyte counts lower (p < 0.001) than in controls while creatine kinase levels did not differ (p = nonsignificant). Using multivariate logistic regression, the following score was identified to have excellent prognostic significance for MC: CRP (mg/l) - 10 x Lyc (%). The area under the receiver-operating characteristic curve was 0.90 +/- 0.05 (p < 0.001). Combined use of CRP levels and relative lymphocyte counts may be helpful in accurately predicting an MC after AMI and should therefore be routinely assessed.