Aims : Traditional echocardiographic parameters for the assessment of suspected cardiac amyloidosis (CA) are of limited diagnostic accuracy. We sought to explore differences and the discriminative value of phasic left atrial strain (LAS) reductions and of regional longitudinal left ventricular (LV) strain alterations (relative apical sparing; RELAPS) in CA and other causes of LV wall thickening (LVH). Methods and results : We included 54 patients with unclear LVH (mean septal diastolic wall thickness 17.8 ± 3.5 mm); CA was bioptically confirmed in 35 patients (8 mATTR, 6 wtATTR, 20 AL, and 1 AA amyloidosis) and LVH in 19 subjects. We analysed RELAPS as well as LA reservoir (LASr), conduit (LAScd), and contraction strain (LASct) using 2D speckle tracking echocardiography (EchoPAC software, GE). RELAPS was higher (1.37 ± 0.94 vs. 0.86 ± 0.29, P < 0.007), whereas atrial mechanics were significantly reduced in CA (LASr, LAScd, and LASct: 9.7 ± 5.2%, −6.5 ± 3.5%, and −5.0 ± 4.1% in CA; and 22.7 ± 7.8%, −13.9 ± 5.2%, and −13.0 ± 5.5% in LVH, respectively; P < 0.001 each). With an area under the curve (AUC) of 0.91 [95% confidence interval (CI) 0.82–0.99], LASr showed a higher diagnostic accuracy in discriminating CA than RELAPS (AUC 0.74, 95% CI 0.59–0.88). LASr and LAScd remained significantly associated with CA in a multivariate regression model. Conclusion : Phasic LAS was significantly reduced in patients with CA and showed a higher diagnostic accuracy in discriminating CA than RELAPS. The additional assessment of phasic LAS may be useful to rule in the possible diagnosis of CA in patients with unclear LVH.

Background “Classical” echocardiographic signs of Fabry cardiomyopathy (FC), such as left ventricular hypertrophy (LVH), posterolateral strain impairment (PLSI), and papillary muscle hypertrophy may be of limited diagnostic accuracy in clinical practice. Our aim was to evaluate the diagnostic value of left atrial (LA) strain impairment compared to “classical” echocardiographic findings to discriminate FC. Methods In standard echocardiographic assessments, we retrospectively analyzed the diagnostic value of the “classical” red flags of FC as well as LA strain in 20 FC patients and in 20 subjects with other causes of LVH. Receiver operating characteristic (ROC) curve analysis was performed to assess the respective diagnostic accuracy. Results FC was confirmed in 20 patients by genetic testing. In the LVH group, 12 patients were classified by biopsy to have hypertrophic cardiomyopathy, two had hypertensive heart disease, and six LVH combined with borderline myocarditis. Global and regional left ventricular (LV) strain was not significantly different between groups while LA strain was significantly impaired in FC (Left atrial reservoir strain (LASr) 19.1%±8.4 in FC and 25.6%±8.9 in LVH, p = 0.009; left atrial conduction strain (LAScd) -8.4%±4.9 in FC and -15.9%±8.4 in LVH, p < 0.01). LAScd, with an area under the curve (AUC) of .81 (95% confidence interval [CI] .66–.96) showed the highest diagnostic accuracy to discriminate FC. The PLSI pattern showed an AUC of .49, quantification of papillary muscle hypertrophy an AUC of .47. Conclusion Adding LA strain analysis to a comprehensive echocardiographic work-up of unclear LVH may be helpful to identify FC as a possible cause.

Layer-specific left ventricular (LV) strain alterations have been suggested as a specific finding in Fabry disease (FD). Our study aimed to assess the diagnostic value of layer-specific radial strain (RS) indices compared to the established LV regional strain pattern in cardiac amyloidosis (CA) and FD, i.e. apical sparing and posterolateral strain deficiency (PLSD). We retrospectively analysed the global, subendocardial, subepicardial LV radial strain, the corresponding strain gradient, as well as the regional and global longitudinal strain. The diagnostic accuracy of the diverse LV strain analyses was comparatively assessed using receiver operating characteristic curve and multivariable regression analyses. In 40 FD and 76 CA patients, CA featured more reduced layer strain values [global RS −12.3 (−15.6 to −9.6) in CA vs. −16.7 (−20.0 to −13.6) in FD; P < 0.001; subendocardial RS −22.3 (−27.4 to −15.9) vs. −28.3 (−31.8 to −23.6), P < 0.001; subepicardial RS −6.6 (−8.6 to −4.7) in CA vs. −8.9 (−11.7 to − 6.5) in FD; P < 0.001]. Global radial and longitudinal strain held an area under the curve (AUC) of 0.75 (0.66–0.84) and AUC 0.73 (0.63–0.83). While the apical sparing and PLSD strain pattern showed the highest accuracy as single parameters [AUC 0.87 (0.79–0.95) and 0.81 (0.72–0.89), P < 0.001], the combination of subendocardial RS and the apical sparing pattern featured the highest diagnostic accuracy [AUC 0.92 (0.87–0.97)]. Combining radial strain-derived parameters to the established strain pattern apical sparing and PLSD improve the diagnostic accuracy in the echocardiographic assessment in suspected storage disease.

Background: Aortic stenosis is a common finding in cardiac amyloidosis (CA). Younger patients often remain asymptomatic. If unrecognized, this can lead to serious complications such as heart failure. Progression of aortic stenosis can be accelerated in patients with chronic kidney disease and need for dialysis. Perioperative risk in these patients is often high due to the underlying systemic disease. Case summary: A 40-year-old Caucasian man with known AA amyloidosis, highly active Ankylosing Spondylitis and need for chronic dialysis due to end-stage chronic renal failure presented for echocardiographic routine exam without reporting any cardiac symptoms. At the last visit 4 years ago, a normal heart valve function was noted and no echocardiographic follow-up was performed in the following. Now, rapid progression with severe aortic valve and mitral valve stenosis was stated and the patient underwent combined aortic and mitral surgical valve replacement following discussion in the multidisciplinary cardiology meeting. Macroscopic examination of the valves revealed significant calcification and histological examination showed the high presence of amyloid by Congo-red staining and immunohistological staining for AA-Amyloid. Both valve prosthetic devices showed normal function as well as a normal left ventricular ejection fraction in initial post-operative transoesophageal echocardiography. After prolonged and complicated post-operative course in the intensive care unit the patient died 3 months after surgery due to intractable multiorgan failure in combined severe abdominal septic and cardiogenic shock. Discussion: Concomitant CA and chronic dialysis can accelerate the onset of severe aortic valve stenosis. Young patients, as in this case, often stay asymptomatic, perioperative risk increases with duration of chronic dialysis and severity of valve stenosis. This increases the need for regular short-term echocardiographic examinations even in clinical stable patients.

Infective endocarditis is a severe, mostly bacterial disease characterized by high mortality and morbidity. Both diagnosis and therapy are difficult, and mortality rates have not improved over decades. We review diagnostic and management problems, the disease's modern face in the era of widespread cardiac implants and valve interventions, and current diagnostic and therapeutic recommendations. Die infektiöse Endokarditis (IE) ist eine schwere, meist bakterielle Erkrankung – oft tödlich oder mit einer hohen Morbidität belastet. Diagnose und Therapie der IE gehören zu den schwierigsten Aufgaben in der Kardiologie. Dieser Beitrag stellt die diagnostischen Herausforderungen dieser sehr oft zu spät erkannten und behandelten Krankheit in den Vordergrund und beschreibt aktuelle Erscheinungsformen, Diagnosestrategien und Behandlungsoptionen.

Background: While previous studies indicate an association between chest pain and favorable clinical outcomes in patients with pulmonary embolism (PE), the extent and underlying mechanisms of this effect remain inadequately defined. Methods: We investigated the prognostic value of chest pain with regard to in-hospital adverse outcomes and the association of chest pain with age and sex in consecutive patients with confirmed PE enrolled in a single-center registry between 2008 and 2019. Results: Of 858 patients (52% female) included in this study, 435 (51%) had chest pain at presentation. Chest pain was more prevalent in younger individuals aged 18–34 years (74%) compared to patients >34 years (46%). The prevalence of coronary artery disease (CAD) was similar in patients with and without chest pain (17.0% vs. 16.1%). Chest pain patients less frequently presented with elevated troponin levels (p < 0.001) or signs of right heart strain (RHS; p = 0.007) but more frequently exhibited imaging signs of pulmonary infarction (p = 0.001). Chest pain was associated with lower risk of adverse outcome (OR 0.35 [95% CI: 0.19–0.65]) and in-hospital mortality (OR 0.31 [95% CI: 0.13–0.74]). Multivariable models confirmed a prognostic effect independent of sex, comorbidities and results of risk stratification algorithms. Conclusions: In acute PE, chest pain is a favorable prognostic marker irrespective of sex. Chest pain patients are less likely to suffer from myocardial ischemia or show signs of RHS but more frequently show imaging signs of pulmonary infarction, suggesting pleuritic irritation rather than myocardial ischemia as the likely cause of pain.