Diagnostic value of left ventricular layer strain and specific regional strain patterns in cardiac amyloidosis and Fabry disease.

Aims: 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). Methods and results: 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)]. Conclusion: 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.

Right heart and left atrial strain to differentiate cardiac amyloidosis and Fabry disease.

Echocardiographic differentiation of cardiac amyloidosis (CA) and Fabry disease (FD) is often challenging using standard echocardiographic parameters. We retrospectively analyzed the diagnostic accuracy of right heart and left atrial strain parameters to discriminate CA from FD using receiver operating characteristic curve analyses and logistic regression models. A total of 47 FD and 88 CA patients with left ventricular wall thickening were analyzed. The comparison of both cardiomyopathies revealed significantly reduced global and free wall longitudinal right ventricular strain (RVS; global RVS: CA - 13 ± 4%, n = 67, vs. FD - 18 ± 4%, n = 39, p < 0.001) as well as right atrial strain (RAS; reservoir RAS: CA 12 ± 8%, n = 70, vs. FD 26 ± 9%, n = 40, p < 0.001) and left atrial strain (LAS) in CA patients. Individually, global RVS as well as phasic LAS and RAS showed the highest diagnostic accuracy to distinguish CA and FD. The best diagnostic accuracy was achieved by combining the age, basal RV diameter, global RVS, and reservoir and conduit RAS (area under the curve 0.96 [95% CI 0.90-1.00]). Differential echocardiographic diagnostic work-up of patients with suspected CA or FD can be improved by integrating structural and functional parameters of the right heart and the left atrium.Trial registration: DRKS00027403.

Diagnostic value of papillary muscle hypertrophy and mitral valve thickness to discriminate cardiac amyloidosis and Fabry disease.

BACKGROUND: Cardiac amyloidosis (CA) and Fabry disease (FD) cause myocardial damage but may also affect the valvular and subvalvular apparatus. We aimed to evaluate the diagnostic accuracy of new echocardiographic indices including mitral valve thickness and papillary muscle (PM) hypertrophy to differentiate CA and FD. METHODS: In patients with confirmed CA and FD, a detailed assessment of valvular function, mitral valve leaflet thickness and PM area as well as PM left ventricular area ratio (PM/LV-ratio) was performed in offline analyses. Receiver operating characteristic curve analyses were conducted to determine the diagnostic accuracy of mitral valve thickness, PM hypertrophy, and PM/LV-ratio to distinguish CA from FD. RESULTS: We retrospectively analyzed a cohort of 129 patients (FD n = 49, CA n = 80). CA patients showed significantly more thickened mitral valve leaflets (4.1 ± 1.3 mm vs. 2.9 ± 1.1 mm, p < 0.001) and a higher PM area [4.0 (3.1-4.6) mm2 vs. 2.8 (2.1-4.6) mm2, p = 0.009] with a comparable PM/LV-ratio in both groups. Mitral valve thickness showed the highest diagnostic accuracy to discriminate CA [AUC 0.77 (95% CI 0.67-0.87)]. The prevalence of aortic, tricuspid, and pulmonary valve regurgitation was significantly higher in CA (aortic regurgitation ≥ II° 13% vs. 4%, tricuspid regurgitation≥ II° 19% vs. 8%, p < 0.001). CONCLUSION: Our results suggest that the assessment of mitral valve thickness may be a new useful echocardiographic parameter to differentiate CA and FD, whereas papillary muscle hypertrophy and PM/LV-ratio showed a limited diagnostic performance to discriminate CA. German clinical trials registry: DRKS00027403.

Impact of body mass index on worsening of diastolic function and impairment of left atrial strain in the general female urban population: a subanalysis of the Berlin female risk evaluation echocardiography follow-up study.

Background: The association of body mass index (BMI) with diastolic dysfunction (DD) is well described in the literature. However, there is conflicting evidence and long-term follow-up data regarding effects of BMI on preclinical DD and left atrial (LA) function are scarce, highlighting the importance of early detection tools, such as myocardial strain. Purpose: The aim of our study was to prospectively analyze the impact of clinical and demographic parameters, especially of BMI, on worsening of diastolic function and left atrial strain (LAS) in an urban population of women with a low prevalence of cardiovascular risk factors. Methods and Results: An extensive clinical and echocardiographic assessment comprising the analysis of phasic LAS using two-dimensional speckle-tracking echocardiography (2D STE) was performed in 258 participants of the Berlin Female Risk Evaluation (BEFRI) trial between October 2019 and December 2020 after a mean follow-up period of 6.8 years. We compared clinical and echocardiographic parameters stratifying women by BMI < or ≥25 kg/m2, and we analyzed the impact of demographic characteristics on the worsening of DD and LA mechanics in the longer-term follow-up using univariate and multivariate regression analyses. 248 women were suitable for echocardiographic analysis of LAS using 2D STE. After a mean follow-up time of 6.8 years, LA reservoir strain (LASr) and LA conduit strain (LAScd) were significantly reduced in participants with a BMI ≥25 kg/m2 compared with women with a BMI <25 kg/m2 at baseline (30 ± 8% vs. 38 ± 9%, p < 0.0001; -14 ± 7% vs. -22 ± 8%, p < 0.0001). 28% of the overweighted women presented a deterioration of diastolic function at the time of follow-up in contrast with only 7% of the group with a BMI <25 kg/m2 (p < 0.0001). BMI remained significantly associated with LAS reductions after adjustment for other risk factors in multivariate regression analyses. Conclusion: Overweight and obesity are related to impaired LAS and to a worsening of diastolic function after a long-term follow-up in a cohort of randomly selected women.

Comparison of procedural characteristics of percutaneous annuloplasty and edge-to-edge repair for the treatment of severe tricuspid regurgitation.

Background: In recent years, new interventional therapies for tricuspid regurgitation (TR) demonstrated their effectiveness in reducing TR severity and improving symptoms. Currently, tricuspid transcatheter edge-to-edge repair (T-TEER) and percutaneous annuloplasty are the most widely used techniques in Europe. In this retrospective study, we compared procedural characteristics and learning curves of both TR devices in a real-world cohort. Material and methods: Eligible patients with severe to torrential TR underwent either percutaneous annuloplasty or T-TEER as recommended by the local heart team. Patients with combined mitral and tricuspid interventions were excluded from the analysis. The study focused on procedural characteristics, TR reduction and learning curves. Results: A total of 122 patients underwent either percutaneous annuloplasty (n = 64) or T-TEER (n = 58) with a technical and device success rate of 98% and 97%, respectively. Reasons for technical failure included right coronary artery (RCA) dissection prior to percutaneous annuloplasty, and two single leaflet device attachments (SLDA) during T-TEER implantation. The mean improvement of TR severity was 2.4 ± 0.8 degrees after T-TEER and 2.5 ± 0.8 after percutaneous annuloplasty. T-TEER procedures were shorter in terms of both procedure time and radiation exposure, while percutaneous annuloplasty, although taking longer, showed a significant reduction in procedure time over the course of the analysed period. Conclusion: In summary, both interventional therapies reduce TR severity by approximately two degrees when used in the appropriate anatomy. The learning curve for annuloplasty group showed a significant decrease of procedure times.

Procedural success of transcatheter annuloplasty in ventricular and atrial functional tricuspid regurgitation.

Background: Transcatheter annuloplasty is meant to target annular dilatation and is therefore mainly applied in functional tricuspid regurgitation (TR). Due to recent recognition of varying disease pathophysiology and differentiation of ventricular and atrial functional TR (VFTR and AFTR), comparative data regarding procedural success for both disease entities are required. Methods: In this consecutively enrolled observational cohort study, 65 patients undergoing transcatheter annuloplasty with a Cardioband® device were divided into VFTR (n = 35, 53.8%) and AFTR (n = 30, 46.2%). Procedural success was assessed by comparing changes in annulus dilatation, vena contracta (VC) width, effective regurgitation orifice area (EROA), as well as reduction in TR severity. Results: Overall, improvement of TR by at least two grades was achieved in 59 patients (90.8%), and improvement of TR by at least three grades was realised in 32 patients (49.2%). Residual TR of ≤2 was observed in 52 patients (80.0%). No significant differences in annulus diameter reduction [VFTR: 11 mm (9-13) vs. AFTR: 12 mm (9-16), p = 0.210], VC reduction [12 mm (8-14) vs. 12 mm (7-14), p = 0.868], and EROA reduction [0.62 cm2 (0.45-1.10) vs. 0.54 cm2 (0.40-0.70), p = 0.204] were reported. Improvement by at least two grades [27 (90.0%) vs. 32 (91.4%), p = 1.0] and three grades [14 (46.7%) vs. 18 (51.4%), p = 0.805] was similar in VFTR and AFTR, respectively. No significant difference in the accomplishment of TR grade of ≤2 [21 (70.0%) vs. 31 (88.6%), p = 0.118] was noted. Conclusion: According to our results from a real-world scenario, transcatheter annuloplasty with the Cardioband® device may be applied in both VFTR and AFTR with evidence of significant procedural TR reduction.

Phasic left atrial strain to predict worsening of diastolic function: Results from the prospective Berlin Female Risk Evaluation follow-up trial.

Purpose: The predictive value of maximum left atrial volume index (LAVI), phasic left atrial strain (LAS) and other standard echocardiographic parameters assessing left ventricular (LV) diastolic function to discriminate a future worsening of diastolic function (DD) in patients at risk is unclear. We aimed to prospectively assess and compare the clinical impact of these parameters in a randomly selected study sample of the general urban female population. Methods and results: A comprehensive clinical and echocardiographic evaluation was performed in 256 participants of the Berlin Female Risk Evaluation (BEFRI) trial after a mean follow up time of 6.8 years. After an assessment of participants' current DD status, the predictive impact of an impaired LAS on the course of DD was assessed and compared with LAVI and other DD parameters using receiver operating characteristic (ROC) curve and multivariate logistic regression analyses. Subjects with no DD (DD0) who showed a decline of diastolic function by the time of follow-up showed a reduced LA reservoir (LASr) and conduit strain (LAScd) compared to subjects who remained in the healthy range (LASr 28.0% ± 7.0 vs. 41.9% ± 8.5; LAScd -13.2% ± 5.1 vs. -25.4% ± 9.1; p < 0.001). With an area under the curve (AUC) of 0.88 (95%CI 0.82-0.94) and 0.84 (95%CI 0.79-0.89), LASr and LAScd exhibited the highest discriminative value in predicting worsening of diastolic function, whereas LAVI was only of limited prognostic value [AUC 0.63 (95%CI 0.54-0.73)]. In logistic regression analyses, LAS remained a significant predictor for a decline of diastolic function after controlling for clinical and standard echocardiographic DD parameters, indicating its incremental predictive value. Conclusion: The analysis of phasic LAS may be useful to predict worsening of LV diastolic function in DD0 patients at risk for a future DD development.GRAPHICAL ABSTRACT.

Thirty-day outcomes of the Cardioband tricuspid system for patients with symptomatic functional tricuspid regurgitation: The TriBAND study.

BACKGROUND: Severe tricuspid regurgitation (TR) has limited treatment options and is associated with high morbidity and mortality. AIMS: We evaluated the safety and effectiveness of the Cardioband tricuspid valve reconstruction system from the ongoing European single-arm, multicentre, prospective TriBAND post-market clinical follow-up study. METHODS: Eligible patients had chronic symptomatic functional TR despite diuretic therapy and were deemed candidates for transcatheter tricuspid repair by the local Heart Team. RESULTS: Sixty-one patients had ≥severe functional TR. At baseline, 85% of patients were in NYHA Class III-IV, 94% had ≥severe TR (core laboratory-assessed) with 6.8% EuroSCORE II and 53% LVEF. Device success was 96.7%. At discharge, 59% (p<0.001) of patients achieved ≤moderate TR and 78% had at least one grade TR reduction. At 30 days, all-cause mortality and composite MAE rates were 1.6% and 19.7%, respectively; septolateral annular diameter was reduced by 20%, where 69% of patients achieved ≤moderate TR and 85% of patients had at least one grade TR reduction (all p<0.001). Mid-RVEDD, RA volume, and IVC diameter decreased by 10% (p=0.005), 21% (p<0.001), and 11% (p=0.022), respectively; 74% were in NYHA Class I-II (p<0.001) with improvements in overall KCCQ score by 17 points (p<0.001). CONCLUSIONS: In the TriBAND study, the Cardioband tricuspid system demonstrated favourable outcomes at discharge and 30 days in a challenging patient population with symptomatic ≥severe functional TR. Results showed significant reductions in annular diameter and TR severity, accompanied by early evidence of right heart remodelling and improvements in functional status and quality of life.

[Incidence of nosocomial infections at a neonatal intensive care unit: a six-year surveillance study]. / Incidencia de infecciones en una unidad de cuidados intensivos neonatales: estudio de vigilancia de 6 años.

INTRODUCTION: Nosocomial infection is a frequent complication in neonatal intensive care units (NICUs) attending patients who require lengthy hospitalization and frequent invasive techniques. PATIENTS AND METHODS: This study is part of a prospective surveillance program for nosocomial infection in Spain. All patients admitted to NICUs between June 1999 and March 2005 were observed. CDC criteria were used as the standard definition for nosocomial infection. RESULTS: A total of 1236 neonates (58% male) were admitted during the surveillance period, involving 19,420 days in the NICU. The average birth weight was 1947.6 +/- 1009.5 g and average gestational age was 32.9 +/- 5.4 weeks. The most frequent associated pathology was respiratory distress (23.06%). A total of 316 nosocomial infections were diagnosed in 226 neonates, 76.7% affecting premature neonates (< 1500 g). The most frequent location was bacteremia (56.3%), and there was a predominance of coagulase-negative staphylococci (46.05%). Gram-negative microorganisms were isolated in 32.1% of the cases (Escherichia coli and Pseudomonas aeruginosa were the most frequent pathogens). Overall incidence of nosocomial infection was 25.6%. Overall mortality was 6.6%, with higher mortality in the group with nosocomial infections (8.7%). CONCLUSIONS: Nosocomial infection rates are acceptable, with a typical epidemiological pattern for these units. Presence of a central catheter increased the risk. A program to promote proper hand washing should be considered. We do not recommend a continuing surveillance strategy in these units.

Incidencia de infecciones en una unidad de cuidados intensivos neonatales: estudio de vigilancia de 6 años / Incidence of nosocomial infections at a neonatal intensive care unit: a six-year surveillance study

Introducción. La infección nosocomial (IN) es una complicación frecuente en unidades de cuidados intensivos neonatales (UCIN), que atienden a pacientes con largas estancias hospitalarias y sometidos a frecuentes técnicas invasivas. Pacientes y métodos. Este trabajo forma parte de un estudio prospectivo multicéntrico nacional de vigilancia de IN. Se observan todos los ingresos en UCIN entre junio de 1999 y marzo de 2005. Se vigila la aparición de infecciones siguiendo los criterios diagnósticos de los Centers for Disease Control and Prevention (CDC). Resultados. En el período de estudio ingresaron 1.236 neonatos (58% varones), con un total de 19.420 días de estancias. El peso medio al nacimiento fue de 1.947,6 6 1.009,5 g y la edad gestacional media fue de 32,9 6 5,4 semanas. La patología más frecuente fue la dificultad respiratoria neonatal (23,06%). Se diagnosticaron 316 IN en 226 neonatos, el 76,7% de los cuales afectaron a prematuros de menos de 1.500 g. La localización más frecuente fue la bacteriemia (56,3%), con claro predominio del Staphylococcus coagulasa-negativo como responsable (46,05%). Los gramnegativos fueron aislados en el 32,1% de los casos (Escherichia coli y Pseudomonas aeruginosa, los más frecuentes). La incidencia acumulada de IN fue de 25,6% y la densidad de incidencia del 16,3 ‰. La mortalidad global afectó al 6,6% de los neonatos, mayor en el subgrupo de infectados (8,7%) que en los que no se infectaron (6,2%). Conclusiones. Se detectaron cifras de IN aceptables, con un patrón epidemiológico típico de estas unidades. Se debe considerar cuidadosamente el cateterismo central e intensificar los programas de higiene de las manos. No recomendamos la estrategia de vigilancia ininterrumpida en estas unidades (AU)

Introduction. Nosocomial infection is a frequent complication in neonatal intensive care units (NICUs) attending patients who require lengthy hospitalization and frequent invasive techniques. Patients and methods. This study is part of a prospective surveillance program for nosocomial infection in Spain. All patients admitted to NICUs between June 1999 and March 2005 were observed. CDC criteria were used as the standard definition for nosocomial infection. Results. A total of 1236 neonates (58% male) were admitted during the surveillance period, involving 19,420 days in the NICU. The average birth weight was 1947.6 6 1009.5 g and average gestational age was 32.9 6 5.4 weeks. The most frequent associated pathology was respiratory distress (23.06%). A total of 316 nosocomial infections were diagnosed in 226 neonates, 76.7% affecting premature neonates (< 1500 g). The most frequent location was bacteremia (56.3%), and there was a predominance of coagulase-negative staphylococci (46.05%). Gram-negative microorganisms were isolated in 32.1% of the cases (Escherichia coli and Pseudomonas aeruginosa were the most frequent pathogens). Overall incidence of nosocomial infection was 25.6%. Overall mortality was 6.6%, with higher mortality in the group with nosocomial infections (8.7%). Conclusions. Nosocomial infection rates are acceptable, with a typical epidemiological pattern for these units. Presence of a central catheter increased the risk. A program to promote proper hand washing should be considered. We do not recommend a continuing surveillance strategy in these units (AU)