Search

Awareness of transthyretin amyloid cardiomyopathy (ATTR-CM) has increased over the years due to diagnostic and therapeutic developments. Timely initiation of novel disease-modifying treatments improves both morbidity and mortality, which underlines the necessity for a prompt diagnosis. Nevertheless, early diagnosis of ATTR-CM remains challenging. This is a retrospective observational cohort study of patients diagnosed with ATTR-CM. Between 2016 and 2023, 87 patients were diagnosed with cardiac amyloidosis of which 65 (75%) patients with ATTR-CM and 22 (25%) patients with light chain amyloidosis. This study included 65 ATTR-CM patients (mean age 77 ± 7 years; 86% male) of whom 59 (91%) with wild-type ATTR-CM (ATTRwt) and six (9%) with variant ATTR-CM. We observed a surge in ATTR-CM diagnoses from 3 patients/year (2016–2020) to 16 patients/year (2021–2023), driven by ATTRwt. Nevertheless, the interval between the onset of heart failure symptoms and ATTR-CM diagnosis has not changed significantly (2016–2020 27.3 months [18.6–62.4]; 2021–2023 30.0 months [8.6–57.2]; p = 0.546), driven by time to referral. Red flags for ATTR-CM preceded diagnosis by several years: left ventricular hypertrophy (79%, 5.8 years [3.3–7.0]) and carpal tunnel syndrome (49%, 6.8 years [2.3–12.1]). Despite the presence of typical red flags, symptom-to-diagnosis duration has remained similar driven by time to referral. Improved recognition of red flags for ATTR-CM could reduce the time to diagnosis and improve overall recognition. [ABSTRACT FROM AUTHOR]

Cardiac amyloidosis (CA)—mostly transthyretin-related (ATTR-CA)—has recently gained interest in cardiology. Bone scintigraphy (BS) is one of the main screening tools for ATTR-CA but also used for various other reasons. The objective was to evaluate whether all CA cases are detected and what happens during follow-up. All routine BS performed at the Maastricht University Medical Center (May 2012–August 2020) were screened for the presence of CA. Scans performed for suspected CA were excluded. A Perugini stage ≥1 was classified as positive necessitating further examination. The electronic medical record system was evaluated for any contact with cardiology or other specialists until 2021. Of the 2738 BS evaluated, 40 scans (1.46%; median age 73.5 [IQR: 65.8–79.5], 82.5% male) were positive (Perugini grade 1: 31/77.5%, grade 2: 6/15%, grade 3: 3/7.5%); the potential diagnosis ATTR-CA was not seen in 38 patients (95%) by the nuclear medicine specialist. During follow-up, 19 out of those 40 patients (47.5%) underwent cardiac evaluation without diagnosing CA. Available echocardiograms of patients with a positive BS showed left ventricular hypertrophy, a preserved ejection fraction, and diastolic dysfunction ≥2 in 9/47%, 10/53%, and 4/21% of patients, respectively. Additionally, 20 (50%) patients presented to at least one specialty with symptoms indicative of cardiac amyloidosis. The prevalence of a positive BS indicating potential CA in an unselected population is low but substantial. The majority was not detected which asks for better awareness for CA of all involved specialists to ensure appropriate treatment and follow-up. [ABSTRACT FROM AUTHOR]

The Simpson's method is the standard technique to determine left ventricular (LV) ejection fraction (EF) on echocardiography. The large inter-observer variability of measuring LVEF is well documented but not fully understood. A graphical analysis was used to elaborate what contributes to the inter-observer difference. Forty-two cardiologists (32 male, 39 ± 7 years) evaluated the LVEF using the Simpson's method on 15 different echocardiograms (2 and 4 chamber view (2CH/4CH)); the program did not show the result of EF to prevent a bias. End-diastolic (ED) and end-systolic (ES) frames were predefined ensuring measurement at the same time point of the cardiac cycles. After standardization of the LV contour, the differences of the individual contours compared to a reference contour were measured. Also, the spreading of lateral/medial mitral annulus contours and the apex were depicted. A significant spreading of LV-contours was seen with larger contours leading to higher EFs (p < 0.001). Experience did not influence the determination of LVEF. ED-volumes showed more spreading than ES-volumes ((3.6 mm (IQR: 2.6–4.0) vs. 3.4 mm (IQR: 2.8–3.8), p < 0.001). Also, the differences were larger for the 2CH compared to the 4CH (p < 0.001). Variability was significantly larger for lateral than septal wall (p < 0.001) as well as the anterior compared to the inferior wall (p < 0.001). There was a relevant scattering of the apex and medial/ lateral mitral annulus ring. There was a large variability of LV-volumes and LVEF as well as position of mitral valve ring and apex. There were global differences (apical 2CH or 4CH), regional aspects (LV walls) and temporal factors (ED vs. ES). Thus, multiple factors contributed to the large variability. Trial registration: The study was registered at "Netherlands Trial Register" (www.trialregister.nl; study number: NL5131). [ABSTRACT FROM AUTHOR]

Loop diuretics remain the cornerstone of congestion management in contemporary chronic heart failure care. However, their use is not supported by high quality data, and there is doubt about the safety in the outpatient heart failure setting. Still, congestion is related to a worse outcome, and there is general consensus among experts that congestion should not be tolerated in heart failure patients. Recommendations in international guidelines, regarding decongestion strategies in chronic heart failure, are limited. Thus, there is an emerging need for clinical decision-making support about the best strategy for using loop diuretics and decongestion in the chronic setting. The present review provides a comprehensive overview over the evidence of chronic loop diuretic use. Strategies for the assessment of congestion in the outpatient setting and decongestion algorithm are provided to assist health care specialists in delivering high-quality heart failure care. [ABSTRACT FROM AUTHOR]

Low adherence to cardiac rehabilitation (CR) might be improved by remote monitoring systems that can be used to motivate and supervise patients and tailor CR safely and effectively to their needs. The main objective of this study was to evaluate the feasibility of a smartphone-guided training system (GEX) and whether it could improve exercise capacity compared to CR delivered by conventional methods for patients with coronary artery disease (CAD). A prospective, randomized, international, multi-center study comparing CR delivered by conventional means (CG) or by remote monitoring (IG) using a new training steering/feedback tool (GEx System). This consisted of a sensor monitoring breathing rate and the electrocardiogram that transmitted information on training intensity, arrhythmias and adherence to training prescriptions, wirelessly via the internet, to a medical team that provided feedback and adjusted training prescriptions. Exercise capacity was evaluated prior to and 6 months after intervention. 118 patients (58 ± 10 years, 105 men) with CAD referred for CR were randomized (IG: n = 55, CG: n = 63). However, 15 patients (27 %) in the IG and 18 (29 %) in the CG withdrew participation and technical problems prevented a further 21 patients (38 %) in the IG from participating. No training-related complications occurred. For those who completed the study, peak VO improved more ( p = 0.005) in the IG (1.76 ± 4.1 ml/min/kg) compared to CG (−0.4 ± 2.7 ml/min/kg). A newly designed system for home-based CR appears feasible, safe and improves exercise capacity compared to national CR. Technical problems reflected the complexity of applying remote monitoring solutions at an international level. [ABSTRACT FROM AUTHOR]

There is some evidence that long-term high-intensity endurance training might be associated with deterioration in cardiac function and might impose a potential risk for cardiovascular events. Thus, the intention was to retrospectively evaluate the cardiac status in former endurance athletes, particularly right ventricular (RV) dimension and function, to reveal potential cardiac damage. A group of 12 former world-class swimmers (45 ± 1.5 years) was examined 24.9 ± 4.3 years after cessation of high-intensity endurance training. They underwent history taking, physical examination, ECG, exercise testing and echocardiography. Furthermore, functional and echocardiography data that were also available from former evaluations were included in the analysis. There was a significant decline in exercise capacity. LV function was normal with a decrease in septal thickness to 9.1 ± 1.3 ( p < 0.05) and LV diastolic diameter to 48.9 ± 5.6 ( p < 0.05). Still, there was a remaining septal hypertrophy. RV function was 55.3 ± 4.2 % and there were normal RV dimensions adjusted for body surface area. 25 years after the cessation of endurance training there was a normal RV and LV function with a normalization of almost all diameters, still there was a mild LV hypertrophy in some athletes. Consequently, no relevant long-term cardiac remodeling after intensive endurance training was depicted in this group of athletes. [ABSTRACT FROM AUTHOR]

Congestive heart failure is frequent and leads to reduced exercise capacity, reduced quality of life (QoL), and depression in many patients. Cardiac resynchronization therapy (CRT) and implantable cardioverter defibrillators (ICD) offer therapeutic options and may have an impact on QoL and depression. This study was performed to evaluate physical and mental health in patients undergoing ICD or combined CRT/ICD-implantation (CRT-D). Echocardiography, spiroergometry, and psychometric questionnaires [Beck Depression Inventory, General World Health Organization Five Well-being Index (WHO-5), Brief Symptom Inventory and 36-item Short Form (SF-36)] were obtained in 39 patients (ICD: 17, CRT-D: 22) at baseline and 6-month follow-up (FU) after device implantation. CRT-D patients had a higher NYHA class and broader left bundle branch block than ICD patients at baseline. At FU, ejection fraction (EF), peak oxygen uptake, and NYHA class improved significantly in CRT-D patients but remained unchanged in ICD patients. Patients with CRT-D implantation showed higher levels of depressive symptoms, psychological distress, and impairment in QoL at baseline and FU compared to ICD patients. These impairments remained mostly unchanged in all patients after 6 months. Overall, these findings imply that there is a need for careful assessment and treatment of psychological distress and depression in ICD and CRT-D patients in the course of device implantation as psychological burden seems to persist irrespective of physical improvement. [ABSTRACT FROM AUTHOR]

Three-dimensional (3D)-imaging provides important information on cardiac anatomy during electrophysiological procedures. Real-time updates of modalities with high soft-tissue contrast are particularly advantageous during cardiac procedures. Therefore, a beat to beat 3D visualization of cardiac anatomy by intracardiac echocardiography (ICE) was developed and tested in phantoms and animals. An electronic phased-array 5-10 MHz ICE-catheter (Acuson, AcuNav™/Siemens Medical Solutions USA/64 elements) providing a 90° sector image was used for ICE-imaging. A custom-made mechanical prototype controlled by a servo motor allowed automatic rotation of the ICE-catheter around its longitudinal axis. During a single heartbeat, the ICE-catheter was rotated and 2D-images were acquired. Reconstruction into a 3D volume and rendering by a prototype software was performed beat to beat. After experimental validation using a rigid phantom, the system was tested in an animal study and afterwards, for quantitative validation, in a dynamic phantom. Acquisition of beat to beat 3D-reconstruction was technically feasible. However, twisting of the ICE-catheter shaft due to friction and torsion was found and rotation was hampered. Also, depiction of catheters was not always ensured in case of parallel alignment. Using a curved sheath for depiction of cardiac anatomy there was no congruent depiction of shape and dimension of static and moving objects. Beat to beat 3D-ICE-imaging is feasible. However, shape and dimension of static and moving objects cannot always be displayed with necessary steadiness as needed in the clinical setting. As catheter depiction is also limited, clinical use seems impossible. [ABSTRACT FROM AUTHOR]

Many age-related diseases are associated with, and may be promoted by, cardiac fibrosis. Transforming growth factor (TGF)-β, hypoxia-induced factor (HIF), and the matrix metalloproteinase (MMP) system have been implicated in fibrogenesis. Thus, we investigated whether age is related to these systems and to atrial fibrosis. Right atrial appendages (RAA) obtained during heart surgery ( n = 115) were grouped according to patients’ age (<50 years, 51–60 years, 61–70 years, or >70 years). Echocardiographic ejection fractions (EF) and fibrosis using Sirius-red-stained histological sections were determined. TGF-β was determined by quantitative RT-PCR and hypoxia-related factors [HIF1α, the vascular endothelial growth factor (VEGF)-receptor, CD34 (a surrogate marker for microvessel density), the factor inhibiting HIF (FIH), and prolyl hydroxylase 3 (PHD 3)] were detected by immunostaining. MMP-2 and -9 activity were determined zymographically, and mRNA levels of their common tissue inhibitor TIMP-1 were determined by RT-PCR. Younger patients (<50 years) had significantly less fibrosis (10.1% ± 4.4% vs 16.6% ± 8.3%) than older individuals (>70 years). While HIF1α, FIH, the VEGF-receptor, and CD34 were significantly elevated in the young, TGF-β and PHD3 were suppressed in these patients. MMP-2 and -9 activity was found to be higher while TIMP-1 levels were lower in older patients. Statistical analysis proved age to be the only factor influencing fibrogenesis. With increasing age, RAAs develop significantly more fibrosis. An increase of fibrotic and decrease of hypoxic signalling and microvessel density, coupled with differential expression of MMPs and TIMP-1 favouring fibrosis may have helped promote atrial fibrogenesis. [ABSTRACT FROM AUTHOR]

In computed tomography (CT) several contrast media with different iodine concentrations are available. The aim of this study is to prospectively compare contrast media with iodine concentrations of 300, 370 and 400 mg iodine/ml for chest- CT. 300 consecutive patients were prospectively enrolled, under a waiver of the local ethics committee. The first (second, third) 100 patients, received contrast medium with 300 (370, 400) mg iodine/ml. Injection protocols were adapted for an identical iodine delivery rate (1.3 mg/s) and total iodine load (33 g) for all three groups. Standardized MDCT of the chest (16 x 0.75 mm, 120 kVp, 100 mAseff.) was performed. Intravascular attenuation values were measured in the pulmonary trunk and the ascending aorta; subjective image quality was rated on a 3-point-scale. Discomfort during and after injection was evaluated. There were no statistically significant differences in contrast enhancement comparing the three contrast media at the pulmonary trunk (p = 0.3198) and at the ascending aorta (p = 0.0840). Image quality (p = 0.0176) and discomfort during injection (p = 0.7034) were comparable for all groups. General discomfort after injection of contrast media with 300 mg iodine/ml was statistically significant higher compared to 370 mg iodine/ml (p = 0.00019). Given identical iodine delivery rates of 1.3 g/s and iodine loads of 33 g, contrast media with concentrations of 300, 370 and 400 mg iodine/ml do not result in different intravascular enhancement in chest-CT. [ABSTRACT FROM AUTHOR]

Background: Knowledge of the anatomy of the coronary venous system (CVS) is important for planning of cardiac interventions like cardiac resynchronization therapy or percutaneus mitral annuloplasty. Different methods have been used for preprocedural visualization of the CVS. However, limited data is available comparing invasive retrograde coronary sinus angiography (CSA) and non-invasive multi slice computed tomography (MSCT). Thus, a comparison of retrograde CSA and ECG-gated MSCT for the visualization of the CVS in patients with congestive heart failure (CHF) was performed.Methods: 20 patients (male: 11) with CHF underwent CSA and MSCT (16 x 0.75 mm collimation, tube voltage: 120 kV, tube current: 550 mAs(eff)). Both methods were compared with respect to vessel diameter and visibility.Results: Vessel visualization was better using retrograde CSA except for middle cardiac vein and small veins which were better seen with MSCT. Overall, there was a trend that MSCT detected more vessels. Vessel diameters were larger measured using retrograde CSA, but only statistically significant for the coronary sinus and middle cardiac vein.Conclusion: Whereas MSCT is more suitable for an overview, retrograde CSA offers a more detailed visualization of the CVS including marginal and posterior veins than MSCT. Thus, retrograde CSA allows a better display of target vessels commonly used for cardiac interventions. Overall, these two imaging techniques offer complementary information. [ABSTRACT FROM AUTHOR]

Information on the anatomy of the cardiac venous system (CVS) is increasingly important for cardiac resynchronization therapy or percutaneous transvenous mitral valve annuloplasty. Three-dimensional (3D) imaging can further improve the understanding of the relationship of cardiac structures. This study was performed to validate the accuracy of rotational coronary sinus angiography (CSA) displaying the 3D anatomy of the CVS compared to ECG-gated, contrast-enhanced, cardiac dual-source computed tomography (DSCT). Five domestic pigs (60 kg) underwent DSCT using a standardized examination protocol. Using a standard C-arm for fluoroscopy, a rotational CSA was obtained and 3D-image reconstructions performed. Side branches were identified using both methods and enumerated. Vessel visibility was estimated for each side branch and great cardiac vein/anterior interventricular vein. Also, vessel diameters were measured at distinct landmarks, i.e., side branching. The amount of contrast medium was determined and the effective radiation exposure of both methods was calculated. There was no significant difference regarding the vessel diameter of the great cardiac vein/anterior interventricular vein or its side branches. Also, estimation of vessel visibility was not different between the two imaging modalities. Estimated radiation exposure and amount of contrast medium were lower for rotational CSA. In conclusion, a 3D reconstruction of rotational CSA images is possible. All parts of the CVS are well depicted, allowing a 3D overview of the CVS anatomy. On-site 3D visualization might improve decision making during cardiac interventions. In contrast to DSCT, rotational CSA does not demonstrate the anatomy of the mitral annulus or the course of the left circumflex artery. [ABSTRACT FROM AUTHOR]

The purpose was to evaluate a new semi-automated 3D region-growing segmentation algorithm for functional analysis of the left ventricle in multislice CT (MSCT) of the heart. Twenty patients underwent contrast-enhanced MSCT of the heart (collimation 16 x 0.75 mm; 120 kV; 550 mAseff). Multiphase image reconstructions with 1-mm axial slices and 8-mm short-axis slices were performed. Left ventricular volume measurements (end-diastolic volume, end-systolic volume, ejection fraction and stroke volume) from manually drawn endocardial contours in the short axis slices were compared to semi-automated region-growing segmentation of the left ventricle from the 1-mm axial slices. The post-processing-time for both methods was recorded. Applying the new region-growing algorithm in 13/20 patients (65%), proper segmentation of the left ventricle was feasible. In these patients, the signal-to-noise ratio was higher than in the remaining patients (3.2+/-1.0 vs. 2.6+/-0.6). Volume measurements of both segmentation algorithms showed an excellent correlation (all P