Multimodality Imaging Reveals Divergent Responses of Left and Right Heart to Treatment in Cardiac Amyloidosis.
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ABSTRACT:
SUBMITTER: Poller W
PROVIDER: S-EPMC8289130 | biostudies-literature |
REPOSITORIES: biostudies-literature
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| S-EPMC6710901 | biostudies-literature
Project description:Background: Cardiac amyloidosis (CA) manifests in a hypertrophic phenotype with a poor prognosis, making differentiation from hypertrophic cardiomyopathy (HCM) challenging and delaying early treatment. The extent to which magnetic resonance imaging (MRI) quantifies the right atrial strain (RAS) and strain rate (RASR), providing valuable diagnostic information, is not yet clinically established. Aims: This study assesses diagnostic differences in the longitudinal RAS and RASR between CA and HCM patients, control subjects (CTRL) and CA subtypes in addition to the impact of atrial fibrillation (AF) on the right atrial function in CA patients. The RAS and RASR of tricuspid regurgitation (TR) patients are used to assess the potential for diagnostic overlap. Methods: RAS and RASR quantification was conducted via MRI feature-tracking for biopsy-confirmed CA patients with subtypes identified. Strain parameters were compared for CTRL, HCM and TR patients. Post hoc testing identified intergroup differences. Results: In total, 41 CA patients were compared to 47 CTRL, 20 HCM and 31 TR patients. Reservoir (R), conduit and booster RAS and RASRs allow for significant differentiation (p < 0.001) between CA and HCM patients (R: 10.6 ± 14.3% vs. R: 33.5 ± 16.3%) and CTRL (R: 44.6 ± 15.7%). Booster and reservoir RAS and RASRs qualified as reliable diagnostic tests (AUC > 0.8). CA patients with AF, in contrast to sinus rhythm, demonstrated a significantly impaired reservoir RAS and RASR and booster RASR. The discriminative power of RAS for CA vs. TR was insufficient (R: 10.6% ± 14.3% vs. 7.0% ± 6.0%, p = 0.069). Differentiation between 21 transthyretin and 20 light-chain amyloidosis subtypes was not achievable (R: 0.7% ± 1.0% vs. 0.7% ± 1.0%, p = 0.827). Conclusion: The MRI-derived RAS and RASR are impaired in CA patients and may support noninvasive differentiation between CA, HCM and CTRL.
| S-EPMC9775378 | biostudies-literature
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| S-EPMC8819573 | biostudies-literature
Project description:Right ventricular (RV) and left ventricular (LV) myocardium differ in their pathophysiological response to pressure-overload hypertrophy. In this report we use microarray and proteomic analyses to identify pathways modulated by LV-aortic banding (AOB) and RV-pulmonary artery banding (PAB) in the immature heart. Newborn New Zealand White rabbits underwent banding of the descending thoracic aorta [LV-AOB; n = 6]. RV-PAB was achieved by banding the pulmonary artery (n = 6). Controls (n = 6 each) were sham-manipulated. After 4 (LV-AOB) and 6 (RV-PAB) wk recovery, the hearts were removed and matched RNA and proteins samples were isolated for microarray and proteomic analysis. Microarray and proteomic data demonstrate that in LV-AOB there is increased transcript expression levels for oxidative phosphorylation, mitochondria energy pathways, actin, ILK, hypoxia, calcium, and protein kinase-A signaling and increased protein expression levels of proteins for cellular macromolecular complex assembly and oxidative phosphorylation. In RV-PAB there is also an increased transcript expression levels for cardiac oxidative phosphorylation but increased protein expression levels for structural constituents of muscle, cardiac muscle tissue development, and calcium handling. These results identify divergent transcript and protein expression profiles in LV-AOB and RV-PAB and provide new insight into the biological basis of ventricular specific hypertrophy. The identification of these pathways should allow for the development of specific therapeutic interventions for targeted treatment and amelioration of LV-AOB and RV-PAB to ameliorate morbidity and mortality.
| S-EPMC3833994 | biostudies-literature
Project description:Cardiac amyloidosis is a form of restrictive cardiomyopathy resulting in heart failure and potential risk on arrhythmia, due to amyloid infiltration of the nerve conduction system and the myocardial tissue. The prognosis in this progressive disease is poor, probably due the development of cardiac arrhythmias. Early detection of cardiac sympathetic innervation disturbances has become of major clinical interest, because its occurrence and severity limits the choice of treatment. The use of iodine-123 labelled metaiodobenzylguanidine ([I-123]MIBG), a chemical modified analogue of norepinephrine, is well established in patients with heart failure and plays an important role in evaluation of sympathetic innervation in cardiac amyloidosis. [I-123]MIBG is stored in vesicles in the sympathetic nerve terminals and is not catabolized like norepinephrine. Decreased heart-to-mediastinum ratios on late planar images and increased wash-out rates indicate cardiac sympathetic denervation and are associated with poor prognosis. Single photon emission computed tomography provides additional information and has advantages for evaluating abnormalities in regional distribution in the myocardium. [I-123]MIBG is mainly useful in patients with hereditary and wild-type ATTR cardiac amyloidosis, not in AA and AL amyloidosis. The potential role of positron emission tomography for cardiac sympathetic innervation in amyloidosis has not yet been identified.
| S-EPMC6394628 | biostudies-literature
Project description:While patients with transthyretin cardiac amyloidosis (ATTR-CA) typically present with concentric or asymmetric hypertrophy, a small percentage of ATTR-CA is known to present with 'atypical' cardiac morphologies such as eccentric hypertrophy or even no hypertrophy. However, detailed report of multimodality assessments of ATTR-CA with no ventricular hypertrophy is lacking. Herein, we report detailed multimodality assessments of an 81-year-old Japanese woman with heart failure and history of carpal tunnel syndrome and lumbar canal stenosis, presenting no ventricular hypertrophy and negative 99m technetium-pyrophosphate scintigraphy, who was eventually diagnosed as having wild-type ATTR-CA. Our case highlights the role of multimodality assessments for early diagnosis of ATTR-CA in patients with atypical cardiac morphologies and also emphasizes the limitations of bone scintigraphy and the importance of considering ATTR-CA in patients with non-cardiac manifestations of ATTR amyloidosis.
| S-EPMC10053176 | biostudies-literature
Project description:BackgroundImprovements in outcomes for patients with congenital heart disease (CHD) have increased the need for diagnostic and interventional procedures. Cumulative radiation risk is a growing concern. MRI-guided interventions are a promising ionizing radiation-free, alternative approach.PurposeTo assess the feasibility of MRI-guided catheterization in young patients with CHD using advanced visualization passive tracking techniques.Study typeProspective.PopulationA total of 30 patients with CHD referred for MRI-guided catheterization and pulmonary vascular resistance analysis (median age/weight: 4 years / 15 kg).Field strength/sequence1.5T; partially saturated (pSAT) real-time single-shot balanced steady-state free-precession (bSSFP) sequence.AssessmentImages were visualized by a single viewer on the scanner console (interactive mode) or using a commercially available advanced visualization platform (iSuite, Philips). Image quality for anatomy and catheter visualization was evaluated by three cardiologists with >5 years' experience in MRI-catheterization using a 1-5 scale (1, poor, 5, excellent). Catheter balloon signal-to-noise ratio (SNR), blood and myocardium SNR, catheter balloon/blood contrast-to-noise ratio (CNR), balloon/myocardium CNR, and blood/myocardium CNR were measured. Procedure findings, feasibility, and adverse events were recorded. A fraction of time in which the catheter was visible was compared between iSuite and the interactive mode.Statistical testsT-test for numerical variables. Wilcoxon signed rank test for categorical variables.ResultsNine patients had right heart catheterization, 11 had both left and right heart catheterization, and 10 had single ventricle circulation. Nine patients underwent solely MRI-guided catheterization. The mean score for anatomical visualization and contrast between balloon tip and soft tissue was 3.9 ± 0.9 and 4.5 ± 0.7, respectively. iSuite provided a significant improvement in the time during which the balloon was visible in relation to interactive imaging mode (66 ± 17% vs. 46 ± 14%, P < 0.05).Data conclusionMRI-guided catheterizations were carried out safely and is feasible in children and adults with CHD. The pSAT sequence offered robust and simultaneous high contrast visualization of the catheter and cardiac anatomy.Level of evidence2 TECHNICAL EFFICACY STAGE: 1.
| S-EPMC8247035 | biostudies-literature
Project description:A 74-year-old man underwent echocardiographic exam for hypertension screening. A fixed plurilobulated mass originating from the right ventricular lateral wall and occupying half of the cavity was incidentally diagnosed. On cardiac magnetic resonance (CMR) it appeared homogeneous, intermediate-to-high signal on T1-weighted, and diffusely hyperintense on T2-weighted images. First pass enhancement was late and heterogeneous and no late gadolinium enhancement was present. Computed tomography (CT) showed no extracardiac infiltration, the feeding artery was a branch of therightcoronary artery. The tumor was excised and histological examination demonstrated a hemangioma of the cavernous type. The postoperative course was uneventful. From 1998 to 2014, four cardiac hemangiomas have been diagnosed in our Department, accounting for 8.7% of all primary cardiac tumors and for 9.5% of all benign forms; estimated population prevalence was 0.11/100.000 inhabitants/year. The hemodynamic consequences of unoperated cardiac hemangiomas cannot be predicted and therefore, resection is recommended.
| S-EPMC5353457 | biostudies-literature
Project description: Not available
| S-EPMC6302035 | biostudies-literature
Project description:Amyloidosis is a heterogeneous group of diseases characterized by localized or systemic deposition of insoluble extracellular fibrillary proteins in organs and tissues. Several types of amyloid can infiltrate the heart resulting in a restrictive cardiomyopathy, heart failure, and atrial and ventricular arrhythmias. Scintigraphy is a noninvasive method that may facilitate early diagnosis, distinguish various forms of cardiac amyloid, and may be useful in following disease burden. The amyloid-specific tracers presented in this article have been used with planar imaging and/or single-photon emission computed tomography. To date, there are no approved cardiac amyloid tracers although investigational tracers are currently under examination. This article serves to review the current nuclear imaging modalities available in the detection of cardiac amyloid.
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