Publications by authors named "Paul R Scully"

Background: Extracellular volume fraction (ECV) is a marker for myocardial fibrosis and infiltration, can be quantified using cardiac computed tomography (ECV), and has prognostic utility in several diseases. This study aims to map out regional differences in ECV to obtain greater insights into the pathophysiological mechanisms of ECV expansion and its clinical implications.

Methods: Three prospective cohorts were included: patients with aortic stenosis (AS) and coexisting AS and transthyretin cardiac amyloidosis were referred for a transcatheter aortic valve replacement and had ECG-gated CT angiography and Technetium-99m-labelled 3,3-diphosphono-1,2-propanodicarboxylic acid scintigraphy to differentiate between the 2 cohorts.

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Although transcatheter aortic valve implantation (TAVI) has revolutionised the landscape of treatment for aortic stenosis, there exists a cohort of patients where TAVI is deemed futile. Among the pivotal high-risk trials, one-third to half of patients either died or received no symptomatic benefit from the procedure at 1 year. Futility of TAVI results in the unnecessary exposure of risk for patients and inefficient resource utilisation for healthcare services.

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Article Synopsis
  • The study investigated the impact of wild-type transthyretin cardiac amyloidosis (ATTR) on patients with severe aortic stenosis (AS) undergoing transcatheter aortic valve implantation (TAVI), identifying diagnostic and management challenges.
  • Researchers analyzed data from 583 patients, comparing groups with AS, ATTR, AS-ATTR, and age-matched controls, assessing factors like cardiac remodeling and function using NT-proBNP as a main measurement.
  • Results showed that patients with AS-ATTR experienced more severe cardiac issues compared to those with just AS, suggesting that the combined condition is at an early stage of amyloid infiltration, yet still resembles more advanced ATTR complications, indicating potential benefits from ATTR-targeted treatments.
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Background: Older patients with severe aortic stenosis (AS) are increasingly identified as having cardiac amyloidosis (CA). It is unknown whether concomitant AS-CA has worse outcomes or results in futility of transcatheter aortic valve replacement (TAVR).

Objectives: This study identified clinical characteristics and outcomes of AS-CA compared with lone AS.

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Objectives: The purpose of this study was to validate computed tomography measured ECV (ECV) as part of routine evaluation for the detection of cardiac amyloid in patients with aortic stenosis (AS)-amyloid.

Background: AS-amyloid affects 1 in 7 elderly patients referred for transcatheter aortic valve replacement (TAVR). Bone scintigraphy with exclusion of a plasma cell dyscrasia can diagnose transthyretin-related cardiac amyloid noninvasively, for which novel treatments are emerging.

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Objectives: To assess whether single-photon emission computed tomography (SPECT/CT) quantification of bone scintigraphy would improve diagnostic accuracy and offer a means of quantifying amyloid burden.

Background: Transthyretin-related cardiac amyloidosis is common and can be diagnosed noninvasively using bone scintigraphy; interpretation, however, relies on planar images. SPECT/CT imaging offers 3-dimensional visualization.

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Aims: Cardiac amyloidosis is common in elderly patients with aortic stenosis (AS) referred for transcatheter aortic valve implantation (TAVI). We hypothesized that patients with dual aortic stenosis and cardiac amyloid pathology (AS-amyloid) would have different baseline characteristics, periprocedural and mortality outcomes.

Methods And Results: Patients aged ≥75 with severe AS referred for TAVI at two sites underwent blinded bone scintigraphy prior to intervention (Perugini Grade 0 negative, 1-3 increasingly positive).

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Aims: Marathon running is a popular ambition in modern societies inclusive of non-athletes. Previous studies have highlighted concerning transient myocardial dysfunction and biomarker release immediately after the race. Whether this method of increasing physical activity is beneficial or harmful remains a matter of debate.

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Purpose Of Review: This review article discusses the evolution of extracellular volume (ECV) quantification using both cardiovascular magnetic resonance (CMR) and computed tomography (CT).

Recent Findings: Visualizing diffuse myocardial fibrosis is challenging and until recently, was restricted to the domain of the pathologist. CMR and CT both use extravascular, extracellular contrast agents, permitting ECV measurement.

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Background: Calcific aortic stenosis (cAS) affects 3% of individuals aged >75 years, leading to heart failure and death unless the valve is replaced. Wild-type transthyretin cardiac amyloid is also a disorder of ageing individuals. Prevalence and clinical significance of dual pathology are unknown.

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