The Zero Calcium Score Paradox.

Our study presents a case of angina with a zero calcium score yet severe coronary stenosis from noncalcified plaque. We highlight the limitation of otherwise prognostically powerful coronary calcium score as a singular predictive tool especially when used in symptomatic patients.

Long-Term Imaging and Clinical Outcomes of Surgical Left Atrial Appendage Occlusion With AtriClip.

Surgical left atrial appendage (LAA) occlusion with an AtriClip (AtriCure, West Chester, Ohio) is frequently performed for stroke prophylaxis in patients with atrial fibrillation (AF). We conducted a retrospective analysis of all patients with long-standing persistent AF who underwent hybrid convergent ablation and LAA clipping. Contrast-enhanced cardiac computed tomography was performed at 3 to 6 months after LAA clipping to assess the degree of complete closure and the residual LAA stump. A total of 78 patients (64 ± 10 years, 72% male) underwent LAA clipping as part of hybrid convergent AF ablation, from 2019 to 2020. Median size of AtriClip used was 45 mm. Mean LA size was 4.6 ± 1 cm. At 3-to-6 months follow-up computed tomography, 46.2% of patients (n = 36) had a residual stump proximal to the deployed LAA clip. Mean depth of residual stump was 3.95 ± 5.5 mm, with 19% of patients (n = 15) having a stump depth of ≥10 mm and 1 patient requiring more endocardial LAA closure owing to large stump depth. During 1-year follow-up, 3 patients developed stroke; device leak of 6 mm was noted in 1 patient; and none of the patients had a thrombus proximal to the clip. In conclusion, high incidence of residual LAA stump was observed with AtriClip. Larger studies with long-term follow-up are needed to better assess the thromboembolic implications of a residual stump after AtriClip placement.

99mTc Bone-Avid Tracer Cardiac Scintigraphy: Role in Noninvasive Diagnosis of Transthyretin Cardiac Amyloidosis.

Transthyretin cardiac amyloidosis (ATTR-CA) is an overlooked cause of heart failure, with substantial morbidity and mortality. The emergence of several novel therapies has fueled the interest in early and accurate diagnosis of ATTR-CA so that potentially life-saving pharmacologic therapy can be administered in a timely manner. The most promising imaging modality and biomarker is SPECT imaging with technetium 99m (99mTc)-radiolabeled bone-seeking tracers, which have high specificity in the diagnosis of ATTR-CA, potentially obviating biopsy. In this article, the authors provide a focused review on the use of 99mTc pyrophosphate (PYP), 3,3-diphosphono-1,2-propanodicarboxylic acid (DPD), and hydroxymethylene diphosphonate (HMDP) for diagnosis of ATTR-CA, present a systematic approach to interpretation of the scans, and highlight several common pitfalls to illustrate important diagnostic principles for accurate interpretation of these images. The authors indicate when to use endomyocardial biopsy for the diagnosis of cardiac amyloidosis and conclude with a section on quantitation of 99mTc-PYP/DPD/HMDP imaging.

Echocardiographic indices of left ventricular function and filling pressure are not related to blood pool activity on pyrophosphate scintigraphy.

BACKGROUND: Pyrophosphate (PYP) imaging has a high diagnostic accuracy for transthyretin cardiac amyloidosis (ATTR-CA). Indeterminate findings are often reported due to persistent blood pool activity, presumed to be from low cardiac output. We evaluated the relationship between blood pool activity on PYP imaging and echocardiographic indices of cardiac function. METHODS: Clinical and imaging data of 189 patients referred for PYP scintigraphy were evaluated. All patients underwent planar imaging and SPECT (diagnostic standard). Among those with a negative PYP SPECT, persistent left ventricular blood pool activity on planar images was inferred by a visual score ≥2 or a heart-to-contralateral (HCL) ratio ≥ 1.5. Absence of blood pool activity was inferred when both visual score was < 2 and HCL was < 1.5. Left ventricular ejection fraction (LVEF), global longitudinal strain (GLS), stroke volume index (SVi), and left atrial pressure (LAP) were calculated from standard transthoracic echocardiograms. RESULTS: ATTR-CA was present in 43 (23%) patients. Among those with a negative PYP SPECT, 11 patients had significant blood pool activity. Patients with ATTR-CA had a lower LVEF, SVi, and GLS, with a higher LAP, compared to those without ATTR-CA. Among those without ATTR-CA, there were no significant differences in these parameters. CONCLUSION: Approximately 8% of patients with a negative PYP SPECT have significant blood pool activity. Measures of cardiac function are not different among those with and without blood pool activity. PYP SPECT should be routinely performed in all patients to avoid false image interpretation.

Mechanism and management of leaks arising after left atrial appendage closure (LAAC).

This article reviews the latest available data in regard to the diagnosis, management, and intervention of both central and peri-device leaks that arise after left atrial appendage closure (LAAC). The aim of this article is to have a better understanding of both addressing leaks arising after LAAC, and which interventions and closure methods are best served for each type of residual leak based on etiology, size, and operator experience.

A Rare Case of New-Onset Atrial Fibrillation Presenting as Biatrial Inflammation Seen on 18F-FDG-PET.

Isolated atrial myocarditis, a rare clinical entity, is presented in this case report as the diagnosis in a patient with new-onset atrial fibrillation. Our findings emphasize the potential for atrial arrhythmias and their unusual presentation; the role of multimodal imaging, especially 18F-FDG-PET/CT, in diagnosis; and considerations for long-term treatment strategies.

Harnessing imaging biomarkers to refine individualized cardiovascular disease risk: a case-based discussion.

Traditional models of cardiovascular risk assessment rely on population-level risk factors and may not accurately capture individualized risk. Imaging biomarkers such as plaque characterization and pericoronary fat inflammation may offer refined risk prediction and allow physicians to personalize care-plans for cardiovascular disease prevention. The integration of plaque morphology and pericoronary inflammation into clinical care is highlighted using a case-based discussion. This article reviews the existing body of evidence supporting the use of novel biomarkers in an individualized comprehensive risk assessment algorithm.

Inter-observer reproducibility and intra-observer repeatability in 99mTc-pyrophosphate scan interpretation for diagnosis of transthyretin cardiac amyloidosis.

AIM: The purpose of this study was to determine the inter- and intra-observer variability in 99mtechnetium-pyrophosphate (99mTc-PYP) scan interpretation for diagnosis of transthyretin cardiac amyloidosis (ATTR). METHODS AND RESULTS: Our study cohort comprised 100 consecutive subjects referred for 99mTc-PYP imaging based on clinical suspicion of ATTR cardiac amyloidosis. Myocardial 99mTc-PYP uptake was assessed by both visual (comparison of myocardial to rib uptake) and semi-quantitative (heart-to-contralateral lung uptake ratio, H:CL) methods. Twenty scans were analyzed twice, at least 48 hours apart, by each of two independent observers. Patients with visual scores of ≥ 2 on planar imaging as well as myocardial uptake on SPECT/CT were classified as ATTR positive. Diagnosis of ATTR by visual 99mTc-PYP grade was perfectly reproducible [concordance: positive and negative scans 100% (53/53 and 47/47, respectively). Both inter- and intra-observer correlations for H:CL ratio (r2 = 0.90, 0.99 (Observer 1) and 0.98 (Observer 2), respectively) and repeatability values on Bland-Altman plots were excellent. The coefficient of variation (%) for Observers 1 and 2 was 3.21 (2.14 to 4.29) and 7.49 (4.95 to 10.09), respectively. In addition, there was 100% concordance in positive and negative scan interpretation by visual grading between novice CV imagers (< 3 years' experience) and an experienced CV imager (10 years' experience). CONCLUSIONS: This study showed excellent inter-observer reproducibility and intra-observer repeatability of 99mTc-PYP visual scan interpretation and H:CL ratio for diagnosis of cardiac ATTR amyloidosis. Cardiac ATTR amyloidosis can be diagnosed reliably using 99mTc-PYP SPECT/CT by novice and experienced CV imagers.

Myocardial Composition in Light-Chain Cardiac Amyloidosis More Than 1 Year After Successful Therapy.

OBJECTIVES: The goals of this study were to characterize myocardial composition during the active and remission phases of light-chain (AL) cardiac amyloidosis. BACKGROUND: Cardiac dysfunction in AL amyloidosis is characterized by dual insults to the myocardium from infiltration and toxicity from light chains during the active phase and by infiltration alone in the remission phase. METHODS: Prospectively enrolled subjects with cardiac AL amyloidosis (21 remission AL amyloidosis; age: 63.4 ± 7.3 years; 47.6% male; and 48 active AL amyloidosis; age: 62.5 ± 7.4 years; 60.4% male) underwent contrast-enhanced cardiac magnetic resonance with T1 and T2 mapping and measurement of extracellular volume (ECV). By definition, serum free light-chain levels were normal for at least 1 year following successful AL therapy in the remission group and abnormal in the active group. RESULTS: Myocardial ECV was similarly expanded in the remission and active AL amyloidosis groups (0.488 ± 0.082 vs 0.519 ± 0.083, respectively; P = 0.15). However, myocardial T2 relaxation times (47.7 ± 3.2 ms vs 45.5 ± 3.0 ms; P = 0.008) as well as native T1 times (1,368 ms [IQR: 1,290-1,422 ms] vs 1,264 ms [IQR: 1,203-1,380 ms]; P = 0.024) were significantly higher in the remission compared to the active AL amyloidosis group. CONCLUSIONS: Myocardial ECV is substantially expanded in the active AL and remission AL cardiac amyloidosis groups, but native T1 values were higher, suggesting a different myocardial composition. There is no evidence of myocardial edema in active AL cardiac amyloidosis. Future phenotyping studies of AL cardiac amyloidosis need to consider complementary myocardial markers that define the interstitial milieu in addition to changes in extracellular volume. (Molecular Imaging of Primary Amyloid Cardiomyopathy; NCT02641145).

Biventricular Strain Imaging with Cardiac MRI in Genotyped and Histology Validated Amyloid Cardiomyopathy.

Cardiac amyloidosis (CA) is a common and potentially fatal infiltrative cardiomyopathy. Contrast-enhanced cardiac MRI (CMR) is used as a diagnostic tool. However, utility of CMR for the comprehensive analysis of biventricular strains and strain rates is not reported as extensively as echocardiography. In addition, RV strain analysis using CMR has not been described previously. OBJECTIVES: We sought to study the global and regional indices of biventricular strain and strain rates in endomyocardial biopsy (EMB)-proven, genotyped cases of CA. METHODS: A database of 80 EMBs was curated from 2012 to 2019 based on histology. A total of 19 EMBs positive for CA were subjected to further tissue-characterization with histology, and compared with four normal biopsy specimens. Samples were genotyped for ATTR- or AL-subtypes. Five patients, with both echocardiography and contrast-enhanced CMR performed 72-h apart, were subjected to comprehensive analysis of biventricular strain and strain-rates. RESULTS: Histology confirmed that the selected samples were indeed positive for cardiac amyloid. Echocardiography showed reduced global and regional left-ventricular (LV) longitudinal strain indices. CMR with tissue-characterization of LV showed global reductions in circumferential, radial and longitudinal strains and strain-rates, following a general trend with the echocardiographic findings. The basal right-ventricular (RV) segments had reduced circumferential strains with no changes in longitudinal strain. CONCLUSIONS: In addition to providing a clinical diagnosis of CA based on contrast clearance-dynamics, CMR can be a potent tool for accurate functional assessment of global and regional changes in strain and strain-rates involving both LV and RV. Further studies are warranted to validate and curate the strain imaging capacity of CMR in CA.

Normal Variants and Pitfalls in Cardiac PET/CT.

Positron emission tomography and/or computed tomography (PET/CT) MPI is a powerful imaging modality for the assessment of cardiovascular diseases. It offers several advantages over single-photon emission computed tomography (SPECT) MPI including robust attenuation correction and absolute quantification of radiotracer activity. PET MPI has a large evidence base and is the only clinical tool to evaluate coronary microvascular dysfunction. In addition, the clinical use and evidence base for 2-deoxy-2-[18F]fluoro-D-g1ucose (18F-FDG) cardiac PET imaging for inflammation and metabolism imaging is rising exponentially. In order to gain from the advances of this sophisticated quantitative technique, a high-quality scan is critical. It is important for readers to recognize a poor-quality scan, identify artifacts contributing to the poor image quality, and understand how to correct them prior to reporting the results. In this review, we will discuss some normal variants and pitfalls in cardiac PET/CT radionuclide MPI, myocardial viability, and inflammation imaging.