Artificial intelligence-based opportunistic detection of coronary artery stenosis on aortic computed tomography angiography in emergency department patients with acute chest pain.

Aims: To evaluate a deep-learning model (DLM) for detecting coronary stenoses in emergency room patients with acute chest pain (ACP) explored with electrocardiogram-gated aortic computed tomography angiography (CTA) to rule out aortic dissection. Methods and results: This retrospective study included 217 emergency room patients (41% female, mean age 67.2 years) presenting with ACP and evaluated by aortic CTA at our institution. Computed tomography angiography was assessed by two readers, who rated the coronary arteries as 1 (no stenosis), 2 (<50% stenosis), or 3 (≥50% stenosis). Computed tomography angiography was categorized as high quality (HQ), if all three main coronary arteries were analysable and low quality (LQ) otherwise. Curvilinear coronary images were rated by a DLM using the same system. Per-patient and per-vessel analyses were conducted. One hundred and twenty-one patients had HQ and 96 LQ CTA. Sensitivity, specificity, positive predictive value, negative predictive value (NPV), and accuracy of the DLM in patients with high-quality image for detecting ≥50% stenoses were 100, 62, 59, 100, and 75% at the patient level and 98, 79, 57, 99, and 84% at the vessel level, respectively. Sensitivity was lower (79%) for detecting ≥50% stenoses at the vessel level in patients with low-quality image. Diagnostic accuracy was 84% in both groups. All 12 patients with acute coronary syndrome (ACS) and stenoses by invasive coronary angiography (ICA) were rated 3 by the DLM. Conclusion: A DLM demonstrated high NPV for significant coronary artery stenosis in patients with ACP. All patients with ACS and stenoses by ICA were identified by the DLM.

Real-Life Evaluation of an Algorithm for the Diagnosis of Cardiac Amyloidosis.

OBJECTIVE: To evaluate the real-life use of a modified Gillmore algorithm with a "one-stop-shop" approach, bone scintigraphy (BS), a monoclonal gammopathy test (GT), a salivary gland biopsy (SGB), and genetic testing performed at the same time for the diagnosis of cardiac amyloidosis at the French National Reference Centre for Cardiac Amyloidosis (Henri Mondor Hospital, Créteil, France). METHODS: This retrospective cohort study included a total of 1222 patients with suspected amyloidosis who underwent BS and GT between June 2008 and May 2019. RESULTS: Of 1222 patients, 349 had no cardiac uptake on BS and negative GT (BS-/GT-), 276 were BS-/GT positive (GT+), 420 patients were BS+/GT-, and 177 were BS+/GT+. Our one-stop-shop check-up enabled us to diagnose 892 (72.9%) patients; 330 (27.0%) patients required additional examinations, such as mass spectrometry and/or a cardiac biopsy. This subset notably included 112 patients with amyloid light chain amyloidosis. More than 64% of the patients with transthyretin amyloidosis or another type of amyloidosis were diagnosed during the one-stop shop visit. Sensitivity and specificity of BS for transthyretin amyloidosis diagnosis was 99% and 96%, respectively. For amyloid light chain diagnosis, sensitivity and specificity were 100% and 76%, respectively, for GT and 54% and 100%, respectively, for SGB. Of 910 transthyretin genetic tests, 205 (17%) detected mutations. CONCLUSION: The results of our real-life cohort study confirmed the ability of a one-stop-shop approach with a modified Gillmore algorithm to diagnose cardiac amyloidosis and the interest of simultaneous testing for earlier diagnosis. The SGB has diagnostic value because it is easy, quick, and less invasive than a cardiac biopsy.

Correlation between Pulmonary Artery Pressure and Vortex Duration Determined by 4D Flow MRI in Main Pulmonary Artery in Patients with Suspicion of Chronic Thromboembolic Pulmonary Hypertension (CTEPH).

Chronic thromboembolic pulmonary hypertension (CTEPH) is one of the causes of pulmonary hypertension (PH) and requires invasive measurement of the mean pulmonary artery pressure (mPAP) during right heart catheterisation (RHC) for the diagnosis. 4D flow MRI could provide non-invasive parameters to estimate the mPAP. Twenty-five patients with suspected CTEPH underwent cardiac MRI. Mean vortex duration (%), pulmonary distensibility, right ventricular volumes and function were measured using 4D flow MRI and cine sequences, and compared with the mPAP measured by RHC. The mPAP measured during RHC was 33 ± 16 mmHg (10−66 mmHg). PH (defined as mPAP > 20 mmHg) was present in 19 of 25 patients (76%). A vortical flow was observed in all but two patients (92%) on 4D flow images, and vortex duration showed good correlation with the mPAP (r = 0.805; p < 0.0001). Youden index analysis showed that a vortex duration of 8.6% of the cardiac cycle provided a 95% sensitivity and an 83% specificity to detect PH. Reliability for the measurement of vortex duration was excellent for both intra-observer ICC = 0.823 and inter-observer ICC = 0.788. Vortex duration could be a useful parameter to non-invasively estimate mPAP in patients with suspected CTEPH.

[Cardiac imaging in infiltrative cardiomyopathies. What cardiovascular imaging modalities to propose in hypertrophic cardiomyopathies ?] / Rôle de l'imagerie cardiaque dans les cardiomyopathies infiltratives: Quels examens d'imagerie demander devant une hypertrophie ventriculaire gauche ?

Infiltrative cardiomyopathies are abnormal accumulations or depositions of different substances in cardiac tissue leading to its dysfunction, first diastolic, then systolic. The different infiltrative cardiomyopathies are amyloidosis (both light chain amyloidosis and transthyretin amyloidosis variants), lysosomal and glycogen storage disorders (Fabry-Anderson disease), and iron overload (hemochromatosis and thalassemia associated with blood transfusions), as well as inflammatory diseases such as sarcoidosis. We also evoke hypereosinophilic syndrome associated with endomyocardial fibrosis. Echocardiography is the first essential step after interrogatory and clinical examination and may help the cardiologist as a screening tool. Cardiac MRI is the second fundamental step towards the diagnosis especially due to the late gadolinium enhancement and to the T1-mapping. Cardiac amyloidosis diagnosis also requires the use of nuclear imaging. Cardiac CT-Scan may be useful for estimating the amyloid load, identify potential cardiac thrombus and rule out associated coronaropathy.

Describing mode of death in three major cardiac amyloidosis subtypes to improve management and survival.

BACKGROUND: The three main cardiac amyloidosis (CA) types have different progression and prognosis. Little is known about the mode of death (MOD) which is commonly attributed to cardiovascular causes in CA. Improving MOD's knowledge could allow to adapt patient care. OBJECTIVE: This retrospective study describes the MOD that occurred during long-term follow-up in CA patients in light-chain (AL), transthyretin hereditary (ATTRv) or wild-type (ATTRwt). MATERIAL AND METHODS: Patients referred to and cared for, at the French referral centre for CA, Henri Mondor Hospital, Créteil between 2010 and 2016 were included. Clinical information surrounding patient deaths were investigated and centrally evaluated by two blinded clinical committees which classified MOD as cardiovascular, non-cardiovascular or unknown and sub-classified it depending on its subtype. RESULTS: From the 566 patients included, 187 had AL, 206 ATTRv and 173 ATTRwt. During the 864 patient-year follow-up, 160 (28%) deaths occurred, with median survival time of 17.3 months (interquartile range 5.1-35.4). The most frequent MOD was cardiovascular (64%) of which worsening heart failure occurred most frequently and for which, 69% were of AL subtype, 79% ATTRv and 76% ATTRwt. Sudden death also occurred more frequently in AL subtype accounting for 29% of AL deaths. Non-cardiovascular MOD occurred in 26% of patients overall. Among these, infection was the most common non-cardiovascular MOD in any type of CA (80%). CONCLUSIONS: Mortality is high during natural course of CA and differs between subtypes. The main MOD were worsening heart failure, sudden death and infection, opening room to optimise management.

Breast arterial calcifications on mammography: a new marker of cardiovascular risk in asymptomatic middle age women?

OBJECTIVES: To determine whether breast arterial calcification (BAC) detected on mammography can predict the presence of coronary artery calcification (CAC) on CT in women. METHODS: Women explored with both mammography and thoracic CT from 2009 to 2018 were retrospectively included. Women were separated in 3 categories (no BAC, few BAC, and marked BAC) using a specific 12-point scale. Similar scale was used to evaluate the amount of CAC on CT. The mean sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and diagnostic accuracy of BAC for the detection of CAC were calculated. Statistical significance was assessed with Pearson's chi-squared test and Fisher's exact test as appropriate. RESULTS: A total of 507 women (mean age: 62 years ± 16) were included. Patients with high amount of BAC were older (72 ± 11 vs. 59 ± 15 years old; p < .0001), were more frequently hypertensive (66% vs. 31%; p < .0001), and had more frequently renal failure (21% vs. 6%; p < .0003) than patients without BAC. The sensitivity, specificity, PPV, NPV, and diagnostic accuracy of BAC for the detection of women with marked CAC were 53.1%, 87.6%, 55.0%, 86.7%, and 79.9%, respectively. The highest diagnostic accuracy was obtained in patients under 60 years: 84.2% for detection of CAC and 93.2% for detection of women with marked CAC. CONCLUSION: The presence of BAC on mammography was linked to the presence of CAC and may be used as a cardiovascular marker in patient less than 60 years. KEY POINTS: • The diagnostic accuracy of breast arterial calcification (BAC) to detect the presence of coronary artery calcification (CAC) was 70.4% and reached to 79.9% to detect women with high amount of CAC. • Highest diagnostic accuracy of BAC to detect CAC (93.2%) was noticed in women under 60 years. • The presence of BAC on mammography may be used as a cardiovascular risk marker in women, especially under 60 years.

Abdominal imaging in ICU patients with viral pneumonia: Are findings in COVID-19 patients really different from those observed with non-SARS-CoV-2 viral pneumonia?

Purpose: To evaluate and compare the prevalence and type of abdominal involvements identified on CT scans in COVID-19 critically ill patients to those observed in critically ill patients with non-SARS-CoV-2 viral pneumonia. Methods: Monocentric IRB approved retrospective study comparing all abdominal CT scans performed for patients admitted in the ICU with COVID-19 and those performed in a historical cohort of ICU patients with non-SARS-CoV-2 viral pneumonia. For each patient, gallbladder abnormality, acute pancreatitis signs, acute adrenal infarction, renal infarcts, bowel wall thickening and CT scan signs of bowel ischemia were assessed. Results were then compared between critically ill COVID-19 and non-COVID-19 patients (Chi-2 or Fisher exact tests for categorical data and Student t-test or Mann-Whitney U test for continuous data as appropriate). Results: Ninety-nine COVID-19 patients and 45 non-COVID-19 patients were included.No difference was found between the rate of abnormal findings comparing COVID-19 patients and patients with other viral pneumonia (63/99 [64%] vs 27/45 [61%], p=0.94). Acute pancreatitis signs were more commonly seen in COVID-19 patients but without statistically difference between groups (14/99 [14%] vs 3/45 [6.7%], p=0.31). Bowel wall thickening was slightly more commonly seen in patients with other viral pneumonia (18/99 [18%] vs 11/45 [24%], p=0.52), however ischemic features were observed in higher rate in the COVID-19 group, although without reaching statistically significant differences (7/99 [7.1%] vs 2/45 [4.4%], p=0.75). Conclusion: The rate and severity of abdominal involvement demonstrated by CT in ICU patients hospitalized for COVID-19 although high were not different to that observed in patients with other severe viral pneumoniae.

Assessing Cardiac Amyloidosis Subtypes by Unsupervised Phenotype Clustering Analysis.

BACKGROUND: Cardiac amyloidosis (CA) is a set of amyloid diseases with usually predominant cardiac symptoms, including light-chain amyloidosis (AL), hereditary variant transthyretin amyloidosis (ATTRv), and wild-type transthyretin amyloidosis (ATTRwt). CA are characterized by high heterogeneity in phenotypes leading to diagnosis delay and worsened outcomes. OBJECTIVES: The authors used clustering analysis to identify typical clinical profiles in a large population of patients with suspected CA. METHODS: Data were collected from the French Referral Center for Cardiac Amyloidosis database (Hôpital Henri Mondor, Créteil), including 1,394 patients with suspected CA between 2010 and 2018: 345 (25%) had a diagnosis of AL, 263 (19%) ATTRv, 402 (29%) ATTRwt, and 384 (28%) no amyloidosis. Based on comprehensive clinicobiological phenotyping, unsupervised clustering analyses were performed by artificial neural network-based self-organizing maps to identify patient profiles (clusters) with similar characteristics, independent of the final diagnosis and prognosis. RESULTS: Mean age and left ventricular ejection fraction were 72 ± 13 years and 52% ± 13%, respectively. The authors identified 7 clusters of patients with contrasting profiles and prognosis. AL patients were distinctively located within a typical cluster; ATTRv patients were distributed across 4 clusters with varying clinical presentations, 1 of which overlapped with patients without amyloidosis; interestingly, ATTRwt patients spread across 3 distinct clusters with contrasting risk factors, biological profiles, and prognosis. CONCLUSIONS: Clustering analysis identified 7 clinical profiles with varying characteristics, prognosis, and associations with diagnosis. Especially in patients with ATTRwt, these results suggest key areas to improve amyloidosis diagnosis and stratify prognosis depending on associated risk factors.

Echocardiographic Patterns of Left Ventricular Diastolic Function in Cardiac Amyloidosis: An Updated Evaluation.

AIMS: Multimodal imaging has allowed cardiac amyloidosis (CA) to be increasingly recognised as a treatable cause of heart failure with preserved ejection fraction, but its prognosis remains poor due to late diagnosis. To assess the left ventricular diastolic function (LVDF) patterns in a large contemporary CA cohort according to the current recommendations and to identify their determinants. METHODS AND RESULTS: We conducted a monocentric, observational study on a cohort of CA patients from a tertiary CA referral centre. Diastolic function was analysed using standard echocardiography and clinical, laboratory and survival parameters were collected. Four hundred and sixty-four patients with one of the three main type of CA were included: 41% had grade III diastolic dysfunction (restrictive mitral pattern), 25% had grade II diastolic dysfunction, and 25% had grade I diastolic dysfunction; 9% were unclassified. No difference was found between the main CA types. After multivariate analyses, grades II and III were independently associated with dyspnoea, elevated NT-proBNP, cardiac infiltration and systolic dysfunction (global longitudinal strain). Grade I patients had a better prognosis. CONCLUSIONS: All LVDF patterns can be observed in CA. One quarter of CA patients have grade I LVDF, reflecting the emergence of earlier stage-related phenotypes with a better prognosis.

Should We Quantify Valvular Calcifications on Cardiac CT in Patients with Infective Endocarditis?

BACKGROUND: Evaluate the impact of valvular calcifications measured on cardiac computed tomography (CCT) in patients with infective endocarditis (IE). METHODS: Seventy patients with native IE (36 aortic IE, 31 mitral IE, 3 bivalvular IE) were included and explored with CCT between January 2016 and April 2018. Mitral and aortic valvular calcium score (VCS) were measured on unenhanced calcium scoring images, and correlated with clinical, surgical data, and 1-year death rate. RESULTS: VCS of patients with mitral IE and no peripheral embolism was higher than those with peripheral embolism (868 (25-1725) vs. 6 (0-95), p < 0.05). Patients with high calcified mitral IE (mitral VCS > 100; n = 15) had a lower rate of surgery (40.0% vs.78.9%; p = 0.03) and a higher 1-year-death risk (53.3% vs. 10.5%, p = 0.04; OR = 8.5 (2.75-16.40) than patients with low mitral VCS (n = 19). Patients with aortic IE and high aortic calcifications (aortic VCS > 100; n = 18) present more frequently atypical bacteria on blood cultures (33.3% vs. 4.8%; p = 0.03) than patients with low aortic VCS (n = 21). CONCLUSION: The amount of valvular calcifications on CT was associated with embolism risk, rate of surgery and 1-year risk of death in patients with mitral IE, and germ's type in aortic IE raising the question of their systematic quantification in native IE.

History of extracardiac/cardiac events in cardiac amyloidosis: prevalence and time from initial onset to diagnosis.

AIMS: Cardiac amyloidosis (CA) has a poor prognosis which is aggravated by diagnostic delay. Amyloidosis extracardiac and cardiac events (AECE and ACE) may help improve CA diagnosis and typing. The aim of this study was to compare AECE and ACE between different CA types and assess their relationship with survival. METHODS AND RESULTS: This retrospective cohort study conducted in France from June 2008 to May 2019, at the Henry Mondor Hospital. This cohort included 983 patients with CA. Mean age at inclusion was 73.1 ± 11.4 years, 726 (75.1%) were male and the mean body mass index was 24.5 ± 4.1 kg/m2 . Among them, 321 had immunoglobulin light chain (AL) amyloidosis, 434 had wild-type transthyretin (ATTRwt), and 212 had hereditary transthyretin (ATTRv). The first AECE and/or ACE occurred at a mean age of 63 ± 11 years for AL and ATTRv, and 70 ± 12 years for ATTRwt (P < 0.01). The median (Q1-Q3) delay between declaration of the first events and diagnosis varied from 11.1 (5.9; 34.8) months for AL to 92.2 (39.0; 174.7) months for ATTRwt (P < 0.01). The nature of the onset of AECE or ACE varied based on amyloidosis type, heart failure symptoms for AL (26%) and integumentary symptoms for ATTRv with cardiologic or mixed phenotype (39%) and ATTRwt (42%). In AL and ATTRwt, a short delay between the onset of the first AECE or ACE and diagnosis was associated with reduced survival rate (log-rank test P-value <0.01). CONCLUSIONS: This study highlights the impact of amyloidosis type and evolution on diagnostic delay and on prognosis. Physicians must be aware and vigilant in front of extracardiac and cardiac events to considerably improve early diagnosis of amyloidosis.

Cardiovascular phenotypes predict clinical outcomes in sickle cell disease: An echocardiography-based cluster analysis.

This study sought to link cardiac phenotypes in homozygous Sickle Cell Disease (SCD) patients with clinical profiles and outcomes using cluster analysis. We analyzed data of 379 patients included in the French Etendard Cohort. A cluster analyses was performed based on echocardiographic variables, and the association between clusters, clinical profiles and outcomes was assessed. Three clusters were identified. Cluster 1 (n = 123) patients had the lowest cardiac output, mild left cardiac cavities remodeling, mild diastolic dysfunction, and higher tricuspid regurgitation velocity (TRV). They were predominantly female and displayed the most altered functional limitation. Cluster 2 (n = 102) patients had the highest cardiac output and the most remodeled cardiac cavities. Diastolic function and TRV were similar to cluster 1. These patients had a higher blood pressure and a severe hemolytic anemia. Cluster 3 (n = 154) patients had mild left cardiac cavities remodeling, normal diastolic function and lowest TRV values. They were younger with the highest hemoglobin value. Right heart catheterization was performed in 94 patients. Cluster 1 (n = 33) included the majority of pre-capillary PH whilst cluster 2 (n = 34) included post-capillary PH. No PH was found in cluster 3 (n = 27). After a follow-up of 11.4 ± 2 years, death occurred in 41 patients (11%). Cluster 2 patients had the worst prognosis with a 19% mortality rate versus 12% in cluster 1 and 5% in cluster 3 (p log-rank = 0.003). Cluster analysis of echocardiography variables identified three hemodynamic and clinical phenotypes among SCD patients, each predicting a different prognosis.

Study of Thoracic CT in COVID-19: The STOIC Project.

Background There are conflicting data regarding the diagnostic performance of chest CT for COVID-19 pneumonia. Disease extent at CT has been reported to influence prognosis. Purpose To create a large publicly available data set and assess the diagnostic and prognostic value of CT in COVID-19 pneumonia. Materials and Methods This multicenter, observational, retrospective cohort study involved 20 French university hospitals. Eligible patients presented at the emergency departments of the hospitals involved between March 1 and April 30th, 2020, and underwent both thoracic CT and reverse transcription-polymerase chain reaction (RT-PCR) testing for suspected COVID-19 pneumonia. CT images were read blinded to initial reports, RT-PCR, demographic characteristics, clinical symptoms, and outcome. Readers classified CT scans as either positive or negative for COVID-19 based on criteria published by the French Society of Radiology. Multivariable logistic regression was used to develop a model predicting severe outcome (intubation or death) at 1-month follow-up in patients positive for both RT-PCR and CT, using clinical and radiologic features. Results Among 10 930 patients screened for eligibility, 10 735 (median age, 65 years; interquartile range, 51-77 years; 6147 men) were included and 6448 (60%) had a positive RT-PCR result. With RT-PCR as reference, the sensitivity and specificity of CT were 80.2% (95% CI: 79.3, 81.2) and 79.7% (95% CI: 78.5, 80.9), respectively, with strong agreement between junior and senior radiologists (Gwet AC1 coefficient, 0.79). Of all the variables analyzed, the extent of pneumonia at CT (odds ratio, 3.25; 95% CI: 2.71, 3.89) was the best predictor of severe outcome at 1 month. A score based solely on clinical variables predicted a severe outcome with an area under the curve of 0.64 (95% CI: 0.62, 0.66), improving to 0.69 (95% CI: 0.6, 0.71) when it also included the extent of pneumonia and coronary calcium score at CT. Conclusion Using predefined criteria, CT reading is not influenced by reader's experience and helps predict the outcome at 1 month. ClinicalTrials.gov identifier: NCT04355507 Published under a CC BY 4.0 license. Online supplemental material is available for this article. See also the editorial by Rubin in this issue.

The use of cardiac computed tomography angiography in the assessment of percutaneous left atrial appendage closure - Review and experts recommendations endorsed by the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle.

Atrial fibrillation is the most common cause of arrhythmia which is responsible for over 15% of ischemic strokes, most of these being secondary to migration of a left atrial appendage (LAA) thrombus. In patient with contraindication to anticoagulant therapy, percutaneous closure system placement may be indicated. Cardiac computed tomography (CT) angiography plays a central role in the initial assessment as well as in the follow-up. The purpose of the pre-implantation cardiac CT angiography is to evaluate the anatomy of the LAA in order to select the most suitable prosthesis and check for any contraindication to device implantation. Image analysis is divided into four steps that include analysis of the approach; search for a thrombus in the LAA; investigation of the anatomy of the LAA (morphology of the LAA, dimensions of the LAA and choice of device) and cardiac and thoracic assessments. Follow-up involves CT examination to check for correct placement of the device and to detect any complications. On the basis of the results of currently available published research, a panel of experts has issued recommendations regarding cardiac CT angiography prior to percutaneous LAA closure device placement, which were further endorsed by the Société française d'imagerie cardiaque et vasculaire diagnostique et interventionnelle (SFICV).

Diagnostic Value of Extracellular Volume Quantification and Myocardial Perfusion Analysis at CT in Cardiac Amyloidosis.

Background CT can provide information regarding myocardial perfusion and expansion of the extracellular space, which is relevant to patients with cardiac amyloidosis (CA). Purpose To evaluate the role of CT in the diagnosis and prognosis of CA. Materials and Methods In this prospective study (Commission National de l'Informatique et des Libertés registration no. 1431858), participants with CA, participants with nonamyloid cardiac hypertrophy (NACH), and participants without hypertrophy were included between April 2017 and December 2018. The confirmed diagnosis of CA was determined according to established criteria (ie, proven with positive bone scintigraphy or endomyocardial biopsy). All participants were imaged with dynamic CT perfusion imaging at whole-heart cardiac CT. Extracellular volume measured at CT and myocardial perfusion parameters calculated on CT perfusion maps were compared among different participant groups. Differences between continuous data were tested using the unpaired t test, Mann-Whitney rank-sum test, or the Kruskal-Wallis test. Results A total of 84 participants with CA, 43 participants with NACH, and 33 participants without hypertrophy were included. Participants with CA exhibited a higher value of extracellular volume measured at CT (mean, 54.7% ± 9.7 [standard deviation]) than participants with NACH (mean, 34.6% ± 9.1; P < .001) and participants without hypertrophy (mean, 35.9% ± 9.9; P = .001). Mean myocardial blood volume and mean myocardial blood flow were lower in participants with CA (mean myocardial blood volume: 4.05 mL/100 g of myocardium ± 0.80; mean myocardial blood flow: 73.2 mL/100 g of myocardium per minute ± 25.7) compared to participants with NACH (mean myocardial blood volume: 5.38 mL/100 g of myocardium ± 1.20, P < .001; mean myocardial blood flow: 89.6 mL/100 g of myocardium per minute ± 31.3, P = .007) and participants without hypertrophy (mean myocardial blood volume: 5.68 mL/100 g of myocardium ± 1.05; mean myocardial blood flow: 106.3 mL/100 g of myocardium per minute ± 29.8; P < .001 for both). Extracellular volume measured at CT (hazard ratio >0.56 vs ≤0.56 = 4.2 [95% CI: 1.4, 11.8]), mean slope (hazard ratio ≤3.0 sec-1 vs >3.0 sec-1 = 0.2 [95% CI: 0.1, 0.8]), and time to peak (hazard ratio >20 seconds vs ≤20 seconds = 11.6 [95% CI: 1.3, 101.6]) were predictive of mortality in participants with CA. Conclusion Participants with cardiac amyloidosis exhibited an increase in extracellular volume at CT and abnormal CT perfusion parameters. Extracellular volume and several perfusion parameters were predictive of mortality. © RSNA, 2021 Online supplemental material is available for this article. See also the editorial by Zimmerman in this issue.

18F-sodium fluoride PET/MRI myocardial imaging in patients with suspected cardiac amyloidosis.

BACKGROUND: We evaluated the diagnostic performance of 18F-NaF PET/MRI in patients with suspected cardiac amyloidosis (CA). METHODS: Twenty-seven consecutive patients underwent myocardial PET 1 hour after injection of 4 MBq/kg 18F-NaF with simultaneous MRI including cine-MRI, T1 and T2 mapping, first-pass and late gadolinium enhancement (LGE). 18F-NaF uptake was measured visually and semi-quantitatively by calculating myocardium-to-blood pool (M/B) ratios. CA was confirmed histologically. RESULTS: Transthyretin (TTR)-CA was diagnosed in 16 patients, light-chain (AL)-CA in 7, and no-CA in 4. Visual interpretation of 18F-NaF images revealed a relative increase in myocardial uptake in only 3 patients, all with TTR CA, and a relative decrease in 13, including 7 AL CA, 3 no-CA, and 3 TTR CA. M/B ratios were significantly higher in TTR CA (1.00 ± 0.12) than in AL CA (0.81 ± 0.06, P = 0.001) or in no-CA (0.73 ± 0.16, P = 0.006). The optimal M/B cut-off to distinguish TTR CA from AL CA was ≥ 0.90 (Fischer, P = 0.0005). By comparison, classification of patients using 99mTc-HMDP heart-to-mediastinum ratios with the previously published cut-off ≥ 1.21 reached higher significance (P < 0.0001). Among MRI parameters, myocardial T1, LGE score, and extracellular volume were higher in CA than in no-CA patients, 1409 ± 76 vs 1278 ± 35 ms (P = 0.004), 10.35 ± 5.30 vs 3.50 ± 3.42 (P = 0.03), and 46 ± 10 vs 33 ± 8 % (P = 0.01), respectively. CONCLUSION: 18F-NaF PET/MRI shows good diagnostic performance when semi-quantification is used. However, contrast is low and visual interpretation may be challenging in routine. PET/MRI could constitute a one-stop-shop evaluation of amyloid load and cardiac function in patients needing rapid work-up.

Natural history and impact of treatment with tafamidis on major cardiovascular outcome-free survival time in a cohort of patients with transthyretin amyloidosis.

AIMS: Hereditary (ATTRv) and wild-type (ATTRwt) transthyretin amyloidosis are severe and fatal systemic diseases, characterised by amyloid fibrillar accumulation principally in the heart or peripheral nerves (or both). Since 2012, tafamidis has been used in France to treat patients with ATTRv with neuropathy (alone or combined with cardiomyopathy). Recently, the Phase III ATTR-ACT trial showed that tafamidis decreased the relative risk of mortality in ATTR amyloidosis with cardiomyopathy. The aims of this study were to assess the clinical characteristics of ATTR amyloidosis in a real-life population in comparison to the population included in the ATTR-ACT trial and to assess the impact of tafamidis treatment on major cardiovascular outcome (MCO)-free survival time without cardiac decompensation, heart transplant, or death. METHODS AND RESULTS: From June 2008 to November 2018, 648 patients with ATTR amyloidosis (423 ATTRwt and 225 ATTRv) consecutively referred to the French Referral Center for cardiac amyloidosis were included. A total of 467 (72%) patients matched the inclusion criteria of the ATTR-ACT trial. For the 631 patients with cardiomyopathy, tafamidis treatment was associated with a longer median MCO-free survival time (n = 98): 1565 (1010-2400) days vs. 771 (686-895) days without treatment (log-rank P < 0.001). This association was confirmed after considering confounding factors (age at inclusion, N-terminal pro-B-type natriuretic peptide and amyloidosis type) with a propensity score (hazard ratio 0.546; P = 0.0132). CONCLUSION: In a large cohort of ATTRwt and ATTRv patients, representative of the inclusion criteria of the ATTR-ACT trial, the present results show an association between tafamidis treatment and a lower occurrence of cardiovascular outcomes in a real-life population.