Xuezhu Wang MD , Kaini Shen MD , Yuke Zhang BS , Yajuan Gao MD , Bowei Liu BS , Yubo Guo BS , Chao Ren MM , Zhenghai Huang RA , Xiao Li MD , Long Chang MD , Haiyan Ding PhD , Hui Zhang PhD , Zhuang Tian MD , Marcus Hacker MD , Shuyang Zhang MD , Yining Wang MD , Jian Li MD , Xiang Li PhD , Li Huo MD
{"title":"Molecular Stratification of Light-Chain Cardiac Amyloidosis With 18F-Florbetapir and 68Ga-FAPI-04 for Enhanced Prognostic Precision","authors":"Xuezhu Wang MD , Kaini Shen MD , Yuke Zhang BS , Yajuan Gao MD , Bowei Liu BS , Yubo Guo BS , Chao Ren MM , Zhenghai Huang RA , Xiao Li MD , Long Chang MD , Haiyan Ding PhD , Hui Zhang PhD , Zhuang Tian MD , Marcus Hacker MD , Shuyang Zhang MD , Yining Wang MD , Jian Li MD , Xiang Li PhD , Li Huo MD","doi":"10.1016/j.jcmg.2024.10.001","DOIUrl":"10.1016/j.jcmg.2024.10.001","url":null,"abstract":"<div><h3>Background</h3><div>Cardiac involvement in amyloid light chain (AL) amyloidosis significantly influences prognosis, necessitating timely diagnosis and meticulous risk stratification.</div></div><div><h3>Objectives</h3><div>This prospective study aimed to delineate the molecular phenotypes of AL–cardiac amyloidosis (CA) by characterizing fibro-amyloid deposition using <sup>18</sup>F-florbetapir and gallium-68-labeled fibroblast activation protein inhibitor (<sup>68</sup>Ga-FAPI)-04 positron emission tomography (PET)/computed tomography (CT) imaging. The authors also proposed a novel molecular stratification methodology for prognosis.</div></div><div><h3>Methods</h3><div>Patients with confirmed AL-CA underwent echocardiography and <sup>18</sup>F-florbetapir and <sup>68</sup>Ga-FAPI-04 PET/CT imaging. Cardiac amyloid burden was quantified as <sup>18</sup>F-florbetapir cardiac amyloid volume and total cardiac amyloid. Meanwhile, cardiac fibroblast activation protein (FAP) was quantified as <sup>68</sup>Ga-FAPI-04 cardiac fibroblast activation protein volume (CFV) and total cardiac fibroblast activation protein (TCF). PET/CT metrics were calculated in correlation to clinical and echocardiographic markers and their association with overall survival (OS) evaluated.</div></div><div><h3>Results</h3><div>Among the 38 patients enrolled (median age: 58 years; 76.3% male), all patients exhibited amyloid deposition, and 86.8% (33 of 38) patients exhibited cardiac fibroblast activation. Cardiac amyloid burden was correlated with Mayo stage and several echocardiography metrics (<em>P <</em> 0.05). In addition, there was a correlation between CFV and N-terminal pro–B-type natriuretic peptide level (<em>P <</em> 0.05). Thirteen deaths occurred over a median follow-up of 24.8 months. Higher CFV and TCF were associated with shortened OS, particularly in Mayo stage III. In multivariable analysis, higher TCF was a primary determinant for shortened OS.</div></div><div><h3>Conclusions</h3><div>The study underscores that higher TCF on <sup>68</sup>Ga-FAPI-04 PET/CT imaging might be a correlated factor of worse clinical outcome in newly diagnosed AL-CA, and this metric seems to be a molecular imaging tool complementary to <sup>18</sup>F-florbetapir imaging. The combination might offer a holistic understanding of molecular attributes, assisting in clinical decision-making.</div></div>","PeriodicalId":14767,"journal":{"name":"JACC. Cardiovascular imaging","volume":"18 3","pages":"Pages 323-336"},"PeriodicalIF":12.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142965085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Tara Shrout Allen MD, MSc, Mikaila Reyes MAS, Jonathan M. Kermanshahchi BA, Julie O. Denenberg MA, Joseph Yeboah MD, Matthew A. Allison MD, MPH, Michael H. Criqui MD, MPH, Harpreet S. Bhatia MD, MAS
{"title":"Coronary Artery Calcium Distribution and Cardiovascular Risk","authors":"Tara Shrout Allen MD, MSc, Mikaila Reyes MAS, Jonathan M. Kermanshahchi BA, Julie O. Denenberg MA, Joseph Yeboah MD, Matthew A. Allison MD, MPH, Michael H. Criqui MD, MPH, Harpreet S. Bhatia MD, MAS","doi":"10.1016/j.jcmg.2024.10.005","DOIUrl":"10.1016/j.jcmg.2024.10.005","url":null,"abstract":"","PeriodicalId":14767,"journal":{"name":"JACC. Cardiovascular imaging","volume":"18 3","pages":"Pages 392-394"},"PeriodicalIF":12.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142784990","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Milind Y. Desai MD, MBA , Yuichiro Okushi MD , Kathy Wolski MPH , Jeffrey B. Geske MD , Anjali Owens MD , Sara Saberi MD, MS , Andrew Wang MD , Paul C. Cremer MD, MS , Mark Sherrid MD , Neal K. Lakdawala MD , Albree Tower-Rader MD , David Fermin MD , Srihari S. Naidu MD , Kathy L. Lampl MD , Amy J. Sehnert MD , Steven E. Nissen MD , Zoran B. Popovic MD, PhD
{"title":"Mavacamten-Associated Temporal Changes in Left Atrial Function in Obstructive HCM","authors":"Milind Y. Desai MD, MBA , Yuichiro Okushi MD , Kathy Wolski MPH , Jeffrey B. Geske MD , Anjali Owens MD , Sara Saberi MD, MS , Andrew Wang MD , Paul C. Cremer MD, MS , Mark Sherrid MD , Neal K. Lakdawala MD , Albree Tower-Rader MD , David Fermin MD , Srihari S. Naidu MD , Kathy L. Lampl MD , Amy J. Sehnert MD , Steven E. Nissen MD , Zoran B. Popovic MD, PhD","doi":"10.1016/j.jcmg.2024.08.005","DOIUrl":"10.1016/j.jcmg.2024.08.005","url":null,"abstract":"<div><h3>Background</h3><div>In severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HCM), the VALOR-HCM (A Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy) trial showed that mavacamten reduced the eligibility for septal reduction therapy with sustained improvement in left ventricular outflow tract gradients. Mavacamten also resulted in favorable cardiac remodeling, including improvement in biomarkers (eg, N-terminal pro–B-type natriuretic peptide and troponin T). However, the impact of mavacamten on left atrial (LA) function is unknown.</div></div><div><h3>Objectives</h3><div>The aim of this study was to assess serial changes in LA strain measures in patients enrolled in the VALOR-HCM trial.</div></div><div><h3>Methods</h3><div>VALOR-HCM included 112 symptomatic patients with obstructive HCM (mean age 60 years; 51% male). Patients assigned to receive mavacamten at baseline (n = 56) continued therapy for 56 weeks and those assigned to placebo transitioned to mavacamten (n = 52) from week 16 to week 56. Echocardiographic LA strain (reservoir, conduit, and contraction) was measured by using a vendor-neutral postprocessing software.</div></div><div><h3>Results</h3><div>At baseline, the mean LA volume index (LAVI) and LA strain values (conduit, contraction, and reservoir) were 41.3 ± 16.5 mL/m<sup>2</sup>, −11.8% ± 6.5%, −8.7% ± 5.0%, and 20.5% ± 8.7%, respectively (all worse than reported normal). LAVI significantly improved by −5.6 ± 9.7 mL/m<sup>2</sup> from baseline to week 56 (<em>P <</em> 0.001). There was a significant (<em>P <</em> 0.05) improvement in absolute LA strain values from baseline to week 56 (conduit [−1.7% ± 6%], contraction [−1.2% ± 4.5%], and reservoir [2.8% ± 7.7%]). Patients originally receiving placebo had no differences in LA measurements up to week 16. There was no significant improvement in LA strain values (conduit [−0.9% ± 3.8%], contraction [−0.4% ± 3.4%], and reservoir [1.4% ± 6.1%]; all; <em>P =</em> not significant) from baseline to week 56 in patients with history of atrial fibrillation.</div></div><div><h3>Conclusions</h3><div>In VALOR-HCM, mavacamten resulted in an improvement in LAVI and LA strain at week 56, suggesting sustained favorable LA remodeling and improved function, except in the atrial fibrillation subgroup. Whether the advantageous LA remodeling associated with long-term treatment with mavacamten results in a favorable impact on the observed high burden of atrial tachyarrhythmias in HCM remains to be proven. (A Study to Evaluate Mavacamten in Adults With Symptomatic Obstructive Hypertrophic Cardiomyopathy Who Are Eligible for Septal Reduction Therapy [VALOR-HCM]; <span><span>NCT04349072</span><svg><path></path></svg></span>)</div></div>","PeriodicalId":14767,"journal":{"name":"JACC. Cardiovascular imaging","volume":"18 3","pages":"Pages 251-262"},"PeriodicalIF":12.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142144083","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Activated Fibroblast Imaging in AL Amyloid Cardiomyopathy","authors":"Rodney H. Falk MD, Osnat Itzhaki Ben Zadok MD","doi":"10.1016/j.jcmg.2024.12.003","DOIUrl":"10.1016/j.jcmg.2024.12.003","url":null,"abstract":"","PeriodicalId":14767,"journal":{"name":"JACC. Cardiovascular imaging","volume":"18 3","pages":"Pages 337-339"},"PeriodicalIF":12.8,"publicationDate":"2025-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143535144","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":1,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}