Dania Mohty1, Vincent Petitalot2, Julien Magne2, Bahaa M Fadel3, Cyrille Boulogne2, Dounia Rouabhia4, Chahrazed El Hamel5, David Lavergne6, Thibaud Damy7, Victor Aboyans2, Arnaud Jaccard6. 1. Department of Cardiology, Dupuytren University Hospital, Limoges, France; Heart Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia. Electronic address: dania.mohty@chu-limoges.fr. 2. Department of Cardiology, Dupuytren University Hospital, Limoges, France. 3. Heart Center, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia. 4. Laval University, Medical Department, Quebec, Canada. 5. Department of Nephrology, Dupuytren University Hospital, Limoges, France. 6. Department of Hematology, National Reference Center of Light-chain Systemic Amyloidosis, Dupuytren University Hospital, Limoges, France. 7. Department of Cardiology, AP-HP, Henri Mondor University Hospital, Creteil, France.
Abstract
BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography. METHODS: A total of 77 patients (67±10 years, 60% men) with confirmed AL and 39 healthy controls were included. All standard 2D echocardiographic and 3D-LA parameters were obtained. RESULTS: Fourteen patients (18%) were in Mayo Clinic (MC) stage I, 30 (39%) in stage II, and 33 (43%) in stage III at initial evaluation. There was no significant difference among the MC stages groups in terms of age, gender, or cardiovascular risk factors. As compared to patients in MC II and MC I, those in MC III had significantly larger indexed 3D-LA volumes (MCIII: 46±15mL/m2, MC II: 38±12mL/m2, and MC I: 23±9mL/m2, p<0.0001), lower 3D-LA total emptying fraction (3D-tLAEF) (21±13% vs. 31±15% vs. 43±7%, respectively, p<0.0001), and worse 3D peak atrial longitudinal strain (3D-PALS) (11±9% vs. 18±13% vs. 20±7%, respectively, p=0.007). Two-year survival was significantly lower in patients with 3D-tLAEF <+34% (p=0.003) and in those with 3D-PALS <+14% (p=0.034). Both parameters provided incremental prognostic value over maximal LA volume in multivariate analysis. CONCLUSION: Functional LA parameters are progressively altered in AL patients according to the MC stage. A decrease in 3D-PALS is associated with worse outcome, independently of LA volume.
BACKGROUND: Systemic light chain amyloidosis (AL) is characterized by the extracellular deposition of amyloid fibrils. Transthoracic echocardiography is the modality of choice to assess cardiac function in patients with AL. Whereas left ventricular (LV) function has been well studied in this patient population, data regarding the value of left atrial (LA) function in AL patients are lacking. In this study, we aim to examine the impact of LA volumes and function on survival in AL patients as assessed by real-time 3D echocardiography. METHODS: A total of 77 patients (67±10 years, 60% men) with confirmed AL and 39 healthy controls were included. All standard 2D echocardiographic and 3D-LA parameters were obtained. RESULTS: Fourteen patients (18%) were in Mayo Clinic (MC) stage I, 30 (39%) in stage II, and 33 (43%) in stage III at initial evaluation. There was no significant difference among the MC stages groups in terms of age, gender, or cardiovascular risk factors. As compared to patients in MC II and MC I, those in MC III had significantly larger indexed 3D-LA volumes (MCIII: 46±15mL/m2, MC II: 38±12mL/m2, and MC I: 23±9mL/m2, p<0.0001), lower 3D-LA total emptying fraction (3D-tLAEF) (21±13% vs. 31±15% vs. 43±7%, respectively, p<0.0001), and worse 3D peak atrial longitudinal strain (3D-PALS) (11±9% vs. 18±13% vs. 20±7%, respectively, p=0.007). Two-year survival was significantly lower in patients with 3D-tLAEF <+34% (p=0.003) and in those with 3D-PALS <+14% (p=0.034). Both parameters provided incremental prognostic value over maximal LA volume in multivariate analysis. CONCLUSION: Functional LA parameters are progressively altered in AL patients according to the MC stage. A decrease in 3D-PALS is associated with worse outcome, independently of LA volume.
Authors: Sharmila Dorbala; Yukio Ando; Sabahat Bokhari; Angela Dispenzieri; Rodney H Falk; Victor A Ferrari; Marianna Fontana; Olivier Gheysens; Julian D Gillmore; Andor W J M Glaudemans; Mazen A Hanna; Bouke P C Hazenberg; Arnt V Kristen; Raymond Y Kwong; Mathew S Maurer; Giampaolo Merlini; Edward J Miller; James C Moon; Venkatesh L Murthy; C Cristina Quarta; Claudio Rapezzi; Frederick L Ruberg; Sanjiv J Shah; Riemer H J A Slart; Hein J Verberne; Jamieson M Bourque Journal: J Nucl Cardiol Date: 2019-12 Impact factor: 5.952
Authors: Francesco Bandera; Anita Mollo; Matteo Frigelli; Giulia Guglielmi; Nicoletta Ventrella; Maria Concetta Pastore; Matteo Cameli; Marco Guazzi Journal: Front Cardiovasc Med Date: 2022-01-13
Authors: Tobias Dittrich; Axel Benner; Christoph Kimmich; Fabian Aus dem Siepen; Kaya Veelken; Arnt V Kristen; Tilmann Bochtler; Hugo A Katus; Carsten Müller-Tidow; Ute Hegenbart; Stefan O Schönland Journal: Haematologica Date: 2019-01-17 Impact factor: 9.941