Dania Mohty1,2,3, Sarah Pradel4, Julien Magne4,5, Bahaa Fadel6, Cyrille Boulogne4, Vincent Petitalot4, Safaa Raboukhi4, Nicole Darodes4, Thibaud Damy7, Victor Aboyans4,5, Arnaud Jaccard8. 1. Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France. dania.mohty@chu-limoges.fr. 2. Faculté de médecine de Limoges, INSERM 1094, 2, Rue Marcland, 87000, Limoges, France. dania.mohty@chu-limoges.fr. 3. Department of Hematology, French National Reference Center of AL Amyloidosis Pôle «Onco-Hématologie», CHU Dupuytren, Limoges, France. dania.mohty@chu-limoges.fr. 4. Department of Cardiology, CHU Dupuytren, Service Cardiologie, 2 Avenue Martin Luther-King, 87042, Limoges, France. 5. Faculté de médecine de Limoges, INSERM 1094, 2, Rue Marcland, 87000, Limoges, France. 6. King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia. 7. Department of Cardiology, AP-HP, Henri Mondor University-Hospital, Créteil, France. 8. Department of Hematology, French National Reference Center of AL Amyloidosis Pôle «Onco-Hématologie», CHU Dupuytren, Limoges, France.
Abstract
BACKGROUND: Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL. METHODS AND RESULTS: Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3 mm) was assessed in all included patients. Overall, 42% had LVT at the time of diagnosis. Compared to patients without LVT, those with LVT were older and had a more advanced NYHA functional class (63% in patients with NYHA III-IV vs. 33% in NYHA I-II, p < 0.001). They also had higher left ventricular (LV) wall thickness and mass, larger left atrium, higher mitral annulus E/E' ratio and systolic pulmonary artery pressures, and lower LV ejection fraction (all p < 0.05). Patients with more advanced Mayo Clinic stage had a higher incidence of LVT: 58% in stage III vs. 45% in stage II and 5% in stage I (p < 0.001). During a median follow-up of 2 years, 79 deaths occurred. The presence of LVT was significantly associated with reduced 5-year survival (32 ± 7 vs. 64 ± 6%). In multivariate analysis, after adjusting for age, gender, NYHA functional class, and LV ejection fraction, LVT remained significantly associated with higher all-cause mortality (hazard ratio 1.90, 95% CI 1.10-3.34, p = 0.02). CONCLUSION: Left heart valve thickening is common in patients with AL and is associated with worse functional class, LV systolic and diastolic function, and more advanced stage of the disease. In addition, LVT appears to be a powerful marker of all-cause mortality.
BACKGROUND:Left heart valve thickening (LVT) was described in patients with light-chain amyloidosis (AL). This phenomenon reflects likely infiltration of the valve by amyloid proteins. However, the prevalence of LVT and its prognostic value have not been investigated in patients with AL. METHODS AND RESULTS: Comprehensive transthoracic echocardiography was performed at baseline in 150 patients [median age 68 (33-87) years; 59% male] with confirmed AL. The presence of abnormal mitral and/or aortic valve thickening (>3 mm) was assessed in all included patients. Overall, 42% had LVT at the time of diagnosis. Compared to patients without LVT, those with LVT were older and had a more advanced NYHA functional class (63% in patients with NYHA III-IV vs. 33% in NYHA I-II, p < 0.001). They also had higher left ventricular (LV) wall thickness and mass, larger left atrium, higher mitral annulus E/E' ratio and systolic pulmonary artery pressures, and lower LV ejection fraction (all p < 0.05). Patients with more advanced Mayo Clinic stage had a higher incidence of LVT: 58% in stage III vs. 45% in stage II and 5% in stage I (p < 0.001). During a median follow-up of 2 years, 79 deaths occurred. The presence of LVT was significantly associated with reduced 5-year survival (32 ± 7 vs. 64 ± 6%). In multivariate analysis, after adjusting for age, gender, NYHA functional class, and LV ejection fraction, LVT remained significantly associated with higher all-cause mortality (hazard ratio 1.90, 95% CI 1.10-3.34, p = 0.02). CONCLUSION:Left heart valve thickening is common in patients with AL and is associated with worse functional class, LV systolic and diastolic function, and more advanced stage of the disease. In addition, LVT appears to be a powerful marker of all-cause mortality.
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