1. Prevalence and prognostic impact of left-sided valve thickening in systemic light-chain amyloidosis
- Author
-
Bahaa M. Fadel, Nicole Darodes, Victor Aboyans, Cyrille Boulogne, Thibaud Damy, Julien Magne, Vincent Petitalot, Dania Mohty, Arnaud Jaccard, Sarah Pradel, Safaa Raboukhi, Cardio-vascular diseases, Clinical sciences, Service de cardiologie [CHU Limoges], CHU Limoges, Neuroépidémiologie Tropicale (NET), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Service de Chirurgie Thoracique et Vasculaire - Médecine vasculaire [CHU Limoges], Unité fonctionnelle insuffisance cardiaque, and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est (UPE)
- Subjects
Male ,Comorbidity ,030204 cardiovascular system & hematology ,VALVE ,Ventricular Dysfunction, Left ,0302 clinical medicine ,Prevalence ,echocardiography ,Immunoglobulin Light-chain Amyloidosis ,030212 general & internal medicine ,Aged, 80 and over ,Ejection fraction ,Amyloidosis ,Hazard ratio ,General Medicine ,Organ Size ,Middle Aged ,Prognosis ,3. Good health ,Survival Rate ,medicine.anatomical_structure ,Aortic Valve ,Cardiology ,Mitral Valve ,Female ,Thickening ,France ,Cardiology and Cardiovascular Medicine ,Adult ,medicine.medical_specialty ,Left sided ,Risk Assessment ,Sensitivity and Specificity ,survival ,03 medical and health sciences ,medicine.artery ,Internal medicine ,medicine ,Humans ,Light chains ,In patient ,Heart valve ,Aged ,business.industry ,Reproducibility of Results ,medicine.disease ,Pulmonary artery ,[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie ,business - Abstract
International audience; 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.
- Published
- 2017
- Full Text
- View/download PDF