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Aortic Stenosis and Cardiac Amyloidosis JACC Review Topic of the Week JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY VOL. 74, NO. 21, 2019 ª 2019 BY THE AMERICAN COLLEGE OF CARDIOLOGY FOUNDATION PUBLISHED BY ELSEVIER THE PRESENT AND FUTURE JACC REVIEW TOPIC OF THE WEEK Aortic Stenosis and Cardiac Amyloidosis JACC Review Topic of the Week a,b,c a,d e,f e Julien Ternacle, MD, PHD, Laura Krapf, MD, Dania Mohty, MD, PHD, Julien Magne, PHD, b,c b,c b,c b,c Annabelle Nguyen, MD, Arnault Galat, MD, Romain Gallet, MD, PHD, Emmanuel Teiger, MD, PHD, a a g a Nancy Côté, PHD, Marie-Annick Clavel, PHD, DVM, François Tournoux, MD, Philippe Pibarot, PHD, DVM, b,c Thibaud Damy, MD, PHD ABSTRACT The prevalence of calcific aortic stenosis (AS) and of cardiac amyloidosis (CA) increases with age, and their association is not uncommon in the elderly. The identification of CA is particularly challenging in patients with AS because these 2 conditions share several features. It is estimated that #15% of the AS population and #30% of the subset with low-flow, low-gradient pattern may have CA. In patients with AS, CA is associated with increased risk of heart failure, mortality, and treatment futility with aortic valve replacement. In case of suspicion of CA, it is thus crucial to confirm the diagnosis to guide therapeutic management of AS and eventually implement recently developed pharmacological treatment dedicated to transthyretin amyloidosis. Given the high surgical risk of patients with AS and concomitant CA, trans- catheter aortic valve replacement may be preferred to surgery in these patients. (J Am Coll Cardiol 2019;74:2638–51) © 2019 by the American College of Cardiology Foundation. ortic stenosis (AS) is the most common patient’s life expectancy close to that of the age- A valvular heart disease (VHD), with a preva- and sex-matched general population. lence >4% in octogenarians. The pressure Cardiac amyloidosis (CA) is characterized by overload associated with AS leads to development of extracellular deposit of amyloid fibrils within the left ventricular (LV) concentric hypertrophy, impair- myocardium and other cardiac structures; this con- ment of LV diastolic and systolic function, and ulti- dition afflicts #25% of octogenarians (1,2). CA shares mately, heart failure (HF). In the absence of several common features with AS, but its prognosis treatment, symptomatic severe AS is associated with generally is worse than severe AS alone (3). Recent poor prognosis. However, aortic valve replacement studies suggest that the coexistence of AS and CA is may change the natural history of AS and restore a more common than previously anticipated (4,5). The From the aInstitut Universitaire de Cardiologie et de Pneumologie de Québec/Québec Heart and Lung Institute, Université Laval, Québec City, Québec, Canada; bReferral Center for Cardiac Amyloidosis, Mondor Amyloidosis Network, GRC Amyloid Research Institute and Cardiology Department, APHP Henri Mondor Hospital, Créteil, France; cINSERM Unit U955, Team 8, Paris-Est Creteil University, Val-de-Marne, Créteil, France; dCardiology Department, Centre d’Accueil et de Soins Hospitaliers, Hôpital Max Fourestier, Nanterre, France; eCHU Limoges, Hôpital Dupuytren, Service Cardiologie, and INSERM 1094, Faculté de médecine de Limoges, Limoges, France; fKing Faisal Specialist Hospital & Research Center, Heart Center, Riyadh, Saudi Arabia; and the Listen to this manuscript’s gCardiology Department, Centre Hospitalier de l’Université de Montréal, Montréal, Québec, Canada. Dr. Ternacle has received a audio summary by fellowship grant from AREMCAR. Dr. Clavel has core laboratory contracts with Edwards Lifesciences, for which she receives no Editor-in-Chief direct compensation; and has received a research grant from Medtronic. Dr. Tournoux has received consulting honorarium from Dr. Valentin Fuster on Alnylam and Akcea. Dr. Pibarot holds the Canada Research Chair in Valvular Heart Diseases and a Foundation Scheme Grant JACC.org. (FDN-143225 from Canadian Institutes of Health Research); has received a grant from the Foundation of the Quebec Heart and Lung Institute; has echo core laboratory contracts with Edwards Lifesciences, for which he receives no direct compensation; and has a research contract with Medtronic. Dr. Damy has received consulting honorarium, research grants, and travel and congress funding from Pfizer, Alnylam, Ionis, Akcea, Sanofi, Prothena, and GlaxoSmithKline; and has received consultant honorarium from Neurimmune. All other authors have reported that they have no relationships relevant to the contents of this paper to disclose. Manuscript received August 1, 2019; revised manuscript received September 13, 2019, accepted September 16, 2019. ISSN 0735-1097/$36.00 https://doi.org/10.1016/j.jacc.2019.09.056 JACC VOL. 74, NO. 21, 2019 Ternacle et al. 2639 NOVEMBER 26, 2019:2638– 51 Aortic Stenosis and Cardiac Amyloidosis associated with AS, especially in men age >70 ABBREVIATIONS HIGHLIGHTS years (4,5). Patients with AS rarely have the AND ACRONYMS CA may be present in #15% of patients AL-CA type, which is related to the accumu- AL = light-chain amyloidosis with AS. lation of an amyloidogenic light chain variant AS = aortic stenosis (k-orl-) produced by plasma cell dyscrasia. Clinical and imaging “red flags” for CA AVR Cardiac deposition of amyloid substance = aortic valve should be systematically searched in replacement can infiltrate any cardiovascular structure patients with AS. CA = cardiac amyloidosis (Figure 1). Infiltration of myocardium and hTTR = hereditary valves is frequent and often becomes the “red Transcatheter rather than surgical AVR transthyretin may be preferred in patients with CA. flag” that raises the suspicion of CA (Table 1) LV = left ventricle (14–16). LV myocardial amyloid infiltration Recently developed pharmacological LVEF = left ventricular starts from the base to the apex and results in treatment for transthyretin CA should be ejection fraction increased biventricular wall thickness and instituted as soon as diagnosis is SAVR = surgical aortic valve stiffness, which ultimately leads to hyper- replacement confirmed. trophic restrictive cardiomyopathy with se- TAVR = transcatheter aortic vere impairment of LV diastolic function and valve replacement combination of AS and CA complicates the diagnosis longitudinal systolic function (Figures 1 and 2) TTR = transthyretin and therapeutic management of both conditions. (10). The most prevalent type of HF associ- wtTTR = wild-type Recently, effective pharmacotherapies have been ated with CA is diastolic HF with preserved transthyretin developed to halt and reverse the course of trans- left ventricular ejection fraction (LVEF), but nearly thyretin (TTR) CA (6). There is thus an urgent need to one-third of patients present with reduced ejection standardize and optimize the diagnostic evaluation fraction (17). These features are also common in pa- and therapeutic management of CA in AS, and vice tients with severe AS, which may mask the presence versa (7,8). The aim of this review is to provide an of a concomitant CA. Aortic valve infiltration by am- overview of current knowledge on: 1) the patho- yloid substance may contribute to the initiation and physiology and prevalence of CA in the AS popula- progression of AS (14,18). Infiltration of amyloid tion; 2) the diagnostic and therapeutic management within the atria walls contributes to the high preva- of CA in patients with AS; and 3) the diagnostic and lence of supraventricular arrhythmia and leads therapeutic management of AS in patients with to atrioventricular electromechanical dissociation, concomitant CA. which is associated with increased risk of thrombo- embolic events and HF decompensation (19,20). PATHOPHYSIOLOGY OF CA Microvascular amyloid infiltration contributes to the myocardial dysfunction and ischemia, therefore Amyloidosis corresponds to the extracellular accu- explaining the frequent dissociation between the mulation of amyloid fibrils within otherwise normal absence of epicardial coronary lesion and the elevated organs (Figure 1). The 2 more prevalent proteins troponin levels (21,22). The conduction disorders involved in CA are transthyretin (TTR-CA) and the caused by amyloid infiltration of conduction tissue immunoglobulin light chain (or AL-CA) (9). There are often occurs early in the course of the disease (23). 2 types of systemic TTR-CA: senile or wild-type Pericardial effusion is also a common feature of CA. transthyretin (wtTTR) and hereditary transthyretin (hTTR). Senile or wtTTR-CA is more prevalent in pa- PREVALENCE OF CA tients age >80 years (1) and is related to the deposi- tion of genetically unaltered transthyretin. The The exact prevalence of CA in the general population mechanism of this dysregulation is unknown, but it is as well as in patients with AS is likely underestimated likely more complex and multifaceted than solely the because of the absence of systematic screening, the aging process. Hereditary TTR-CA is a genetic disease complexity of diagnosis confirmation, and the over- with an autosomal dominant pattern and is observed lap with other cardiac disease phenotypes. The in younger patients than those with wtTTR-CA (10). prevalence of CA reported in previous studies for the Among the 120 mutations, some variants are more general population ranges from 5% to 74% (1,2,14,17). prevalent in CA such as the ATTR pVal142Ile mutation This wide interstudy variability is related to differ- also called the Afro-American mutation (11). ences in population selection criteria and diagnostic Depending on the mutation, the main phenotypic
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