Cardiac amyloidosis: an unusual cause of low flow–low gradient aortic stenosis with preserved ejection fraction

Haloui, Fairouz; Salaün, Erwan; Maysou, Laurie-Anne; Dehaene, A.; Habib, Gilbert

doi:10.1093/ehjci/jev342

Cited by 5 publications

(2 citation statements)

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“…Several T1 mapping approaches have been developed to quantify diffuse fibrosis. Extracellular volume fraction (ECV), which corrects for blood pool and the plasma gadolinium volume of distribution, offers the best reproducibility and can predict outcomes as least as strongly as LV ejection fraction [ 94 , 95 ].…”

Section: The Role Of Advanced Imagingmentioning

confidence: 99%

“…Recent case reports and case series have reported coexisting cardiac amyloid in patients with severe aortic stenosis—typically wild-type transthyretin (wtATTR) [ 95 – 97 ]. Its prevalence and prognostic significance are currently unknown but require further investigation because they may influence the management of AS in terms of decisions surrounding intervention, procedure performance and the use of specific amyloid therapies.…”

Section: The Role Of Advanced Imagingmentioning

confidence: 99%

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Aortic Stenosis, a Left Ventricular Disease: Insights from Advanced Imaging

et al. 2016

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Aortic stenosis (AS) is the most common primary valve disorder in the elderly with an increasing prevalence. It is increasingly clear that it is also a disease of the left ventricle (LV) rather than purely the aortic valve. The transition from left ventricular hypertrophy to fibrosis results in the eventual adverse effects on systolic and diastolic function. Appropriate selection of patients for aortic valve intervention is crucial, and current guidelines recommend aortic valve replacement in severe AS with symptoms or in asymptomatic patients with left ventricular ejection fraction (LVEF) <50 %. LVEF is not a sensitive marker and there are other parameters used in multimodality imaging techniques, including longitudinal strain, exercise stress echo and cardiac MRI that may assist in detecting subclinical and subtle LV dysfunction. These findings offer potentially better ways to evaluate patients, time surgery, predict recovery and potentially offer targets for specific therapies. This article outlines the pathophysiology behind the LV response to aortic stenosis and the role of advanced multimodality imaging in describing it.

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Section: The Role Of Advanced Imagingmentioning

confidence: 99%

Section: The Role Of Advanced Imagingmentioning

confidence: 99%

Aortic Stenosis, a Left Ventricular Disease: Insights from Advanced Imaging

et al. 2016

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Advances in the diagnosis and treatment of transthyretin amyloidosis with cardiac involvement

et al. 2019

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Workup and Management of Patients With Paradoxical Low-Flow, Low-Gradient Aortic Stenosis

Annabi

Clisson

Clavel

2018

Curr Treat Options Cardio Med

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About 60% of patients with paradoxical low-flow, low-gradient (PLF-LG) aortic stenosis (AS) have a severe disease that justifies aortic valve replacement (AVR). The first step in patients with symptomatic PLF AS should be to rule out measurement errors and treat hypertension. The second step is to distinguish pseudo-severe from true severe AS (TSAS). The third step is to select the optimal treatment modality at the right time. Regarding the second step, projected aortic valve area calculated using stress echocardiography is superior to traditional severity criteria (AVA < 1.0 cm and mean gradient ≥ 40 mmHg) to unmask TSAS and predict outcomes. Aortic valve calcification score quantitated by computed tomography is helpful to identify TSAS by applying thresholds of 2000 and 1200 AU, respectively, for men and women. This modality should be considered, particularly if stress echocardiography is either not feasible or inconclusive. Once AS severity is confirmed, a risk stratification based on symptomatic status and the importance of left ventricular (LV) systolic impairment will guide therapeutic decision. Symptomatic assessment should not solely rely on patient-reported symptom status, but rather include an objective exercise test. The presence of symptomatic PLF-LG TSAS is a class IIa indication for AVR in the guidelines. In asymptomatic patients, a markedly reduced stroke volume, the presence of myocardial fibrosis by cardiac magnetic resonance imaging, a poor longitudinal LV function as assessed by speckle tracking echocardiography, and/or a moderate to severe LV diastolic dysfunction are predictors of poor outcome in PLF-LG patients and may indicate the need of early AVR. The type of AVR should be discussed within a multidisciplinary team, bearing in mind that transcatheter AVR (TAVR) is superior to medical treatment in inoperable patients. Furthermore, TAVR may be a useful alternative to surgical AVR (SAVR) in high-risk patients. Nevertheless, the potential benefits of TAVR, including the lower risk of severe patient-prosthesis mismatch, should be weighed against the risk of paravalvular regurgitation, which is likely poorly tolerated by patients with PLF-LG who often harbor a small and non-compliant LV cavity.

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Cardiac amyloidosis: an unusual cause of low flow–low gradient aortic stenosis with preserved ejection fraction

Cited by 5 publications

References 0 publications

Aortic Stenosis, a Left Ventricular Disease: Insights from Advanced Imaging

Aortic Stenosis, a Left Ventricular Disease: Insights from Advanced Imaging

Advances in the diagnosis and treatment of transthyretin amyloidosis with cardiac involvement

Workup and Management of Patients With Paradoxical Low-Flow, Low-Gradient Aortic Stenosis

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