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Submitted on 17 Jan 2021
Author(s): Imane ESSAKET
Affiliations: Department of Cardiology, University Hospital Mohammed the VIth of Marrakesh
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Abstract: We report the case of a 49-year-old female, with a history of arterial hypertension for 12 years on ACE inhibitors , and that reported chest pain 6 months before admission with positive us-troponin T levels and normal coronary angiography at that time . She was admitted to the cardiac care unit for acute heart failure (HF).On anamnesis, she reported exertional dyspnea class II of the NYHA and worsening peripheral edema over the previous two months. Upon admission, the dyspnea worsened and appeared on minimal exertion evolving 5 days before admission with paroxysmal nocturnal dyspnea, peripheral edema and increased abdominal circumference, apparently decreased urine output, and lower limb paresthesia. Physical examination showed normal blood pressure at 114/81 mmHg, heart rate (HR) 67 bpm and 95% oxygen saturation in room air. Laboratory assessment was unremarkable except for elevated of ultrasensitive Troponin T level at 194ng/ml NV(<19 ng/ml) . The chest X-ray showed alveolar interstitial infiltrate with a butterfly pattern.  A 12-leadECG showed sinus rhythm ,HR 67 bpm, first-degree atrioventricular block, low voltage in the frontal leads and pseudo-infarctus pattern in the precordial leads. Transthoracic echocardiography revealed biatrial dilatation (Figure 2), impaired LV systolic function (ejection fraction 40% by Simpson's method) ,with an apex-base gradient on the LV strain pattern (Figure 3)), the mitral flow showed a restrictive filling pattern with decrease deceleration and reduced A wave .Given the findings of very suggestive features on the echocardiography, low voltage on the ECG, and apex-base gradient on LV strain pattern, a diagnosis of amyloidosis was considered., regardless of the abdominal fat pad and salivary gland biopsy that did not show the presence of amyloid deposits.
Laboratory tests showed increased beta-2 microglobulin .
Based on the above findings, a diagnosis of systemic amyloidosis with cardiac involvement was suspected. The type of fibril involved was not yet determined, and 99mTc-DPD scintigraphy did not show any uptake of the radiotracer.
She had good clinical improvement with standard medical management of heart failure with reduced ejection fraction. Though further evaluation and specific treatment for systemic amyloidosis were planned. Although the patient was in sinus rhythm and had no intracavitary thrombus, the question of putting her on anticoagulation was discussed. Further clarifications on its reason are displayed below.
Our patient presents typical clinical, biological and echocardiographic and LV strain features of cardiac amyloidosis. Although the patient was in sinus rhythm, the question of anticoagulation indication was a clinical challenge to be solved, as multiple pathophysiological features seem to explain the left atrial thrombosis and systemic thromboembolism in cardiac amyloidosis patients such as the endomyocardial injury, the blood hypercoagulability due to hyperviscosity caused by paraprotein production, and eventual associated malignancy. Furthermore, there is an atrial blood stasis that could be explained by severe biatrial dilation, impaired atrial emptying, amyloid infiltration of the atrial walls and increased atrial afterload. It is well known that thrombosis occurs when there is an abnormality of blood flow, of the endothelium, or of factors involved with coagulation itself-the classic Virchow's triad.
In cardiac amyloidosis it seems likely that at least the first two of these criteria may be satisfied. To support this statement, a study found a high frequency of intracardiac thrombosis in autopsies or explanted cases of cardiac amyloidosis, all types confound[1]. In the latter, intracardiac thrombosis was identified in 33% of cases. Interestingly, an evidence of atrial electromechanical dissociation was described by Dubrey and al. in patients with cardiac amyloidosis and atrial thrombus whilst in sinus rhythm [2]. The patients described in this case series were three women with NYHA class IV heart failure caused by primary (amyloid light chain) cardiac amyloidosis, and had extensive infiltration of both the ventricular and atrial myocardium. All three showed either a reduced or an absent A wave on the Doppler mitral valve velocity spectrum. An increase in ventricular end diastolic pressures resulting in a restrictive filling pattern has been proposed as the cause of the reduced atrial contribution, as a result of an increase in atrial afterload. The E wave deceleration times in these three cases were significantly shorter than those reported for normal subjects of the same age; supporting the argument that a restrictive physiology contributes to the absence of an atrial contribution. An alternative theory to explain the reduced A wave is that atrial contraction is severely impaired or absent because of amyloid infiltration of the atrial walls. Our patient had a similar mitral flow pattern profile.
To better assess the LA function in cardiac amyloidosis, Kotaro and al. used speckle-tracking echocardiography in 124 patients with CA and sinus rhythm. They concluded that LA function was severely impaired and highly correlated with LV deformation. Differences in LA function between amyloid subtypes suggest that amyloid aetiology plays a role in the pathophysiology of cardiac dysfunction in CA. Unfortunately, due to lack of routine practice routine of LA strain in our setting, this technique couldn’t be performed in our patient.
Regarding the occurrence of AF in cardiac amyloidosis patients, a study aiming to assess prevalence, incidence, risk factors and prognostic significance of AF in AL, m-ATTR and wt-ATTR cardiac amyloidosis found that the prevalence of AF varies widely according to etiology with a mean value of 15% that reaches 40% in wt-ATTR amyloidosis [4]. Age, HF, LV ejection fraction, left atrial size and right atrial pressure were the main independent risk factors, while wall thickness and etiology were not the main independent risk factors. Our patient had three of these factors namely HF, low ejection fraction and left atrial dilation.
For the choice of the anticoagulant therapy, and following our review, there is a scarcity of literature regarding the superiority of an agent over the other (vitamin K antagonists versus NOACs). However the benefice/risk balance of the anticoagulation should take into consideration the risk of cerebral amyloid angiopathies.

Summary: The effect of amyloidosis on the quality of life and mortality rate of patients is substantial. CA leads to higher rates of atrial arrhythmia and an increased risk of intracardiac thrombus formation. However, atrial mechanical dysfunction due to protein deposition in amyloidosis may lead to thrombus formation in the absence of atrial arrhythmia. We hereby describe a case of cardiac amyloidosis in sinus rythm and discuss the anticoagulation necessity in this case.
References: 1.Feng D, Edwards W, Oh J, Chandrasekaran K, Grogan M, Martinez M et al. Intracardiac Thrombosis and Embolism in Patients With Cardiac Amyloidosis. Circulation. 2007;116(21):2420-2426.
2.Dubrey S, Pollak A, Skinner M, H Falk R. Atrial thrombi occurring during sinus rhythm in cardiac amyloidosis: evidence for atrial electromechanical dissociation. Br HeartJ 1995;74:541-544
3.Nochioka K, Quarta C, Claggett B, Roca G, Rapezzi C, Falk R et al. Left atrial structure and function in cardiac amyloidosis. European Heart Journal - Cardiovascular Imaging. 2017;.
4.Longhi S, Quarta C, Milandri A, Lorenzini M, Gagliardi C, Manuzzi L et al. Atrial fibrillation in amyloidotic cardiomyopathy: prevalence, incidence, risk factors and prognostic role. Amyloid. 2015;22(3):147-155.
5. Russo D, Limite L, Arcari L, Autore C, Musumeci M. Predicting the Unpredictable. Journal of the American College of Cardiology. 2019;73(22):2910-2911.
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