Natural History and Disease Progression of Early Cardiac Amyloidosis Evaluated by Echocardiography

Zadok, Osnat Itzhaki Ben; Eisen, Alon; Shapira, Yaron; Monakier, Daniel; Iakobishvili, Zaza; Schwartzenberg, Shmuel; Abelow, Aryeh; Ofek, Hadass; Kazum, Shirit; Ben‐Avraham, Binyamin; Hamdan, Ashraf; Bental, Tamir; Sagie, Alik; Kornowski, Ran; Vaturi, Mordehay

doi:10.1016/j.amjcard.2020.07.050

Cited by 13 publications

(18 citation statements)

References 25 publications

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“…showed that increased wall thickness and diastolic dysfunction develop in cardiac amyloidosis over a time course of several years. 31 In AS patients, however, the echocardiographic findings can be similar. Since the diagnosis of ATTR cardiac amyloidosis was performed at enrolment, we cannot know whether the patients were positive for ATTR cardiac amyloidosis before TAVR, but we assume that these subjects had a milder form of the disease at the time of TAVR.…”

Section: Discussionmentioning

confidence: 98%

Transthyretin cardiac amyloidosis in patients after TAVR: clinical and echocardiographic findings and long term survival

et al. 2021

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Aims The aim of this study was to examine the prevalence of amyloid transthyretin (ATTR) cardiac amyloidosis in patients 1–2 years after trans‐catheter aortic valve replacement (TAVR) and to assess their clinical and echocardiographic outcome and long‐term survival. Methods and results We enrolled 88 patients, mean age 81 years, 534 (390–711) days after TAVR. Patients underwent a Tc99m‐PYP scintigraphy for the diagnosis of ATTR cardiac amyloidosis. Eleven (12.5%) participants were diagnosed with ATTR cardiac amyloidosis. Eighty eight per cent of patients without amyloidosis were in New York Heart Association Classes 1–2 after TAVR, compared with 64% patients with ATTR cardiac amyloidosis (P = 0.022). There were no differences in left ventricular (LV) ejection fraction (P = 0.69) between patients with and without ATTR cardiac amyloidosis at enrolment. The LV mass index and pulmonary artery pressure were significantly higher in patients with ATTR cardiac amyloidosis (P = 0.046 and P = 0.002, respectively). Global longitudinal strain and myocardial work efficiency were significantly lower in patients with ATTR cardiac amyloidosis (P = 0.031 and P = 0.048, respectively). We assessed changes in echocardiographic data, from the time of TAVR to enrolment, and as expected, there was a significant decrease in aortic valve gradient in both groups. There was a significant reduction in LV mass and LV mass index and improvement in basal segment LV strain in the ATTR cardiac amyloidosis negative group (P = 0.045, P = 0.046 and 0.023, respectively). However, in the ATTR cardiac amyloidosis group the change in LV mass and LV mass index and LV basal strain values was not significant (P = 0.24, P = 0.13 and P = 0.35, respectively). The were no significant changes in other echocardiographic parameters in both groups. The patients were followed for 1150 (1086–1221) days after enrolment. Twenty seven patients had at least one cardiac hospitalization during of follow up, of them seven were with ATTR cardiac amyloidosis and 20 patients without amyloidosis (P = 0.017). Eighteen patients (20%) died during follow up; 12 (14%) patients died due to cardiac causes. There was no difference in all‐cause and cardiac mortality between patients with and without ATTR cardiac amyloidosis (P = 0.6 and P = 0.53, respectively). Conclusions The long‐term survival after TAVR is not significantly affected by the presence of ATTR cardiac amyloidosis. However, the clinical course of these patients and the LV hemodynamic improvement is less favourable. This hypothesis‐generating study suggests screening for ATTR cardiac amyloidosis in patients who underwent TAVR and have limited clinical or echocardiographic improvement, because they may potentially improve with new therapies for ATTR cardiac amyolidosis.

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Section: Discussionmentioning

confidence: 98%

Transthyretin cardiac amyloidosis in patients after TAVR: clinical and echocardiographic findings and long term survival

et al. 2021

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“…The diagnosis criteria of ATTR-CA and AL-CA has been previously described [ 4 ]. Cardiac ATTR was defined as the combination of symptoms with an echocardiogram consistent with or suggestive of cardiac amyloidosis, grade 2 or 3 cardiac uptake on 99mTc-DPD scintigraphy in the absence of a monoclonal gammopathy [ 6 ].…”

Section: Methodsmentioning

confidence: 99%

“…The shared clinical and imaging characteristics of AL-CA and ATTR-CA, which most frequently present at advanced or end-stage amyloid heart disease, have often resulted in the combination of both diseases under one umbrella. However, important differences do exist, and most probably derivate from their specific etiology and pathophysiology [ 4 , 5 ]. Defining these differences may pave the way for an earlier, targeted diagnosis of AL or ATTR CA and improve our understanding of disease progression and management.…”

Section: Introductionmentioning

confidence: 99%

Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis

et al. 2021

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Aims To compare the baseline cardiovascular characteristics of immunoglobulin light-chain (AL) and amyloid transthyretin (ATTR) cardiac amyloidosis (CA) and to investigate patients’ contemporary cardiac outcomes. Methods Single-center analysis of clinical, laboratory, echocardiographic and cardiac magnetic resonance imaging (CMRi) characteristics of AL and ATTR-CA patients’ cohort (years 2013–2020). Results Included were 67 CA patients of whom 31 (46%) had AL-CA and 36 (54%) had ATTR-CA. Patients with ATTR-CA versus AL-CA were older (80 (IQR 70, 85) years versus 65 (IQR 60, 71) years, respectively, p<0.001) with male predominance (p = 0.038). Co-morbidities in ATTR-CA patients more frequently included diabetes mellitus (19% versus 3.0%, respectively, p = 0.060) and coronary artery disease (39% versus 10%, respectively, p = 0.010). By echocardiography, patients with ATTR-CA versus AL-CA had a trend to worse left ventricular (LV) ejection function (50 (IQR 40, 55)% versus 60 (IQR 45, 60)%, respectively, p = 0.051), yet comparable LV diastolic function. By CMRi, left atrial area (31 (IQR 27, 36)cm2 vs. 27 (IQR 23, 30)cm2, respectively, p = 0.015) and LV mass index (109 (IQR 96, 130)grams/m2 vs. 82 (IQR 72, 98)grams/m2, respectively, p = 0.011) were increased in patients with ATTR-CA versus AL-CA. Nevertheless, during follow-up (median 20 (IQR 10, 38) months), patients with AL-CA were more frequently admitted with heart failure exacerbations (HR 2.87 (95% CI 1.42, 5.81), p = 0.003) and demonstrated increased mortality (HR 2.51 (95%CI 1.19, 5.28), p = 0.015). Conclusion Despite the various similarities of AL-CA and ATTR-CA, these diseases have distinct baseline cardiovascular profiles and different heart failure course, thus merit tailored-cardiac management.

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“…Typically, LVH in ATTR-CA is slowly progressive, allowing for early detection of disease prior to significant symptom onset. In a study by Itzhaki Ben Zadok et al, echocardiograms in AL and ATTR-CA patients analyzed in the years prior to a formal diagnosis found LVH ≥ 12 mm in 79% of patients more than 3 years prior to diagnosis ( 16 ). Additionally, ATTR-CA patients developed LVH earlier than AL-CA patients prior to their formal diagnosis, possibly related to the rapid disease progression seen with AL-CA as compared to ATTR-CA ( 12 ).…”

Section: Echocardiographymentioning

confidence: 99%

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

Scheel

Mukherjee

Hays

et al. 2022

Front. Cardiovasc. Med.

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Cardiac amyloidosis (CA) is an infiltrative cardiomyopathy resulting from deposition of misfolded immunoglobulin light chains (AL-CA) or transthyretin (ATTR-CA) proteins in the myocardium. Survival varies between the different subtypes of amyloidosis and degree of cardiac involvement, but accurate diagnosis is essential to ensure initiation of therapeutic interventions that may slow or potentially prevent morbidity and mortality in these patients. As there are now effective treatment options for CA, identifying underlying disease pathogenesis is crucial and can be guided by multimodality imaging techniques such as echocardiography, magnetic resonance imaging, and nuclear scanning modalities. However, as use of cardiac imaging is becoming more widespread, understanding optimal applications and potential shortcomings is increasingly important. Additionally, certain imaging modalities can provide prognostic information and may affect treatment planning. In patients whom imaging remains non-diagnostic, tissue biopsy, specifically endomyocardial biopsy, continues to play an essential role and can facilitate accurate and timely diagnosis such that appropriate treatment can be started. In this review, we examine the multimodality imaging approach to the diagnosis of CA with particular emphasis on the prognostic utility and limitations of each imaging modality. We also discuss how imaging can guide the decision to pursue tissue biopsy for timely diagnosis of CA.

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Natural History and Disease Progression of Early Cardiac Amyloidosis Evaluated by Echocardiography

Cited by 13 publications

References 25 publications

Transthyretin cardiac amyloidosis in patients after TAVR: clinical and echocardiographic findings and long term survival

Transthyretin cardiac amyloidosis in patients after TAVR: clinical and echocardiographic findings and long term survival

Differences in the characteristics and contemporary cardiac outcomes of patients with light-chain versus transthyretin cardiac amyloidosis

Multimodality Imaging in the Evaluation and Prognostication of Cardiac Amyloidosis

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