A Critical Review of Echocardiographic Findings for Diagnosing Cardiac Amyloidosis

Barberato, Silvio Henrique; Beck, Adenalva Lima de Souza; Hotta, Viviane Tiemi; Rassi, Daniela do Carmo

doi:10.36660/ijcs.20240047

Silvio Henrique Barberato

Conception and design of the research

Cardioeco, Centro de Diagnóstico Cardiovascular, Curitiba, PR – Brazil

https://orcid.org/0000-0002-0711-7995

Adenalva Lima de Souza Beck

acquisition of data

analysis and interpretation of the data

writing of the manuscript and critical revision of the manuscript for intellectual content

Instituto de Cardiologia e Transplantes do Distrito Federal (ICTDF-DF), Brasília, DF – Brazil

Hospital Sírio Libanês, Brasília, DF – Brazil

https://orcid.org/0000-0002-5903-987X

Viviane Tiemi Hotta

acquisition of data

analysis and interpretation of the data

writing of the manuscript and critical revision of the manuscript for intellectual content

Instituto do Coração HC-FMUSP, Unidade Clínica de Miocardiopatias e Doenças da Aorta, São Paulo, SP – Brazil

https://orcid.org/0000-0002-9236-3815

Daniela do Carmo Rassi

acquisition of data

analysis and interpretation of the data

writing of the manuscript and critical revision of the manuscript for intellectual content

Faculdade de Medicina da Universidade Federal de Goiás, Goiânia, GO – Brazil

https://orcid.org/0000-0002-9131-0439

Mailing Address: Silvio Henrique Barberato Cardioeco, Centro de Diagnóstico Cardiovascular. Avenida República Argentina, 1336, conj 215. Postal code: 80620-010. Curitiba, PR – Brazil E-mail: silviohb@cardiol.br

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

Editor responsible for the review: Marcus Vinicius Simoes

Figures (4)
Tables (2)

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Central Illustration
: A Critical Review of Echocardiographic Findings for Diagnosing Cardiac Amyloidosis

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Figure 1
– Conventional echocardiographic findings suggestive of CA in a 71-year-old patient with arterial hypertension and previous myocardial revascularization admitted with HFpEF. Increased LV wall thickness (A and B), pleural effusion (A), interatrial septum thickening (c), LV diastolic dysfunction with a restrictive pattern (E/A 2.7, average E/e' 25.8) (D, E, and F) and "triple 5" sign (E). This patient was diagnosed with light chain amyloidosis, confirmed by anatomopathological examination of bone marrow biopsy

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Figure 2
– Echocardiographic indexes in a patient with light chain amyloidosis. A) APS evaluated by speckle-tracking echocardiography; reduction of longitudinal strain can be seen in the basal and medium segments in the light red color. Longitudinal strain in the apical segments is preserved, as evidenced by the dark red color in the parametric cardiac map. LVEF derived from Simpson´s method is 62.9%, and GLS is - 14.4%. The LVEF/GLS ratio is 4.4. B) Septal apical-to-basal longitudinal strain (SAB) ratio calculation in the same patient. Segmental longitudinal strain obtained in the 4-chamber view was 8.5 in the inferior septal basal wall and 21.6 in the septal apical wall. Thus, SAB was 2.5. Both findings are suggestive of the diagnosis of CA. AUC, area under curve. LVEF: Left ventricle ejection fraction; GLS: global longitudinal strain; HCM: hypertrophic cardiomyopathy; EF: ejection fraction; AUC: LS: EDV: ESV:

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Figure 3
– Patient with increased LV wall thickness suspected of having CA. The calculated IWT score summed 9 points, derived from RWT = 0.78 (A), TAPSE = 12.6mm (B), E/e′ = 12.6 (C and D), GLS = -7.4% (E), and SAB = 21 (E). This is consistent with a high likelihood of having CA. TAPSE: tricuspid annular plane systolic excursion; E/e´: the ratio of the early mitral inflow (E) to early mitral annular (e´) velocities; GLS: global longitudinal strain; SAB: septal apical-to-basal longitudinal strain ratio

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Table 1
– Relative utility of echocardiographic parameters for the diagnosis of CA

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Table 2
– Variable cut-Offs for calculation of the AL and IWT scores

Echocardiographic parameter	Abnormal	Practical points	Caveats
Conventional findings
LV WT	Increased (≥ 12mm) in the absence of a plausible cause	Disproportionate wall thickening in the absence of high ECG voltage is suggestive of CA.	Low specificity. If normal, it does not rule out CA.
RWT	>0.42	“Typical” concentric LV remodeling in opposition to eccentric or asymmetric. RWT is part of the multiparametric IWT and AL scores.	Asymmetry does not rule out CA
Myocardial echogenicity	Increased (granular “sparkling” appearance)	Only valid when not using harmonic imaging in the echocardiogram. Highly suggestive in conjunction with severely reduced GLS.	Low sensitivity and specificity. It can occur in other causes of hypertrophy.
RV “hypertrophy” and dysfunction	Increased free RV WT (> 7mm). Decreases in TAPSE (< 16mm) and tricuspid TDI s´ (< 10cm/s)	Increased WT occurs in intermediate stages. RV dysfunction can occur before evidence of LV involvement	Low specificity
Atrial size and function	Atrial enlargement and dysfunction	Provides insight into risk for stroke or arterial embolism.	Low specificity
Interatrial septum and Cardiac valves	Thickening of the interatrial septum (> 5mm) and valves	Suggestive of the diagnosis, especially if along with myocardial “sparkling”.	Low specificity
Pericardial effusion	Often mild effusion	Suggestive of the diagnosis when accompanied by other typical findings.	Low specificity
Diastolic dysfunction	Typically, grade ≥ II diastolic dysfunction	Early stage: average E/e´> 13 is one of the first echocardiographic signs of CA. Later stage: restrictive physiology. Correlate with severity of symptoms and prognosis.	Low specificity
TDI of mitral annulus	The “5-5-5” sign	Highly suggestive of the diagnosis.	Low sensitivity in early stages
MCF	20–30%	Independent of chamber size and geometry. It appears to have the best diagnostic performance among non-deformation parameters.	Validation only in TTR-CA
Speckle-tracking echocardiography
LV GLS	< 16	Sensitive marker of subclinical dysfunction. CA patients tend to have lower GLS values than HCM patients with similar LV wall thickness. GLS is part of the multiparametric IWT and AL scores.	Non-specific
APS ratio	>1	Relative preservation of apical LS compared to basal and mid-LV LS (“cherry-on-top” sign on the bullseye map).	APS can be found in other hypertrophic phenotypes in the absence of CA and even in healthy subjects.
SAB	>2.1	SAB is part of the multiparametric IWT score.	Modest accuracy in real-world cohorts
LVEF/GLS	>4.1	Highest accuracy for the diagnosis of CA in comparison to APS and SAB	Modest accuracy in real-world cohorts

	Cut-Off	Points
AL score: indicative of AL-CA if ≥ 5 points
RWT	>0,52	2
E/e´	>10	2
TAPSE, mm	≤ 19	1
GLS, %	≥ - 14	1
IWT score: indicative of TTR-CA if ≥ 8 points
RWT	>0,6	3
E/e´	>11	1
TAPSE, mm	≤ 19	2
GLS, %	≥ - 13	1
SAB	>2.9	2

[1] Mailing Address: Silvio Henrique Barberato Cardioeco, Centro de Diagnóstico Cardiovascular. Avenida República Argentina, 1336, conj 215. Postal code: 80620-010. Curitiba, PR – Brazil E-mail: silviohb@cardiol.br

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A Critical Review of Echocardiographic Findings for Diagnosing Cardiac Amyloidosis

Abstract