Potential use of cardiac magnetic resonance imaging in differential diagnosis of cardiomyopathies due to light-chain amyloidosis and transthyretin amyloidosis

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Abstract

BACKGROUND: Cardiac amyloidosis is a serious progressive disease with a high mortality rate. The differential diagnosis of cardiomyopathies due to amyloid light-chain (AL) amyloidosis and transthyretin (ATTR) amyloidosis is important for selecting the optimal treatment strategy.

AIM: The aim of this study was to evaluate the capabilities of cardiac magnetic resonance imaging in the differential diagnosis of cardiomyopathies due to AL and ATTR amyloidosis.

MATERIALS AND METHODS: A retrospective analysis of the medical records of 25 patients with a confirmed diagnosis of amyloid cardiomyopathy was performed. Patients were divided into two groups according to the type of amyloidosis, with group 1 including patients with cardiomyopathy due to AL amyloidosis and group 2 including patients with cardiomyopathy due to ATTR amyloidosis. All patients underwent contrast-enhanced cardiac magnetic resonance imaging. Volumetric and linear cardiac parameters, ventricular function, and late gadolinium enhancement patterns were assessed. Standard statistical methods were used, and differences were considered significant at p <0.05.

RESULTS: Group 2 showed a more significant thickening of the myocardial walls compared to group 1 (interventricular septum: 18 [17; 18] vs. 14.5 mm [12.8; 16.0], p <0.01, posterior wall of the left ventricle: 14 [13; 17] vs. 10.5 mm [10; 12.3], p <0.01). The indexed mass of the left ventricle myocardium was 110 [92; 125] in group 2 and 85 mm [69.3; 91.8] in group 1 (p <0.01). In group 2, late gadolinium enhancement with a transmural left ventricle pattern was more frequently observed in the basal and mid-lower-lateral segments, whereas in group 1, a subendocardial pattern of late gadolinium enhancement was more frequent in the mid-anterior and lower-lateral segments (p <0.05). In addition, frequency of simultaneous contrast enhancement in the subendocardial layers of the interventricular septum on the left ventricle and right ventricle sides was higher in group 2 (100% of cases vs. 50%, p <0.01). Late gadolinium enhancement of the right ventricle was also more common in group 2 (100 vs. 58%, p <0.05), especially in the interventricular septum and inferior wall area (p <0.05). Semi-quantitative assessment of LGE using the Query Amyloid Late Enhancement (QALE) showed greater contrast enhancement in group 2: 13 [12; 14] vs. 10.5 [1.75; 12], p <0.01), and a score greater than 13 differentiated between cardiomyopathy due to AL amyloidosis and ATTR amyloidosis with a sensitivity of 69% and a specificity of 83%.

CONCLUSIONS: Cardiac MRI identifies typical features of cardiomyopathies due to AL amyloidosis and ATTR amyloidosis for their differential diagnosis. Further research is needed to confirm diagnostic accuracy of the patterns identified.

About the authors

Zainab M. Magomedova

Pirogov Municipal Clinical Hospital № 1; Sechenov First Moscow State Medical University

Author for correspondence.
Email: magomedova.zainab.97@mail.ru
ORCID iD: 0000-0001-6753-1525
SPIN-code: 5271-4915

MD, radiologist of the Department of Magnetic Resonance and Computed Tomography of the Clinical Hospital No. 1 named after N.I. Pirogov; postgraduate student of the Cardiology Department, Functional and Ultrasound Diagnostics at I.M. Sechenov Moscow State Medical University (Sechenov University).

Russian Federation, Moscow; Moscow

Tatyana V. Nikiforova

S.S. Yudin City Clinical Hospital

Email: attrcmp@gmail.com
ORCID iD: 0000-0003-3072-8951
SPIN-code: 4997-0330

MD, cardiologist

Russian Federation, Moscow

Dmitry Y. Shchekochikhin

Pirogov Municipal Clinical Hospital № 1; Sechenov First Moscow State Medical University

Email: agishm@list.ru
ORCID iD: 0000-0002-8209-2791
SPIN-code: 3753-6915

MD, Cand. Sci. (Medicine), Assistant Professor

Russian Federation, Moscow; Moscow

Ekaterina S. Pershina

Pirogov Municipal Clinical Hospital № 1; Sechenov First Moscow State Medical University

Email: pershina86@mail.ru
ORCID iD: 0000-0002-3952-6865
SPIN-code: 7311-9276

MD, Cand. Sci. (Medicine), Deputy Chief Physician for or Strategic Development and Head, Associate Professor of the Department of Cardiology, Functional and Ultrasound Diagnostics), Senior Researcher at the Institute of Personalized Cardiology

Russian Federation, Moscow; Moscow

Konstantin V. Kovalev

Pirogov Municipal Clinical Hospital № 1

Email: radix606@yandex.ru
ORCID iD: 0009-0004-4841-041X

MD, radiologist of the Department of Magnetic Resonance and Computed Tomography

Russian Federation, Moscow

Khadizhat S. Abdulmazhidova

Sechenov First Moscow State Medical University

Email: abdulmazhidova.kh@mail.ru
ORCID iD: 0009-0008-5064-7802

student

Russian Federation, Moscow

Daria S. Rassechkina

Sechenov First Moscow State Medical University

Email: rassechkina@yandex.ru
ORCID iD: 0009-0007-8825-8485

MD, resident of the Department of Cardiology, Functional and Ultrasound Diagnostics

Russian Federation, Moscow

Alexander E. Grachev

National Medical Research Center of Hematology

Email: gra4al@yandex.ru
ORCID iD: 0000-0001-7221-9392
SPIN-code: 4281-3923

MD, Cand. Sci. (Medicine), Hematologist

Russian Federation, Moscow

Irina G. Rekhtina

National Medical Research Center of Hematology

Email: rekhtina.i@blood.ru
ORCID iD: 0000-0002-7944-6202
SPIN-code: 4920-7144

MD, Dr. Sci. (Medicine), Head of the Department of Hematology and Chemotherapy of Plasma Cell Dyscrasias, Hematologist

Russian Federation, Moscow

Susanna D. Sarkisyan

Sechenov First Moscow State Medical University

Email: sysanna.sarkisyan.2001@mail.ru
ORCID iD: 0000-0002-6454-1370

student

Russian Federation, Moscow

Alexey N. Volovchenko

Sechenov First Moscow State Medical University

Email: dr.volovchenko@mail.ru
ORCID iD: 0000-0002-0923-735X
SPIN-code: 4120-8740

MD, Cand. Sci. (Medicine), Head of the Cardiology Department at the Cardiology Clinic, Assistant Professor at the Department of Cardiology, Functional and Ultrasound Diagnostics

Russian Federation, Moscow

Valentin E. Sinitsyn

Lomonosov Moscow State University

Email: vsini@mail.ru
ORCID iD: 0000-0002-5649-2193
SPIN-code: 8449-6590

MD, Dr. Sci. (Medicine), Professor, Head of the Department of Radiology and Therapy, Head of the Department of Radiology at the Faculty of Fundamental Medicine and the Interdisciplinary Scientific and Educational School

Russian Federation, Moscow

Denis A. Andreev

Sechenov First Moscow State Medical University

Email: dennan@mail.ru
ORCID iD: 0000-0002-0276-7374
SPIN-code: 8790-8834

MD, Dr. Sci. (Medicine), Head of the Department of Cardiology, Functional and Ultrasound Diagnostics

Russian Federation, Moscow

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Supplementary files

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2. 3. Images of delayed cardiac magnetic resonance imaging in patients with transthyretin amyloidosis. Subendocardial accumulation of contrast agent in the interventricular septum from the left and right ventricles (red dotted lines).

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3. 4. Images of delayed-contrast magnetic resonance imaging of the heart in patients with transthyretin amyloidosis. Transmural accumulation of contrast agent in the basal and middle regions (lower lateral segments), subendocardial accumulation in the basal region (anterior, anterolateral, lower segments) of the left ventricular myocardium (white arrows), subendocardial accumulation of contrast agent in the interventricular septum of the myocardium from the right ventricle (yellow arrow).

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4. 5. Images of delayed-contrast magnetic resonance imaging of the heart in amyloidosis of the light chains. Subendocardial accumulation of contrast agent in the basal and middle part (lower-lateral segments) of the left ventricular myocardium (white arrows).

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5. 8. Images of delayed-contrast magnetic resonance imaging of the heart in patients with transthyretin amyloidosis. a — transmural accumulation of contrast agent in the basal lateral segments and intramural in the basal lower segment of the left ventricular myocardium (white arrows), QALE score — 15 points. b is a circular subendocardial accumulation of contrast agent in all segments of the middle myocardium of the left ventricle (white arrows), the QALE score is 10 points.

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6. Fig. 1. Box plot of the linear measures of cardiac magnetic resonance imaging in Groups 1 and 2. AL, light-chain amyloidosis (Group 1); ATTR, transthyretin amyloidosis (Group 2); IVS, interventricular septum; LVPW, left ventricular posterior wall.

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7. Fig. 2. Distribution of late gadolinium enhancement cases in the right ventricle in the groups. RV, right ventricle; AL, light-chain amyloidosis (Group 1); ATTR, transthyretin amyloidosis (Group 2); LGE, late gadolinium enhancement.

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8. Fig. 6. Box plot of the Query Amyloid Late Enhancement (QALE) score in Groups 1 and 2. AL, light-chain amyloidosis (Group 1); ATTR, transthyretin amyloidosis (Group 2).

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9. Fig. 7. ROC curve for the Query Amyloid Late Enhancement (QALE) score. Solid line: QALE score, area under the curve (AUC) 0.83 (95٪ confidence interval: 0.64–0.97); sensitivity 69٪; specificity 83٪.

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