Rapid Fire Session

Session: Rapid Fire Session 2: Multicenter Clinical Trials and Cardiomyopathy (Monitor 10)

Transthyretin Amyloid Cardiomyopathy Patients Undergoing Tafamidis Therapy Assessed by Advanced Serial Cardiac Magnetic Resonance Imaging

Thursday, February 5, 2026

3:52 PM - 3:59 PM

Location: Galapagos / Aruba / Ilhabela (2nd Lower Level)

Presenting Author(s)

Cassady Palmer, BSc

Research Associate
The Christ Hospital Health Network
Cincinnati, Ohio, United States

Primary Author(s)

Cassady Palmer, BSc

Research Associate
The Christ Hospital Health Network
Cincinnati, Ohio, United States

Co-Author(s)

SS

Suzanne Smart, BSc, CCRP

Research Associate
The Ohio State University, United States
CS

Christian Schmidt, PhD

Biomedical Statistician
The Christ Hospital Health Network, United States
KZ

Karolina Zareba, M.D.

Assistant Professor of Medicine, Associate Program Director, Cardiovascular Diagnostics Training Program
The Ohio State University Wexner Medical Center
Columbus, Ohio, United States
Deborah Kwon, MD, FSCMR

Director of Cardiac MRI
Cleveland Clinic
Cleveland, Ohio, United States
WM

Wojciech M Mazur, MD

Director of Advanced Cardiac Imaging
The Christ Hospital Health Network
Cincinnati, Ohio, United States

Background: Transthyretin amyloid cardiomyopathy is caused by the accumulation of misfolded transthyretin-derived amyloid fibrils, which leads to progressive ventricular wall thickening and severe heart failure. The heterogeneity of the disease adds to the perplexity of this disease. We conducted a multi-center investigation utilizing cardiac magnetic resonance imaging for the purpose of evaluating the stabilization of cardiac ATTR in patients undergoing Tafamidis treatment.

Methods: A prospective multicenter investigation, in which participants were followed and assessed utilizing cardiac imaging parameters 1-3 year following Tafamidis. All subjects underwent a comprehensive baseline clinical echocardiogram, PYP, and gene sequencing (as these are standard of care). In addition, all patients underwent baseline and follow cardiac MRI with simultaneous capture of biomarkers.

Results: A total of 23 patients positive of ATTR were analyzed. Patients were mostly male (n=21; 91% ) with majority classified as wild type ATTR (n=18; 90%). Baseline KCCQ-12 physical function score was calculated at 68.3 ± 28.3 with significant decrease at follow up at 63.2 ± 32.5 (p=0.024). Baseline CMR calculated LV ejection was 51.7 ± 8.6 and significantly reduced at follow up 44.8 ± 9.4 (p=0.048). The baseline median CMR LV mass index was calculated at 84.4 (73.7, 92.1) at follow up measured at 83 (66.3, 105.5) (p=0.650). Parametric mapping did not demonstrate any significant changes over time, as global ECV at baseline demonstrated increased values calculated at 52.9 ± 14.4 and at follow up calculated at 56.6 ± 14.6 (p =0.567). There were no significant differences between baseline CMR LV strain metrics (baseline GLS -9.85 ± 3.32, follow up -9.13 ± 2.4; p= 0.232) (baseline GCG -13.06 ± 3.24, follow up -12.50 ± 2.22; p= 0.310) (baseline GRS 19.48 ± 6.76, follow up 18.12 ± 4.39; p=0.234).

Conclusion: Our multi-center real-world study evaluating both functional and tissue characteristic changes in ATTR patients undergoing Tafamidis therapy, demonstrated that while there was reduction to left ventricular ejection fraction this did not translate to subclinical changes to global strain indices. In addition, there were no significant changes to left ventricular mass or tissue characterization. A larger cohort of patients are needed in future studies to elucidate our findings of our study.

Baseline Patient Characteristics and Functional Assessments

Patient Demographics	N=23
Enrolling Site, n (%) TCH OSU CCF	8 (35) 5 (22) 10 (43)
Sex (Male), n (%)	21 (91)
Race, n (%) Black or African American White	5 (22) 18 (78)
Hispanic Ethnicity, n (%)	0 (0)

Amyloid History
Endomyocardial biopsy, n (%) ATTR Amyloid Result	2 (10) 2 (100)
TcPYP, n (%)	20 (95)
Visual Myocardial Uptake, n (%)	17 (100)
Planar Grade, n (%) 2 3	4 (24) 13 (76)
Heart to Contralateral Ratio, median (IQR)	1.69 (1.48, 1.82)
SPECT/CT, n (%) Diffuse Focal	12 (57) 9 (90) 1 (10)
Serum FLC kappa, median (IQR)	25 (22.2, 38.7)
Serum FLC lambda, median (IQR)	20.8 (16.4, 23.6)
Serum FLC ratio, median (IQR)	1.41 (1.24, 1.97)
Bone marrow biopsy, n (%)	1 (5)
Type of Mutation, n (%) Wild Type Variant	21 (91) 2 (9)
Mutation Variants, n (%) Val142Ile	2
Prior Treatments, n (%)	0 (0)
Medical History
Hypertension	17 (81)
Hyperlipidemia	17 (81)
Diabetes	5 (25)
Smoking Status Former Never	12 (55) 10 (45)
NYHA Class I II III	3 (14) 12 (57) 6 (29)
Atrial Fibrillation Chronic Paroxysmal Permanent	14 (64) 7 (50) 6 (43) 1 (7)
Coronary artery disease	7 (32)
COPD	2 (9)
PVD	2 (9)
Cerebrovascular accident (stroke)	1 (5)
Myocardial Infarction LAD	2 (9) 1
Carpal Tunnel	20 (91)
Spinal stenosis	11 (50)
Biceps tendon rupture	2 (9)
Surgical
PCI LCx	3 (14) 2
CABG	1 (5)
Carpal tunnel release	15 (71)
Spinal Stenosis	7 (33)
Knee replacement	7 (33)
Shoulder surgery	4 (19)
Hip surgery	4 (19)

Labs	Baseline assessment	Follow-up	p-value
Serum creatinine	1.27 ± 0.38	1.30 ± 0.40	0.929
GFR	56.8 ± 16.5	56.4 ± 21.9	0.962
Hematocrit	41.7 ± 5.3	43.0 ± 3.0	^0.025
Hemoglobin	13.6 ± 2.0	14.1 ± 1.1	0.0495
Sodium	139.3 ± 3.3	139.6 ± 3.1	0.314
BUN	27.2 ± 12.6	24.1 ± 10.6	0.657
Prealbumin	22.5 ± 11.2	N = 1	NA
Calcium	9.40 ± 0.46	9.47 ± 0.53	0.370
NT-pro BNP	1971 ± 2551	1528 ± 1195	0.648
Troponin T	73.2 ± 231.5	NA	NA
TTR Tetrameric Stability	NA	NA	NA

6MWT	Baseline assessment	Follow-up	p-value
Systolic BP	125.9 ± 14.7	125.0 ± 14.4	0.707
Diastolic BP	72.3 ± 9.9	70.1 ± 9.9	0.491
Heart Rate	74.3 ± 12.0	78.8 ± 10.7	^0.004
Dyspnea Change	3.38 ± 3.34	1.80 ± 2.44	^0.109
Dyspnea (pre-test)	0.63 ± 1.13	1.80 ± 2.90	^0.063
Dyspnea (post-test)	4.00 ± 3.56	3.60 ± 3.47	^1.000
Fatigue Change	1.53 ± 2.09	0.90 ± 1.10	0.705
Fatigue (pre-test)	1.59 ± 1.80	2.60 ± 2.99	0.159
Fatigue (post-test)	3.13 ± 2.83	3.50 ± 3.44	0.322
SpO2 Change	0.06 ± 1.29	-1.33 ± 2.29	0.153
SpO2 (pre-test)	96.8 ± 2.3	95.9 ± 2.8	0.578
SpO2 (post-test)	96.8 ± 2.0	94.6 ± 4.1	0.153
Supplemental Oxygen	0	0	NA
Number of full 60-meter laps	5.18 ± 1.80	5.50 ± 2.37	0.678
Final partial lap	17.9 ± 16.2	22.5 ± 31.8 (N=2)	N A
Total Distance	296.1 ± 110.5	334.5 ± 143.9	0.860

KCCQ-12	Baseline assessment	Follow-up	p-value
Physical Function Score	68.3 ± 28.3	63.2 ± 32.5	0.024
Symptom Frequency Score	72.9 ± 25.4	79.4 ± 21.0	^0.576
Quality of Life Score	56.3 ± 34.9	65.8 ± 27.3	^0.516
Social Limitation Score	65.4 ± 35.4	75.9 ± 32.5	^0.367
Overall Score	66.6 ± 28.5	72.4 ± 25.4	^0.562

^ Signed-rank test instead of paired t-test due to violation of assumptions.Cardiac MRI functional and tissue characteristic values

Cardiac MRI	Baseline assessment	Follow-up	p-value
LVEDV	176.5 ± 41.2	170.4 ± 40.9	0.281
LVEDV Index	87.0 ± 14.7	81.9 ± 16.8	0.238
LVESV	84.8 ± 25.0	94.4 ± 30.8	^0.975
LVESV Index	41.8 ± 9.7	45.7 ± 11.9	0.469
LVEF	51.7 ± 8.6	45.1 ± 9.1	0.049
LV Mass	166 (145, 203)	159 (151, 216)	0.996
LV Mass Index	84.4 (73.7, 92.1)	83 (66.3, 105.5)	0.650
RVEDV	169.1 ± 43.9	166.0 ± 48.1	0.562
RVEDV Index	82.2 ± 19.3	80.5 ± 24.6	0.614
RVESV	88.0 ± 30.6	90.0 ± 30.2	0.802
RVESV Index	42.6 ± 15.2	43.1 ± 14.6	0.502
RVEF	48.5 ± 8.3	45.5 ± 10.8	0.569
LA Area	29.5 ± 10.0	26.5 ± 6.1	0.285
LA Volume	78.7 ± 51.3	74.0 ± 39.8	0.575
RA Area	24.6 ± 6.1	25.4 ± 6.0	0.316
RA Volume	56.4 ± 37.1	51.4 ± 33.5	^ 0.109
Aortic Regurgitation None Mild Moderate	11 (92) 1 (8) 0	10 (83) 1 (8) 1 (8)	1.00
Mitral Regurgitation None Mild	9 (69) 4 (31)	8 (62) 5 (38)	1.00
Tricuspid Regurgitation None Mild	10 (77) 3 (23)	12 (92) 1 (8)	0.500

Cardiac MRI Strain (%)	Baseline assessment	Follow-up	p-value
Global circumferential	-13.75 ± 3.97	-12.65 ± 2.23	0.310
Endocardial circumferential	-17.26 ± 5.90	-15.35 ± 4.70	0.208
Epicardial circumferential	-12.15 ± 4.28	-10.86 ± 3.85	0.199
Global radial	21.40 ± 9.47	18.42 ± 4.39	0.234
Endocardial radial	27.57 ± 11.83	22.23 ± 8.00	0.143
Epicardial radial	16.55 ± 6.55	14.35 ± 5.57	0.203
Global longitudinal	-9.58 ± 3.33	-9.05 ± 2.12	0.232
Endocardial longitudinal	-10.43 ± 3.12	-10.46 ± 3.07	0.211
Epicardial longitudinal	-10.21 ± 3.56	-9.85 ± 2.79	^0.742
Peak Systolic Strain Rates
Global circumferential	-0.78 ± 0.32	-0.73 ± 0.21	^0.742
Endocardial circumferential	-0.93 ± 0.31	-0.90 ± 0.36	0.859
Epicardial circumferential	-0.62 ± 0.23	-0.58 ± 0.21	0.864
Global radial	1.53 ± 0.86	1.28 ± 0.53	^0.859
Endocardial radial	2.31 ± 1.54	1.89 ± 0.99	0.691
Epicardial radial	1.25 ± 0.68	1.02 ± 0.54	0.556
Global longitudinal	-0.53 ± 0.23	-0.59 ± 0.17	0.582
Endocardial longitudinal	-0.67 ± 0.28	-0.73 ± 0.23	0.641
Epicardial longitudinal	-0.59 ± 0.24	-0.65 ± 0.29	0.584
Early Diastolic Strain Rates
Global circumferential	0.55 ± 0.18	0.57 ± 0.27	0.900
Endocardial circumferential	0.66 ± 0.23	0.68 ± 0.34	0.857
Epicardial circumferential	0.48 ± 0.19	0.50 ± 0.25	0.845
Global radial	-1.15 ± 0.43	-1.06 ± 0.56	0.643
Endocardial radial	-1.62 ± 0.69	-1.41 ± 0.66	0.254
Epicardial radial	-0.70 ± 0.65	-0.82 ± 0.45	0.672
Global longitudinal	0.40 ± 0.14	0.35 ± 0.16	0.504
Endocardial longitudinal	0.54 ± 0.16	0.47 ± 0.22	0.583
Epicardial longitudinal	0.53 ± 0.22	0.46 ± 0.27	^0.547
Peak Late Diastolic Strain Rates
Global circumferential	0.58 ± 0.24	0.56 ± 0.18	0.397
Endocardial circumferential	0.74 ± 0.38	0.66 ± 0.41	`
Epicardial circumferential	0.52 ± 0.24	0.48 ± 0.32	^0.766
Global radial	-0.71 ± 0.29	-0.66 ± 0.22	0.223
Endocardial radial	-0.93 ± 0.51	-0.81 ± 0.47	0.119
Epicardial radial	-0.67 ± 0.32	-0.54 ± 0.38	0.074
Global longitudinal	0.38 ± 0.19	0.42 ± 0.21	^0.625
Endocardial longitudinal	0.45 ± 0.19	0.63 ± 0.30	0.219
Epicardial longitudinal	0.43 ± 0.21	0.56 ± 0.30	0.668

Cardiac MRI Mapping	Baseline assessment	Follow-up	p-value
T1 Mapping – SAX base
Septal Native T1	1198 ± 144	1194 ± 130	0.321
Global Native T1	1218 ± 136	1193 ± 129	0.431
Blood Pool native T1	1760 ± 194	1598 ± 266	0.067
Septal Post contrast T1	397 ± 105	324 ± 104	0.038
Global post contrast T1	411 ± 109	336 ± 104	0.159
Blood pool post contrast T1	380 ± 107	333 ± 103	0.200
Septal Extracellular Volume	51.1 ± 15.4	59.3 ± 19.1	0.070
Global Extracellular volume	50.2 ± 13.5	56.6 ± 14.6	0.567
T1 Mapping – SAX Mid
Septal Native T1	1213 ± 154	1179 ± 111	0.471
Global Native T1	1177 ± 129	1151 ± 104	0.496
Blood Pool native T1	1761 ± 207	1666 ± 151	0.246
Septal Post contrast T1	384 ± 102	343 ± 116	0.221
Global post contrast T1	408 ± 81	374 ± 121	0.617
Blood pool post contrast T1	380 ± 107	339 ± 111	0.401
Septal Extracellular Volume	53.7 ± 11.8	54.2 ± 17.0	1.000^
Global Extracellular volume	48.4 ± 11.9	48.1 ± 12.6	0.857
T1 Mapping – SAX Apex
Septal Native T1	1211 ± 147	1173 ± 98	0.690
Global Native T1	1161 ± 129	1165 ± 107	0.248
Blood Pool native T1	1775 ± 204	1697 ± 175	0.272
Septal Post contrast T1	446 ± 121	351 ± 104	0.628
Global post contrast T1	445 ± 86	394 ± 124	0.704
Blood pool post contrast T1	424 ± 108	338 ± 109	0.650
Septal Extracellular Volume	49.8 ± 12.2	51.3 ± 13.8	0.056
Global Extracellular volume	44.5 ± 9.9	50.0 ± 11.5	0.113
T1 Mapping – HLA
Septal Native T1	1188 ± 142	1141 ± 177	0.423
Global Native T1	NA	NA	---
Blood Pool native T1	1709 ± 180	1638 ± 105	0.215
Septal Post contrast T1	386 ± 96	313 ± 83	0.011
Global post contrast T1	NA	NA	---
Blood pool post contrast T1	371 ± 77	306 ± 45	0.033
Septal Extracellular Volume	53.3 ± 16.6	54.0 ± 13.4	0.448
Global Extracellular volume	NA	NA	---
Mean ECV
Septal mean ECV	52.0 ± 13.8	56.4 ± 17.2	0.169
Global mean ECV	49.2 ± 11.7	53.0 ± 13.2	0.558

^ Signed-rank test instead of paired t-test due to violation of assumptions.CMR quantified LV mass demonstrating baseline and follow up
Septal and mean global extracellular volumes at baseline and follow up
Global CMR feature tracking strain values at baseline and follow up