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Table 3 Cardiac characteristics at enrollment in symptomatic patients with a predominantly cardiac or mixed phenotype according to genotype category

From: Clinical and genetic profile of patients enrolled in the Transthyretin Amyloidosis Outcomes Survey (THAOS): 14-year update

 

ATTRwt amyloidosis (n = 1142)

Cardiac mutations (n = 307)

Other mutations (n = 718)

Heart failure, n (%)

1012 (88.6)

283 (92.2)

449 (62.5)

NYHA functional classa, n (%)

   

 I

107 (10.5)

27 (9.5)

78 (17.4)

 II

612 (59.8)

140 (49.3)

220 (49.1)

 III

278 (27.2)

102 (35.9)

133 (29.7)

 IV

26 (2.5)

15 (5.3)

17 (3.8)

Abnormal ECG, n (%)

947 (82.9)

218 (71.0)

415 (57.8)

Atrial fibrillation/flutter, n (%)

420 (36.8)

56 (18.2)

53 (7.4)

Pacemaker implanted, n (%)

179 (15.7)

26 (8.5)

33 (4.6)

ICD implanted, n (%)

59 (5.2)

16 (5.2)

12 (1.7)

NT-proBNP (pg/mL), n

775

127

253

 Median (10th, 90th percentile)

2573.0 (731.0, 9041.0)

2648.0 (664.0, 8889.0)

1557.0 (213.0, 8633.0)

Troponin I (ng/mL), n

133

57

58

 Median (10th, 90th percentile)

0.1 (0.0, 0.3)

0.1 (0.0, 0.5)

0.1 (0.0, 0.6)

Troponin T (ng/mL), n

626

86

215

 Median (10th, 90th percentile)

0.1 (0.0, 0.2)

0.1 (0.0, 0.3)

0.0 (0.0, 0.1)

  1. Cardiac mutations include Val122Ile, Leu111Met, Thr60Ala, and Ile68Leu. Other mutations include Val30Met and non-Val30Met excluding cardiac mutations
  2. aDenominator for NYHA functional class is the total of non-missing records
  3. ATTRwt amyloidosis = wild-type transthyretin amyloidosis; ECG = electrocardiogram; ICD = implantable cardioverter-defibrillator; NP-proBNP = N-terminal pro-B-type natriuretic peptide; NYHA = New York Heart Association; SD = standard deviation