Table 1.
Demographic, clinical and biochemical characteristics at baseline in all patients and in patients classified according to non‐invasive fibrosis assessment using cardiac magnetic resonance imaging and serum carboxy‐terminal propeptide of procollagen type I
| Total (n = 209) | LGE− (n = 144) | LGE+ (n = 65) | P‐value | |
|---|---|---|---|---|
| Demographics | ||||
| Age at diagnosis (years) | 54 ± 13 (18–80) | 54 ± 13 | 54 ± 12 | NS |
| Male sex | 136/209 (65%) | 93/144 (65%) | 43/65 (66%) | NS |
| Heart rate (bpm) | 75 ± 17 | 72 ± 13 | 76 ± 15 | NS |
| Hypertension | 84/209 (40%) | 56/144 (39%) | 28/65 (43%) | NS |
| Diabetes mellitus | 21/209 (10%) | 16/144 (11%) | 5/65 (8%) | NS |
| Atrial fibrillation | 52/209 (25%) | 33/144 (23%) | 16/65 (25%) | NS |
| LBBB | 52/209 (25%) | 36/144 (25%) | 16/65 (25%) | NS |
| NSVT | 59/209 (28%) | 34/144 (24%) | 25/65 (39%) | 0.032 |
| Duration of symptoms (months) | 2 [0–5] | 2 [0–4] | 2 [0–7] | NS |
| Presentation in outpatient clinic setting | 165/209 (79%) | 115/144 (80%) | 50/65 (77%) | NS |
| Genetic diagnostic yield | ||||
| Core panel + TTN | 136/209 (65%) | 95/144 (66%) | 41/65 (63%) | NS |
| Pathogenic mutation | 29/136 (14%) | 18/95 (19%) | 11/41 (27%) | NS |
| TTN | 16/29 (8%) | 8/18 (44%) | 8/11 (73%) | NS |
| LMNA | 3/29 (1%) | 1/18 (6%) | 2/11 (18%) | NS |
| Presentation | ||||
| Family history of DCM | 35/209 (17%) | 23/144 (16%) | 12/65 (19%) | NS |
| NYHA class III or IV | 61/209 (29%) | 34/144 (34%) | 27/65 (42%) | 0.013 |
| Out of hospital cardiac arrest | 13/209 (6%) | 8/144 (6%) | 5/65 (8%) | NS |
| Lab | ||||
| AST (U/L) | 24 [19–33] | 24 [18–32] | 25 [20–36] | NS |
| ALT (U/L) | 26 [20–35] | 26 [20–34] | 29 [21–38] | NS |
| Alkaline phosphatase (U/L) | 82 [64–99] | 83 [67–100] | 79 [63–95] | NS |
| eGFR (mL/min/1.73 m2) | 72 [61–86] | 75 [64–88] | 68 [56–80] | 0.008 |
| NT‐proBNP (ng/L) | 557 [191–1636] | 367 [128–1228] | 1032 [360–3078] | <0.001 |
| CRP (mg/L) | 3 [1–7] | 3 [2–6] | 3 [0–9] | NS |
| hs‐TnT (ng/L) | 11 [7–25] | 10 [6–18] | 19 [10–42] | 0.001 |
| PICP (ng/mL) | 78 [64–102] | 77 [63–97] | 85 [66–110] | NS |
| PIIINP (ng/mL) | 4 [3.2–6.4] | 4 [3.1–5.6] | 5 [3.5–7.4] | 0.005 |
| Medication | ||||
| Beta‐blocker | 174/209 (83%) | 122/144 (85%) | 52/65 (80%) | NS |
| ACE inhibitor/ARB | 185/209 (89%) | 124/144 (86%) | 58/65 (89%) | NS |
| Loop diuretic | 112/209 (54%) | 73/144 (51%) | 39/65 (60%) | NS |
| Aldosterone antagonist | 74/209 (35%) | 49/144 (34%) | 25/65 (39%) | NS |
| CMR | ||||
| LVEDVi (mL/m2) | 136 ± 53 | 135 ± 56 | 138 ± 47 | NS |
| LVESVi (mL/m2) | 92 ± 50 | 90 ± 53 | 97 ± 45 | NS |
| LVEF (%) | 34 ± 12 | 35 ± 12 | 32 ± 12 | 0.04 |
| Stroke volume, indexed (mL/m2) | 43 ± 14 | 44 ± 14 | 41 ± 14 | NS |
| LV mass index (g/m2) | 75 ± 27 | 74 ± 28 | 76 ± 23 | NS |
| RVEDVi (mL/m2) | 89 ± 32 | 88 ± 34 | 89 ± 26 | NS |
| RVESVi (mL/m2) | 48 ± 27 | 48 ± 29 | 49 ± 23 | NS |
| RVEF (%) | 47 ± 14 | 47 ± 14 | 45 ± 13 | NS |
| LAVI (mL/m2) | 54 ± 24 | 53 ± 24 | 56 ± 22 | NS |
| Endomyocardial biopsy | ||||
| Cardiac inflammation | 71/209 (34%) | 55/132 (42%) | 16/64 (25%) | 0.027 |
| CD3 (cells/mm2) | 6 [3–9] | 6 [4–10] | 5 [3–7] | 0.003 |
| CD45 (cells/mm2) | 9 [4–12] | 10 [6–14] | 8 [5–11] | 0.020 |
| Collagen volume fraction (%) | 7 [4–11] | 6 [4–10] | 9 [4–14] | 0.009 |
ACE, angiotensin‐converting enzyme; ALT, alanine aminotransferase; ARB, angiotensin receptor II blocker; AST, aspartate aminotransferase; CMR, cardiac magnetic resonance; CRP, C‐reactive protein; DCM, dilated cardiomyopathy; eGFR, estimated glomerular filtration rate; hs‐TnT, high‐sensitivity troponin T; LAVI, indexed left atrial volume; LBBB, left bundle branch block; LGE, late gadolinium enhancement; LVEDVi, indexed left ventricular end‐diastolic volume; LVEF, left ventricular ejection fraction; LVESVi, indexed left ventricular end‐systolic volume; NSVT, non‐sustained ventricular tachycardia; NT‐proBNP, N‐terminal pro B‐type natriuretic peptide; NYHA, New York Heart Association; PICP, carboxy‐terminal propeptide of procollagen type I; PIIINP, amino‐terminal propeptide of procollagen type III; RVEDVi, indexed right ventricular end‐diastolic volume; RVEF, right ventricular ejection fraction; RVESVi, indexed right ventricular end‐systolic volume.