1Medizinische Hochschule Hannover Kardiologie und Angiologie Hannover, Deutschland; 2Medizinische Hochschule Hannover Hämatologie und Onkologie Hannover, Deutschland; 3Medizinische Hochschule Hannover Zentrum Innere Medizin, Abteilung Nephrologie Hannover, Deutschland; 4Medizinische Hochschule Hannover Neurologie Hannover, Deutschland
Background: Cardiac amyloidosis (CA) leads to progressive heart failure if left untreated. Most common forms are cardiac transthyretin (ATTR-) and light chain (AL-) amyloidosis. Awareness and number of patients have risen due to improved diagnostics and treatment options. A structured and standardized clinical work-up and follow-up ensures optimal patient care and enables analysis of clinical routine registry data for better understanding of the disease, and to identify patients at high-risk for clinical worsening and poor outcomes. The aim of this analysis of medical data at first presentation in the amyloidosis center is to characterize clinical parameters and outcomes in patients with CA primarily located in Lower Saxony.
Methods: Patients referred for highly suspected or confirmed CA were enrolled in this registry. All data obtained within clinical routine (e.g. patient history, clinical examination, ECG, echocardiography, laboratory data) at first presentation (baseline) were analyzed using descriptive statistics. All-cause mortality is reported by Kaplan-Meier survival analysis.
Results: 188 patients were presented to our center between 2018 -2023, 150 with ATTR-, 27 with AL- and 1 with AA-amyloidosis. Of ATTR patients, 102 (68%) had wtATTR, 18 (12%) hATTR, and 30 ATTR without known genotype (20%). The most common mutation detected in hATTR was Val50Met (n=10). The majority of patients were male (wtATTR 92%; hATTR 67%, AL 62%). CA was diagnosed in 145 patients. Of those, hATTR and AL patients were younger than wtATTR patients. Cardiac biomarkers were higher in wtATTR vs. hATTR as well as in AL vs. all ATTR patients, and renal function was better in hATTR vs. ATTRwt (Table 1). Patients with AL-amyloidosis and ATTR-amyloidosis not genotyped showed the worst survival in Kaplan-Meier-survival-analysis (Figure 1, log-rank-test, p<0.01) most likely based on advanced cardiac amyloidosis within these populations.
Conclusions: Structured and standardized clinical work-up and follow-up data of patients with different forms of cardiac amyloidosis provide important information on disease severity and outcomes. This should enable better risk-stratification and optimal patient care which has to be proven in further studies.
Table 1. Baseline characteristics of patients with CA
|
Variable |
ATTRwt, n=85 |
hATTR, n=10 |
ATTR (not genotyped) n=26 |
ATTR all, n=121 |
AL, n=24 | |||
|
| ||||||||
|
Age [years] |
79 (54-89) |
67 (35-88) |
81 (57-89) |
78 (54-89) |
67 (40-85) | |||
|
NYHA class ≥ 3 |
22% |
29% |
67% |
31% |
54% | |||
|
Atrial Fibrillation |
76% |
25% |
71% |
65% |
25% | |||
|
Pacemaker |
19% |
13% |
8% |
16% |
0% | |||
|
NT-proBNP [ng/l] |
2203 (218-21741) |
929 (444-2763)** |
3412 (65-28698) |
2646 (65-28698) |
10837 (117-35000)## | |||
|
cTroponin T [ng/l] |
55 ± 32 |
37 ± 14* |
58 ± 25 |
54 ± 30 |
127 ± 93# | |||
|
eGFR [ml/min/1.73] |
55 ± 17 |
67 ± 24* |
45 ± 18 |
54 ± 18 |
52 ± 27 | |||
|
LVEF [%] |
48 ± 10 |
52 ± 5 |
44 ± 14 |
47 ± 11 |
48 ± 13 | |||
|
LV GLS [%] |
-12 ± 5 |
-15 ± 5 |
-12 ± 5 |
-12 ± 5 |
-12 ± 5 | |||
|
IVSEd [cm] |
1.7 ± 0.3 |
1.7 ± 0.2 |
1.6 ± 0.3 |
1.6 ± 0.3 |
1.4 ± 0.2 | |||
|
LVEDD [cm] |
4.7 ± 0.7 |
3.8 ± 1 |
4.7 ± 0.6 |
4.6 ± 0.7 |
4 ± 0.9 | |||
*P<0.05 vs. ATTRwt, **P<0.01 vs. ATTRwt, #P<0.05 vs. ATTR all, ##P<0.01 vs. ATTR all