Allied Professionals (Non-physician submissions only) -> Clinical Research D-MP03 - From Patients to Procedures: The Spectrum of Care (ID 23) Moderated ePoster

D-MP03-04 - ECG Monitoring in Patients with Rare Cardiac Diseases (ID 33)

Disclosure
 Z. Fingrova: Nothing relevant to disclose.

Abstract

Background: Patients with rare cardiac diseases such as cardiac amyloidosis, sarcoidosis and Anderson-Fabry disease (FD) often develop clinically relevant arrhythmias.
Objective: The aim of the study was to identify and quantify early rhythm disorder on 24hr Holter monitoring.
Methods: We prospectively analyzed 24 hr Holter ECG monitoring in 35 consecutive patients (18 males; median age 71 (57-84) y) diagnosed with cardiac amyoidosis, 27 patients (median age 38males; 22 - 54 y) confirmed with sarcoidosis and finally 88 (47 males; median age 53 (19 - 79 y) patients with FD.
Results: Out of all patients, persistent atrial fibrillation was detected in 2 (6%) patients with amyloidosis and 4 (5%) with FD. 3 (3%) FD patients were permanently paced because of advanced AV block. Patients with sarcoidosis manifested significantly lower rates of supraventricular premature beats (SPB) and short episodes of supraventricular tachycardia (SVT); p < 0.05, Table. Patients with amyloidosis and FD manifested similar occurrence of SPB. Median of ventricular premature beats (VPB) per 24hr was the lowest in patients with sarcoidosis (p < 0.05). However, proportion of patients with significant number of VPB was comparable across all three study groups, Table. In patients with FD and amyloidosis were found episodes of non-sustained ventricular tachycardia (nsVT). Other significant arrhythmias were not detected.
Conclusion: According to results of ECG monitoring, patients with amyloidosis and Fabry disease manifest more advanced cardiac involvement than patients with sarcoidosis. Regular follow-up with Holter ECG monitoring is recommended in all tested diseases.
Study population
SPB / 24 hr> 50 SPB / 24 hrSalvo SPBVPB / 24 hr> 50 VPB / 24 hrnsVT
Amyloidosis79 (24 -274)25 (71%)8 (23%)34 (4 -158)15 (43%)3 (9%)
Sarcoidosis

2

(1 - 9)

5

(19%)
1 (4%)

2

(0 - 13)

6

(22%)
0 (0%)
FD

45

(5 - 160)
34 (39%)9 (10%)

37

(1 - 48)

21

(24%)
11 (13%)

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