Pseudo-delta wave occurred in 5.5% of Anderson-Fabry patients and was linked to larger left atrial diameter, QRS fragmentation, and pathological QTc, not short PR.
What is the prevalence of the pseudo-delta wave in Anderson-Fabry disease and is it associated with a short PR interval or disease stage?
The pseudo-delta wave is a rare but identifiable ECG feature in Anderson-Fabry disease that reflects intraventricular conduction tissue disease rather than an accessory pathway.
Absolute Event Rate: 0% vs 0%
Abstract Background Electrocardiographic (ECG) abnormalities in Anderson-Fabry disease (AFD) result from progressive glycosphingolipid accumulation in the myocardium. A hallmark finding is PR interval shortening, which can complicate the differential diagnosis with ventricular pre-excitation, potentially leading to unnecessary electrophysiological studies (EPS). The presence of an initial slurred QRS complex, mimicking a delta wave, further challenges differentiation. Aims This study aimed to assess the prevalence of the pseudo-delta wave in AFD, evaluate its association with a short PR interval, identify related clinical and instrumental features, and determine its correlation with disease stage. Methods We conducted a retrospective multicenter study including 220 AFD patients. Clinical, echocardiographic, and 12-lead ECG data were analyzed. The pseudo-delta wave was defined as initial QRS slurring, altering the QRS slope before the R-wave peak in at least two contiguous leads. Results The study population consisted of 220 patients, including 89 males (40%), with a median age of 44 years (IQR 8–86). The classical phenotype was observed in 148 patients (67%). A pseudo-delta wave was detected in 12 patients (5.5%), both in those with (n=10) and without (n=2) left ventricular hypertrophy (LVH) on echocardiography. Compared to patients without a pseudo-delta wave (see Table), those with this feature had a significantly larger left atrial diameter (40 mm vs. 35 mm, p=0.007), a higher prevalence of QRS complex fragmentation (42% vs. 11%, p=0.011), and a pathological QTc interval (35% vs. 25%, p=0.013). Conversely, no significant differences were found in the presence of a short PR interval (8% vs. 9%, p=1) or PR duration (140 ms vs. 143 ms, p=0.852). There were also no differences in age, sex, or AFD phenotype. Conclusion The pseudo-delta wave is a rare but identifiable feature in AFD. It is more frequently associated with specific ECG and echocardiographic abnormalities characteristic of intermediate to advanced stages of the disease. Moreover, its inconsistent association with a short PR interval supports the hypothesis that it does not result from the presence of an atrioventricular accessory pathway but rather reflects glycosphingolipid accumulation in the intraventricular conduction tissue.
Serratore et al. (Sat,) reported a other. Pseudo-delta wave occurred in 5.5% of Anderson-Fabry patients and was linked to larger left atrial diameter, QRS fragmentation, and pathological QTc, not short PR.