Key result
In patients with NPPA gene mutation, progression to atrial standstill was associated with extensive right atrial low voltage (99.5%) and scarring (57.5%) on electroanatomic mapping and LGE-MRI.
Why the study?
How do electroanatomic mapping and LGE-MRI characterize the evolution of the arrhythmic substrate in patients with NPPA gene mutation-induced arrhythmogenic atrial cardiomyopathy?
Population
5 patients with isolated arrhythmogenic atrial cardiomyopathy caused by Natriuretic Peptide Precursor A gene…
Design
Case_series
Authors
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Hypothesis-generating for scarring-driven progression in NPPA cardiomyopathy; requires validation before influencing management.
Observational (n=5)
How do electroanatomic mapping and LGE-MRI characterize the evolution of the arrhythmic substrate in patients with NPPA gene mutation-induced arrhythmogenic atrial cardiomyopathy?
Progressive atrial scarring and fibrosis constitute the structural substrate for the evolution of atrial arrhythmias to atrial standstill in NPPA-related genetic atrial cardiomyopathy.
Disertori et al. (2014) conducted an observational in Isolated arrhythmogenic atrial cardiomyopathy (n=5). NPPA gene mutation was evaluated on Atrial arrhythmic substrate (low voltage and atrial wall scarring). In patients with NPPA gene mutation, progression to atrial standstill was associated with extensive right atrial low voltage (99.5%) and scarring (57.5%) on electroanatomic mapping and LGE-MRI.
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