Introduction Relations between voltage mapping and diagnosis or genetic background in patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. Objective We investigate if diagnosis or genetic background were linked to voltage mapping in ARVC. Method 70 patients with proved or suspected ARVC undergoing 3D endocardial mapping and genetic testing have been retrospectively included. Localisation and extension of bipolar low voltage areas were correlated to ARVC diagnosis and presence of a culprit mutation. Results 44/70 fulfilled ARVC Task Force criteria and 25/70 had culprit mutations. Endocardial (38/44 vs 16/26, p=0.02) and especially infero-lateral scars (31/44 vs 9/26, p=0.003) were more often present in patients fulfilling Task force criteria vs suspected ARVC, with larger scars (area 23±27 vs 8±11 cm2, p=0.04, perimeter 17±10 vs 11±7 cm, p=0.03) (sensitivity 86%). Mutated patients had more infero-lateral (19/25 vs 21/45, p=0.01), multiple (12/20 vs 11/34, p=0.04) and larger scars (perimeter 21±10 vs 12±7 cm, p=0.01) vs non mutated patients. In patients with ARVC diagnosed according to the Task Force criteria, there was a trend toward more infero-lateral (p=0.09) and larger scars (p=0.08) in mutated cases. PKP2-mutated cases tended to have less ourflow tract (p=0.08) and less multiple scars (p=0.09) vs other mutations. Conclusion 3D endocardial mapping could have an important role for ARVC diagnosis and may be able to detect minor forms with otherwise insufficiant criteria for diagnosis. More frequent and larger infero-lateral scars are present in mutated patients with bordeline differences according to the mutated genes. Funding Acknowledgement Type of funding source: None
We report the case of a reciprocating tachycardia using a purely retrograde decremental slow-conducting accessory pathway. High density 3D mapping of the atrial insertion reveals a large area of specific potentials at the level of the tricuspid annulus, which can be regarded as an accessory conduction network
Background The relations between voltage mapping and diagnosis or genetic background of patients with arrhythmogenic right ventricular cardiomyopathy (ARVC) have not been investigated so far. Objective To investigate if diagnosis or genetic background were linked to voltage mapping in ARVC. Method 97 patients with proved or suspected ARVC undergoing 3D endocardial mapping and genetic testing have been retrospectively included. Presence, localisation and extension of low voltage areas were correlated to ARVC diagnosis and presence of a culprit mutation. Results 68 patients (70%) fulfilled ARVC diagnosis according to the Task Force criteria and 43 (44%) had ARVC-causal mutations. 78 (80%) presented with some bipolar or unipolar endocardial scar. 60/ 78 patients with endocardial scar (77%) fulfilled the criteria for a definitive ARVC diagnosis versus 8/19 patients without scar (42%) (p=0.003). In the 68 patients with a definitive diagnosis of ARVC, the presence of endocardial scar was similar whether an ARVC-causal mutation was present or not (35/40 vs 25/28, p=ns). While there was slightly more infero-lateral scars in patients carrying a pathogenic genetic variant (34/40 vs 18/28, p=0.04), there was no difference for right ventricular outflow tract (24/40 vs 17/28) and apical scars (12/40 vs 11/28) or for multiple scars (26/35 vs 14/25 patients with scars). Scar extension was greater in patients with pathogenic variants (bipolar 12±10 vs 6±10%, p=0.02, and unipolar 22±13 vs 12±15%, p=0.01). Conclusion 3D endocardial mapping could have an important role for refining ARVC diagnosis. Trends for larger and more infero-lateral scars were observed in mutated patients, without difference according to the mutated genes. Funding Acknowledgement Type of funding sources: None.
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