Electroanatomical features of biopsy-proven cardiac amyloidosis

FUNDING ACKNOWLEDGEMENTS: Type of funding sources: None. BACKGROUND: Cardiac amyloidoses (CA) are an increasingly recognized group of infiltrative cardiomyopathies associated with high risk of heart failure, thromboembolic events, arrhythmias, and sudden death. Endomyocardial biopsy may be required to differentiate the amyloid type (mainly, Immunoglobulin light chain [AL] versus transthyretin-related [ATTR]) in some cases. PURPOSE: to provide a first description of electroanatomical characteristics of amyloid cardiomyopathy, and relate them to endomyocardial biopsy findings. METHODS: we enrolled ten consecutive patients (median age, 68 [63-77]; male, 50%) in an observational, retrospective study. All of them had a clinical diagnosis of CA, but a diagnosis of CA type was hampered by the presence of inconclusive or discordant laboratory-imaging findings (abnormal serum free light chain assay and positive bone scintigraphy, n=5; ambiguous imaging results, n=4; abnormal serum free light chain assay and TTR gene mutation, n=1). Therefore, all patients underwent right ventricular high-density electroanatomical mapping (EAM) using a multipolar catheter (Advisor HD Grid, Abbott), and EAM-guided endomyocardial biopsy (EMB). We recorded electrogram features at EMB sampling site, and explored their correlation with histological findings with mixed effect models. RESULTS: The clinical, electroanatomical, and histological features of enrolled patients according to the EMB-proven type of CA (AL, n=6; ATTR, n=4) are resumed in Table. Electrogram amplitudes in both the bipolar and the unipolar configuration were generally normal both in the overall right ventricle, and at EMB sites. We found a significant correlation between both unipolar and bipolar electrogram amplitude and the percentage of both amyloid tissue (p<0.01 and p=0.016, respectively) and fibrous tissue (both p<0.01) at EMB (Figure). We observed the strongest linear association (i.e., highest R2) between unipolar electrogram amplitude and amyloid tissue, as well as between bipolar electrogram amplitude and fibrous tissue. On the other hand, electrogram duration and the number of electrogram peaks were unrelated to histological findings. The unipolar voltage cutoff that best identified regions with >15% amyloid tissue infiltration according to Youden index was 9.1 mV (sensitivity, 43%; specificity, 100%; accuracy, 77%). CONCLUSION: CA is associated with normal electrogram amplitudes using ahigh density mapping catheter and conventional voltage cutoffs. Nevertheless, unipolar electrogram amplitude is strongly related to the extent of amyloid infiltration, and unipolar voltage<9.1 mV is 100% specific for >15% amyloid tissue deposition. [Figure: see text] [Figure: see text]