The Association of Atrial Tachyarrhythmias with Isolated Atrial Amyloid Disease: Preliminary Observations in Autopsied Heart SpecimensAriyarajah V.a · Steiner I.c · Hájková P.c · Khadem A.b · Kvasnicka J.d · Apiyasawat S.e · Spodick D.H.f
Divisions of aCardiology and bElectrophysiology, Department of Medicine, St. Boniface General Hospital, University of Manitoba, Winnipeg, Man., Canada; cFingerland Department of Pathology and dFirst Department of Internal Medicine, Charles University Faculty of Medicine and Faculty Hospital, Hradec Králové, Czech Republic; eDivision of Electrophysiology, Department of Medicine, Columbia University College of Physicians and Surgeons, New York, N.Y., and fDivision of Cardiology, Department of Medicine, Saint Vincent Hospital, University of Massachusetts, Worcester, Mass., USA
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Objective: Isolated atrial amyloidosis (IAA) is associated with atrial tachyarrhythmias. However, only a few studies have appraised atrial tachyarrhythmias and atrial depolarization abnormalities in connection with high-grade IAA. We conducted a collaborative retrospective study to assess this association. Methods: One hundred consecutive autopsied hearts were studied histologically for IAA. To increase the specificity for atrial depolarization abnormalities in this preliminary study, we excluded those specimens with intermediate amyloid involvement, i.e. IAA grades 1 and 2 (grade 0 = absent or trivial deposits; grade 1 = small deposits; grade 2 = moderate deposits; grade 3 = dense, large deposits). We then screened for baseline, premortem electrocardiograms (ECGs) to assess rhythm. In those with sinus rhythm, the P wave axis, duration, dispersion and terminal force in V1 were determined under magnification. Results: Of the 27 premortem ECGs corresponding to the autopsy specimens with grades 3 (sample) or 0 (controls) IAA, 9 showed sinus rhythm, 13 showed atrial fibrillation, 1 showed atrial flutter and 4 were uninterpretable. Fourteen autopsied hearts (52%) were positive for grade 3 IAA. Ten of those had atrial tachyarrhythmias (9 atrial fibrillation and 1 atrial flutter) compared to 4 of the 13 hearts with grade 0 IAA (72 vs. 31%, respectively; p = 0.03). Although there was excellent interobserver agreement using intraclass correlation coefficients, there were no significant differences in P wave measurements among the small number of patients with sinus rhythm for grade 3 versus grade 0 IAA. Conclusion: In a collaborative, preliminary, pilot assessment of autopsied hearts for which premortem ECGs were necessarily screened retrospectively, significantly more hearts with high-grade IAA were associated with atrial tachyarrhythmias compared to those with low-grade IAA. A larger study with an appropriately matched autopsy control group is needed to confirm these and previous observations.
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