Evaluation of Structural Progression in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

作者：Thomas P. Mast, Cynthia A. James, Hugh G Calkins, Arco J. Teske, Crystal Tichnell, Brittney A. Murray, Peter Loh, Stuart D. Russell, Birgitta K. Velthuis, Daniel Philip Judge, Dennis Dooijes, Ryan James Tedford, Jeroen F. van der Heijden, Harikrishna S. Tandri, Richard N.W. Hauer, Theodore P. Abraham, Pieter A. Doevendans, Anneline S.J.M. te Riele, Maarten Jan Cramer · 发表于：JAMA Cardiology · 年份：2017 · DOI：10.1001/jamacardio.2016.5034 · 被引用次数：75 · 研究领域：Cardiovascular Effects of Exercise、Trauma Management and Diagnosis、Eosinophilic Disorders and Syndromes

Importance: Considerable research has described the arrhythmic course of arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C). However, objective data characterizing structural progression, such as ventricular enlargement and cardiac dysfunction, in ARVD/C are relatively scarce. Objectives: To define the extent of structural progression, identify determinants of structural progression, and determine the association between structural progression and electrocardiographic (ECG) changes in patients with ARVD/C. Design, Setting, and Participants: In this cohort study, first- and last-available echocardiograms of 85 patients with ARVD/C fulfilling 2010 Task Force diagnostic criteria (TFC) from a transatlantic ARVD/C registry were retrospectively compared to assess structural disease progression. Right ventricular (RV) size and systolic function between baseline and last follow-up were compared. The RV size was determined by RV outflow tract dimension, and RV and left ventricular (LV) systolic function were determined by RV fractional area change (RV-FAC) and LV ejection fraction (LVEF), respectively. Multivariable logistic regression was used to study associations between baseline characteristics and the occurrence of structural progression. Main Outcomes and Measures: The main outcome was the change in variables indicating structural progression. Secondary outcomes were the correlation with electrical progression and identification of the association between baselin...