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QUARTERLY FOCUS ISSUE: HEART FAILURE: RIGHT VENTRICULAR CARDIOMYOPATHY

Prevalence and Pathophysiologic Attributes of Ventricular Dyssynchrony in Arrhythmogenic Right Ventricular Dysplasia/Cardiomyopathy

Laurens F. Tops, MD^_*,, Kalpana Prakasa, MD^_*, Harikrishna Tandri, MD^_*, Darshan Dalal, MD^_*, Rahul Jain, MD^_*, Veronica L. Dimaano, MD^_*, David Dombroski, MD, Cynthia James, PhD^_*, Crystal Tichnell, MGC^_*, Amy Daly, MSc^_*, Frank Marcus, MD, Martin J. Schalij, MD, Jeroen J. Bax, MD, David Bluemke, MD, Hugh Calkins, MD^_* and Theodore P. Abraham, MD^_*,_*

^_* Division of Cardiology, Department of Medicine, Johns Hopkins Hospital, Baltimore, Maryland
Department of Radiology, Johns Hopkins Hospital, Baltimore, Maryland
Department of Cardiology, Leiden University Medical Center, Leiden, the Netherlands
Sarver Heart Center, University of Arizona College of Medicine, Tucson, Arizona

Manuscript received February 24, 2009; revised manuscript received April 6, 2009, accepted April 14, 2009.

^_* Reprint requests and correspondence: Dr. Theodore P. Abraham, Johns Hopkins University, 600 North Wolfe Street, Carnegie 568, Baltimore, Maryland 21287 (Email: tabraha3{at}jhmi.edu).

Objectives: This study sought to investigate the prevalence and mechanisms underlying right ventricular (RV) dyssynchrony in arrhythmogenic right ventricular dysplasia/cardiomyopathy (ARVD/C) using tissue Doppler echocardiography (TDE).

Background: An ARVD/C is characterized by fibrofatty replacement of RV myocardium and RV dilation. These pathologic changes may result in electromechanical dyssynchrony.

Methods: Echocardiography, both conventional and TDE, was performed in 52 ARVD/C patients fulfilling Task Force criteria and 25 control subjects. The RV end-diastolic and -systolic areas, right ventricular fractional area change (RVFAC), and left ventricular (LV) volumes and function were assessed. Mechanical synchrony was assessed by measuring differences in time-to-peak systolic velocity (T_SV) between the RV free wall, ventricular septum, and LV lateral wall. An RV dyssynchrony was defined as the difference in T_SV between the RV free wall and the ventricular septum, >2 SD above the mean value for control subjects.

Results: The mean difference in RV T_SV was higher in ARVD/C compared with control subjects (55 ± 34 ms vs. 26 ± 15 ms, p < 0.001). Significant RV dyssynchrony was not noted in any of the control subjects. Based on a cutoff value of 56 ms, significant RV dyssynchrony was present in 26 ARVD/C patients (50%). Patients with RV dyssynchrony had a larger RV end-diastolic area (22 ± 5 cm² vs. 19 ± 4 cm², p = 0.02), and lower RVFAC (29 ± 8% vs. 34 ± 8%, p = 0.03) compared with ARVD/C patients without RV dyssynchrony. No differences in QRS duration, LV volumes, or function were present between the 2 groups.

Conclusions: An RV dyssynchrony may occur in up to 50% of ARVD/C patients, and is associated with RV remodeling. This finding may have therapeutic and prognostic implications in ARVD/C.

Key Words: arrhythmogenic right ventricular dysplasia/cardiomyopathy • echocardiography • ventricular dyssynchrony

Abbreviations and Acronyms   ARVD/C = arrhythmogenic right ventricular dysplasia/cardiomyopathy   CI = confidence interval   ECG = electrocardiogram   LV = left ventricle/ventricular   RV = right ventricle/ventricular   RVEDA = right ventricular end-diastolic area   RVESA = right ventricular end-systolic area   RVFAC = right ventricular fractional area change   SE = strain echocardiography   TDE = tissue Doppler echocardiography   Tsv = time-to-peak systolic velocity

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J. Am. Coll. Cardiol. 2009 54: A26. [Full Text] [PDF]