Arrhythmogenic Right Ventricular Dysplasia

Arrhythmogenic Right Ventricular Dysplasia :
Echocardiographic Findings in Patients

Meeting Task Force Criteria for Arrhythmogenic Right Ventricular Dysplasia
New Insights From the Multidisciplinary Study of Right Ventricular Dysplasia

Danita M. Yoerger, MD,* Frank Marcus, MD,† Duane Sherrill, PHD,† Hugh Calkins, MD,‡
Jeffery A. Towbin, MD,§ Wojciech Zareba, MD, PHD,
Michael H. Picard, MD,*

for the
Multidisciplinary Study of Right Ventricular Dysplasia Investigators
Boston, Massachusetts; Tucson, Arizona; Baltimore, Maryland; Houston, Texas; and Rochester, New York

OBJECTIVES

The purpose of this study was to quantify the echocardiographic abnormalities in probands
who were newly diagnosed with arrhythmogenic right ventricular dysplasia (ARVD).
BACKGROUND The diagnosis of ARVD remains challenging. The Multidisciplinary Study of Right
Ventricular Dysplasia was initiated to characterize the cardiac structural, clinical, and genetic
aspects of ARVD.
METHODS

Detailed echocardiograms were performed in 29 probands and compared with echoes from 29
normal control patients matched for age, gender, body size, and year of echo. Right atrial
(RA) and right ventricular (RV) chamber dimensions, RV regional function, and the presence
of morphologic abnormalities (hyper-reflective moderator band, trabecular derangement, and
sacculations) were assessed. The RV systolic function was calculated as RV fractional area
change (FAC).

RESULTS
The RV dimensions were significantly increased, and RV FAC was significantly decreased in
probands versus control patients (27.2
16 mm vs. 41.0
7.1 mm, p  0.0003).
The right ventricular outflow tract (RVOT) was the most commonly enlarged dimension in
ARVD probands (37.9
6.6 mm) versus control patients (26.2
4.9 mm, p  0.00001).
A RVOT long-axis diastolic dimension 30 mm occurred in 89% of probands and 14% of
controls. The RV morphologic abnormalities were present in many probands (trabecular
derangement in 54%, hyper-reflective moderator band in 34% and sacculations in 17%) but
not in controls.

CONCLUSIONS
Probands with ARVD have significant RA and RV enlargement and decreased RV function,
which can be easily assessed on standard echocardiographic imaging. These parameters
should be measured when ARVD is suspected and compared with normal values.

Journal of the American College of Cardiology Vol. 45, No. 6, 2005
© 2005
by the American College of Cardiology Foundation ISSN 0735-1097/05/$30.00
Published by Elsevier Inc. doi:10.1016/j.jacc.2004.10.070

Criteria for Diagnosis of Right Ventricular Dysplasia (1)

I. Global and/or regional dysfunction and structural alterations*

• Major
  • Severe dilatation and reduction of right ventricular ejection fraction
    with no (or only mild) left ventricular impairment
  • Localized right ventricular aneurysms (akinetic or dyskinetic areas
    with diastolic bulging)
  • Severe segmental dilatation of the right ventricle
• Minor
• Mild global right ventricular dilatation and/or ejection fraction
  reduction with normal left ventricle
• Mild segmental dilatation of the right ventricle
  Regional right ventricular hypokinesia

II. Tissue characterization of wall

• Fibrofatty replacement of myocardium on endomyocardial biopsy

III. Repolarization abnormalities

• Minor
• Inverted T waves in right precordial leads (V2 and V3) in people
  age 12 yrs, in absence of right bundle branch block

IV. Depolarization/conduction abnormalities

• Major
• Epsilon waves or localized prolongation (110 ms) of the QRS
  complex in right precordial leads (V1–V3)
• Minor
• Late potentials (signal-averaged ECG)

V. Arrhythmias

• Minor
• Left bundle branch block type ventricular tachycardia (sustained
  and nonsustained) by ECG, Holter, or exercise testing
  Frequent ventricular extrasystoles (1,000/24 h) (Holter)

VI. Family history

• Major
• Familial disease confirmed at necropsy or surgery
• Minor
• Family history of premature sudden death (35 yrs) due to
  suspected right ventricular dysplasia
• Familial history (clinical diagnosis based on present criteria)

Arrhythmogenic right ventricular dysplasia diagnosis: two major criteria or one major
and two minor criteria, or four minor criteria. *Detected by echocardiography,
angiography, magnetic resonance imaging, or radionuclide scintigraphy.
ECG  electrocardiogram.

(1) – McKenna WJ, Thiene G, Nava A, et al.
Diagnosis of arrhythmogenic
right ventricular dysplasia/cardiomyopathy.
Task Force of the Working
Group Myocardial and Pericardial Disease of the European
Society of Cardiology and of the Scientific Council on Cardiomyopathies
of the International Society and Federation of Cardiology. Br
Heart J 1994;71:215– 8.