Arrhythmogenic right ventricular cardiomyopathy/dysplasia clinical presentation and diagnostic evaluation: results from the North American Multidisciplinary Study.
BACKGROUND
Prior reports on patients with arrhythmogenic right ventricular cardiomyopathy/dysplasia (ARVC/D) focused on individuals with advanced forms of the disease. Data on the diagnostic performance of various testing modalities in newly identified individuals suspected of having ARVC/D are limited.


OBJECTIVE
The purpose of the Multidisciplinary Study of Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia was to study the clinical characteristics and diagnostic evaluation of a large group of patients newly identified with ARVC/D.


METHODS
A total of 108 newly diagnosed patients with suspected ARVC/D were prospectively enrolled in the United States and Canada. The patients underwent noninvasive and invasive tests using standardized protocols that initially were interpreted by the enrolling center and adjudicated by blind analysis in six core laboratories. Patients were followed for a mean of 27 +/- 16 months (range 0.2-63 months).


RESULTS
The clinical profile of these newly diagnosed patients differs from the profile of reported patients with more advanced disease. There was considerable difference in the initial and final classification of the presence of ARVC/D after the diagnostic tests were evaluated by the core laboratories. Final clinical diagnosis was 73 affected, 28 borderline, and 7 unaffected. Individual tests agreed with the final diagnosis in 50% to 70% of the 73 patients with a final classification of affected.


CONCLUSIONS
The clinical profile of 108 newly diagnosed probands with suspected ARVC/D indicates that a combination of diagnostic tests is needed to evaluate the presence of right ventricular structural, functional, and electrical abnormalities. Echocardiography, right ventricular angiography, signal-averaged ECG, and Holter monitoring provide optimal clinical evaluation of patients suspected of ARVC/D.
Marcus
Frank I
FI
Section of Cardiology, University of Arizona, 1501 N. Campbell Avenue, Tucson, Arizona 85724-0001, USA. fmarcus@email.arizona.edu
Zareba
Wojciech
W
Calkins
Hugh
H
Towbin
Jeffrey A
JA
Basso
Cristina
C
Bluemke
David A
DA
Estes
N A Mark
NA
Picard
Michael H
MH
Sanborn
Danita
D
Thiene
Gaetano
G
Wichter
Thomas
T
Cannom
David
D
Wilber
David J
DJ
Scheinman
Melvin
M
Duff
Henry
H
Daubert
James
J
Talajic
Mario
M
Krahn
Andrew
A
Sweeney
Michael
M
Garan
Hasan
H
Sakaguchi
Scott
S
Lerman
Bruce B
BB
Kerr
Charles
C
Kron
Jack
J
Steinberg
Jonathan S
JS
Sherrill
Duane
D
Gear
Kathleen
K
Brown
Mary
M
Severski
Patricia
P
Polonsky
Slava
S
McNitt
Scott
S
D000328:Adult; D000792:Angiography; D019571:Arrhythmogenic Right Ventricular Dysplasia / Q000175:diagnosis*; D002170:Canada; D004452:Echocardiography; D004562:Electrocardiography; D015716:Electrocardiography, Ambulatory; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D014481:United States; D055815:Young Adult