PerCar - Perinatal Cardiology

Pagine ed articoli di informazioni sulle cardiopatie congenite dal feto al giovane adulto. Potete usare questa sezione per potere interagire con i medici

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


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.

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).

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.

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.

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