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A Textbook on ARVD


ARRHYTHMOGENIC
RIGHT VENTRICULAR
CARDIOMYOPATHY / DYSPLASIA



Editors:

Andrea Nava, MD
Department of Cardiology
University of Padua Medical School
Padova, Italy

Lino Rossi, MD
Department of Pathological Anatomy
University of Milan
I.R.C.C.S. Ospedale Maggiore
Milano, Italy

and

Gaetano Thiene, MD
Department of Pathology
University of Padua Medical School
Padova, Italy


494 pages

International Congress Series No. 1122
ISBN 0 444 82447 2

1997, Elsevier Science B.V.
P.O. Box 211
1000 AE Amsterdam
The Netherlands



Foreword

Arrhythmogenic right ventricular cardiomyopathy (formerly named dysplasia) can be viewed as a lucky combination of clinical serendipity and well-planned anatomical, etiological and pathogenetic investigations.

In 1964, in two curiously forgotten papers (one in English, the second in French) by myself and associates, the clinical hemodynamic and pathologica1 findings of a cardiomyopathy were presented, neither coronary nor inflammatory in nature, affecting four young people, with arrhythmias, heart failure and sudden death in three of them. These patients had practically functional exclusion of the right ventricle, the pulmonary circulation being maintained by a strong atrial contraction, a spontaneous form of ”Fontan heart”. The main features were the electrocardiogram with inverted T wave in the right precordial leads, the absence of coronary artery disease and a patchy fibrosis of the right ventricle. In 1977 Fontaine et al. described, with more complete and extensive investigations, the main clinical features, among them the severe ventricular arrhythmias.

To date, the disease has been thoroughly studied in its pathological aspects by Thiene et al. within the large autopsy material of the well-known target project ”Juvenile Sudden Death” of the Veneto Region, Italy. The carefully oriented analysis of a large population of familiar forms has permitted the identification of the genetic loci by Nava and Danieli in Padua. In the same period the clinical echocardiographic and hemodynamic data, as well as the study of the type, and characteristics of the ventricular arrhythmias, have provided the standardization of the diagnostic criteria, through investigations mainly performed in Paris and Padua. A first insight into the molecular basis of arrhythmogenic right ventricular cardiomyopathy represents a further step to understanding the pathogenetic mechanisms of the disease.

Many problems are still opened: the real incidence of the syndrome, which does not appear to be a ”Venetian disease” as suggested by some authors; the link between the patients and healthy carriers; the controversy between an antenata1 or a postnatal onset; the identification of minor forms.

All these and other pending questions explain why this cooperative volume is the right book at the right moment, just 1 year after the first world congress on the topic, held in Paris.

It is not simply a hope that the next decade should clear most of the problems discussed in this superb book.

Sergio Dalla Volta, M.D.
Ordinary Professor of Cardiology
Director, Division of Cardiology
University of Padua Medical School
Padova, Italy






Contents


Introduction: past, present and future
F. I. Marcus

History of the disease
L. Rossi


Clinical aspects

Incidence and natural history
A. Nava, B. Bauce and L. Daliento

Clinical presentation and arrhythmias
G.F. Buja, M. Miorelli and P. Turrini

Sudden death
D. Corrado, C. Basso and G. Thiene

Left ventricular involvement, progression of the disease and prognosis
B. Pinamonti and F. Camerini

The disease in children and adolescents
L. Daliento, P. Turrini, M.C. Baratella and A. Nava


Pathology

Morbid anatomy
C. Basso, D. Corrado, L. Rossi and G. Thiene

Pathologic experience in the USA
R. Virmani, A.P. Burke and A. Farb

Pathologic experience Japan: outer layer involvement of the left ventriculum myocardium and selective preservation of the right ventricular trabeculae
S. Kawai, Y. Nakazato, R. Okada, N. Sueyoshi and T. Murai

Similarities and differences with Chagas’ heart disease
M. A. Rossi

Apoptosis and the heart
T.N. James

Ultrastructural observations
M. Sekiguchi, O. Kinoshita and Y. Yazaki


Etiopathogenesis

Pathobiology
M. Valente, F. Calabrese, A. Angelini, A.L.P. Caforio, C. Basso and G. Thiene

Familiar occurrence
A. Nava, A. Rampazzo, C. Villanova, M Muriago and G.F. Buja

A first insight into molecular genetics
G.A. Danieli, A. Nava and A. Rampazzo


Diagnostic Evolution

Diagnostic criteria
S. Sharma, A.S. Coonar and W.J. Mc Kenna

Electrocardiographic features
A. Nava, G.F. Buja and D. Corrado

Characteristics and differential diagnosis of arrhythmias
O.A. Obel and E. Rowland

Signal-averaged electrocardiogram
A.F. Folino, L. Dal Corso, L. Oselladore and A. Nava

Echocardiographic features
C. Blomstrom-Lundqvist

Diagnostic and evolutive aspects in concealed forms
R. Scognamiglio, G. Fasoli, M. Palisi, M. Marin and S. Dalla Volta

Contrast cineangiocardiography
C. Leclercq, H. Le Breton, P. Mabo and J. C. Daubert

Clinical and pathological relevance of endomyocardial biopsy
A. Angelini, C. Basso, P. Turrini and G. Thiene

Magnetic resonance imaging
T. Wichter, M. G. Lentschig, P. Reimer, M. Borggrefe and G. Breithardt

Radionuclide angiography
D. Le Guludec, M. Slama, M Faraggi, R. Frank, M. H. Bourguignon, G. Fontaine and G. Motte

Radionuclide imaging of sympathetic innervation
T. Wichter, H. Lereh, M. Schafers, M. Borpg refe, O. Sehober and G. Breithardt


Therapy

The treatment of ventricular arrhythmias
G. Fontaine, O. Zenati, J. Tonet, F. Hidden, C. Himbert and R. Frank

Cardioverter-defibrillator therapy
T. Wichter, D. Bocker, M. Borggrefe, D. Hammel, G. Breithardt and M. Block

Catheter ablation of ventricular tachycardia
T. Wichter, G. Hindricks, H. Kottkamp, G. Breithardt and M. Borggrefe

Surgical approaches
G. M. Guiraudon, C.M.J. Guiraudon and G.J. Klein


Peculiarities

Infundibular tachycardia
B.B. Lerman, K.M. Stein and S.M. Markowitz

Re-entry circuits and zones of slow conduction
W.G. Stevenson and P.L. Friedman

Apparent right bundle branch block, ST segment elevation and sudden cardiac death
J. Brugada, P. Brugada and R. Brugada

Syndrome of right bundle branch block, ST segment elevation and sudden death: evidence of an organic substrate
B. Martini, D. Corrado, A. Nava and G. Thiene

The Naxos disease
N.I. Protonotarios and A.A. Tsatsopoulou

MIBG scintigraphy in sportsmen
R. Bettini, M. Camerani, S. Severi and F. Furlanello

The disease in competitive athletes
F. Furlanello, A. Bertoldi, R. Bettini, G.B. Durante and G. Vergara


Index of authors

Keyword index

 
             
         
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Revisor:  Prof. G.A.Danieli.
Last update:  May, 6th, 2003.

 
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