Who has discovered ARVC/D ? (Barry Maron Cardiostim 2008)

For the sake of History

The introductory historical chapter by Thiene Nava and Marcus of the recent book Arrhythmogenic RV Cardiomyopathy/Dysplasia needs at its turn to be strongly clarified (1) !

The authors are quoting the first description of Lancisi concerning a clinico-pathological report of a family with obvious ARVD. However, I found in the work of Leonardo da Vinci the first impressive drawing representing the body of a young woman open from sternum to pubis showing the whole internal anatomy. The heart is cut on a four cavity chamber view with normal left ventricle and septum. In sharp contrast the right ventricle shows a major dilatation associated with definite thinness and segmental irregularity. A more complete description of this famous picture is described in the Website www.guyfontaine.com

The authors are also quoting one of the Dalla Volta's interesting original papers reported in the American Heart Journal in 1961, indicating that one of these patients survived 35 years before heart transplant (2). A slice of the ventricle at the junction of a thick moderator band to the septum shows on Fig.3 of this book, a typical pathologic pattern of end stage ARVD.

Following is the surprising paragraph:

“Finally, some historical note. It was in 1961 that Pr Sergio Dalla Volta from the University of Padua reported cases with “Auricularization of the right ventricular pressure” with an amazing fibrofatty, nonischemic pathology of the right ventricle [27]. One of those patients survived until 1995, and underwent transplantation due to end-stage cardiac failure. Interestingly the heart specimen showed severe right ventricular enlargement with nearly normal left ventricle (Fig.3).”

Dalla Volta 1961 paper could not report on the "fibrofatty replacement" of the right ventricle since the specimen was only available after heart transplant 35 years later!

In addition, the two patients of the 1961 paper are classified by Dalla Volta as fibroblastic cardiomyopathy for the first and myocardial infarction with RV extension for the second confirmed by autopsy (2). Therefore, it is not correct to say that the specimen reported in Fig.3 pertain one of these two patients!

 However, the origin of this interesting specimen is related to another patient reported 4 years later by the same first author in a different paper (3). In this second paper the case N°4 shows on the ECG inverted T waves in the right precordial leads and parietal bloc with prolongation of QRS duration in lead V1 and V2 as reproduced below.

Figure legend : ECG : Dipped P wave in Leads II, aVF and V1. Transseptal subepicardial ischemia
(Click on the ECG to enlarge it and even further with the glass magnifier. Experts will discover Epsilon waves on other leads not stressed in Yellow !)

 

This description suggests that Dalla Volta was thinking of another case of ischemic cardiomyopathy extending to the right ventricle as reported in the 1961 paper.

In Conclusion Dalla Volta described 4 cases of a “new clinical entity” with auricularization of the pressure curves in the right ventricle corresponding to a new category of cardiomyopathy.

He did not saw the histopathologic pattern which is the structural background of ARVD, he was not intrigued by the ECG abnormality of parietal block in the right ventricle in sinus rhythm and the nature  of ventricular arrhythmias in the only case who presented this rhythm disorder !

 References :

(1) Thiene G, Nava A, Marcus FI.

Introduction: Arrhythmogenic Right Ventricular Cardiomyopathy/Dysplasia clarified

In - Arrhythmogenic Right Ventricular Cardiomyopathy / Dysplasia

Marcus FI, Nava A, Thiene G Eds.

Springer-Verlag Italia 2007 p.1

(2) Dalla Volta S, Battaglia G, Zerbini E

“Auricularization” of right ventricular pressure curve.

Amer Heart J 1961:61;25-33 

(3) Dalla Volta S, Fameli O, Maschio G

Le Syndrome clinique d’auricularisation du ventricule droit (A propos de 4 cas personnels)

Arch Mal Cœur Vaiss 1965 : 58 ; 1129-53

After my complains Dr Marcus indicated that he has not reviewed this first chapter but that it will be corrected in the next edition of the book.

In addition, Dr Marcus always explains in his lectures how he met GF and spent a sabbatical year in 1980 in Paris to better study this interesting new clinical concept. This lead to the classical paper Right Ventricular Dysplasia published in Circulation in 1982.