Arrhythmogenic Cardiomyopathy: A Historical Overview

Abstract

4 Italy may rightly claim the discovery of arrhythmogenic cardiomyopathy (AC) as a distinct heredofamilial morbid entity. In 1738 Giovanni Maria Lancisi posthumously published in Naples the book De Motu Cordis et Aneurysmatibus. Lancisi was Professor of Anatomy at the University “La Sapienza” in Rome and personal doctor (archiater) of the Pope. In Chapter V of the book entitled De Hereditaria ad Cordis Aneurysmata Constitutione: De Cordis Prolapsu (On the Hereditary Predisposition to Cardiac Aneurysms: Cardiac Prolapse), he reported some examples of such morbid entities and described the history of a family with disease recurrence in 4 generations, all featuring signs and symptoms that were in keeping with what nowadays we call AC: palpitations, dilatation and aneurysms of the right ventricle (RV), heart failure, and sudden death (Fig. 1). Thus, the first description of AC dates back nearly two and a half centuries earlier than modern observations. The first recent pathologic description wasmade by Laennec, as reported in his bibliography by Saintignon in 1904. In Middlemarch, published in 1871 by George Eliot, the protagonist Dr Lydgate, talking to his patient, says “you are suffering from what is called fatty degeneration of the heart, a disease which was first described by Laennec. it is my duty to tell you that death from the disease is often sudden..” In 1905 Sir William Osler reported a case of a nearly 40-year-old man who died suddenly