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(Circulation. 2005;111:1199-1201.)
© 2005 American Heart Association, Inc.

Special Report

25th Anniversary of the International Long-QT Syndrome Registry

An Ongoing Quest to Uncover the Secrets of Long-QT Syndrome

Arthur J. Moss, MD; Peter J. Schwartz, MD

From the Cardiology Unit of the Department of Medicine, University of Rochester Medical Center, Rochester, NY (A.J.M.), and the Department of Cardiology, IRCCS Policlinico S. Matteo and University of Pavia, Pavia, Italy (P.J.S.).

Correspondence to Arthur J. Moss, MD, Heart Research Follow-up Program, Box 653, University of Rochester Medical Center, Rochester, NY 14642. E-mail heartajm@heart.rochester.edu

Key Words: long-QT syndrome • electrocardiography • electrophysiology • genetics

An extract of the first 250 words of the full text is provided, because this article has no abstract.

	Introduction

 
Today, it is exceptional to find a major cardiology congress without a session devoted to the long-QT syndrome (LQTS), but it was quite different 25 years ago when the minuscule knowledge about LQTS was paralleled only by the minute number of investigators interested in what seemed to be little more than a medical oddity. Partly by chance, the two of us had actually and independently developed a rather burning interest and curiosity for this often lethal hereditary disorder that is spiced by several unique features.^1–4 We joined forces with the goal of unraveling this mysterious disease by establishing a prospective International Registry for LQTS. The main objectives were those to gain insight into the natural history, clinical course, and efficacy of current and novel therapies. When molecular biology techniques matured to the point of making possible the identification of disease-causing genes and disease-causing mutations, what became essential was the availability of numerous and well-developed clinical pedigrees providing clear separation between "affected" and "nonaffected" individuals. This is what the Registry was able to provide and where it played a decisive role in sharing with molecular biologists the ideal material for their analysis.

In 1979, when the Registry was established, it did not escape us that this long-term project was likely to contribute to a better understanding and management of LQTS. Quite frankly, however, we did not anticipate the explosion of knowledge that would result from the genetic and molecular findings of the 1990s and the central role that the Registry, with . . . [Full Text of this Article]

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