This Article Free upon publication Full Text Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Alert me to new issues of the journal Download to citation manager Request Permissions Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Khairy, P. Articles by Landzberg, M. J. Search for Related Content PubMed Articles by Khairy, P. Articles by Landzberg, M. J. Related Collections Pediatric and congenital heart disease, including cardiovascular surgery

(Circulation. 2008;117:2311-2312.)
© 2008 American Heart Association, Inc.

Editorial

Adult Congenital Heart Disease

Toward Prospective Risk Assessment of a Multisystemic Condition

Paul Khairy, MD, PhD; Michael J. Landzberg, MD

From the Adult Congenital Heart Center, Montreal Heart Institute, Montreal, Canada (P.K.), and Boston Adult Congenital Heart (BACH) Service, Children’s Hospital Boston, Boston, Mass (P.K., M.J.L.).

Reprint requests to Dr Paul Khairy, Adult Congenital Heart Center, Montreal Heart Institute, 5000 Bélanger St, Montreal, Quebec, Canada, H1T 1C8. E-mail paul.khairy@umontreal.ca

Key Words: Editorials • heart defects, congenital • kidney • prognosis

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

	Introduction

 
It has long been recognized that manifestations of congenital heart disease extend beyond the cardiovascular system. The extracardiac ramifications of congenital heart disease are multifaceted and bidirectional. The intricate interplay between congenital heart physiology, bi- or univentricular heart function, early and/or ongoing cyanosis, surgical and residual sequelae, and multisystemic effects are increasingly appreciated. Examples include the well-characterized neurological, hematologic, respiratory, skeletal, and hepatic consequences of cyanosis¹; thromboembolic complications in surgically palliated univentricular hearts²; and dysregulation of the autonomic nervous system in various forms of congenital heart disease.³

Article p 2320

In the present issue of Circulation, Dimopoulos and associates⁴ provide original and important insights regarding the relationship between congenital heart disease and impaired renal function. They examine the prevalence of renal dysfunction across a wide-spectrum of adult congenital heart defects and explore implications on overall death rates.

	Methodological Considerations

 
Heterogeneity of the Study Population
A general and common challenge in congenital heart disease research that encompasses a broad spectrum of cardiac disorders is the complexity and marked heterogeneity of the study population. From a methodological perspective, there is often a trade-off between heterogeneity of the patient population and the precision of a given statistical inference. When the objective is to provide a global perspective on a novel association, such as renal dysfunction and death in adults with congenital heart disease, however, heterogeneity of the patient population may be advantageous. The nonrestrictive inclusion criteria enhance generalizability of the findings to a broad target population. Although the reported overall 9.3% prevalence of moderate to severe renal . . . [Full Text of this Article]