INTRODUCTION

We, the authors of this second Consensus Conference Report on Adult Congenital Heart Disease (ACHD), are grateful to the Canadian Cardiovascular Society (CCS) and its Council for the opportunity to update and assemble the document which follows.

Important advances have been made in the field of adult congenital cardiology since the 1996 (1) Consensus Conference Report was published, which led to the decision to provide an update to the recommendations. As well, over 160 new references have been added. Sections on Marfan syndrome, Single Ventricle, and Cyanotic Patients have been added. Prevalence, genetics, pregnancy, and arrhythmias for each specific cardiac lesion have been incorporated. The recommendations which follow are the best available given present knowledge.

These recommendations have been written for cardiologists, cardiac surgeons, and other health care professionals who are not experts in this field. This is important to state, since an audience more or less knowledgeable about the subject would require a different amount of background information and depth of treatment of the material.

We panelists are convinced that the interests of any but the most simple patients are best served by involving what we call "national or regional ACHD centres". The knowledge and experience in the care of these patients should be focused, so that competence and skills become available as quickly as possible. This recommendation is not intended to stand in the way of involving local physicians in the care of these patients as collaborating members of a team with the best interests of the patients at heart. Almost all these patients require primary care. Many would benefit from periodic contact with a cardiologist in their community, along with their specialist at the national or regional ACHD centre.

One of the problems and challenges of ACHD is the large number of different lesions and situations one may encounter. Those of us specializing in the area have worked hard to attain the competence and confidence we have, yet regularly continue to be unclear about managing individual patients. We have great respect for the seemingly endless scenarios we encounter.

Patients with congenital heart disease (CHD) are interesting to have in one's practice, but this should be done either collaboratively with a national or regional ACHD centre, or after one has concluded that the patient does not need such a referral. The natural interest in "collecting a few congenitals" should be resisted until this test has been run. This principle applies as much in a surgical as a medical practice.

Canada is fortunate to have a nation-wide group of national and regional ACHD centres called the CACH Network (see Appendix IV). We encourage Canadian readers to make use of these facilities and the skills and experience they represent. More information can be obtained on the Internet at www.cachnet.org.

Another aspect of this consensus conference update is that it will remain available on the Internet at www.cachnet.org and on this website.

In keeping with the origins of the panelists, this document has been endorsed by the most important societies with an interest in this field (see opening page).

We have written this material in as user-friendly a fashion as possible. We envisaged a clinician looking up a lesion, and wishing to see the recommendations "at a glance", rather than having to refer to other sections of the report. This has led to some repetition for the reader who begins at the beginning, and ends at the end. The repetitive portions are printed in italics to reduce frustrations resulting from this style.

We also committed to NOT writing a textbook, even though a good and current one is needed for an audience such as ours. We have focused on the principles of management of these patients, leaving latitude where possible for the clinician to exercise judgement. We wish to guide, but not to constrain unduly.

We have given weight to our management recommendations. The scales which we used are shown in Appendix III. We have used standards similar to those used in past CCS Consensus Conferences.

We hope these recommendations will be found helpful to the patients in whose interests we have written them, and to those who care for them. Canadians have made important contributions to the management of patients with CHD. We join with our international colleagues in hoping this report will follow in this tradition.

Choice of Panelists

The CCS invited Dr. Gary Webb (president of the Canadian Adult Congenital Heart Network) and Dr. Judith Therrien to lead the process, and endorsed the membership of the primary and secondary panels. As is apparent from the panel memberships, the Grown-Up Congenital Heart Working Group of the European Society of Cardiology and the International Society for Adult Congenital Cardiac Disease contributed many members to this process. While published in Canada, this is truly an international document. Further, the panels were selected so as receive input from various interested groups (adult/pediatric cardiology, cardiac surgery, obstetrics, genetics, nurse practitioner), the various regions of Canada, the United States, Europe and Japan.

Most of the panelists worked very hard reviewing many drafts, and offering suggestions for improvement. The panel had almost no difficulty in reaching agreement on the statements made. Debate occurred only where there was insufficient data to decide a point.

A glossary defining the many unusual terms used in this field has been prepared as a companion document.