All physicians practicing medicine encounter patients suffering from cardiovascular disease. This book has been outlined in such a way that vascular surgeons, general internists, neurologists and cardiologists should be able to use it. The book covers the complete scope of cardiac diseases in addition to chapters on hypertension and atherosclerosis. In many patients there is a family history of cerebrovascular accidents, myocardial infarction or peripheral arterial disease. Also in patients reporting collaps, palpitations and arrhythmias the family is crucial and can provide clues to a genetic cause of the disease. This book is published to guide physicians in the process of determining whether a genetic component is likely to be present. Furthermore, information is provided what the possibilities and limitations of DNA diagnostic techniques are. Finally, the importance of newly identified categories of potential patients, i. e. gene carriers without symptoms or any inducible sign of disease, is highlighted. For some patients a genetic diagnosis is essential to determine appropriate therapy and for counseling? In some other diseases DNA diagnostic tools are available but the relevant for the patients may be less clear. In other families the search for a disease causing gene is ongoing and the possibilities to find genes and to unravel the pathophysiology of the disease is limited by the lack of patients. To give insight into the current state of genetic diagnostics, the authors have classified the cardiovascular diseases.

Improving our insights into the genetic predisposition to cardiovascular disease is one of the most important challenges in our field in the next millennium, not only to unravel the cause of disease but also to improve the selection of patients for particular treatments. Nowadays, for example, subjects with a cholesterol above a particular plasma level are exposed to a cholesterol lowering regime based upon the beneficial outcome of epidemiological studies which include subjects not prone to the disease, despite a plasma cholesterol above the accepted level. Identification of the patients who are genetically predisposed to the consequences of this disorder will reduce the number of subjects unnecessarily treated and, hence, the costs of health care. Because in most cardiovascular diseases the genetic component is a consequence of more than one gene defect, only limited progress has as yet been made in identifying subjects genetically at risk. For example, in hypertension only in less than 10% of the patients the genetic defect has been identified. It has been known for quite some time that in heart and blood vessels fetal genes are as high blood pressure and upregulated or induced when they are exposed to such disorders ischemia. Little is known about the function of these genes in the cardiac and vascular adaptation to these disorders; only guesses can be made.

Four years ago-in December 1997-the first European Science Foundation Workshop on Cardiovascular Specific Gene Expression was held in Maastricht. It was hardly possible to imagine the progress in the field in those four years. In 1997, gene expression was still an art focused on individual genes; in 200 I, many labs have access to micro-array facilities to determine the expression of thousands of genes simultaneously. In 1997, gene expression was an area of fundamental research in basis molecular biology laboratories; in 200 I, clinical cardiovascular research has incorporated gene expression approaches. In 1997, the interpretation of a gene expression experiment was usually straightforward; in 200 I, advanced bioinformatics tools are needed to approach the extreme complexities of genetic control of cell and tissue function. The second symposium in this series is focused on Cardiovascular Genomics. New Pathophysiological Concepts. The organizing committee chose to invite a group of renown scientists and young investigators around four topics of eminent importance in cardiovascular research. These topics reflect the major present-day clinical cardiovascular problems: atherosclerosis, hypertension, arrhythmias and heart failure. In addition to these four disease-driven topics, the workshop has sessions on gene expression methodologies and cellular transplant approaches to cardiovascular disease.

The electrocardiogram (ECG) remains the most accessible and inexpensive diagnostic tool to evaluate the patient presenting with symptoms suggestive of acute myocardial ischemia. It plays a crucial role in decision making about the aggressiveness of therapy especially in relation to reperfusion therapy, because such therapy has resulted in a considerable reduction in mortality from acute myocardial infarction. Several factors play a role in the amount of myocardial tissue that can be salvaged by reperfusion therapy, such as the time interval between onset of coronary occlusion and reperfusion, site and size of the jeopardized area, type of reperfusion attempt (thrombolytic agent or an intracoronary catheter intervention), presence or absence of risk factors for thrombolytic agents, etc. Most important in decision making on reperfusion therapy and the type of intervention is to look for markers indicating a higher mortality rate from myocardial infarction. The ECG is a reliable, inexpensive, non-invasive instrument to obtain that information. Recently it has become clear that both in anterior and inferior myocardial infarction, the ECG frequently allows not only to identify the infarct related coronary artery, but also the site of occlusion in that artery and therefore the size of the jeopardized area. Obviously, the more proximal the occlusion, the larger the area at risk and the more aggressive the reperfusion attempt.

The first invasive evaluation of cardiac arrhythmias in humans was performed in 1967 in Paris (Prof. P. Coumel) and Amsterdam (Prof. D. Durrer). This was the start of a rapid increase in our knowledge of the diagnosis, mechanism and treatment of cardiac arrhythmias. In that same year Prof. Hein J.J. Wellens became cardiologist in the Wilhelmina Gasthuis in Amsterdam. Initially in Amsterdam (1967-1977) and later on in Maastricht (from 1977), he was the driving force for many breakthroughs in clinical cardiac electrophysiology. With an active interplay between the knowledge derived from the 12-lead electrocardiogram and the recordings made with invasive electrophysiology, he composed new ideas leading to major contributions in clinical cardiac electrophysiology and, more generally, in arrhythmology. He published over 650 scientific papers and 14 books, and had numerous functions within scientific boards of prestigious journals. In addition he trained more than 120 cardiologists in clinical cardiac electrophysiology. On the occasion of the congress `2000, Future of Arrhythmology: Lessons From the Past, Promises For Tomorrow', we highlight the scientific work of Prof. Hein J.J. Wellens. A selection of more than 60 articles over the whole time span has been selected. These articles are accompanied by comments from an expert, co-worker and/or former fellow in order to place the paper in a scientific time frame, including the relationship of the author with Prof. Hein J.J. Wellens.

All physicians practicing medicine encounter patients suffering from cardiovascular disease. This book has been outlined in such a way that vascular surgeons, general internists, neurologists and cardiologists should be able to use it. The book covers the complete scope of cardiac diseases in addition to chapters on hypertension and atherosclerosis. In many patients there is a family history of cerebrovascular accidents, myocardial infarction or peripheral arterial disease. Also in patients reporting collaps, palpitations and arrhythmias the family is crucial and can provide clues to a genetic cause of the disease. This book is published to guide physicians in the process of determining whether a genetic component is likely to be present. Furthermore, information is provided what the possibilities and limitations of DNA diagnostic techniques are. Finally, the importance of newly identified categories of potential patients, i. e. gene carriers without symptoms or any inducible sign of disease, is highlighted. For some patients a genetic diagnosis is essential to determine appropriate therapy and for counseling? In some other diseases DNA diagnostic tools are available but the relevant for the patients may be less clear. In other families the search for a disease causing gene is ongoing and the possibilities to find genes and to unravel the pathophysiology of the disease is limited by the lack of patients. To give insight into the current state of genetic diagnostics, the authors have classified the cardiovascular diseases.

Four years ago-in December 1997-the first European Science Foundation Workshop on Cardiovascular Specific Gene Expression was held in Maastricht. It was hardly possible to imagine the progress in the field in those four years. In 1997, gene expression was still an art focused on individual genes; in 200 I, many labs have access to micro-array facilities to determine the expression of thousands of genes simultaneously. In 1997, gene expression was an area of fundamental research in basis molecular biology laboratories; in 200 I, clinical cardiovascular research has incorporated gene expression approaches. In 1997, the interpretation of a gene expression experiment was usually straightforward; in 200 I, advanced bioinformatics tools are needed to approach the extreme complexities of genetic control of cell and tissue function. The second symposium in this series is focused on Cardiovascular Genomics. New Pathophysiological Concepts. The organizing committee chose to invite a group of renown scientists and young investigators around four topics of eminent importance in cardiovascular research. These topics reflect the major present-day clinical cardiovascular problems: atherosclerosis, hypertension, arrhythmias and heart failure. In addition to these four disease-driven topics, the workshop has sessions on gene expression methodologies and cellular transplant approaches to cardiovascular disease.

The electrocardiogram (ECG) remains the most accessible and inexpensive diagnostic tool to evaluate the patient presenting with symptoms suggestive of acute myocardial ischemia. It plays a crucial role in decision making about the aggressiveness of therapy especially in relation to reperfusion therapy, because such therapy has resulted in a considerable reduction in mortality from acute myocardial infarction. Several factors play a role in the amount of myocardial tissue that can be salvaged by reperfusion therapy, such as the time interval between onset of coronary occlusion and reperfusion, site and size of the jeopardized area, type of reperfusion attempt (thrombolytic agent or an intracoronary catheter intervention), presence or absence of risk factors for thrombolytic agents, etc. Most important in decision making on reperfusion therapy and the type of intervention is to look for markers indicating a higher mortality rate from myocardial infarction. The ECG is a reliable, inexpensive, non-invasive instrument to obtain that information. Recently it has become clear that both in anterior and inferior myocardial infarction, the ECG frequently allows not only to identify the infarct related coronary artery, but also the site of occlusion in that artery and therefore the size of the jeopardized area. Obviously, the more proximal the occlusion, the larger the area at risk and the more aggressive the reperfusion attempt.

Improving our insights into the genetic predisposition to cardiovascular disease is one of the most important challenges in our field in the next millennium, not only to unravel the cause of disease but also to improve the selection of patients for particular treatments. Nowadays, for example, subjects with a cholesterol above a particular plasma level are exposed to a cholesterol lowering regime based upon the beneficial outcome of epidemiological studies which include subjects not prone to the disease, despite a plasma cholesterol above the accepted level. Identification of the patients who are genetically predisposed to the consequences of this disorder will reduce the number of subjects unnecessarily treated and, hence, the costs of health care. Because in most cardiovascular diseases the genetic component is a consequence of more than one gene defect, only limited progress has as yet been made in identifying subjects genetically at risk. For example, in hypertension only in less than 10% of the patients the genetic defect has been identified. It has been known for quite some time that in heart and blood vessels fetal genes are as high blood pressure and upregulated or induced when they are exposed to such disorders ischemia. Little is known about the function of these genes in the cardiac and vascular adaptation to these disorders; only guesses can be made.

Clinical management and signs are the focus of this practical cardiogenetic reference for those who are involved in the care for cardiac patients with a genetic disease. With detailed discussion of the basic science of cardiogenetics in order to assist in the clinical understanding of the topic. The genetic causes of various cardiovascular diseases are explained in a concise clinical way that reinforces the current management doctrine in a practical manner. The authors will cover the principles of molecular genetics in general but also specific to cardiac diseases. They will discuss the etiology, pathogenesis, pathophysiology, clinical presentation, clinical diagnosis, molecular diagnosis and treatment of each cardiogenetic disease separately. Therapy advice, ICD indications, indications for and manner of further family investigation will all be covered, while each chapter will also contain take-home messages to reinforce the key points. The chapters reviewing the different diseases will each contain a table describing the genes involved in each. Each chapter will also contain specific illustrations, cumulatively giving a complete, practical review of each cardiogenetic disease separately. Special emphasis will be given to advice on how to diagnose and manage cardiogenetic diseases in clinical practice, which genes should be investigated and why, and the pros and cons of genetic testing. Guidelines for investigation in families with sudden cardiac death at young age will also be included. This book will be written for the general cardiologist and the clinical geneticist who is involved in cardiac patients and will provide answers to question such as: Which genes are involved and which mutations? What is the effect of the mutation at cellular level? Which genes should be tested and why? What is the value of a molecular diagnosis? Does it influence therapy? When should the first degree relatives be tested and in which way?