This book provides a comprehensive review of the basic science of the mitral valve needed to begin to comprehend both normal and the failing valve. This knowledge is infrequently discussed in the clinical arena, with a rush towards definition of lesions based on limited analysis. It provides a ready single source of the most important basic science subjects pertaining to the valve, and the pathologies found therein, and thus a more complete understanding of the factors that result in dysfunction may be appreciated. Mitral Valve Disease: Basic Sciences and Current Approaches to Management includes chapters on embryology and anatomy, including details of structural, comparative and functional features. Since the valve is completely influenced by genetics and molecular biology, subjects rarely if ever addressed in the clinical arena, these topics receive in-depth attention. Coverage concludes with lengthy discussions of the importance of multi-disciplinary work, along with the importance of the electrophysiological disturbances encountered in some groups of these patients. It therefore is an essential and up-to-date resource for the practicing and trainee cardiac surgeon and any researcher working within this topic. Preface Contents 1: The Mitral Valve: A Brief History References 2: Anatomical Development of the Left Atrioventricular Valvar Complex Introduction The Initial Stages of Development References 3: How Should We Describe the Mitral Valve and Its Component Parts? Introduction The Mitral Valve The Mitral Valvar Complex The Valvar Annulus The Valvar Leaflets The Tendinous Cords The Papillary Muscles References 4: The Anatomy of the Mitral Valve Introduction What Is the Mitral Valve? The Left Atrioventricular Junction The Valvar Leaflets The Tendinous Cords The Papillary Muscles References 5: The Surgical Utility of a Detailed Knowledge of the Basic Sciences Pertaining to the Mitral Valve References 6: The Atrioventricular Valve in the Animal Kingdom Introduction Major Evolutionary Trends The Primitive Atrioventricular Valve Evolutionary Conservation of the Atrioventricular Canal and Valve The Atrioventricular Valve Is Modified by Full Atrial Septation The Atrioventricular Junction Expands Rightwards with Full Ventricular Septation Flow Across the Atrioventricular Valve Increases Manifold with Warm-Bloodedness Thick Compact Walls and Large Papillary Muscles with Multiple Tendinous Cords Evolved Together Blood Vortices in the Left Ventricle of Warm-Blooded Vertebrates Summary of Major Evolutionary Trends The Left Atrioventricular Valve at the Extremes Summary References 7: The Atrioventricular Complex: Function and Dysfunction Introduction The Atrioventricular Complex Through the Cardiac Cycle The Malfunctioning Mitral valve Conclusions References 8: Mitral Valve Pathology Text Normal Histology Age-Associated Changes Rheumatic Disease Infective Endocarditis Non-Bacterial Thrombotic Endocarditis Mitral Valve Prolapse Hypertrophic Cardiomyopathy Related Mitral Valve Disease Congenital Mitral Valve Disease Trauma Papillary Muscle Rupture in Ischaemic Heart Disease Mitral Valve Replacement Future Work References 9: Morphogenetic Aspects of Mitral Valve Development Introductory Remarks Patterning of the Atrioventricular Canal Extracellular Matrix Endocardial-to-Mesenchymal Transformation Sources of Mitral Valve Interstitial Cells Endocardial Cells Second Heart Field-Derived Mesenchyme Epicardial-Derived Cells Neural Crest Cells Bone Marrow-Derived Cells Mitral Valve Growth and Maturation Release of the Leaflets and Formation of the Tension Apparatus Extracellular Matrix (ECM) and Valve Remodelling The Lamellar Mitral Valve Structure Cilia and Cell Polarity in the Mitral Valve Conclusions and Perspectives References 10: Genetics of Mitral Valve Disease Introduction Histology and Pathophysiology of MVP Genetic Basis of Syndromic MVP MVP as Part of Connective Tissue Disorders MVP as Part of Complex Congenital Heart Disease Genetic Basis of Non-Syndromic MVP Pedigree Studies Genome-Wide Association Studies Mitral Valve Prolapse: An Unrecognized Ciliopathy? Future Perspectives Technological Advances Genetic Discoveries Outside of Coding Regions Genetic Basis of Sudden Cardiac Death Risk in MVP Conclusions References 11: Genetics and Genetic Counselling Relevant to Mitral Valve Prolapse Introduction Genetics Familial MVP Cardiomyopathies and Ion Channelopathies (FLNC, LMNA, HCN4, SCN5A) Sporadic and Common MVP Genetic Counselling Definition of Genetic Counselling Generation of Referrals for Genetic Counselling Clinical Evaluation of Family Members What Should Be Included in Counselling Types of Genetic Tests Conclusions References 12: Assessment of Mitral Valve Function: The Valve and The Ventricle Characterisation of Mitral Valve Function Assessment of Mitral Regurgitation Severity The Left Ventricle in Primary Mitral Regurgitation Assessment of Left Ventricular Systolic Function References 13: Arrhythmias in Mitral Valve Prolapse Introduction Early Studies of Arrhythmias in MVP Population Studies Recent Studies of Arrhythmias in MVP Arrhythmia Risk Stratification ECG Findings Echocardiography Cardiac Magnetic Resonance Imaging Electrophysiology Testing Mechanisms of VT/VF and Sudden Cardiac Death in MVP Treatment Options Medical Therapy Catheter Ablation Implantable Cardioverter Defibrillators (ICDs) Mitral Valve Repair Conclusions References 14: The Organisation of Specialist Valve Care Provision References Correction to: The Anatomy of the Mitral Valve Index