Title Pulmonary hypertension in adult congenital heart disease / Konstantinos Dimopoulos, Gerhard-Paul Diller, editors

Published Cham, Switzerland : Springer, [2017] ©2017

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Description 1 online resource Series Congenital heart disease in adolescents and adults, 2364-6659 Congenital heart disease in adolescents and adults. Contents Preface to the Series; Foreword; Preface; Contents; Part I: Pathophysiology and Classification of Pulmonary Hypertension in Adult Congenital Heart Disease; 1: Congenital Heart Defects and Pulmonary Hypertension: The Heath-Edwards Paradigm; 1.1 Introduction; 1.2 The Morphology of PAH Related to CHD: Pulmonary Vascular Disease; 1.3 The Evolution of Pulmonary Vascular Disease in Common Types of CHD; 1.4 Operability and Survival After Cardiac Repair; 1.5 Eisenmenger Syndrome: Viktor Eisenmenger and Paul Wood 1.6 The Effect of PAH on the Heart: RV Adaptation and Maladaptation and the Vicious Cycle of Heterometric Adaptation1.7 The Effect of PAH on the Large Pulmonary Arteries; References; 2: Definition and Classification of Pulmonary Hypertension in Congenital Heart Disease; 2.1 Definition of Pulmonary Hypertension; 2.2 Classification of Pulmonary Hypertension; References; 3: Eisenmenger Syndrome: Pathophysiology and Haematologic Effects; 3.1 Introduction; 3.2 Pathology; 3.3 Haematologic Considerations; 3.3.1 Secondary Erythrocytosis; 3.3.2 Iron Deficiency; 3.3.3 Hyperviscosity 3.3.4 Pulmonary Thrombosis and Haemoptysis3.3.5 Non-haematologic Considerations; 3.3.5.1 Cerebral Vascular Events; 3.3.5.2 Pulmonary Mechanics and Gas Exchange; 3.3.6 Exercise Physiology; 3.3.6.1 Pulmonary Vasodilator Therapy; 3.3.7 Prognosis; References; 4: PAH in Patients with Prevalent Systemic-Pulmonary Shunts and PAH in Patients with Small Cardiac Defects; 4.1 Introduction; 4.2 Classification of PAH Associated with Systemic-to-ƯPulmonary Shunts; 4.3 PAH in Patients with Prevalent Systemic-Pulmonary Shunts and PAH in Patients with Small Cardiac Defects 4.4 PAH Associated with Prevalent Systemic-Pulmonary Shunts4.4.1 Prognostic Relevance of the Appropriate Identification of Correctable versus Non-correctable CHD; 4.4.2 Assessment of Operability; 4.4.3 Treat-and-Repair Approach; 4.5 PAH Associated with Small Cardiac Defects; 4.5.1 Epidemiology; 4.5.2 Clinical Picture; 4.5.3 Therapy; References; 5: Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery; 5.1 Introduction; 5.2 Classification and Epidemiology; 5.3 Mechanisms for the Development of Late Postoperative PAH; 5.3.1 Maladaptation/Developmental Factors 5.3.2 PAH Late After Repair5.4 Genetic Factors; 5.5 Treatment; 5.6 Natural Course: Outcome; References; 6: Segmental Pulmonary Hypertension; 6.1 Introduction; 6.2 Segmental Pulmonary Hypertension: Definition and Classification; 6.2.1 Pulmonary Atresia; 6.2.2 PH in Repaired Pulmonary Atresia; 6.2.3 Unilateral Absence of Pulmonary Artery; 6.2.4 Hemitruncus Arteriosus; 6.2.5 Truncus Arteriosus with Stenosis of a Single Pulmonary Artery; 6.2.6 Large Post-tricuspid Defects with Peripheral Pulmonary Stenosis; 6.2.7 Waterston and Potts Shunts to a Single Lung; 6.2.8 Scimitar Syndrome Summary This book is intended as a comprehensive, practically oriented reference on pulmonary hypertension within the context of adult congenital heart disease (ACHD). After an introductory chapter on pathophysiology, the various types of pulmonary hypertension that may be encountered in ACHD are discussed, highlighting the specifics observed within different patient categories. The diagnostic approach is then addressed in detail, and the last section of the book is devoted to management options, from conservative approaches to interventional treatment and the concept of treat and repair. Management in specific patient subjects, such as pregnant women, Fontan patients, and Down syndrome patients with Eisenmenger syndrome, is fully discussed, and guidance is also provided on palliative care. Pulmonary arterial hypertension related to congenital heart disease (PAH-CHD), despite significant similarities in lung pathohysiology, differs significantly from other types of PAH in terms of mechanism of onset, natural history and management. Mistakes and pitfalls in the management of patients with PAH-CHD are often related to a lack of knowledge or expertise in this condition. Pulmonary Hypertension in Adult Congenital Heart Disease will be a valuable resource and learning tool for all who care for patients with ACHD, both in tertiary practice and general cardiology Bibliography Includes bibliographical references Notes Print version record Subject Pulmonary hypertension. Congenital heart disease. Adulthood. Human beings. Adult Humans Heart Defects, Congenital -- complications Hypertension, Pulmonary -- etiology Hypertension, Pulmonary Heart Defects, Congenital Homo sapiens (species) Cardiothoracic surgery. Respiratory medicine. Cardiovascular medicine. HEALTH & FITNESS -- Diseases -- General. MEDICAL -- Clinical Medicine. MEDICAL -- Diseases. MEDICAL -- Evidence-Based Medicine. MEDICAL -- Internal Medicine. Adulthood Congenital heart disease Pulmonary hypertension Form Electronic book Author Dimopoulos, K. (Konstantinos), editor. Diller, Gerhard-Paul, editor ISBN 9783319460284 3319460285

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