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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-Pulmonary 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
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-Pulmonary 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