Pulmonary Hypertension in Adult Congenital Heart Disease (Congenital Heart Disease in Adolescents and Adults)

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. Front Matter ....Pages i-xv Front Matter ....Pages 1-1 Congenital Heart Defects and Pulmonary Hypertension: The Heath–Edwards Paradigm (Konstantinos Dimopoulos, Elizabeth Orchard, Annalisa Angelini)....Pages 3-22 Definition and Classification of Pulmonary Hypertension in Congenital Heart Disease (Gerhard-Paul Diller)....Pages 23-28 Eisenmenger Syndrome: Pathophysiology and Haematologic Effects (Craig S. Broberg)....Pages 29-44 PAH in Patients with Prevalent Systemic–Pulmonary Shunts and PAH in Patients with Small Cardiac Defects (Alessandra Manes)....Pages 45-60 Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery (Shahin Moledina, Paraskevi Theocharis)....Pages 61-68 Segmental Pulmonary Hypertension (Konstantinos Dimopoulos, Claudia Montanaro, Gerhard-Paul Diller)....Pages 69-89 Pulmonary Vascular Disease in Patients with Fontan-Type Circulation (Lars Idorn, Lars Søndergaard)....Pages 91-104 Post-capillary Pulmonary Hypertension in ACHD (Margarita Brida, Gerhard-Paul Diller)....Pages 105-117 Front Matter ....Pages 119-119 Physical Examination and Electrocardiography in Patients with Pulmonary Arterial Hypertension Due to Congenital Heart Disease: Initial Clinical Assessment (Margherita Ministeri, Natali Chung, Konstantinos Dimopoulos)....Pages 121-148 The Role of Plain Chest Radiography and Computed Tomography (Edward D. Nicol, Michael B. Rubens)....Pages 149-161 Echocardiography in the Diagnosis and Follow-Up of Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease (Pamela Moceri, Wei Li, Konstantinos Dimopoulos)....Pages 163-178 Cardiovascular Magnetic Resonance (Michael A. Quail, Sonya V. Babu-Narayan)....Pages 179-193 Cardiopulmonary Exercise and Six-Minute Walk Testing (Graham Stuart, Reza Ashrafi)....Pages 195-214 Cardiac Catheterization (Massimo Chessa)....Pages 215-226 Front Matter ....Pages 227-227 Conservative Management and Recommendations for Pulmonary Arterial Hypertension Related to Congenital Heart Disease (Heba Nashat, Samantha J. Fitzsimmons, Carl Harries, Konstantinos Dimopoulos, S. John Wort)....Pages 229-251 Pulmonary Vasodilators in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease (Rafael Alonso-Gonzalez, Pilar Escribano-Subías)....Pages 253-266 Perspectives on Shunts in Pulmonary Arterial Hypertension: From Interventions to Create Shunts to the Concept of “Treat-and-Repair” (Alexander R. Opotowsky, Michael J. Landzberg)....Pages 267-278 Eisenmenger Syndrome in Patients with Down Syndrome (Michele D’Alto, Giovanni Maria Di Marco)....Pages 279-289 Pregnancy and Contraception (Lorna Swan)....Pages 291-303 Training and Recommendations for Exercise (Alexander Van de Bruaene, Roselien Buys, Werner Budts)....Pages 305-314 Prognostication in PAH–CHD (Aleksander Kempny)....Pages 315-328 Management of Fontan Patients (Annette Schophuus Jensen, Lars Søndergaard)....Pages 329-340 Palliative Care and End-of-Life Considerations in Patients with PAH–CHD (Laura C. Price, Edith Ubogagu, Laura Bernstein, Jenny Wright, Konstantinos Dimopoulos)....Pages 341-361 PAH in ACHD: Research, Global Perspective and Future Prospects. An Epilogue (George Giannakoulas, Michael A. Gatzoulis)....Pages 363-368