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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.
Diller Gerhard, Royal Bromption Hospital, London, UKKonstantinos Dimopoulos, Royal Bromption Hospital, London, UK
Part 1: Pathophysiology and Classification of Pulmonary Hypertension in Adult Congenital Heart Disease.- 1. Congenital Heart Defects and Pulmonary Hypertension: The Heath Edwards Paradigm.- 2. Definition and Classification of Pulmonary Hypertension in Congenital Heart Disease.- 3. Eisenmenger Syndrome: Pathophysiology and Hematologic Effects.- 4. PAH in Patients with Persistent Systemic-Pulmonary Shunts and PAH in Patients with Small Cardiac Defects.- 5. Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery.- 6. Segmental Pulmonary Hypertension.- 7. Pulmonary Vascular Disease in Patients with Fontan-Type Circulation.- 8. Post-Capillary Pulmonary Hypertension in ACHD.- Part 2: The Diagnosis of Pulmonary Hypertension in Adult Congenital Heart Disease.- 9. Physical Examination and Electrocardiography in Patients with Pulmonary Arterial Hypertension due to Congenital Heart Disease: Initial Clinical Assessment.- 10. The Role of Plain Chest Radiography and Computed Tomography.- 11. Echocardiography in the Diagnosis and Follow-up of Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease.- 12. Cardiovascular Magnetic Resonance.- 13. Cardiopulmonary Exercise and Six-minute Walk Testing.- 14. Cardiac Catheterization.- Part 3: Management of Pulmonary Hypertension in Adult Congenital Heart Disease.- 15. Conservative Management and Recommendations for PAH-CHD.- 16. Pulmonary Vasodilators in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease.- 17. Perspectives on Shunts in Pulmonary Arterial Hypertension: from Interventions to Create Shunts to the Concept of “Treat-and-Repair”.- 18. Eisenmenger Syndrome in Patients with Down’s Syndrome.- 19. Pregnancy and Contraception.- 20. Training and Recommendations for Exercise.- 21. Prognostication in PAH-CHD.- 22. Management of Fontan patients.- 23. Palliative Care and End of Life Considerations in Patients with PAH-CHD.- 24. PAH in ACHD: Research,Global Perspective and Future Prospects: An Epilogue.