Pulmonary Hypertension in Adult Congenital Heart Disease

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.