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.
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.