Pulmonary Hypertension in Adult Congenital Heart Disease

Part I. Pathophysiology and Classification of Pulmonary Hypertension in Adult Congenital Heart Disease

1. Congenital Heart Defects and Pulmonary Hypertension: The Heath–Edwards Paradigm
Konstantinos Dimopoulos, Elizabeth Orchard, Annalisa Angelini

2. Definition and Classification of Pulmonary Hypertension in Congenital Heart Disease
Gerhard-Paul Diller

3. Eisenmenger Syndrome: Pathophysiology and Haematologic Effects
Craig S. Broberg

4. PAH in Patients with Prevalent Systemic–Pulmonary Shunts and PAH in Patients with Small Cardiac Defects
Alessandra Manes

5. Pulmonary Arterial Hypertension in Patients with Previous Reparative Surgery
Shahin Moledina, Paraskevi Theocharis

6. Segmental Pulmonary Hypertension
Konstantinos Dimopoulos, Claudia Montanaro, Gerhard-Paul Diller

7. Pulmonary Vascular Disease in Patients with Fontan-Type Circulation
Lars Idorn, Lars Søndergaard

8. Post-capillary Pulmonary Hypertension in ACHD
Margarita Brida, Gerhard-Paul Diller

Part II. 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
Margherita Ministeri, Natali Chung, Konstantinos Dimopoulos

10. The Role of Plain Chest Radiography and Computed Tomography
Edward D. Nicol, Michael B. Rubens

11. Echocardiography in the Diagnosis and Follow-Up of Patients with Pulmonary Arterial Hypertension Associated with Congenital Heart Disease
Pamela Moceri, Wei Li, Konstantinos Dimopoulos

12. Cardiovascular Magnetic Resonance
Michael A. Quail, Sonya V. Babu-Narayan

13. Cardiopulmonary Exercise and Six-Minute Walk Testing
Graham Stuart, Reza Ashrafi

14. Cardiac Catheterization
Massimo Chessa

Part III. Management of Pulmonary Hypertension in Adult Congenital Heart Disease

15. 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

16. Pulmonary Vasodilators in Patients with Pulmonary Arterial Hypertension Related to Congenital Heart Disease
Rafael Alonso-Gonzalez, Pilar Escribano-Subías

17. 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

18. Eisenmenger Syndrome in Patients with Down Syndrome
Michele D’Alto, Giovanni Maria Marco

19. Pregnancy and Contraception
Lorna Swan

20. Training and Recommendations for Exercise
Alexander Bruaene, Roselien Buys, Werner Budts

21. Prognostication in PAH–CHD
Aleksander Kempny

22. Management of Fontan Patients
Annette Schophuus Jensen, Lars Søndergaard

23. Palliative Care and End-of-Life Considerations in Patients with PAH–CHD
Laura C. Price, Edith Ubogagu, Laura Bernstein, Jenny Wright, Konstantinos Dimopoulos

24. PAH in ACHD: Research, Global Perspective and Future Prospects. An Epilogue
George Giannakoulas, Michael A. Gatzoulis

Nyckelord: Medicine & Public Health, Cardiology, Cardiac Surgery, Pneumology/Respiratory System