Pulmonary Hypertension in Adult Congenital Heart Disease

  • Konstantinos Dimopoulos
  • Gerhard-Paul Diller

Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)

Table of contents

  1. Front Matter
    Pages i-xv
  2. Pathophysiology and Classification of Pulmonary Hypertension in Adult Congenital Heart Disease

    1. Front Matter
      Pages 1-1
    2. Konstantinos Dimopoulos, Elizabeth Orchard, Annalisa Angelini
      Pages 3-22
    3. Shahin Moledina, Paraskevi Theocharis
      Pages 61-68
    4. Konstantinos Dimopoulos, Claudia Montanaro, Gerhard-Paul Diller
      Pages 69-89
    5. Lars Idorn, Lars Søndergaard
      Pages 91-104
    6. Margarita Brida, Gerhard-Paul Diller
      Pages 105-117
  3. The Diagnosis of Pulmonary Hypertension in Adult Congenital Heart Disease

    1. Front Matter
      Pages 119-119
    2. Edward D. Nicol, Michael B. Rubens
      Pages 149-161
    3. Michael A. Quail, Sonya V. Babu-Narayan
      Pages 179-193
    4. Graham Stuart, Reza Ashrafi
      Pages 195-214
    5. Massimo Chessa
      Pages 215-226
  4. Management of Pulmonary Hypertension in Adult Congenital Heart Disease

    1. Front Matter
      Pages 227-227
    2. Heba Nashat, Samantha J. Fitzsimmons, Carl Harries, Konstantinos Dimopoulos, S. John Wort
      Pages 229-251
    3. Michele D’Alto, Giovanni Maria Di Marco
      Pages 279-289
    4. Lorna Swan
      Pages 291-303
    5. Alexander Van de Bruaene, Roselien Buys, Werner Budts
      Pages 305-314
    6. Aleksander Kempny
      Pages 315-328
    7. Annette Schophuus Jensen, Lars Søndergaard
      Pages 329-340
    8. Laura C. Price, Edith Ubogagu, Laura Bernstein, Jenny Wright, Konstantinos Dimopoulos
      Pages 341-361
    9. George Giannakoulas, Michael A. Gatzoulis
      Pages 363-368

About this book


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.


Eisenmenger Syndrome Fontan-type circulation congenital heart disease connective tissue disease management of Fontan patients segmental PAH systemic pulmonary shunts

Editors and affiliations

  • Konstantinos Dimopoulos
    • 1
  • Gerhard-Paul Diller
    • 2
  1. 1.Adult Congenital Heart Centre and Centre for Pulmonary HypertensionRoyal Brompton Hospital and Imperial CollegeLondonUnited Kingdom
  2. 2.University HospitalMünsterGermany

Bibliographic information

  • DOI
  • Copyright Information Springer International Publishing AG 2017
  • Publisher Name Springer, Cham
  • eBook Packages Medicine
  • Print ISBN 978-3-319-46026-0
  • Online ISBN 978-3-319-46028-4
  • Series Print ISSN 2364-6659
  • Series Online ISSN 2364-6667
  • Buy this book on publisher's site