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Eisenmenger Syndrome and Other Types of Pulmonary Arterial Hypertension Related to Congenital Heart Disease

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Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care

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Introduction

Despite recent advances in diagnosis and cardiac surgery for congenital heart disease (CHD), 5–10% of adult patients develop pulmonary arterial hypertension (PAH) [1]. PAH, in turn, impacts quality of life (QoL) and the survival prospects of patients, especially those who develop near-systemic pulmonary arterial pressures and cyanosis (Eisenmenger syndrome) [2]. Improved understanding of the pathophysiology of PAH and potential complications, early diagnosis, and the use of PAH therapies has revolutionized the care of patients with PAH related to CHD (PAH-CHD).

The Wide Spectrum of PAH-CHD

PAH-CHD belongs to group 1 of the international PH guidelines [2, 3]. Within this group, there are four distinct subgroups (Table 1). Eisenmenger syndrome sits at the extreme of the spectrum and is currently the most common type of PAH-CHD in adults. Patients with left-right shunting typically have less severe pulmonary vascular disease (PVD), and some may be amenable to repair of the...

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Authors and Affiliations

  1. Adult Congenital Heart Centre and Centre for Pulmonary Hypertension, Royal Brompton Hospital and the National Heart & Lung Institute, Imperial College, London, UK

    Konstantinos Dimopoulos, Andrew Constantine, Kaushiga Krishnathasan, Favoccia Carla, Matina Prapa & Michael A. Gatzoulis

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  1. Konstantinos Dimopoulos
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  2. Andrew Constantine
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  3. Kaushiga Krishnathasan
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  4. Favoccia Carla
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  5. Matina Prapa
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  6. Michael A. Gatzoulis
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Editors and Affiliations

  1. Children’s Hospital Colorado University of Colorado Denver, School of, Aurora, CO, USA

    Eduardo M. da Cruz

  2. Children's Hospital Colorado University of Colorado School of Medicin, Aurora, CO, USA

    Dunbar Ivy

  3. Department of Cardiac Surgery, German Pediatric Heart Center, Sankt Augustin, Germany

    Viktor Hraska

  4. Pediatric Cardiac Surgery, Children's Hospital Colorado University of Colorado, Aurora, CO, USA

    James Jaggers

Section Editor information

  1. Cincinnati Children’s Hospital Medical Center, Cincinatti, OH, USA

    Gary Webb

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Dimopoulos, K., Constantine, A., Krishnathasan, K., Carla, F., Prapa, M., Gatzoulis, M.A. (2024). Eisenmenger Syndrome and Other Types of Pulmonary Arterial Hypertension Related to Congenital Heart Disease. In: da Cruz, E.M., Ivy, D., Hraska, V., Jaggers, J. (eds) Pediatric and Congenital Cardiology, Cardiac Surgery and Intensive Care. Springer, London. https://doi.org/10.1007/978-1-4471-4999-6_182-2

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