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Intensive Care of the Adult with Congenital Heart Disease pp 273–297Cite as

Critical Care Management of the Adult with Eisenmenger Syndrome and Pulmonary Arterial Hypertension Related to Congenital Heart Disease

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

Abstract

Major advances in the surgical and medical management of congenital heart disease (CHD) in recent decades have meant that an increasing number of children born with CHD survive to adulthood. In the past, lack of early diagnosis and timely repair resulted in a substantial number of patients developing pulmonary vascular disease, especially those with post-tricuspid shunts. Pulmonary arterial hypertension (PAH) related to congenital heart disease (PAH-CHD) is still one the most common types of PAH.

PAH-CHD shares significant similarities in terms of lung pathology to other types of PAH. All pulmonary hypertension (PH), including PAH-CHD, is a major risk factor for individuals who require critical care due to deterioration of their underlying disease, or because they are undergoing essential surgery or other intervention. A multidisciplinary approach, with significant expertise in CHD, PH, anesthetics, and critical care, is essential in order to optimize the otherwise adverse outcome of PAH-CHD patients who are admitted in intensive care or a high dependency unit.

In this chapter, we provide an overview of the critical care and anesthetic management of adults with PH, in particular PAH-CHD.

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Disclosures

Dr. Dimopoulos has acted as consultant for Actelion UK, Pfizer UK, and GSK UK and received unrestricted educational grants from Actelion and Pfizer. Dr. Price has acted as a consultant for Actelion UK and GSK UK and has received educational grants from Actelion. Dr Alexander has no disclosures to declare. Dr. Alonso-Gonzalez has acted as consultants for Pfizer Spain and GSK Spain and received unrestricted educational grants from Actelion and GSK.

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Authors and Affiliations

  1. Adult Congenital Heart Centre and National Centre for Pulmonary Hypertension, National Heart and Lung Institute, Imperial College School of Medicine, London, UK

    Laura C. Price, Rafael Alonso-Gonzalez, David Alexander & Konstantinos Dimopoulos

  2. Department of Anaesthetics, Royal Brompton and Harefield NHS Foundation Trust, Royal Brompton Hospital, London, UK

    Laura C. Price, Rafael Alonso-Gonzalez, David Alexander & Konstantinos Dimopoulos

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  1. Laura C. Price
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  2. Rafael Alonso-Gonzalez
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  3. David Alexander
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  4. Konstantinos Dimopoulos
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Editors and Affiliations

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

    Eduardo da Cruz

  2. Pediatric Intensive Care, Royal Brompton & Harefield NHS Foundation Trust, London, UK

    Duncan Macrae

  3. Adult Congenital Heart Program, Heart Institute, Cincinnati Children Hospital Medical Center, Cincinnati, OH, USA

    Gary Webb

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Price, L.C., Alonso-Gonzalez, R., Alexander, D., Dimopoulos, K. (2019). Critical Care Management of the Adult with Eisenmenger Syndrome and Pulmonary Arterial Hypertension Related to Congenital Heart Disease. In: da Cruz, E., Macrae, D., Webb, G. (eds) Intensive Care of the Adult with Congenital Heart Disease. Congenital Heart Disease in Adolescents and Adults. Springer, Cham. https://doi.org/10.1007/978-3-319-94171-4_15

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  • DOI: https://doi.org/10.1007/978-3-319-94171-4_15

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