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

  • Laura C. Price
  • Rafael Alonso-Gonzalez
  • David Alexander
  • Konstantinos Dimopoulos
Part of the Congenital Heart Disease in Adolescents and Adults book series (CHDAA)


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.


Congenital heart disease Eisenmenger syndrome Pulmonary arterial hypertension Critical care Anesthesia Cyanosis 



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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Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  • Laura C. Price
    • 1
    • 2
  • Rafael Alonso-Gonzalez
    • 1
    • 2
  • David Alexander
    • 1
    • 2
  • Konstantinos Dimopoulos
    • 1
    • 2
  1. 1.Adult Congenital Heart Centre and National Centre for Pulmonary HypertensionNational Heart and Lung Institute, Imperial College School of MedicineLondonUK
  2. 2.Department of AnaestheticsRoyal Brompton and Harefield NHS Foundation Trust, Royal Brompton HospitalLondonUK

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