Canadian Journal of Cardiology


Published:January 19, 2023DOI:


      Outcomes of congenital heart disease have improved markedly over the last 20 years, with survival to adulthood now close to 90%. The mean age of admission to an intensive care unit (ICU) is 40 years. The incidence of hospital and critical care admissions have increased significantly as a consequence of this improved survival. Intensivists are now confronted with the management of complex adult congenital heart disease (ACHD) lesions not only from a cardiac perspective but also extra-cardiac organ consequences of years of abnormal circulation following surgical or palliative correction. Kidney and liver dysfunction, respiratory and hematological abnormalities are very common in this population. ACHD patients can present to the ICU for a vast number of reasons, these are classified in this review as medical non-cardiac, medical cardiac and surgical. Community/hospital acquired infections, cerebrovascular accidents, respiratory failure, alongside arrhythmias and heart failure are responsible for medical admissions. Surgical admissions include post-operative management after correction or palliation, but also medical optimization and work-up for advanced therapies. ICU management of this large heterogeneous group requires a thorough understanding of the pathophysiology in order to apply conventional adult critical care modalities; left ventricular or right ventricular dysfunction, pulmonary hypertension, intracardiac, extracardiac, palliative surgical shunts can be present and require additional consideration.
      This review focuses on the pathophysiology, long term sequelae, and different treatment modalities to supply a framework for the ICU physician caring for these patients. Successful outcome, especially in complex lesions is dependent on early involvement of specialized ACHD centers.
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