Management of the Critically Ill Adult With Congenital Heart Disease.

Survival of adults with congenital heart disease (CHD) has improved significantly over the last 2 decades, leading to an increase in hospital and intensive care unit (ICU) admissions of these patients. Whereas most of the ICU admissions in the past were related to perioperative management, the incidence of medical emergencies from long-term sequelae of palliative or corrective surgical treatment of these patients is rising. Intensivists now are confronted with patients who not only have complex anatomy after congenital cardiac surgery, but also complex pathophysiology due to decades of living with abnormal cardiac anatomy and diseases of advanced age. Comorbidities affect all organ systems, including cognitive function, pulmonary and cardiovascular systems, liver, and kidneys. Critical care management requires an in-depth understanding of underlying anatomy and pathophysiology in order to apply contemporary concepts of adult ICU care to this population and optimize patient outcomes. In this review, the main CHD lesions and their common surgical management approaches are described, and the sequelae of CHD physiology are discussed. In addition, the effects of chronic comorbidities on the management of critically ill adults are explored, and the adjustments of current ICU management modalities and pharmacology to optimize care are discussed.