Recognition, Stabilization, and Management of Children with Pulmonary Hypertension in the Emergency Department
Pulmonary hypertension (PH) is an uncommon but potentially life-threatening condition in children, and improved survival makes it more likely that acute care practitioners will encounter children with PH. This is due in part to better outcomes of extremely preterm infants who develop PH from chronic lung disease and children with congenital heart and lung defects who survive surgery but have residual PH, increased diagnosis of PH due to expanded screening indications, and better diagnostic modalities. This chapter provides an overview of the types of pediatric PH likely to be encountered in the acute care setting and discusses the recognition and initial management of this disease. Specific clinical cases are presented to illustrate various pulmonary hypertensive emergencies in the five categories of PH as classified by the World Health Organization. These consist of (1) pulmonary arterial hypertension, (2) PH with left heart disease, (3) PH associated with lung diseases and/or hypoxemia, (4) PH due to chronic thrombotic and/or embolic disease, and (5) miscellaneous.
KeywordsPulmonary hypertension Congenital heart disease Thromboembolic pulmonary hypertension Sickle cell disease
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