To review the clinical classification, diagnosis, and pathophysiology of pulmonary hypertension
in children, emphasizing the role of right ventricular function
, ventricular interaction, and congenital heart disease
in the evolution and progression of disease, as well as management strategies and therapeutic options.
Critically ill children with pulmonary hypertension
associated with congenital heart disease
are a high-risk population. Congenital cardiac defects resulting in either increased pulmonary blood flow or impaired pulmonary venous drainage predispose patients to developing structural and functional aberrations of the pulmonary vasculature. Mortality from pulmonary hypertension
is most directly related to right ventricular failure.