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Pulmonary hypertension is an abnormal elevation of the pulmonary arterial pressure, which can occur for a number of different reasons. Pulmonary hypertension can be idiopathic or associated with various cardiac, pulmonary, and systemic diseases in infants and children and is often multifactorial in etiology. It is important to identify and treat any known causes of pulmonary hypertension or associated conditions that may exacerbate the disease. Regardless of the etiology, pulmonary hypertension can be associated with significant morbidity and mortality due to right heart failure. While current therapies treat pulmonary hypertension and prolong life, there is no cure to reverse the disease process in many forms of pulmonary hypertension. There have been recent advances in therapeutic options, but there are limited data on use in children.


Pulmonary hypertension is defined as a mean pulmonary artery pressure ≥ 25 mm Hg. It can occur as an isolated process, previously termed “primary pulmonary hypertension,” or can be related to a number of cardiopulmonary or systemic diseases, previously “secondary pulmonary hypertension.” In 1998, the World Health Organization developed a new classification for pulmonary hypertension that categorizes it into 5 major groups based on etiology. This was updated most recently at the World Symposium of Pulmonary Hypertension in Nice in 2013 (Table 486-1). To address the need for a classification system that incorporates more pediatric-specific aspects of pathobiology and related clinical issues, a comprehensive classification of pediatric pulmonary hypertension was proposed at the Panama Pulmonary Vascular Research Institute symposium (PVRI) in 2011, highlighting 10 major categories of pediatric pulmonary vascular disease (Table 486-2).


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