Key result
Critically ill children with pulmonary hypertension associated with congenital heart disease are a high-risk population, with mortality most directly related to right ventricular failure.
This review highlights that in children with pulmonary hypertension and congenital heart disease, mortality is primarily driven by right ventricular failure.
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Warrants specialized monitoring for critically ill children with PH-CHD; leaves open optimal RV-targeted strategies.
OBJECTIVES: 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. DATA SOURCE: MEDLINE, PubMed. CONCLUSIONS: 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.
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Oishi et al. (2016) conducted a review in Pulmonary hypertension in children. Critically ill children with pulmonary hypertension associated with congenital heart disease are a high-risk population, with mortality most directly related to right ventricular failure.
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