Pathobiology of pulmonary hypertension in infants and children

Abstract

Pulmonary vascular disease occurs as a rare primary disorder and a common, frequently major complication of many disease states. But we still cannot define the stimuli and signal transduction pathways which instigate and perpetuate the disease state, and therefore our treatment strategies are reactive, nor proactive. Observational studies on patients with pulmonary hypertension of different aetiologies have identified potentially important structural and functional abnormalities, but whether these are a cause or a consequence of the disease process remains to be determined. Recent genetic studies indicate, for the first time, a primary abnormality in the regulation of pulmonary vascular remodelling in a sub-set of patients with pulmonary hypertension. The aim of research in pathobiology is to discover the molecular mechanisms responsible for the complex vascular changes associated with pulmonary hypertension and in doing so, improve treatment strategies.