• Steven H Abman and Usha Raj.
• The Pediatric Heart Lung Center, Department of Pediatrics, University of Colorado School of Medicine and The Children's Hospital, Aurora CO, United States.
• Prog. Pediatr. Cardiol. 2009 Dec 1; 27 (1-2): 3-6.

AbstractPulmonary hypertension (PH) and related pulmonary vascular diseases contribute to high morbidity and mortality and treatment options remain limited. Despite the availability of new drug therapies, the long-term outcomes of patients with severe PH remain poor. This may be especially true for many children with PH. Although most clinical studies have emphasized studies of adult patients, PH in pediatrics can be devastating and often contributes to poor outcomes in diverse clinical settings in newborns, infants and children. Unfortunately, studies that address the safety and efficacy of PH therapies in children are rare, as most pharmaceutical studies have focused on the adult population and only in patients with a fairly limited range of associated conditions. Thus, pediatric PH has been understudied and little is understood regarding the natural history, mechanisms of disease, and treatment of childhood PH. Limitations regarding current translational approaches to children with PH are partly due to the relatively small numbers of patients with PH associated with specific pediatric disorders at each center; the small number of well-established, multidisciplinary programs in pediatric PH; little communication between translational and clinician-scientists; and limited interactions between existing PH programs. There is clearly a need to develop clinical infrastructure to better define the natural history and course of pediatric PH, to develop new strategies to identify at-risk patients early in their course, and to establish novel approaches to diagnose, monitor disease progression and treat children with PH. This article discusses the rationale, goals and initial steps in the establishment of an interactive network of investigators, care providers and multidisciplinary teams from several pediatric PH centers.

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