Assessment of pulmonary vasoreactivity in children with pulmonary hypertension.
OBJECTIVE
With the current advance in understanding and treatment of pulmonary arterial hypertension in children, pulmonary vasoreactivity testing would navigate the treatment option. An inclusive review of the milestone studies and also recent literature over the last few years on the pulmonary vasoreactivity testing in children will provide the update on various available pulmonary vasodilator agents, markers related to vasoreactivity response, the implication of the testing result on child management and outlook for the long-term outcome.


RESULTS
There continue to be emerging data regarding pulmonary vasodilators for vasoreactivity testing in children and the genetic predictor of pulmonary vasoreactivity response, particularly in children with idiopathic and familial pulmonary hypertension. Despite a recent advance in pulmonary hypertension therapy leading to improved prognosis in children, the novel knowledge on standardized pulmonary vasoreactivity testing in children and its interpretation remain limited and controversial.


CONCLUSIONS
The precise definition of pulmonary vasoreactivity testing remains debatable, particularly in children with pulmonary hypertension related to congenital heart defect. Defining the responder, in order to navigate the treatment option, is frequently dictated by institutional experience and facilities. Meanwhile, the criteria for responder in children with idiopathic pulmonary artery hypertension are reasonably consistent. In general, responders seem to have less severe disease and better prognosis.
Limsuwan
Alisa
A
Division of Pediatric Cardiology, Department of Pediatrics, Ramathibodi Hospital, Mahidol University, Bangkok 10400, Thailand. alimsuwan@yahoo.com
Khowsathit
Pongsak
P
D002648:Child; D006334:Heart Function Tests; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology*; D011237:Predictive Value of Tests; D011379:Prognosis; D014655:Vascular Resistance / Q000187:drug effects*; D014664:Vasodilation / Q000187:drug effects*; D014665:Vasodilator Agents
D014665:Vasodilator Agents