title: Treatment of pulmonary arterial hypertension.
abstract: OBJECTIVE
  To perform a review of the diagnostic and therapeutic management of pulmonary hypertension in the pediatric population, with emphasis on pharmacological factors.
  
  
  METHODS
  Electronic search of publications on the MEDLINE/PubMed, LILACS and Cochrane Collaboration databases. The search strategy adopted gave priority to the identification of clinical trials (controlled or uncontrolled), systematic reviews and directives published during the last 10 years.
  
  
  RESULTS
  Many advances have been incorporated into our understanding of pulmonary hypertension during recent years. Issues related to differences in the pathophysiological mechanism of the disease between different age groups have altered both the treatment and prognosis of patients. The combined effect of more selective vasodilatory properties and antiproliferative action and the employment of new drugs are the basic principles of new treatment proposals. In order to be able to gauge the benefits associated with the use of these new therapies, it is of fundamental importance that all patients have their disease correctly diagnosed, the degree of functional compromise classified and their vascular reactivity capacity established, which is more difficult with pediatric patients.
  
  
  CONCLUSIONS
  To date there is no treatment that can be considered ideal for the management of pulmonary hypertension. With reference to the possibility of employing new drugs, the majority of studies that have been published were undertaken with adult populations. Few data are available on children, and the majority of studies are uncontrolled trials or case series. Taking into account differences that have already been established between different age groups in terms of disease mechanisms and prognostic aspects, it is difficult to claim that these drugs can be incorporated into the treatment of childhood pulmonary hypertension with the same indications and results.
authors.affiliation: UTI Pediátrica, Hospital da Criança Santo Antônio, Complexo Hospitalar Santa Casa, Porto Alegre, RS, Brazil. samantea@santacasa.tche.br
authors.forename: Cláudia Pires
authors.initials: CP
authors.lastname: Ricachinevsky
authors.forename: Sérgio Luís
authors.initials: SL
authors.lastname: Amantéa
mesh_terms: D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000077300:Bosentan; D002648:Child; D065128:Endothelin Receptor Antagonists; D045462:Endothelium-Dependent Relaxing Factors / Q000627:therapeutic use; D011464:Epoprostenol / Q000031:analogs & derivatives / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D009569:Nitric Oxide / Q000627:therapeutic use; D010726:Phosphodiesterase Inhibitors / Q000627:therapeutic use; D010879:Piperazines / Q000627:therapeutic use; D011379:Prognosis; D011687:Purines / Q000627:therapeutic use; D017466:Receptors, Endothelin / Q000627:therapeutic use; D012720:Severity of Illness Index; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000627:therapeutic use; D013450:Sulfones / Q000627:therapeutic use
chemical_list: D000959:Antihypertensive Agents; D065128:Endothelin Receptor Antagonists; D045462:Endothelium-Dependent Relaxing Factors; D010726:Phosphodiesterase Inhibitors; D010879:Piperazines; D011687:Purines; D017466:Receptors, Endothelin; D013449:Sulfonamides; D013450:Sulfones; D009569:Nitric Oxide; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D000077300:Bosentan