Objective: To perform a review of the diagnostic and therapeutic management of pulmonary hypertension in the pediatric population, with emphasis on pharmacological factors.Sources: 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.Summary of the findings: 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.J Pediatr (Rio J). 2006;82(5 Suppl):S153-65: Pulmonary arterial hypertension, pulmonary hypertension, nitric oxide, bosentan, sildenafil.
ResumoObjetivo: Estabelecer uma revisão acerca do manejo diagnóstico e terapêutico da hipertensão pulmonar na população pediátrica, com ênfase nos aspectos farmacológicos.Fontes de dados: Busca eletrônica de publicações nas bases de dados MEDLINE/PubMed, LILACS e Cochrane Collaboration. Estabeleceu-se uma estratégia de busca priorizando a identificação de ensaios clínicos (controlados ou não controlados), revisões sistemáticas e diretrizes publicados nos últimos 10 anos.Síntese dos dados: Muitos avanços têm sido incorporados ao conhecimento da hipertensão pulmonar nos últimos anos. Aspectos relativos a diferenças nos mecanismos fisiopatológicos da doença entre as diferentes faixas etárias têm modificado o tratamento e o prognós-tico dos pacientes. Uma ação combinada de propriedades vasodilatadoras mais seletivas e ação antiproliferativa e o emprego de novas drogas representam princípios fundamentais das novas propostas terapêuti-cas. Para considerar benefícios as...