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RESUMO
A hipertensão pulmonar primária (HPP) é uma doença grave que atinge preferencialmente mulheres jovens. A compreensão da sua f'isiopatologia tem permitido a introdução de importantes avanços terapêuticos, nomeadamente a anticoagulação, os vasodilatadores orals e a prostaciclina intravenosa contínua, nos doentes que não respondem a urn teste agudo de vasodilatação durante o cateterismo dircilo. Umu via de administração atractiva para os prostanóidcs parece ser a via inalatória recorrendo a arálogos estáveis da prostaciclina, como o iloprost. Outros fármacos e outras vias de adminiotralção encontram-se também em investigação. A transplantação pulmonar ou coração-pulmão permanece como urn último recurso e a septostomia auricular como uma altemativa valida. Os autores revêem a fasiopatologia, a metodologia de diagnóstico, a terapêutica e o prognóstico da HPP.
REV PORT PNEUMOL 2000; VI (5): 383-394
Palavras-chave:
Hipertensão pulmonar primána
Bloqueantes do cálcio
Epoprostcnol
Prostanóides
lloprost
ABSTRACT
Primary pulmonary hypertension (PPH) is a serious disease affecting mainly young women. The understanding of its physiopathology bas led to the development or therapeutic advances, namely anticoagulation, oral vasodilators and continuous intravenous prostacyclio for those patients that do not react to an acute vasodilation test during right heart cathetberization. An attractive administration route see.ris to be the Jnbalative one, using more stable prostacyclin analogues like iloprost. Other drugs and administration routes are currently beeing investigated. Lung or heart-lung transplantation remains as a last resource and baJion blade atrial septostomy as a valid alternative. The authors review the physiopathology, diagnostic methodology, therapeutic option and prognosis of PPH.
REV PORT PNEUMOL 2000; VI (5): 383-394
Key-words:
Primary pulmonary hypertension
Calcium channel blockers
Epopros tenol
Prostanoids
lloprost
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