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Primary pulmonary hypertension.

Authors
  • Oliaro, E
  • Grosso Marra, W
  • Orzan, F
Type
Published Article
Journal
Minerva cardioangiologica
Publication Date
Nov 01, 2000
Volume
48
Issue
11
Pages
361–378
Identifiers
PMID: 11214428
Source
Medline
License
Unknown

Abstract

Primary Pulmonary Hypertension (PPH) is a rare disease that progressively increases pulmonary arterial pressure and pulmonary vascular resistance to the point of right heart failure, in the absence of secondary causes of the disease. The following specific risk factors that can trigger PPH have been identified and examined: appetite depressant drugs, oral contraceptives and hyperuricaemia. Familial PPH transmitted by an incomplete penetrance dominant autosomic mechanism is responsible for about 6% of PPH cases. Recent research groups have identified the gene responsible for familial PPH. Since pulmonary vasoconstriction is the prime cause of PPH, vasodilation of the pulmonary arterial circulation system (using prostacyclin in the form of epoprostenol or iloprost) is the main aim of treatment.

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