Primary pulmonary hypertension is a rare, progressive disease with a median age at diagnosis of 36 years and a mean survival of 2.8 years from diagnosis.
Primary pulmonary hypertension (PPH) is defined clinically by a sustained elevation of pulmonary arterial pressure (>25 mm Hg at rest or >30 mm Hg during exercise) without a demonstrable cause. Symptoms of PPH are largely non-specific but may include worsening shortness of breath, chest pain, syncope, fatigue, and peripheral oedema. It is a rare disorder with an estimated incidence of 2-3 per million per year. 1 PPH shows a female bias with a F:M ratio of 2.3:1. The median age at diagnosis is 36 years but it may occur at any age. PPH is a progressive, often fatal, disease with mean survival from diagnosis of 2.8 years. Clues to the aetiology of the disease have been suggested by environmental stimuli associated with the development of severe pulmonary hypertension, pathologically indistinguishable from PPH. The association with appetite suppressants, in particular the fenfluramine/ dexfenfluramine group, 3 suggested a role of re-uptake inhibition of serotonin. Severe PPH may also follow HIV-1 infection, and PPH is associated with autoimmune thyroid disease and the presence of circulating anti-Ku and antinuclear antibodies. 1
Nung Rudarakanchana (Thu,) conducted a review in Primary pulmonary hypertension. Primary pulmonary hypertension is a rare, progressive disease with a median age at diagnosis of 36 years and a mean survival of 2.8 years from diagnosis.
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