[Epidemiology, diagnostic criteria, prognosis of pulmonary arterial hypertension with qualification criteria for the obstinate disease in Japan]

Abstract

The incidence of new cases of idiopathic pulmonary arterial hypertension(IPAH) is reported to be one or two per 1,000,000 of the population. The 5-year survival of patients with IPAH has improved from about 30% to 60% in Japan. The estimated prevalence of IPAH is now 10 to 20 per 1,000,000. The actual reported prevalence of IPAH is 7.5 in 1,000,000. In our facility, the 4-year survival of patients with IPAH for the last 4 years was 91% compared to 5-year survival of 74% for the last 8 years. Other epidemiologic features and the diagnostic criteria of pulmonary arterial hypertension are also discussed.