Primary pulmonary hypertension: an Italian multicenter study. A retrospective epidemiological survey in the period 1975-1985.

An epidemiological retrospective survey on primary pulmonary hypertension was undertaken in Italy in 1985-6. The aim of the study was to obtain clinical and laboratory data on patients observed in the major cardiological Institutions, both Universities and Hospital Clinical Centers, in the period 1975-85. Forms were sent to 67 centers and twenty-three of them communicated their data on 124 cases of primary pulmonary hypertension. The diagnosis was considered certain or probable in 91 patients by the referring center. The mean age of patients was 38.5 years, with an overall female-to-male ratio of 2:1. Data were subjected to statistical analysis which confirmed the poor prognosis of the disease particularly for patients with high pulmonary resistances, low cardiac output and in class 3-4 of the NYHA. Half of the 124 cases were treated with calcium antagonists after 1980, but the results of therapy in modifying the clinical course of the disease could not be assessed by the study. Like all retrospective studies even the Italian Multicenter Study is biased but it can be considered as an useful preliminary investigation which can form the basis for a prospective registry of primary pulmonary hypertension cases.