Prognosis of patients with primary pulmonary hypertension

Primary pulmonary hypertension is a rare disease entity. Clinical evaluations of such patients were deemed inadequate during a multi-center study in Japan (1976). The prognoses of our patients in Keio Hospital were evaluated, especially in terms of their clinical pictures and hemodynamic backgrounds. The study group consisted of 15 patients, who fulfilled the clinical criteria of primary pulmonary hypertension according to the Research Committee of the Ministry of Health and Welfare in Japan. There were four males and 11 females, whose age ranged from 16 to 72 years and averaged 34.7 years. Follow-up periods from the onsets were from 15 to 152 months, and they averaged 51 months. Nine of the 15 patients were deceased, and autopsies were performed in eight. The periods from onsets to deaths were between 15 and 152 months, and averaged 53 months (4 years and 5 months). Except for four cases followed for less than 2 years, there were seven who survived over three years and four patients who were deceased in this period, but there were no differences between these groups in terms of their clinical pictures, such as age, sex and symptomatology at onset. Survivors included three patients associated with thyroid disease, one with liver cirrhosis, and one with the Sjoegren syndrome. Hemodynamically, heart rates and pulmonary artery pressures did not differ among six survivors more than 2 years after the hemodynamic evaluations and five patients who were deceased within this period. In the deceased group, however, cardiac output was low and arterio-venous differences in oxygen content were high. In conclusion, it was difficult to estimate life expectancy by means of the clinical pictures at the times of onset. Pulmonary artery pressure, in other words pulmonary hypertension, did not relate to their prognoses. Cardiac outputs and arteriovenous differences in oxygen content were the hemodynamic parameters affecting their prognoses.