原発性肺高血圧症の1家系

The familial occurence of primary pulmonary hypertension

　原発性肺高血圧症（以下PPHと略す）の成因については不明な点が多く，一方家族性の報告は少ない。今回我々は家族性と思われるPPHの症例を経験したので報告する。

We have experienced familiar primary pulmonary hypertension. The first patient was found to have PPH at age 48 who had been healthy till age 40 when he developed hemoptysis, palpitation and dyspnea on exersion. His young brothers age 34, male cousin age 48 and female cousin age 36 had similar symptous.

Right heart catheterization revealed no intracardiac shunt and pulmonary pressure was elevated. Wedge pulmonary pressure was normal in one case and could not be measured in other cases.