Scherpereel A, Steenhouwer F, Quandalle P, Dennetière S, Haftel Y, Declercq N, Demarcq J M
Service de Soins intensifs cardiologiques, Centre Hospitalier de Roubaix.
Rev Mal Respir. 1997 Nov;14(5):409-12.
The authors present the case of three young female patients, of the same family, with all quickly fatal Primary Pulmonary Hypertension (PPH). Their clinical signs and paraclinical investigations were suggestive of a PPH and excluded all others causes of Pulmonary Hypertension. These observations recall the possible familial cases of this rare and very severe disease. It emphasizes as well the possible revelation of the disease during or after a pregnancy. So the members of this family, essentially first the young women who wish to have children, need a medical detection.
作者介绍了同一家庭中三名年轻女性患者的病例，她们均患有迅速致死的原发性肺动脉高压（PPH）。她们的临床症状和辅助检查提示为PPH，并排除了所有其他导致肺动脉高压的原因。这些观察结果让人想起这种罕见且非常严重疾病可能存在的家族性病例。这也强调了该疾病在孕期或产后可能会显现出来。因此，这个家庭的成员，尤其是那些想要孩子的年轻女性，需要进行医学检查。
