Madan S, Boonstra A, Berger R M
Sophia Kinderziekenhuis/Academisch Ziekenhuis, afd. Kindergeneeskunde, onderafd. Kindercardiologie, Dr. Molewaterplein 60, 3015 GJ Rotterdam.
Ned Tijdschr Geneeskd. 2001 Sep 22;145(38):1836-42.
Primary pulmonary hypertension (PPH) is a rare, progressive and life-threatening pulmonary vascular disease of unknown aetiology, which results in elevated pulmonary vascular resistance, pulmonary hypertension and eventually right ventricle failure. PPH occurs in all age groups, but most frequently in women aged 20-30 years old. The mean survival after the onset of symptoms is 2-3 years. Although the disease remains incurable at present, major advances in treatment options have been made during the last 10 years. This had led to a considerable improvement in extended survival and the quality of life in a substantial number of PPH patients. At present the most important treatment options include anticoagulants, calcium channel antagonists, chronic prostacyclin therapy (cost of this only reimbursed by health insurers in the Netherlands since 1999) and if needs be lung or heart-lung transplantation. Chronic continuous intravenous administration of epoprostenol is burdensome for the patient and requires adequate patient selection and a well-developed intramural and extramural infrastructure.
原发性肺动脉高压（PPH）是一种病因不明的罕见、进行性且危及生命的肺血管疾病，可导致肺血管阻力升高、肺动脉高压，最终引发右心室衰竭。PPH可发生于所有年龄组，但最常见于20至30岁的女性。症状出现后的平均生存期为2至3年。尽管目前该疾病仍无法治愈，但在过去10年中治疗方法取得了重大进展。这使得大量PPH患者的生存期显著延长，生活质量也有了相当大的改善。目前最重要的治疗方法包括抗凝剂、钙通道拮抗剂、慢性前列环素治疗（自1999年起，荷兰的健康保险公司仅报销此项治疗费用），必要时可进行肺移植或心肺移植。持续静脉输注依前列醇对患者来说负担较重，需要对患者进行充分筛选，并具备完善的院内和院外基础设施。
