Fauzi A R
Med J Malaysia. 2000 Dec;55(4):529-37; quiz 538.
Primary pulmonary hypertension (PPH) is a rare disease. The annual incidence in the West is 1-2 cases per million population per year. A recent WHO symposium in 1998 has produced a consensus on classification, methods of screening, risk assessment and treatment. PPH is a diagnosis of exclusion after all other secondary causes of pulmonary hypertension are ruled out. Current treatment strategy involves acute vasodilator drug trial where positive responders are treated with high dose calcium channel blockers and anticoagulation. Those who do not show positive response may be commenced on intravenous prostacyclin. Surgical treatment is one option for patients with severe PPH or for symptomatic relief. Prognosis in general is very poor.
原发性肺动脉高压（PPH）是一种罕见疾病。在西方，其年发病率为每百万人口每年1 - 2例。1998年世界卫生组织最近召开的一次研讨会就分类、筛查方法、风险评估和治疗达成了共识。PPH是在排除所有其他继发性肺动脉高压病因后做出的排除性诊断。当前的治疗策略包括急性血管扩张剂药物试验，对有阳性反应者用高剂量钙通道阻滞剂和抗凝治疗。那些没有显示出阳性反应的患者可能开始静脉注射前列环素。手术治疗是重度PPH患者或为缓解症状的一种选择。总体预后非常差。
