Rozkovec A, Montanes P, Oakley C M
Br Heart J. 1986 May;55(5):449-58. doi: 10.1136/hrt.55.5.449.
Factors predicting life expectancy in primary pulmonary hypertension have not been well defined. Thirty four cases of primary pulmonary hypertension that had been followed up until death or for at least five years were reviewed retrospectively. Patients were divided into three groups: 18 patients who died within five years of presentation to hospital; 12 who survived more than five years; and four who improved and who lived for more than five years. The age at onset was similar in the three groups and, like symptoms and sex, did not predict life expectancy. Right heart failure during the course of the disease was associated with a poor outcome. Radiographic evidence of cardiac enlargement and evidence of right heart strain on electrocardiogram at presentation was also predictive of survival for less than five years. Pulmonary arteriolar resistance was higher and cardiac output lower in those with the shortest survival times. There was no relation between pulmonary artery pressure and length of survival. Systemic resistance varied directly with pulmonary resistance and served to maintain systemic pressure. Presentation in or after pregnancy and patency of the foramen ovale were associated with longer survival. In four patients there was evidence of regression of the disease by cardiac catheterisation and lung histology. Primary pulmonary hypertension is a heterogeneous condition in which life expectancy varies widely.
原发性肺动脉高压患者预期寿命的预测因素尚未明确界定。回顾性分析了34例原发性肺动脉高压患者，这些患者均随访至死亡或至少随访了5年。患者被分为三组：18例在入院后5年内死亡；12例存活超过5年；4例病情改善且存活超过5年。三组患者的发病年龄相似，症状和性别也与预期寿命无关。病程中出现右心衰竭提示预后不良。入院时X线胸片显示心脏增大以及心电图提示右心劳损也提示存活时间不足5年。存活时间最短的患者肺小动脉阻力更高，心输出量更低。肺动脉压与存活时间无关。体循环阻力与肺循环阻力呈正相关，并有助于维持体循环压力。妊娠期间或产后发病以及卵圆孔未闭与较长的存活时间相关。4例患者经心导管检查和肺组织学检查显示病情有缓解迹象。原发性肺动脉高压是一种异质性疾病，其预期寿命差异很大。
