重度原发性肺动脉高压或艾森曼格综合征成人患者的血流动力学与生存率比较。

Comparison of the hemodynamics and survival of adults with severe primary pulmonary hypertension or Eisenmenger syndrome.

作者信息

Hopkins W E, Ochoa L L, Richardson G W, Trulock E P

机构信息

Cardiovascular Division, Department of Medicine, Washington University School of Medicine, St. Louis, MO, USA.

出版信息

J Heart Lung Transplant. 1996 Jan;15(1 Pt 1):100-5.

PMID:8820089

Abstract

BACKGROUND

To date, lung or heart-lung transplantation remains the only definitive treatment for most adults with severe primary pulmonary hypertension or Eisenmenger syndrome. Although the hemodynamic derangements and clinical history of adults with severe primary pulmonary hypertension have been well documented, those of adults with Eisenmenger syndrome have not.

METHODS

We evaluated hemodynamics and nontransplantation survival of 100 adults with severe pulmonary hypertension (34 +/- 9 years, 73 women and 27 men; 57 with primary pulmonary hypertension, 37 with Eisenmenger syndrome, and six with a previously repaired congenital heart defect) followed up by the lung transplant or adult congenital heart services at Washington University.

RESULTS

Despite a trend toward greater pulmonary artery pressures (107 +/- 20 versus 97 +/- 21 mm Hg, p = 0.06), patients with Eisenmenger syndrome had greater systemic cardiac indexes (2.7 +/- 0.6 versus 2.2 +/- 0.8 L/min/m2, p < 0.05) and lower mean right atrial pressures (5 +/- 2 versus 12 +/- 5 mm Hg, p < 0.0001) than patients with primary pulmonary hypertension. Four (11%) patients with Eisenmenger syndrome died and eight (22%) received transplants during the follow-up interval; 13 (23%) patients with primary pulmonary hypertension died, and 31 (54%) received transplants over the same interval. Actuarial survival of patients who did not receive transplants was 97% at 1 year, 89% at 2 years, and 77% at 3 years for patients with Eisenmenger syndrome and 77%, 69%, and 35%, respectively, for patients with primary pulmonary hypertension. Data on hemodynamics from a small number of patients with a previously repaired heart defect and severe pulmonary hypertension were similar to those from patients with primary pulmonary hypertension.

CONCLUSIONS

Our data suggest that adults with Eisenmenger syndrome have a more favorable hemodynamic profile and prognosis than adults with primary pulmonary hypertension.

摘要

背景

迄今为止，肺移植或心肺移植仍是大多数重度原发性肺动脉高压或艾森曼格综合征成年患者的唯一确定性治疗方法。虽然重度原发性肺动脉高压成年患者的血流动力学紊乱和临床病史已有充分记录，但艾森曼格综合征成年患者的情况却并非如此。

方法

我们评估了100例重度肺动脉高压成年患者（年龄34±9岁，女性73例，男性27例；57例原发性肺动脉高压，37例艾森曼格综合征，6例曾接受先天性心脏病修复）的血流动力学和非移植生存率，这些患者由华盛顿大学的肺移植或成人先天性心脏病服务团队进行随访。

结果

尽管艾森曼格综合征患者的肺动脉压力有升高趋势（107±20对97±21 mmHg，p = 0.06），但与原发性肺动脉高压患者相比，其体循环心脏指数更高（2.7±0.6对2.2±0.8 L/min/m²，p < 0.05），平均右心房压力更低（5±2对12±5 mmHg，p < 0.0001）。在随访期间，4例（11%）艾森曼格综合征患者死亡，8例（22%）接受了移植；13例（23%）原发性肺动脉高压患者死亡，31例（54%）在同一时期接受了移植。未接受移植的艾森曼格综合征患者1年、2年和3年的精算生存率分别为97%、89%和77%，原发性肺动脉高压患者分别为77%、69%和35%。少数曾接受心脏缺陷修复且患有重度肺动脉高压患者的血流动力学数据与原发性肺动脉高压患者相似。