Gorenflo M, Ullmann M, Sebening C, Brockmeier K, Hagl S, Ulmer H E, Otto H F, Schnabel P A
Department of Paediatric Cardiology, University Children's Hospital, INF 153, 69120, Heidelberg, Germany.
Virchows Arch. 2002 Sep;441(3):264-70. doi: 10.1007/s00428-002-0647-4. Epub 2002 May 30.
We asked whether a scoring system [index of pulmonary vascular disease (IPVD)] that quantifies the individual pulmonary vascular pathology would relate to postoperative survival in patients with congenital heart disease and pulmonary hypertension (PH).
Lung biopsy specimens from 28 patients at a median age of 6 months (1 month to 21 years) were analysed qualitatively and morphometrically. The IPVD and other morphometric parameters were related to haemodynamic findings and survival.
Mean pulmonary artery pressure (PAP) was 44 mmHg (15-72 mmHg), and the resistance to pulmonary perfusion was 5 U x m(2) (0.9-14 U x m(2)). There were three early (in-hospital) and three late deaths during the follow-up period of 2.5 years (6 months to 7 years). Incipient plexiform lesions were observed in one infant with trisomy 21 and complete atrioventricular septal defect (cAVSD). An IPVD score above the upper critical limit (>2.2) was not observed during the first year of life. On discriminant analysis, morphometric parameters could not predict mortality ( P=0.08).
The IPVD is not helpful to predict surgical mortality during the first year of life. Patients with trisomy 21 and cAVSD may show advanced pulmonary vascular disease in infancy.
我们探讨一种量化个体肺血管病理改变的评分系统[肺血管疾病指数（IPVD）]是否与先天性心脏病合并肺动脉高压（PH）患者的术后生存率相关。
对28例中位年龄为6个月（1个月至21岁）患者的肺活检标本进行定性和形态计量学分析。将IPVD及其他形态计量学参数与血流动力学结果和生存率相关联。
平均肺动脉压（PAP）为44 mmHg（15 - 72 mmHg），肺灌注阻力为5 U x m(2)（0.9 - 14 U x m(2)）。在2.5年（6个月至7年）的随访期内有3例早期（住院期间）死亡和3例晚期死亡。在1例患有21三体综合征和完全性房室间隔缺损（cAVSD）的婴儿中观察到初期丛状病变。在生命的第一年未观察到IPVD评分高于临界上限（>2.2）的情况。经判别分析，形态计量学参数无法预测死亡率（P = 0.08）。
IPVD无助于预测生命第一年的手术死亡率。患有21三体综合征和cAVSD的患者在婴儿期可能表现出晚期肺血管疾病。
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