Pediatric Cardiac Center, Cardiovascular Institute and Fuwai Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 167 Beilishi Road, 100037 Beijing, China.
Int J Cardiol. 2011 Aug 18;151(1):76-83. doi: 10.1016/j.ijcard.2010.04.094. Epub 2010 May 21.
Congenital heart disease with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease (PVD), has been successfully corrected at our institution recently. Whether the PVD is reversible remains unknown. This study aimed to examine the nature of the pulmonary arterial vessels in these selective patients.
All patients with congenital heart disease and severe pulmonary hypertension (PH) were selected using Diagnostic-treatment to undergo radical repair (n=49). Lung biopsy specimens were obtained during operation. The nature of PVD was determined by Heath-Edwards classification system. All specimens were quantitatively analyzed by calculating percentage media wall area, percentage media wall thickness and arteriole density.
Transcutaneous oxygen saturation of all selected patients increased significantly after Diagnostic-treatment (P<0.001). There were no operative deaths. Mean pulmonary artery pressure and pulmonary vascular resistance regressed significantly postoperatively (P<0.001). The incidence of postoperative PH was 59.2% (29/49). Of 49 selected patients with severe PH, 38 (77.6%) showed grade I change, 5 (10.2%) showed grade II change, 4 (8.2%) showed grade III change and only 2 (4%) showed grade IV change with plexiform lesion. The percentage media wall area, percentage media wall thickness and arteriole density were significantly increased in patients associated with PH than in normal subjects (P<0.001). Follow-up data showed the reversal of PVD in these 2 patients with plexiform lesions.
The PVD in these selective patients with congenital heart disease and severe PH using a Diagnostic-treatment-and-Repair strategy is generally reversible and these patients are operable in current era.
先天性心脏病伴接近全身肺脉压升高，既往认为存在不可逆转的肺血管疾病（PVD），但最近我院成功对此类患者进行了根治。目前仍不清楚 PVD 是否可逆。本研究旨在检查这些选择性患者的肺动脉血管性质。
采用诊断-治疗-修复策略选择所有患有先天性心脏病和严重肺动脉高压（PH）的患者（n=49）进行根治性修复。术中获取肺活检标本。采用 Heath-Edwards 分类系统确定 PVD 的性质。通过计算中膜壁面积百分比、中膜壁厚度百分比和小动脉密度对所有标本进行定量分析。
所有入选患者的经皮血氧饱和度在诊断-治疗后均显著升高（P<0.001）。无手术死亡。术后平均肺动脉压和肺血管阻力明显下降（P<0.001）。术后 PH 的发生率为 59.2%（29/49）。49 例严重 PH 患者中，38 例（77.6%）表现为 I 级改变，5 例（10.2%）表现为 II 级改变，4 例（8.2%）表现为 III 级改变，仅 2 例（4%）表现为有丛样病变的 IV 级改变。与正常对照组相比，伴有 PH 的患者中膜壁面积百分比、中膜壁厚度百分比和小动脉密度明显增加（P<0.001）。随访数据显示，2 例有丛样病变的患者的 PVD 逆转。
采用诊断-治疗-修复策略的先天性心脏病伴严重 PH 患者的 PVD 通常是可逆的，在当前时代这些患者是可手术的。
