Academic Medical Centre, Department of Cardiology, Amsterdam, The Netherlands.
Int J Cardiol. 2013 Mar 20;164(1):106-10. doi: 10.1016/j.ijcard.2011.06.084. Epub 2011 Jul 18.
Pulmonary arterial hypertension (PAH) in patients with congenital heart disease (CHD) usually has a homogeneous pressure distribution. More rarely, complex CHD patients have segmental PAH. This is often post-surgically. The characteristics of these patients and their responsiveness to specific pulmonary vasodilator therapy have not been described.
Seven adults with segmental PAH complicating CHD were treated at 3 specialized adult CHD centers between January 2006 and December 2010. Clinical characteristics, six minute walking distances (6 MWD), laboratory tests and images were obtained from medical records and the responses to Bosentan, an endothelin-1 receptor antagonist, were assessed.
All patients (mean age 32 (23-42) years, five females) had a primary diagnosis pulmonary atresia (PA), four with major aortopulmonary collateral arteries (MAPCAs). Four segmental PAH patients had a right pulmonary artery stenosis, two a left pulmonary artery stenosis and one a unilateral MAPCA stenosis. All patients were symptomatic (functional class II or III) and bosentan was started empirically. Bosentan treatment led to a significant improvement in functional class compared to baseline (1.7 ± 0.5 versus 2.4 ± 0.5; p<0.01). Mean 6 MWD (available in 6 patients) increased by 62 m (22-150 m) from 386 ± 135 to 448 ± 133 m (p=0.03) after 12 months treatment. Most improvement was seen in patients with low baseline 6 MWD. Higher baseline exercise heart rate was significantly associated with lesser improvement in 6 MWD (r=-0.91 p=0.01). Laboratory results did not change after initiation of bosentan treatment.
This small retrospective case series suggested a significant improvement of functional class and exercise capacity after bosentan treatment in patients with segmental PAH. These findings warrant a prospective study of the potential benefit of selective pulmonary vasodilator therapy in these complex patients. Therefore, we call on treating physicians to share similar cases.
先天性心脏病（CHD）患者的肺动脉高压（PAH）通常具有均匀的压力分布。更罕见的是，复杂的 CHD 患者存在节段性 PAH。这种情况通常是手术后的。这些患者的特征及其对特定肺动脉扩张剂治疗的反应尚未描述。
2006 年 1 月至 2010 年 12 月，在 3 家专门的成人 CHD 中心治疗了 7 例节段性 PAH 合并 CHD 的成年人。从病历中获得了临床特征、6 分钟步行距离（6MWD）、实验室检查和图像，并评估了内皮素-1 受体拮抗剂波生坦的反应。
所有患者（平均年龄 32（23-42）岁，女性 5 例）均有原发性肺动脉闭锁（PA），4 例有主要体肺侧支循环动脉（MAPCA）。4 例节段性 PAH 患者有右肺动脉狭窄，2 例有左肺动脉狭窄，1 例有单侧 MAPCA 狭窄。所有患者均有症状（功能分级 II 或 III），并开始经验性使用波生坦治疗。与基线相比，波生坦治疗后功能分级显著改善（1.7±0.5 与 2.4±0.5；p<0.01）。6MWD（可用于 6 例患者）从 386±135m 增加到 448±133m（p=0.03），12 个月治疗后增加了 62m（22-150m）。基线 6MWD 较低的患者改善最大。较高的基线运动心率与 6MWD 改善程度显著相关（r=-0.91，p=0.01）。启动波生坦治疗后，实验室结果无变化。
本回顾性小病例系列研究表明，波生坦治疗后，节段性 PAH 患者的功能分级和运动能力有显著改善。这些发现需要前瞻性研究选择性肺动脉扩张剂治疗在这些复杂患者中的潜在益处。因此，我们呼吁治疗医生分享类似的病例。
