Kotlyar Eugene, Sy Raymond, Keogh Anne M, Kermeen Fiona, Macdonald Peter S, Hayward Christopher S, McNeil Keith D, Celermajer David S
St Vincent's Hospital, Sydney, Australia.
Cardiol Young. 2006 Jun;16(3):268-74. doi: 10.1017/S1047951106000114.
Bosentan is efficacious in idiopathic pulmonary arterial hypertension, and the variants associated with connective tissue disease, but not currently approved for treatment of pulmonary arterial hypertension due to Eisenmenger's syndrome. We sought to evaluate its effect in adults with Eisenmenger's syndrome.
We administered bosentan on the basis of compassionate use in 23 patients with Eisenmenger's syndrome, aged 37 plus or minus 14 years. Of the patients, 17 had never received specific treatment for pulmonary arterial hypertension, five were transitioned from treprostinil, and one from beraprost to bosentan. We measured functional class, saturation of oxygen, haemoglobin levels and six-minute walk distance at baseline, one, six months and at most recent follow-up.
Baseline functional class was IV in three, III in fifteen, and II in five patients. At follow-up, with a mean of 15 plus or minus 10 months, 13 of the 23 patients (57%) had improved by at least one functional class, from a median baseline of III to II (p equal to 0.016), mean saturation of oxygen at rest had increased from 81% to 84% (p equal to 0.001), and levels of haemoglobin had decreased from 178 plus or minus 26 grams per litre to 167 plus or minus 19 grams per litre (p equal to 0.001). Overall, the six-minute walk distance did not change from baseline of 335 metres. The distance walked by those not previously receiving specific therapy, however, improved from 318 plus or minus 129 to 345 plus or minus 123 metres (p equal to 0.03).
Treatment of adults with Eisenmenger's syndrome using bosentan significantly improved functional class, saturation of oxygen at rest, and decreased levels of haemoglobin. Treatment with bosentan was associated with improvement in six-minute walk distance in those not previously receiving specific therapy. In patients already in receipt of specific therapy, transition to bosentan resulted in no clinical deterioration.
波生坦对特发性肺动脉高压以及与结缔组织病相关的肺动脉高压有效，但目前尚未获批用于治疗艾森曼格综合征所致的肺动脉高压。我们旨在评估其对患有艾森曼格综合征的成年人的疗效。
我们基于同情用药原则，对23例年龄为37±14岁的艾森曼格综合征患者给予波生坦治疗。其中，17例患者从未接受过肺动脉高压的特异性治疗，5例从曲前列尼尔转换治疗，1例从贝拉普斯转换为波生坦治疗。我们在基线、1个月、6个月及最近一次随访时测量了功能分级、氧饱和度、血红蛋白水平和6分钟步行距离。
基线时，3例患者功能分级为IV级，15例为III级，5例为II级。随访时，平均随访时间为15±10个月，23例患者中有13例（57%）功能分级至少改善了一级，从中位数基线III级改善至II级（p = 0.016），静息时平均氧饱和度从81%升至84%（p = 0.001），血红蛋白水平从178±26克/升降至167±19克/升（p = 0.001）。总体而言，6分钟步行距离与基线时的335米相比没有变化。然而，之前未接受特异性治疗的患者步行距离从318±129米改善至345±123米（p = 0.03）。
使用波生坦治疗患有艾森曼格综合征的成年人可显著改善功能分级、静息时氧饱和度，并降低血红蛋白水平。波生坦治疗使之前未接受特异性治疗的患者6分钟步行距离得到改善。对于已经接受特异性治疗的患者，转换为波生坦治疗未导致临床病情恶化。
