Baptista Rui, Castro Graça, da Silva António Marinho, Monteiro Pedro, Providência Luís Augusto
Unidade de Hipertensão Pulmonar, Serviço de Cardiologia, Hospitais da Universidade de Coimbra, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
Rev Port Cardiol. 2013 Feb;32(2):123-9. doi: 10.1016/j.repc.2012.02.023. Epub 2013 Jan 23.
Bosentan is recommended for symptomatic patients with Eisenmenger syndrome due to simple congenital lesions such as atrial and ventricular septal defects (VSD). However, its long-term efficacy and safety in patients with pulmonary arterial hypertension (PAH) associated with complex congenital heart disease (CHD) is unknown.
We examined the short- and long-term effects and safety profile of bosentan in patients with PAH and complex CHD.
We followed 14 patients with PAH and complex CHD for a mean of four years. Demographic parameters, exercise capacity assessed by the six-minute walking test (6MWT) and oxygen saturation were assessed at baseline, six months and at follow-up.
Mean age was 37.1 ± 11.7 years; 90% were in WHO class III or IV. The most common diagnosis was pulmonary atresia with VSD (35.7%), followed by truncus arteriosus (28.6%), patent ductus arteriosus (21.4%) and transposition of the great arteries (14.3%). After six months of treatment, six-minute walking distance (6MWD) increased from 371.9 to 428.4 m (p=0.005) and functional class was improved (p=0.005). After four years, one patient discontinued bosentan due to side effects and four patients were started on sildenafil, after a mean 38 months of bosentan treatment. Mean 6MWD for patients on bosentan monotherapy (n=8) was 440.1 ± 103.8 m, whereas for patients on bosentan-sildenafil combination therapy (n=4) it was 428.8 ± 96.9 m, after four years of therapy. Two patients died during follow-up.
Bosentan was safe and was associated with improved exercise capacity in patients with PAH and complex CHD. This improvement was sustained for up to four years and the safety profile was similar to simple CHD patients.
对于因房间隔缺损和室间隔缺损（VSD）等简单先天性病变导致艾森曼格综合征的有症状患者，推荐使用波生坦。然而，其在与复杂先天性心脏病（CHD）相关的肺动脉高压（PAH）患者中的长期疗效和安全性尚不清楚。
我们研究了波生坦在PAH合并复杂CHD患者中的短期和长期疗效及安全性。
我们对14例PAH合并复杂CHD患者进行了平均四年的随访。在基线、6个月和随访时评估人口统计学参数、通过6分钟步行试验（6MWT）评估的运动能力和血氧饱和度。
平均年龄为37.1±11.7岁；90%为WHO III或IV级。最常见的诊断是室间隔缺损合并肺动脉闭锁（35.7%），其次是永存动脉干（28.6%）、动脉导管未闭（21.4%）和大动脉转位（14.3%）。治疗6个月后，6分钟步行距离（6MWD）从371.9米增加到428.4米（p=0.005），功能分级得到改善（p=0.005）。四年后，1例患者因副作用停用波生坦，4例患者在接受平均38个月的波生坦治疗后开始使用西地那非。波生坦单药治疗患者（n=8）的平均6MWD为440.1±103.8米，而波生坦-西地那非联合治疗患者（n=4）在治疗四年后的平均6MWD为428.8±96.9米。随访期间有2例患者死亡。
波生坦对PAH合并复杂CHD患者安全且与运动能力改善相关。这种改善可持续长达四年，安全性与简单CHD患者相似。
